Neal C. Dalrymple, MD

• Associate Professor of Radiology
• The University of Texas Health Science Center
• at San Antonio
• San Antonio, Texas

This leads to right ventricular hypertrophy and failure (cor pulmonale) gastritis kas tai per liga discount renagel 400 mg fast delivery, and peripheral edema due to right heart failure is a dominant clinical feature gastritis diet ������ purchase renagel 400mg without a prescription. Type A patients frequently have dominant emphysematous changes gastritis meal plan renagel 800mg visa, while type B patients usually have dominant chronic obstructive bronchitis gastritis diet ���������� cheap 400mg renagel. Most patients chronic gastritis for years purchase 400 mg renagel otc, however gastritis symptoms bloating trusted 800 mg renagel, have varying mixtures of both pathologic changes and clinical features. In the early stages, blood gases are normal at rest, but hypoxemia develops during exercise due to the decreased pulmonary reserve. Administration of oxygen in these patients can remove the respiratory center drive and cause carbon dioxide retention and death (carbon dioxide narcosis). When hypoxemia becomes persistent and severe (Pao2 <60 mm Hg) or associated with cor pulmonale, continuous oxygen therapy is indicated. Chronic infection of the paranasal sinuses also results in absence of the frontal sinuses in this condition. In the male, absence of dynein arms in the microtubules of the sperm tail leads to loss of sperm motility and infertility. Intralobar Sequestration of the Lung (Congenital): In this rare condition a part of the lung receives either no pulmonary arterial supply or no communication with the bronchial tree. The sequestered lobe maintains nutrition via its bronchial arterial supply/The bronchi within the area, however, have no drainage and therefore undergo infection and dilation. The dilated bronchi and bronchioles may be cylindric, fusiform, or saccular and are made more conspicuous by extensive destruction and fibrosis of the intervening lung parenchyma. Bronchiectasis is the result of chronic infection with resulting parenchymal destruction, fibrosis, and abnormal permanent dilation of damaged bronchi. Stagnation of mucus is followed by bronchopneumonia distal to the obstruction, progressing to localized fibrosis and bronchiectasis. Bronchopneumonia, particularly following childhood infections such as measles and whooping cough, which in the past were common antecedents of bronchiectasis. Bronchiectasis occurs today in immunodeficient children who are susceptible to recurrent pulmonary infections. Note the dilated bronchi immediately beneath the pleura, a feature of diagnostic value. Clinical Features Bronchiectasis is a chronic illness with cough, usually productive of a large volume of foul-smelling sputum, and episodic fever. The chronic infection commonly causes clubbing of fingers and hyperglobulinemia and may cause secondary amyloidosis. Common bacteria cultured from bronchiectatic cavities include Staphylococcus aureus, Staphylococcus epidermidis; streptococci of all types, including pneumococci; Haemophilus influenzae; enteric gram-negative bacilli; and anaerobes. Treatment the treatment of bronchiectasis consists of control of infection with antibiotics and removal of predisposing causes such as bronchial obstruction by surgery, when possible. Surgical removal of bronchiectatic areas is important because the permanently dilated bronchi represent foci of almost continuous infection. Chronic diffuse interstitial fibrosis of the lung, showing chronic inflammation and fibrosis of trie interstitium and abnormal air spaces lined by cuboidal cells (honeycomb lung). While all produce the general picture described above, some have individual features that permit specific diagnosis. The end stage is associated with extensive parenchymal destruction, fibrosis, and often the formation of abnormal cystic spaces (honeycomb lung). General Clinical & Pathologic Features Patients with diffuse interstitial lung disease present with dyspnea, tachypnea, and cyanosis. Pulmonary function abnormalities are of the restrictive type, with reduced vital capacity but no airway obstruction. Thickening of the alveolar membrane affects diffusion of oxygen, leading to hypoxemia. Chest x-ray shows a characteristic reticulonodular pattern of interstitial involvement. Etiology Diffuse infiltrative lung disease has many causes Idiopathic pulmonary fibrosis is actually a group of diseases characterized by diffuse interstitial fibrosis occurring without recognized cause. About half the cases of chronic diffuse interstitial lung disease fall into this category. Alhough the cause is unknown, the presence of circulating immune complexes, immunoglobulin deposition in the interstitium in many patients, and the response of early disease to treatment with steroids strongly suggest an immunologic basis. In idiopathic pulmonary fibrosis, alveolar macrophages express several genes that encode for mediators such as fibronectin and platelet-derived growth factor that recruit and stimulate fibroblasts. The production of platelet-derived growth factor by alveolar macrophages in idiopathic pulmonary fibrosis correlates with activation of the c-sis gene in these cells. Clinically, patients present with progressive dyspnea and cough and ventilatory failure of the restrictive type. A defect in oxygen diffusion across the abnormal alveolar membrane may also contribute. Controversy exists about whether this is a variant of usual interstitial pneumonitis or a completely different disease. The course is similar to that of slowly progressive usual interstitial pneumonitis. Lymphocytic Interstitial Pneumonitis (Pseudolymphoma) Lymphocytic interstitial pneumonitis is characterized by extensive infiltration of the interstitium with lymphocytes and plasma cells. It may be diffuse or may involve a single area of lung, producing a mass lesion (pseudolymphoma). The disorder is associated with an increased incidence of primary pulmonary malignant lymphoma; in some cases, the process appears to be a primary low-grade malignant lymphorna. Idiopathic pulmonary fibrosis is classified according to the histologie appearance of the lung, as discussed below. In the acute phase, there is interstitial infiltration with lymphocytes, plasma cells, and macrophages; pulmonary edema; acute alveolar damage; and proteinaceous hyaline membranes. In the acute phase, the disease may respond to treatment with immunosuppressive agents such as corticosteroids. The rate of fibrosis is variable, but the occurrence of fibrosis represents irre ver sibility. The course is characteristically protracted, with respiratory failure occurring many years after onset. When interstitial fibrosis (honeycomb lung) is present, the clinical response to steroids is poor. Hypersensitivity pneumonitis results from inhalation by susceptible individuals of small organic particles (antigens), most commonly spores of thermophilic fungi. Individuals working in a variety of occupations are at risk, and the disease frequently bears the name of these occupations (see Chapter 8). In urban areas, the spores