the Vasculitis Syndromes

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خب ۱7 ® Vasculitis is a clinicopathologic process characterized by inflammationof and damage to blood vessels. 

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a Secondary Vasculitis Syndromes Vasculitis associated with probable etiology Dru Hepatitis C virus-associated cryoglobulinemic vasculitis Hepatitis B virus-associated vasculitis induced vasculitis Cancer-associated vasculitis Vasculitis associated with systemic disease Lupus vasculitis Rheumatoid vasculitis Sarcoid vasculitis La Primary Vasculitis Syndromes Granulomatosis with polyangiitis (Wegener's) Microscopic polyangiitis Eosinophilic granulomatosis with polyangiitis (Churg-Strauss) IgA vasculitis (Henoch-Schontein) Cryoglobulinemic vasculitis Polyarteritis nodosa Kawasaki disease Giant cell arteritis Takayasu arteritis Behcet's disease Cogan’s syndrome Single-organ vasculitis Cutaneous leukocytoclastic angiitis Cutaneous arteritis Primary central nervous system vasculitis Isolated aortitis 

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eS 4 ۱۱۱۱3 ‏ور‎ POTENTIAL MECHANISMS OF VESSEL DAMAGE IN VASCULITIS SYNDROMES Pathogenic immune-complex formation and/or deposition IgA vasculitis (Henoch-Schénlein) Lupus vasculitis Serum sickness and cutaneous vasculitis syndromes Hepatitis C virus-associated cryoglobulinemic vasculitis Hepatitis B virus-associated vasculitis Production of antineutrophilic cytoplasmic antibodies Granulomatosis with polyangiitis (Wegener's) Microscopic polyangiitis Eosinophilic granulomatosis with polyangiitis (Churg-Strauss) Pathogenic T lymphocyte responses and granuloma formation Giant cell arteritis Takayasu arteritis Granulomatosis with polyangiitis (Wegener's) Eosinophilic granulomatosis with polyangiitis (Churg-Strauss) 

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ee ai ‏سس‎ ‎APPROACH TO THE PATIENT: general principles of Diagnosis ® unexplained systemic illness © palpable purpura © pulmonary infiltrates ® microscopic hematuria, © chronic inflammatory sinusitis, ® mononeuritis multiplex, ® unexplained ischemic events © glomerulonephritis with evidence of multisystem disease. 

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© the first step in the workup of a patient with suspected vasculitis is to exclude other diseases that produce clinical manifestations that can mimic vasculitis ® a series of progressive steps that establish the diagnosis of vasculitis and determine, where possible, the category of the vasculitis syndrome 

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خب ۱7 ® The yield of “blind” biopsies of organs with no subjectiveor objective evidence of involvement is very low and should be avoided. ® When syndromes such as polyarteritis nodosa, Takayasu arteritis, or primary central nervous system (CNS) vasculitis are suspected, arteriogram of organs with suspected involvement should be performed. 

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‘Atheroembolc disease ‘Antiglomerular basement membrane disease (Goodpasture's synciome) ‘Amyloidosis Migraine يي ل ۰ 

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© If an offending antigen that precipitates the vasculitis is recognized, the antigen should be removed where possible © If the vasculitis is associated with an underlying disease such as an infection, neoplasm, or connective tissue disease, the underlying disease should be treated 

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خب ۱7 ® glucocorticoids and/or cytotoxic therapy should be instituted immediately in diseases where irreversible organ system dysfunction and high morbidity and mortality rates have been clearly established ® Granulomatosis with polyangiitis (Wegener's) is the prototype of a severe systemic vasculitis requiring such a therapeutic approach 

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© On the other hand, when feasible, aggressive therapy should be avoided for vasculitic manifestations that rarely result in irreversible organ system dysfunction and that usually do not respond to such therapy © For example, idiopathiccutaneous vasculitis 

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خب ۱7 ® Glucocorticoids should be initiated in those systemic vasculitides that cannot be specifically categorized or for which there is no established standard therapy; cytotoxic therapy should be added in these diseases only if an adequate response does not result or if remission can only be achieved and maintained with an unacceptably toxic regimen of glucocorticoids 

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خب ۱7 © When remission is achieved, one should continually attempt to taper glucocorticoids and discontinue when possible © Methotrexate and azathioprine are also associated with bone marrow suppression, and complete blood counts should be obtained every 1-2 weeks for the first 1-2 months after their initiation and once a month thereafter. 