are most frequently found in contaminated forced-air-heating and air-conditioning systems. Allergic angiitis and granulomatosis of Churg and Strauss shows features of eosinophilic pneumonia with asthma combined with a multisystem vasculitis that frequently involves the skin, kidney, and nervous system. Allergic angiitis and granulomatosis of Churg and Strauss is a serious disease with a high mortality rate and requires aggressive immunosuppressive therapy. Fibrous obliteration of bronchioles (bronchiolitis obliterans) is a characteristic change. If the disease is recognized early and the patient removed from the source of antigen, the disease is reversible. With continued exposure, diffuse interstitial fibrosis occurs, leading to end-stage honeycomb lung. Clinical Features Patients present with acute dyspnea, fever, and cough 4-6 hours after exposure to the antigen. As pulmonary fibrosis ensues, the disease goes into its chronic phase, with all the features of diffuse interstitial lung disease. Systemic lupus erythematosus may also be complicated by immune complex deposition in the alveoli, transient patchy lung infiltrates, vasculitis, and acute alveolar damage. In the lung, hemorrhage into the alveoli is the dominant feature; hemosiderincontaining macrophages may be found in the sputum. In the chronic phase, the lung is firm because of marked interstitial fibrosis, brown because of hemosiderin, and may show changes of honeycomb lung. Eosinophilic pneumonias are a group of diseases characterized by eosinophilic pulmonary interstitial infiltrates and peripheral blood eosinophilia. In the chronic phase, there is progressive dyspnea, cough, and right heart failure due to pulmonary fibrosis. Patients almost invariably have evidence of glomerulonephritis, most frequently microscopic hematuria. Treatment by plasmapheresis (to remove the antibody) and steroids has proved effective in a minority of cases. Changes that occur in the lung vary with the type and amount of dust inhaled, particle size, and the presence of other lung diseases, most importantly those associated with cigarette smoking. Some dusts such as coal dust do not evoke a fibrous response (noncollagenous pneumoconioses), whereas others such as silica do (collagenous pneumoconioses). In some patients, inhalation of several different kinds of dust results in mixed disease (eg, anthracosilicosis). There is a variable latent period between exposure to dust and onset of clinical disease that may be as long as 20-30 years. Cobalt Tin1 Kaolin or porcelain clay Ceramics manufacture Diatomaceous earth pneumoconiosis Diatomaceous earth these dusts in pure form tend not to cause fibrosis (noncollagenous pneumoconioses) and have minimal clinical effects. Other dusts, especially silica and asbestos, may lead to severe fibrosis (collagenous pneumoconioses). There may also be insignificant dilation of the respiratory bronchiole ("focal dust emphysema"). Rarely, coal workers develop progressive massive fibrosis when heavy exposure is coupled with a complicating factor such as infection with Mycobacterium tuberculosis (found in 40% of patients with massive fibrosis), significant silica contamination (silica induces fibrosis), or development of allergic responses to various proteins that have passively adsorbed onto the coal dust. Progressive massive fibrosis is characterized by the presence of multiple irregular, firm, homogeneous black fibrous masses in both lungs. More than 1 million workers in the United States are at risk for developing silicosis. Significant pulmonary disease usually occurs with 10-15 years of exposure but may rarely occur after as little as 1 year. Larger crystals (> 5 (im) are caught in the bronchial mucus layer and wafted upward by the ciliary action to be expelled; particles less than 1 jum remain airborne and are exhaled. Silica is toxic to the internal organelle membranes of the macrophages and causes phagolysosomal disruption, cell death, and liberation of free silica particles. Silica crystals are also carried in lymphatics to the hilar lymph nodes, where similar silicotic nodules form. One hypothesis suggests that fibrosis is the result of a fibroblast-stimulating factor liberated by macrophages upon phagocytosis of silica particles. A second hypothesis attributes fibrosis to a lymphokine produced by silica-activated T lymphocytes. Grossly, the silicotic nodule is gray-black (due to associated carbon pigment), hard, and brittle and has concentric rings of hyalinized collagen in cross sec- Silicosis is caused by inhalation of crystalline silicon dioxide (silica) dust particles in the range of 1-5 jum. Nodules are found mainly along lymphatic pathways, especially around the hilum and in the upper lobes. Silica particles are recognized as birefringent needle-shaped crystals in the nodules when examined by polarized light. Clinical Features Silicosis is often asymptomatic, being found incidentally at chest x-ray or histologic examination of lungs and hilar lymph nodes removed for an unrelated reason. Rarely, when patients are exposed to massive amounts of dust, acute lung disease may occur, with alveolar thickening and accumulation of proteinaceous material in the alveoli (acute silicotic proteinosis). More often, there is chronic pulmonary fibrosis with a mild restrictive ventilatory defect, slowly progressive dyspnea, and pulmonary hypertension (cor pulmonale). Complications Progressive massive fibrosis may complicate chronic silicosis, particularly when the level of exposure to dust is high. The disorder is characterized by confluence of silicotic nodules into large masses of fibrous tissue that cause obliteration of vessels and bronchioles. Central necrosis and cavitation may occur in these masses as a result of ischemia. Progressive massive fibrosis commonly involves the upper lobes and is associated with a significant ventilatory defect and respiratory failure. Patients with silicosis have a greatly increased incidence of tuberculosis, believed to be due to the adverse effects of silica dust on macrophage function. Tuberculosis causes extensive necrosis in the nodules, and large numbers of tubercle bacilli can be found in such lesions. Silicosis is also associated with an increased incidence of autoimmune disease, especially progressive systemic sclerosis. It is present in such diverse components of the modern environment as insulation, flame retardants, flooring and roofing materials, water and sewage pipes, and brake linings in vehicles, making low-grade exposure almost universal among urban dwellers. It is estimated that up to 11 million workers in the United States have had significant asbestos exposure since 1940. Asbestos-related disease was first recognized in those with the highest levels of exposure, ie, workers in shipyards and the construction industry. It is becoming clear, however, that lower levels of exposure are also associated with significant risk. Asbestos-related neoplasms occur in families of shipyard workers- due presumably to the presence in the home of contaminated clothing-and in communities with asbestos-based industries (air pollution by asbestos dust).

The lens is derived from surface ectoderm and consists of Glaucoma is defined as an increase in intraocular pressure sufficient to cause degeneration of the optic disk and optic nerve fibers chronic gastritis months cheap renagel amex. Normal intraocular pressure as measured by tonometry (which measures the pressure required to cause flattening of the cornea to a specified amount) is 10-20 mm Hg; elevations in pressure are thought to have a dual effect gastritis fiber buy line renagel, inducing deformational changes in the optic disk plus decreased retinal blood flow gastritis diet vanilla discount generic renagel canada. However chronic gastritis weight loss order 800mg renagel otc, the correlation between intraocular pressure and optic nerve damage is not exact gastritis diet 5 small cheap renagel master card. Glaucoma is the result of an abnormality in the dynamics of aqueous humor circulation chronic gastritis diagnosis generic renagel 800 mg with visa. Aqueous humor production occurs at the ciliary body, partly by diffusion from plasma and partly by ^active secretion by the epithelium of the ciliary processes. Absorption of aqueous humor occurs at the iridocorneal angle by the trabecular meshwork and canal of Schlemm. Glaucoma is a common disorder, with about 2% of all people over 40 years of age affected. Glaucoma may occur as a complication of other diseases affecting the eye (secondary glaucoma) or as a primary disease. It occurs in individuals over 40 years of age and is responsible for over 90% of cases of primary glaucoma. It is characterized by a slow rise in intraocular pressure with subtle microscopic abnormalities in the canal of Schlemm. There is progressive degeneration of the optic disk, an increase in size of the blind spot (scotoma), and peripheral visual field loss, ultimately causing blindness. Treatment with pupillary constrictors such as pilocarpine and laser trabeculoplasty produce temporary improvement. Surgical treatment, which is indicated in severe cases, is successful in about 75% of cases in arresting visual loss. Primary Angle-Closure Glaucoma Angle-closure (closed-angle) glaucoma usually presents acutely. Increasing lens size, a normal occurrence with increasing age, causes forward displacement of the lens, further narrowing the angle. Acute attacks may be precipitated by dilation of the pupil (as in preparation for funduscopy), which further narrows the angle by thickening the periphery of the iris during pupillary dilation. Rapid increase of intraocular pressure causes severe pain, often accompanied by vomiting and rapid visual impairment. Treatment with osmotic agents and pupillary constrictors such as pilocarpine is effective in interrupting the acute attack. Peripheral iridectomy by laser is effective both in the acute relief of symptoms and in preventing further episodes. Anterior lens dislocation often causes obstruction to aqueous flow, leading to acute secondary glaucoma. Posterior dislocation of an intact lens does not cause severe symptoms except visual impairment. If the lens capsule is ruptured, lens protein may enter the bloodstream and stimulate antibody formation (lens protein contains antigens sequestered from the immune system during fetal life and is therefore regarded as foreign). This may result in immunologic endophthalmitis, with lymphocytic infiltration around the ruptured lens. Retinitis pigmentosa is a group of degenerative disorders with variable inheritance, most often recessive. Expression is variable, but retinal degeneration usually begins in early life and progresses slowly to blindness at age 50-60 years. The degeneration begins in the peripheral part of the retina, causing progressive loss of the peripheral visual field. Pathologically, there is disappearance of the rod and cone layer and loss of ganglion cells. Untreated, the detachment progresses, ultimately involving the entire retina and causing blindness. Laser treatment is effective in stopping the progression of retinal detachment and reversing the visual loss. Edema and leakage of blood leads to organization, fibrous traction on the retina, retinal detachment, and blindness. Careful control of oxygen therapy in the premature newborn has reduced the incidence of retrolental fibroplasia. It usually reflects degeneration of optic nerve fibers and has many causes (Table 33-4). Primary optic atrophy-resulting from diseases of the optic disk-is distinguished from secondary optic atrophy, which is due to longstanding edema of the disk caused by increased intracranial pressure. Vascular lesions causing occlusion of the central artery of the retina result in pale retinal infarction. Arterial emboli-either cholesterol emboli, derived from atheromatous plaques in the carotid circulation, or septic emboli in septicemic states such as infective endocarditis-may produce microinfarcts in the retina. In the United States and Europe, it is the most common intraocular malignant neoplasm. Pathology Intraocular malignant melanomas arise in the uveal tract (85% in the choroid, 10% in the ciliary body, 5% in the iris). They are composed of proliferating, invasive melanocytes, with several morphologic subTable 33-4. Optic neuritis Infections: mumps, measles viruses; leprosy; syphilis Demyelinating diseases: multiple sclerosis and variants Ischemia: arteriosclerosis, giant cell arteritis Increased intracranial pressure: chronic low-grade Metabolic disorders: nutritional (vitamin B. Detachment deprives the neuroepithelial layer of its choroidal blood supply and causes degeneration within 4-6 weeks. Spindle cell type A tumor, composed of slender cells with elongated nuclei and no nucleoli, has the best prognosis (85% 10-year survival). Spindle cell type B tumors, composed of more ovoid spindle cells with nucleoli, have a slightly worse prognosis (80% 10-year survival). Epithelioid cell tumors, composed of large pleomorphic round cells with hyperchromatic nuclei, nucleoli, and a high mitotic rate, have a bad prognosis (35% 10-year survival). Over 50% of melanomas of the uveal tract contain mixtures of the above cell types. Clinical Features Melanomas arising in the iris become visible as a black mass in the front of the eye and usually present at an early stage. The combination of early presentation and favorable histologic type gives iris melanomas a high survival rate (nearly 100%) after local surgical removal. Melanomas arising in the ciliary body and choroid generally attain a large size before they are detected. They grow inward into the vitreous, producing detachment of the retina and visual impairment, which