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خب ۱7 ® To lessen toxicity, methotrexate is often given together with folic acid © With the use of daily cyclophosphamide: © minimization of bladder toxicity and prevention of leukopenia 

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© Bladder cancer can occur several years after discontinuation of cyclophosphamide therapy © Bone marrow suppression is an important toxicity of cyclophosphamide © Maintaining the white blood count (WBC) at >3000/L and the neutrophil count >1500/L 

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خب ۱7 GPagraonbinsat oul Paseulitis ofWheampper and lower respiratory tracts together with glomerulonephritis © the mean age of onset is 40 years. ® The histopathologic hallmarks of granulomatosis with polyangiitis (Wegener's) are necrotizing vasculitis of small arteries and veins together with granuloma formation 

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خب ۱7 © Lung involvement typically appears as multiple, bilateral, nodular cavitary infiltrates 

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HIGURE386e-2 Computed tomography of the chest in two patients with grenuiomatesis with polyangitis (Wegener) demonstrating (@)single and (8) mutipie cavitary lung lesions, 

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A FIGURE386e33 Bilateral ground-glass infiltrates Cue to alveolar hemorrhage from pulmonary capilaits as seen in the same patient by (A) chest radiograph and (B) computed tomography. This manifestation can occur in granulomatosis with polyangits (Wegener's) or micio- scopic polyanait. 

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HIGURE 286e1 Bilateral nedular infiltrates seen on computed tomography of the ches: ina 40 year old woman with granulomatosis with polyangitis Wegeners). 

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FIGURE 386e-4 Computed tomography of the chest de 

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FIGURE 3866-5 ۳ tomography of the orbits with granulomatosis / ٩۱8۲ 5( ۵ monstrates inflamr jid sinus through the lamina p right-ey ding f filing 

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خب ۱7 © Granuloma formation is only rarely seen on renal biopsy 

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® Involvement of the upper airways occurs in 95% of patients with granulomatosis with polyangiitis (Wegener's). ® paranasal sinus pain and drainage and purulent or bloody nasal discharge, with or without nasal mucosal ulceration 

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خب ۱7 6 Nasal septal perforation may follow, leading to saddle nose deformity © Serous otitis media © Subglottic tracheal stenosis resulting from active disease 

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Saddle nose deformity in relapsing polychondritis = Saddle nose deformity (arrow) due to cartilage damage in a patient with relay Courtesy of Clement 

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خب ۱7 ® Pulmonary involvement may be manifested as asymptomatic infiltrates or may be clinically expressed as cough, hemoptysis, dyspnea, and chest discomfort. It is present in 85-90% of patients 

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© Eye involvement (52% of patients) may range from a mild conjunctivitis to dacryocystitis, episcleritis, scleritis, granulomatous sclerouveitis, ciliary vessel vasculitis, and retroorbital mass lesions leading to proptosis. 

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© Skin lesions (46% of patients) appear as papules, vesicles, palpable purpura, ulcers, or subcutaneous nodules © Renal disease (77% of patients) generally dominates the clinical picture and, if left untreated, accounts directly or indirectly for most of the mortality rate in this disease 

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خب ۱7 © proteinuria, hematuria, and red blood cell casts, it is clear that once clinically detectable renal functional impairment occurs 

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® Characteristic laboratory findings include a markedly elevated erythrocyte sedimentation rate (ESR), mild anemia and leukocytosis, mild hypergammaglobulinemia (particularly of the IgA class), and mildly elevated rheumatoid factor 

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خب ۱7 © Approximately 90% of patients with active granulomatosis with polyangiitis (Wegener's) have a positive antiproteinase-3 ANCA. © increased incidence of venous thrombotic events 

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خب ۱7 0899985 tissue offers the highest diagnostic yield © Renal biopsy can confirm the presence of pauci-immune glomerulonephritis 

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© The specificity of a positive antiproteinase-3 ANCA for granulomatosis with polyangiitis (Wegener's) is very high, especially if active glomerulonephritis is present. © However, the presence of ANCA should be adjunctive and, with rare exceptions, should not substitute for a tissue diagnosis. False- positive ANCA titers have been reported in certain infectious and neoplastic diseases. 

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TERRGCOMRVIOM ASHE IEW EOlyanstis (Wegener's) symptomatic improvement, with little effect on the ultimate course of the disease. © The development of treatment with cyclophophamide dramatically changed patient outcome 

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© Treatment of granulomatosis with polyangiitis (Wegener's) is currently viewed as having two phases: ® induction, where active disease is put into remission, ® followed by maintenance. The decision regarding which agents to use for induction and maintenance is based upon disease severity together with individual patient factors that include contraindication, relapse history, and comorbidities. 

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خب ۱7 © After 3-6 months of induction treatment, cyclophosphamide should be stopped and switched to another agent for remission maintenance © methotrexate and azathioprine 

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© In patients who are unable to receive methotrexate or azathioprine or who have relapsed through such treatment, mycophenolate mofetil, 1000 mg twice a day, may also sustain remission following cyclophosphamide induction. 