is the usual presenting feature. It occurs almost exclusively in children under 5 years of age, with a frequency of about 1:20,000. Genetic Features (Chapter 18) Inherited cases commonly have bilateral retinoblastoma. Retinoblastoma is associated with a constant karyotypic abnormality (deletion of 13q-). Molecular studies show that a pair of recessive genes is involved, both of which must have undergone mutation to produce the disease. In the hereditary form, one recessive gene is inherited in mutant form; the other suffers an acquired mutation, after which the tumor develops. Microscopically, retinoblastoma is composed of undifferentiated small cells with a high nuclear to cytoplasmic ratio and hyperchromatic nuclei. The presence of Flexner-Winter-steiner rosettes composed of the neoplastic cells arranged in an orderly fashion around a central lumen is a diagnostic feature. Biopsy was necessary to determine whether this was a benign nevus (which it proved to be) or a malignant melanoma. Retinoblastoma, showing a large retinal mass extending into the vitreous with multiple satellite nodules and invasion of the optic nerve. Note the white spot in the right pupil resulting from reflection of light from the retinal tumor. Treatment with radiation and chemotherapy has improved the prognosis somewhat in more advanced cases. A significant number (1-2%) of retinoblastomas undergo spontaneous regression-the most frequent human neoplasm to demonstrate this phenomenon. Regression is associated with cessation of proliferation of the neoplasm followed by fibrosis. Patients with inherited retinoblastomas that have regressed represent a source of transmission of the abnormal gene to the next generation. Neoplasms Chapter 36: Lung cancer (Chapter 36) is responsible for more deaths (over 125,000 annually) in the United States than any other type of cancer. Pneumonia and tuberculosis (Chapter 34) have decreased in importance as a cause of death in developed countries but remain serious problems in developing countries. Pulmonary embolism (Chapter 35) is a life-threatening complication of many clinical states and is usually secondary to venous thrombosis (see Chapter 9). The bronchi divide dichotomously, becoming gradually smaller and more thin-walled as they progress away from the hilum toward the periphery. Bronchioles are less than 2 mm in diameter, have smooth muscle walls, and terminate in the alveoli. The lining epithelium is ciliated columnar in the larger air passages and ciliated cuboidal in the distal bronchioles. Scattered "small granule cells" are present in the bronchi on the basement membrane between epithelial cells; these are neuroendocrine cells that contain serotonin, bombesin, and other polypeptides. Small domeshaped Clara cells in the terminal bronchioles secrete a protein that lines the small air passages. The pulmonary lobule is represented by the structures derived from a small bronchiole, composed of 5-7 terminal bronchioles and the structures distal to them. The pulmonary acinus is represented by the structures arising from a single terminal bronchiole and consists of respiratory bronchioles and alveoli. Respiratory bronchioles are lined by simple cuboidal epithelium and participate in gas exchange. The pleural cavity is lubricated by a small film of pleural fluid that permits movement of the lung in relation to the chest wall. The bronchial arteriolar branches follow the bronchial tree and have a nutritive function. The pulmonary artery divides to produce a network of capillaries, the primary function of which is gas exchange. Microbiologic interpretation requires care because sputum is almost invariably contaminated by saliva, which normally has a rich commensal flora. Pulmonary arteriography displays the major arteries of the lung and is used in the diagnosis of pulmonary embolism. Bronchoscopy also permits biopsy both of the bronchial wall and the lung parenchyma (transbronchial lung biopsy). Normally, 80% of lavage fluid cells are alveolar macrophages, 10-15% T lymphocytes, 1-5% B lymphocytes and plasma cells, 1-3% neutrophils, and 1% eosinophils. Percutaneous fine-needle aspiration under radiologic guidance is used for sampling mass lesions in the lung. Open lung biopsy is an effective method of obtaining representative tissue and is used mainly in diffuse lung disease; it can be done by thoracoscopy or through a limited thoracotomy. Chemical examination of an effusion characterizes it as a transudate or exudate (see Chapter 3. Needle biopsy of the pleura provides a sample of tissue for histologic examination. Gas exchange involves ventilation, the flow of air through the air passages into the acini; perfusion, the flow of blood in the pulmonary circulation to the alveolar capillaries; and diffusion, the passage of oxygen and carbon dioxide from alveolar air to blood and vice versa. Interference with these three processes may produce respiratory failure (Table 34-1). The respiratory center controls the muscles of respiration, the diaphragm, and the intercostal muscles. Ventilation may be quantitated as the volume of gas entering the alveoli per minute. Abnormal Ventilation Impairment of total alveolar ventilation occurs by two mechanisms (Table 34-1): A. This may be due to (1) failure of chest wall expansion as a result of respiratory muscle failure (brain stem, nerve, and muscle diseases) or (2) intrinsic disease in the lung, such as pulmonary fibrosis, that prevents expansion. Obstructive diseases, in which ventilation of the lung is impeded because of obstruction of the air passages. Increased alveolar ventilation caused by hyperventilation washes out carbon dioxide and causes hypocapnia (decreased Pco2). Failure of perfusion of part of the lung results in a decrease in the effective area available for gas exchange, thereby increasing the effective dead space volume. Partial loss of perfusion may be compensated by hyperventilation, which increases total ventilation; this response corrects the tendency to hypercapnia but not the hypoxemia unless the patient is breathing oxygen-enriched air. In diffusion defects, stimulation of the respiratory center by hypoxemia causes hyperventilation and hypocapnia (Table 34-2). It is important to note that the diagnosis of respiratory failure is made by arterial blood gas analysis and not by clinical examination. Respiratory failure may be acute or chronic and results from failure of ventilation, diffusion, or perfusion of the lungs (Table 34-1). The pattern of changes of blood gases may give an indication of the pathogenesis of respiratory failure (Table 34-2).

Treatment of pemphigus vulgaris and pemphigus foliaceus with mycophenolate mofetil gastritis symptoms relief discount 800 mg renagel overnight delivery. Successful treatment with mycophenolate mofetil of four Japanese patients with pemphigus vulgaris gastritis symptoms vs gallbladder cheap 800 mg renagel amex. Treatment of pemphigus vulgaris with mycophenolate mofetil as a steroid-sparing agent gastritis diet spanish generic 800mg renagel visa. Mycophenolate mofetil: a new therapeutic option in the treatment of blistering autoimmune diseases gastritis diet ��������� 800mg renagel amex. Treatment of pemphigus vulgaris and bullous pemphigoid with mycophenolate mofetil monotherapy gastritis diet ���� generic renagel 400 mg on line. Efficacy and safety of long-term mycophenolate sodium therapy in pemphigus vulgaris chronic gastritis meal plan buy renagel 800mg with visa. Enteric-coated mycophenolate sodium as a steroid-sparing agent in pemphigus treatment: a retrospective study. Bullous pemphigoid in late childhood successfully treated with mycophenolate mofetil as an adjuvant therapy. Adjuvant therapy of bullous pemphigoid with mycophenolate mofetil: old drug, new use. Treatment of cicatricial pemphigoid with mycophenolate mofetil as a steroid-sparing agent. Successful treatment of linear IgA disease with mycophenolate mofetil as a corticosteroid sparing agent. Treatment of linear IgA bullous dermatosis of childhood with mycophenolate mofetil. Childhood IgAmediated epidermolysis bullosa acquisita responding to mycophenolate mofetil as a corticosteroid-sparing agent. Successful treatment of subacute cutaneous lupus erythematosus with mycophenolate mofetil. Treatment of resistant discoid lupus erythematosus of the palms and soles with mycophenolate mofetil. Coexisting subacute and systemic lupus erythematosus after terbinafine administration: successful treatment with mycophenolate mofetil. Mycophenolate mofetil for the treatment of cutaneous lupus erythematosus with smoldering systemic involvement. Chilblain lupus erythematosus Hutchinson: successful treatment with mycophenolate mofetil. Nonrenal disease activity following mycophenolate mofetil or intravenous cyclophosphamide as induction treatment for lupus nephritis: findings in a multicenter, prospective, randomized, open-label, parallelgroup clinical trial. Mycophenolate sodium for subacute cutaneous lupus erythematosus resistant to standard therapy. Mycophenolate mofetil: a possible therapeutic agent for children with juvenile dermatomyositis. Mycophenolate mofetil as an effective corticosteroid-sparing therapy for recalcitrant dermatomyositis. Mycophenolate mofetil in the treatment of severe skin manifestations of dermatomyositis: a series of 4 cases. Mycophenolate mofetil (CellCept): an alternative therapy for autoimmune inflammatory myopathy. Experience of mycophenolate mofetil in 10 patients with autoimmune-related interstitial lung disease demonstrates promising effects. Hypomyopathic dermatomyositis associated with interstitial lung disease and good response to mycophenolate mofetil: case-based review. Induction of remission in active anti-neutrophil cytoplasmic antibody-associated vasculitis with mycophenolate mofetil in patients who cannot be treated with cyclophosphamide. Mycophenolate mofetil vs azathioprine for remission maintenance in antineutrophil cytoplasmic antibody-associated vasculitis: a randomized controlled trial. Mycophenolate mofetil is effective for maintenance therapy of hypocomplementaemic urticarial vasculitis. Mycophenolate mofetil is safe, well tolerated, and preserves lung function in patients with connective tissue disease-related interstitial lung disease. Use of mycophenolate mofetil to treat scleroderma-associated interstitial lung disease. Effect of mycophenolate mofetil on pulmonary function in sclerodermaassociated interstitial lung disease. A prospective open-label study of mycophenolate mofetil for the treatment of diffuse systemic sclerosis. Pilot study of anti-thymocyte globulin plus mycophenolate mofetil in recent-onset diffuse scleroderma. Mycophenolate mofetil as first-line treatment improves clinically evident early scleroderma lung disease. Observational study of treatment outcome in early diffuse cutaneous systemic sclerosis. Long-term efficacy and tolerability of mycophenolate mofetil therapy in diffuse scleroderma skin disease. Efficacy of mycophenolate mofetil and oral cyclophosphamide on skin thickness: post hoc analyses from two randomized placebo-controlled trials. Successful long-term treatment of severe atopic dermatitis with mycophenolate mofetil. First experience with enteric-coated mycophenolate sodium (Myfortic) in severe recalcitrant adult atopic dermatitis: an open-label study. Diffuse large B-cell lymphoma of the central nervous system in mycophenolate mofetil-treated patients with systemic lupus erythematosus. Incidence and risk factors for nonmelanoma skin cancer after heart transplantation. Significant risk factors for occurrence of cancer after renal transplantation: a single center cohort study of 1265 cases. Liver enzyme abnormalities in patients with atopic dermatitis treated with mycophenolate mofetil. Four cases of red blood cell aplasia in association with the use of mycophenolate mofetil in renal transplant patients. Acquired and reversible Pelger-Huet anomaly of polymorphonuclear neutrophils in three transplant patients receiving mycophenolate mofetil therapy. Neutrophil dysplasia characterised by a pseudo-Pelger-Huet anomaly occurring with the use of mycophenolate mofetil and ganciclovir following renal transplantation: a report of five cases. Risk of herpes zoster infection in patients with pemphigus on mycophenolate mofetil. Effect of mycophenolate mofetil on the pharmacokinetics of antiretroviral drugs and on intracellular nucleoside triphosphate pools. Glucocorticoids interfere with mycophenolate mofetil bioavailability in kidney transplantation. Blood cyclosporine level soon after kidney transplantation is a major determinant of rejection: insights from the Mycophenolate Steroid-Sparing Trial. Mycophenolate sodium increases cyclosporine blood levels in renal transplant patients. Reactivation of tuberculosis after conversion from azathioprine to mycophenolate mofetil 16 years after renal transplantation. Skin manifestations of systemic lupus erythematosus refractory