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خب ۱7 © Rituximab Induction for Severe Disease © TMP-SMX may be of benefit in the treatment of granulomatosis with polyangiitis (Wegener's) isolated to the sinonasal tissues 

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خب ۱7 © Glomerulonephritis is very common in microscopic polyangiitis, and pulmonary capillaritis often occurs. © The absence of granulomatous inflammation in microscopic polyangiitis is said to differentiate it from granulomatosis with polyangiitis (Wegener's). 

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© The mean age of onset is 57 years of age, and males are slightly more frequently affected than females. © The renal lesion seen in microscopic polyangiitis is identical to that of granulomatosis with polyangiitis (Wegener's 

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خب ۱7 ©). Like granulomatosis with polyangiitis (Wegener's), microscopic polyangiitis is highly associated with the presence of ANCA, which may play a role in pathogenesis of this syndrome 

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‎and Laboratory Manifestations‏ یات ‎Glomerulonephritis occurs in at least 79% of‏ © ‎patients and can be rapidly progressive, leading to renal failure ‎© Hemoptysis may be the first symptom of alveolar hemorrhage ‎© Other manifestations include mononeuritis multiplex and gastrointestinal tract and cutaneousvasculitis. 

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خب ۱7 © Upper airway disease and pulmonary nodules are not typically found in microscopic polyangiitis and, if present, suggest granulomatosis with polyangiitis (Wegener's). 

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® elevated ESR, anemia, leukocytosis, and thrombocytosis. © ANCA are present in 75% of patients with microscopic polyangiitis, with antimyeloperoxidase antibodies being the predominant ANCA associated with this disease. 

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خب ۱7 © The diagnosis is based on histologic evidence of vasculitis or pauci-immune glomerulonephritis in a patient with compatible clinical features of multisystem disease 

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خب ۱7 6 the treatment approach for microscopic polyangiitis is the same as is used for granulomatosis with polyangiitis (Wegener's) 

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خب ۱7 Churg-Strauss Syndrome 8 Churg-Strauss syndrome, also referred to as allergic angiitis and granulomatosis © The mean age of onset is 48 years ® Skin lesions occur in 51% of patients and include purpura in addition to cutaneous and subcutaneous nodules 

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خب ۱7 ® fever, malaise, anorexia, and weight loss © The pulmonary findings in Churg-Strauss syndrome clearly dominate the clinical picture with severe asthmatic attacks and the presence of pulmonary infiltrates 

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خب ۱7 ® Mononeuritis multiplex is the second most common manifestation and occurs in up to 72% of patients. ® Allergic rhinitis and sinusitis ® Clinically recognizable heart disease occurs in 14% of patients and is an important cause of mortality 

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خب ۱7 © The characteristic laboratory finding in virtually all patients with Churg-Strauss syndrome is a striking eosinophilia, which reaches levels >1000 cells/L in >80% of patients 

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Eosinophilic granulomatosis with polyangiitis (Churg a Strauss) Cutaneous ulceration on the elbow of a patient with eosinophilic granulomatosis with polyangiitis (Churg-Strauss). Courtesy of Talmadge E King, Jr, MD. 

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® elevated ESR, fibrinogen, or 2-globulins ® The other laboratory findings reflect the organ systems involved. Approximately 48% of patients with Churg-Strauss syndrome have circulating ANCA that is usually antimyeloperoxidase. 

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خب ۱7 6 the diagnosis of Churg-Strauss syndrome is optimally made by biopsy in a patient with the characteristic clinical manifestations ® Glucocorticoids alone appear to be effective in many patients. 

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خب ۱7 ® In glucocorticoid failure or in patients who present with fulminant multisystem disease, the treatment of choice is a combined regimen of daily cyclophosphamide and prednisone 

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خب ۱7 ® also referred to as classic PAN © Itis a multisystem, necrotizing vasculitis of small and medium-sized muscular arteries in which involvement of the renal and visceral arteries is characteristic.. 

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خب ۱7 © PAN does not involve pulmonary arteries, although bronchial vessels may be involved; granulomas, significant eosinophilia, and an allergic diathesis are not observed 

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خب ۱7 © The vascular lesion in PAN is a necrotizing inflammation of small and medium-sized muscular arteries © The lesions are segmental and tend to involve bifurcations and branchings of arteries. 

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® Nonspecific signs and symptoms are the hallmarks of PAN © Fever, weight loss, and malaise are present in over one-half of cases ® Patients usually present with vague symptoms such as weakness, malaise, headache, abdominal pain, and myalgias that can rapidly progress to a fulminant illness 

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خب ۱7 ® In PAN, renal involvement most commonly manifests as hypertension, renal insufficiency, or hemorrhage due to microaneurysms. 