to multiple treatment modalities: poor results with mycophenolate mofetil. Mycophenolate mofetil is an effective treatment for peristomal pyoderma gangrenosum. Recurrent erythema multiforme/Stevens-Johnson syndrome: response to mycophenolate mofetil. Successful treatment of ulcerated necrobiosis lipoidica with mycophenolate mofetil. Treatment of severe chronic idiopathic urticaria with oral mycophenolate mofetil in patients not responding to antihistamines and/or corticosteroids. The therapeutic effects of CsA in psoriasis were discovered fortuitously in 1979 by Mueller and Hermann2 during a pilot study to investigate CsA efficacy in rheumatoid arthritis and psoriatic arthritis. These investigators observed that in patients with psoriatic arthritis, the psoriasis improved with CsA treatment. Neoral, a more bioavailable and more consistently absorbed microemulsion formulation of CsA, was approved for prophylaxis of organ rejection in the United States in 1995. Neoral was approved for the treatment of rheumatoid arthritis and psoriasis in 1997. Even though CsA is highly effective for all forms of psoriasis and has been used worldwide to treat patients with psoriasis for many years, many dermatologists in the United States are not experienced with its use. Consequently, in this chapter an emphasis will be placed on communicating a proper perspective regarding the use of CsA in the treatment of severe psoriasis, in addition to discussing CsA pharmacology and guidelines for safe use of CsA (Table 17. It is available in original formulation (Sandimmune) or in a more consistently bioavailable microemulsion formulation (Neoral, Gengraf). Neoral is the only formulation of CsA that is approved for the treatment of psoriasis. The oral solution contains 100 mg/mL and should be diluted, preferably with orange juice or apple juice, immediately before it is administered, to make it more palatable. The soft gelatin capsules are much more convenient and are available in 25- and 100-mg strengths. The absolute bioavailability of Neoral has not been determined; however, its bioavailability is 10% to 54% greater than that of Sandimmune. CsA is excreted in human milk, and so breastfeeding should be avoided while on CsA. Gengraf is a subsequent trade name generic product that is considered bioequivalent to Neoral. This is thought to be because of the inhibition of the intracellular enzyme calcineurin by a complex formed between CsA and cyclophilin. A review of its pharmacodynamic and pharmacokinetic properties, and therapeutic use in immunoregulatory disorders. This calcineurin/"signal 1" system creates a highly efficient immunologic response to various antigenic (or superantigen) stimuli. CsA may also directly affect antigen-presenting cells (such as Langerhans cells), mast cells, and keratinocytes. These adhesion molecules enable circulating leukocytes to more effectively migrate through the endothelium into the tissue, and once in the epidermis, inflammatory cells initiate an ongoing inflammatory cascade. First, and probably the best use of CsA, is in patients presenting with widespread, intensely inflammatory psoriasis or with a sudden, severe flare. The second group comprises patients with moderate to severe psoriasis or disabling psoriasis who are (1) unable to tolerate, (2) have contraindications to , or (3) have failed other systemic therapies. In some cases, CsA may allow a patient to achieve sufficient clearing such that a previously ineffective therapy (such as potent topical corticosteroids) may become more useful in maintaining the remission. The third group of patients is those who are experiencing major life events, such as a wedding, where substantial clearing of psoriasis for a short period of time is important. In addition, because CsA is effective for all types of psoriasis, it can be strongly considered for erythrodermic and pustular psoriasis. Heydendael and colleagues compared the efficacy of CsA versus methotrexate in 85 patients with moderate to severe plaque psoriasis in a randomized controlled trial. There was no statistically significant difference between treatment groups in terms of efficacy and the duration of remission. A brief overview of these two uses of CsA is presented in the following sections, serving to further illustrate how this important drug works for a variety of immune-mediated dermatoses. In general, most patients have significant to marked improvement in the first 1 to 2 months of therapy. The majority of patients relapse after discontinuation of CsA, although the timing is variable, and sustained improvement is possible. Patients with cutaneous T-cell lymphoma, including mycosis fungoides, should not be given CsA. There is a commonly held belief that the use of CsA frequently puts psoriasis patients at risk for renal failure or serious renal dysfunction. For example, in the study involving kidney biopsies by Zachariae and associates,85 CsA was used in a manner inconsistent with current established guidelines. Their dosing schedules exceeded the current maximum recommended CsA dosage of 5 mg/kg per day, and CsA was used continuously for longer than 1 year, even when serum creatinine had increased beyond 30% of the baseline value. Historically, the thresh- old for adjusting the CsA dose was elevation of serum creatinine by 50% of baseline. This was based on recommendations from nephrologists that if a 50% increase of creatinine from baseline was not sustained for more than 3 months, elevation of serum creatinine would most likely revert back to normal, and the risk of irreversible damage to the kidneys was unlikely. In many cases, the development of hypertension (mean systolic blood pressure >140 mm Hg, mean diastolic blood pressure >90 mm Hg) during the use of CsA was thought to be because of the direct vasoconstrictive effect of CsA on the vascular smooth muscles in the kidneys. It is not known how much CsA contributes to the risk of skin cancer, as the patients were often exposed to carcinogens. Lamarque and coworkers92 found no increased risk of lymphoproliferative disorders in 1657 psoriasis patients treated with CsA. However, at least three B-cell lymphomas and two cutaneous T-cell lymphomas have been reported after CsA treatment for psoriasis. When duration of treatment was less than 1 year, discontinuation of CsA was followed by spontaneous remission of the lymphoma.

Intracellular copper produces an injury manifested as a progressive microvacuolar fatty change and focal liver cell necrosis gastritis shoulder pain order 400mg renagel otc. It may present gastritis diet 1000 buy cheap renagel on line, usually in late childhood gastritis diet jump 400 mg renagel, as acute hepatitis clinically resembling viral hepatitis gastritis symptoms remedy renagel 400 mg free shipping. With continuing liver cell necrosis diet gastritis adalah purchase renagel master card, it progresses to fibrosis and finally to cirrhosis with chronic liver failure chronic gastritis reflux buy 400 mg renagel. Histochemical methods (rubeanic acid stain) are not always positive because of the abnormal protein binding of the copper in liver cells. Microscopically, the neurons are decreased in number, representing chronic cell necrosis. This is not seen with the naked eye until a late stage of the disease but can be recognized early by slit-lamp examination. The abnormal gene results in hepatic synthesis of an abnormal ccj-antitrypsin molecule that accumulates in the liver cell cytoplasm, appearing as eosinophilic globules. They also stain by immunoperoxidase methods using antibody against ocj-antitrypsin. The demonstration of these globules establishes the diagnosis, which can be further confirmed by absence of oCj-antitrypsin in the blood. Affected patients present in early infancy following feeding of milk (which contains lactose, a disaccharide of glucose and galactose). Diagnosis in a neonate followed by administration of a diet that contains no milk products prevents liver damage. Cavernous Hemangioma Hemangiomas are common incidental findings at surgery, radiologic examination, and autopsy. Histologically, they are composed of large endotheliumlined spaces filled with blood. Peliosis Hepatis Peliosis hepatis is a rare degenerative condition associated with multiple blood-filled spaces in the liver, many of which lack an endothelial lining. Sclerosing Bile Duct Adenoma Bile duct adenoma is uncommon and usually presents as an incidental finding at surgery. Histologically, bile duct adenoma is composed of irregular glands surrounded by collagen. Liver Cell Adenoma Liver cell adenoma is a rare benign neoplasm that occurs mainly in women taking oral contraceptives and athletes taking anabolic steroids; some tumors have regressed when these drugs were withdrawn. Microscopically, liver cell adenomas are composed of cytologically benign hepatocytes arranged in thickened cords. The distinction from a well-differentiated hepatocellular carcinoma may be difficult. Clinically, patients may present with a mass, sudden pain due to infarction, or hemorrhage due to rupture through the liver capsule. Focal Nodular Hyperplasia Focal nodular hyperplasia is another mass lesion of the liver that has been etiologically related to oral contraceptives. Focal nodular hyperplasia most frequently presents as a solitary solid mass lesion, usually subcapsular, well circumscribed, and only rarely larger than 5 cm. On cut section, it is gray-white and typically has a central scar with bands of fibrosis radiating to the periphery. Microscopically, nodules of liver tissue are separated by fibrous bands in which portal tracts containing bile ductules can be identified. It is uncommon in Western Europe and North America (about 8000 cases a year in the United States). Etiology the cause is unknown, but several factors have been implicated: (1) Aflatoxin, a product of the fungus Aspergillus flavus, which grows on improperly stored grain and nuts (including peanuts), is toxic to liver cells. It is present in high levels in grain in Africa and Asia, leading to the suggestion that chronic ingestion of aflatoxin may be at least partially responsible for the high incidence of liver cell carcinoma in these areas; (2) Hepatitis B virus infection is strongly suspected of causing hepatocellular carcinoma. African and Far East countries where hepatocellular carcinoma is common have high rates of hepatitis carriers, probably with vertical transmission of the virus from generation to generation; and (3) Hepatitis C virus infection is also associated with hepatocellular carcinoma. Presentation and risk factors: Hepatocellular carcinoma, cholangiocarcinoma, carcinoma of gallbladder or biliary tract. The increased cell turnover in regenerative nodules of cirrhosis is associated with cytologic abnormalities that have been interpreted as premalignant dysplastic changes. While all types of cirrhosis may be complicated by carcinoma, the association is greatest with hemochromatosis, virus-induced cirrhosis, and alcoholic cirrhosis. Microscopically, the neoplasm is composed of abnormal liver cells of variable differentiation. The cells have enlarged nuclei that show prominent nucleoli and hyperchromatism and may contain bile in the cytoplasm. Invasion of hepatic venous radicles is a typical feature that permits differentiation from adenoma. It may be difficult to distinguish a poorly differentiated hepatocellular carcinoma from metastatic carcinoma. Well-differentiated hepatocellular carcinoma, showing trabeculae of malignant hepatocytes separated by sinusoidal spaces. This is characterized by absence of portal areas and greatly expanded trabeculae composed of several layers of malignant hepatocytes. A rare variant, fibrolamellar carcinoma, has a better prognosis than the usual hepatocellular carcinoma. The entity is defined by the presence of large polygonal cells with abundant eosinophilic cytoplasm separated by broad fibrous bands. Hepatocellular carcinoma, showing a large solitary nodule that is grossly encapsulated except in one area. Rarely, hepatocellular carcinoma may secrete an ectopic hormone, causing hypoglycemia (insulinlike polypeptide), polycythemia (erythropoietin), or hypercalcemia (parathyroid hormone-like polypeptide). Surgical resection is rarely undertaken for the treatment of hepatocellular carcinoma, and chemotherapy and radiotherapy are not very effective. The median survival after diagnosis is 2 months; the 5-year survival rate is almost nil. Europe but has a relatively high incidence in the Far East, where infection with the liver fluke, Clonorchis sinensis, is thought to be a predisposing factor. Grossly, Cholangiocarcinoma presents features indistinguishable from those of hepatocellular carcinoma. The presence of cytoplasmic mucin permits differentiation from hepatocellular carcinoma, which does not secrete mucin. Differentiation from metastatic adenocarcinoma is almost impossible on histologic grounds alone. The progress of disease is often slow, but bloodstream spread ultimately occurs, and the prognosis is poor. Thorium was deposited as refractile crystals in the portal tracts and acted as a carcinogen. The tumor appears as a solid, often very large hemorrhagic mass composed histologically of intercommunicating vascular spaces lined by malignant endothelial cells. Epithelioid Hemangioendothelioma this rare malignant neoplasm of endothelial cells has a slower rate of progression than angiosarcoma. Virtually any malignant neoplasm in the body can metastasize to the liver; those from the gastrointestinal tract (via the portal vein), breast, and lung and malignant melanoma are most common. However, differentiation of hepatocellular carcinoma from metastatic carcinoma is sometimes very difficult. If metastatic spread is from an adenocarcinoma, distinction from primary cholangiocarcinoma of the liver may be impossible unless a primary adenocarcinoma is found elsewhere in the body. Metastatic carcinoma in the liver, showing multiple nodules some of which have central necrosis and umbilication. Infantile hemangioendothelioma is a benign neoplasm of endothelial cells characterized by anastomosing vascular spaces. It is often large and tends to shunt blood from the arterial to the venous side, acting as an arteriovenous fistula and leading to a hyperdynamic circulation; the usual presentation is heart failure in infancy. It is solid, with areas of cystic change; microscopically, mesenchymal hamartoma is composed of bile duct-like structures admixed with disorganized mesenchymal elements. Hepatoblastoma is a malignant neoplasm composed of primitive cells that resemble fetal liver cells. Bile is an alkaline fluid that contains the excretory bilirubin pigments, bile acids and bile salts, cholesterol, inorganic ions, and mucus. Cholesterol, which is insoluble in water, is maintained in solution by the formation of complexes with the hydrophilic bile salts and lecithin. In 70% of patients, the common bile duct and pancreatic duct join at the ampulla of Vater and have a common duodenal opening. Histologically, the entire biliary tract is lined by mucus-secreting columnar epithelium. In the gallbladder, the epithelium is thrown up into delicate folds, and mucous glands are buried deeply in the smooth muscle wall (Aschoff-Rokitansky sinuses). The biliary system stores and delivers the bile secreted by the liver into the duodenum, with the gallbladder acting as a reservoir in which the bile is stored and concentrated (from about 1000 mL/d down to 50 mL/d). The gallbladder is not required for 663 Pain Several types of pain occur in diseases of the extrahepatic biliary system. Biliary colic is severe intermittent pain in the right upper abdomen that radiates to the back and right shoulder. It is caused by increased muscular contraction of the bile duct and occurs when there is bile duct obstruction. Vague epigastric pain (dyspepsia), which may be aggravated by ingestion of fatty foods, is common in patients with gallstones and chronic cholecystitis. Constant right upper abdominal pain, often severe, occurs in acute cholecystitis when extension of inflammation involves the pain-sensitive parietal peritoneum. The biliary system undergoes dilation proximal to the obstruction, and bile backs up in the liver (cholestasis). A: When obstructive jaundice is caused by a calculus in the common bile duct, there is no enlargement of the gallbladder. B: When obstructive jaundice is caused by anything other than gallstones, the gallbladder is enlarged. Oral cholecystography and intravenous cholangiography have a high failure rate in patients with obstructive jaundice because the dye is not excreted in adequate amounts in the bile. Other Radiologic Techniques Ultrasonography and computerized tomography are extremely accurate in detecting dilated bile ducts, calculi in the biliary system, and masses in the head of the pancreas and bile ducts. When a mass is present, a fine needle may be passed into it under radiologic guidance and material aspirated for cytologic diagnosis (fine-needle aspiration biopsy). Functional Evaluation Tests to evaluate obstructive jaundice have been considered in Chapter 42. Chemical examination of bile is of little clinical diagnostic value and is rarely performed. Anatomic abnormalities of the gallbladder include absence, duplication with the presence of two gallbladders, intrahepatic location, and floating gallbladder surrounded by peritoneum and connected to the inferior surface of the liver by a pedicle. A peculiar abnormality is a focal concentric narrowing of the body of the gallbladder, producing an expanded fundus ("phrygian cap" gallbladder). Atresia is failure of development of the lumen in the epithelial cord that ultimately becomes the bile ducts; this failure may be complete or incomplete. The cause of atresia is controversial; some authorities believe it is the result of an intrauterine infection. Atresia commonly involves the extrahepatic bile ducts only; more rarely, the intrahepatic ducts are involved. Complete atresia involving the entire system is associated with a high mortality rate. The liver shows the features of severe large bile duct obstruction with secondary biliary cirrhosis. In cases where atresia involves intrahepatic ducts, liver transplantation represents the only hope of survival. Choledochal cysts are caused by focal dilation, often massive, of the common bile duct. Clinical presentation is with pain, jaundice, and a cystic mass in the right upper quadrant. Recognition of these normal variations is important at cholecystectomy in order to prevent accidental ligation of ducts and blood vessels. Most gallbladder diseases are associated with the formation of gallstones (cholelithiasis). Gallstones are usually formed in the gallbladder and rarely in the common bile duct. Type Mixed Pure cholesterol Combined Pigment Calcium Frequency 80% Chemical Composition Cholesterol, calcium carbonate, calcium bilirubinate Cholesterol Pure cholesterol center, mixed shell Calcium bilirubinate Calcium carbonate Gross Appearance Multiple, small, faceted; variable in size and shape; smooth surface, yellow; laminated on cut section. Solitary, large, oval, white; rough surface; cut section: radiating crystalline structure Solitary or 2 stones; oval or barrel-shaped, yellow, smooth surface Multiple, very small, faceted, black Multiple, amorphous; small grains, rarely large 5% 10% Rare Very rare and combined cholesterol-based gallstones are common and are formed when the concentration of cholesterol is increased or when bile salts are decreased. Middle age, obesity, and multiparity ("fat, fertile females in their forties and fifties") increase the risk to as high as 20%. Oral contraceptives increase biliary cholesterol excretion and predispose to gallstones. Patients with diabetes mellitus also have an increased incidence of cholesterol gallstones, probably related to increased cholesterol levels in bile. Pigment (Bilirubin) Stones: these are uncommon and occur (1) in patients suffering from chronic hemolytic anemias such as sickle cell disease and thalassemia, in whom bilirubin excretion is greatly increased; and (2) in patients with parasitic infestations, most commonly Clonorchis sinensis, in whom the parasite ova form a nidus for pigment stones (see Oriental Cholangiohepatitis, Chapter 42). Asymptomatic Gallstones: Thirty percent or more of patients with gallstones have no symptoms, and gallstones are frequently found incidentally at radiologic examination. Only about 25% of gallstones contain sufficient calcium to be visible on plain x-rays, but ultrasonography and computerized tomography are highly effective at detecting gallstones. The presence of asymptomatic gallstones is not an indication for surgical removal.

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