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‘Table 326-6 Clinical Manifestations Related to Organ System Involvement in Classic Polyarteritis Nodosa ‘Clinical Manifestations al falure, hypertension |arthiti, arthralgia, myalgia [Peripheral neuropathy, mononeuritis muitplex | abdominal pain, uses and vertng, bleeding, bonelinfarcton and perfration,chalecistits, ‏تسیا‎ 1 | kesh, purpura, nodules, cutaneous infarcts, lvedo reticularis, Raynaud's phenomenon | congestive heart faiure, myocar-ial infarction, pericarditis ‏تا‎ ‘ovarian, or epididymal p3in 1 | Cerebral vascular accident, altered mental status, seizure Percent Incidence 43 36 25 23 Organ System Renal Musculoskeletal Peripheral nervous system Gastrointestinal trect skin cardiac Genitourinary Central nervous system 

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© The anemia of chronic disease may be seen, and an elevated ESR is almost always present © Hypergammaglobulinemia may be present, and all patients should be screened for hepatitis B. © Antibodies against myeloperoxidase or proteinase-3 (ANCA) are rarely found in patients with PAN. 

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© The diagnosis of PAN is based on the demonstration of characteristic findings of vasculitis on biopsy material of involved organs ® In the absence of easily accessible tissue for biopsy, the arteriographic demonstration of involved vessels, particularly in the form of aneurysms of small and medium-sized arteries in the renal, hepatic, and visceral vasculature, is sufficient to make the diagnosis. 

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خب ۱7 ® Aneurysms of vessels are not pathognomonic of PAN; furthermore, aneurysms need not always be present, and arteriographic findings may be limited to stenotic segments and obliteration of vessels. 

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۲۱6۷8۲ 38621 Arteriogram of a 40-year-old man with polyarteri- tis nodosa demonstra s in the hepatic circulation 

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® Biopsy of symptomatic organs such as nodular skin lesions, painful testes, and nerve/muscle provides the highest diagnostic yields. © Death usually results from gastrointestinal complications, particularly bowel infarcts and perforation, and cardiovascular causes. 

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خب ۱7 ® Intractable hypertension often compounds dysfunction in other organ systems, such as the kidneys, heart, and CNS, leading to additional late morbidity and mortality in PAN 

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خب ۱7 © Favorable therapeutic results have been reported in PAN with the combination of prednisone and cyclophosphamide 

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خب ۱7 ® In patients with hepatitis B who have a PAN- like vasculitis, antiviral therapy represents an important part of therapy and has been used in combination with glucocorticoids and plasma exchange. 

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® Giant cell arteritis, also referred to as cranial arteritis or temporal arteritis, is an inflammation of medium- and large-sized arteries. © It characteristically involves one or more branches of the carotid artery, particularly the temporal artery 

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® it is a systemic disease that can involve arteries in multiple locations, particularly the aorta and its main branches. © Giant cell arteritis is closely associated with polymyalgia rheumatica, which is characterized by stiffness, aching, and pain in the muscles of the neck, shoulders, lower back, hips, and thighs. 

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® Giant cell arteritis occurs almost exclusively in individuals >50 years. © Giant cell arteritis is most commonly characterized clinically by the complex of fever, anemia, high ESR, and headaches in a patient over the age of 50 years. 

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Visibly enlarged temporal artery in a patient with giant cell arteritis (arrows). Courtesy of Gene G Hunder, MD. 

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® In patients with involvement of the cranial arteries, headache is the predominant symptom and may be associated with a tender, thickened, or nodular artery, which may pulsate early in the disease but may become occluded later. ® Scalp pain and claudication of the jaw and tongue may occur 

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۲۱608۲ 2860-11 ۵ arteriogram demonstrating a long stenotic lesion of the axillary artery in a 75-year-old female with giant cell arteritis. 

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FIGURE386e-12 Magnetic resonance imaging demonstrating extensive san poet disease ofthe tho 

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© A well-recognized and dreaded complication of giant cell arteritis, particularly in untreated patients, is ischemic optic neuropathy, which may lead to serious visual symptoms, even sudden blindness in some patients. 

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® Characteristic laboratory findings in addition to the elevated ESR include a normochromic or slightly hypochromic anemia ® Liver function abnormalities are common, particularly increased alkaline phosphatase levels. 

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© The diagnosis of giant cell arteritis and its associated clinicopathologic syndrome can often be suggested clinically by the demonstration of the complex of fever, anemia, and high ESR with or without symptoms of polymyalgia rheumatica in a patient >50 years 

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خب ۱7 © The diagnosis is confirmed by biopsy of the temporal artery ® biopsy segment of 3-5 cm © Ultrasonography of the temporal artery has been reported to be helpful in diagnosis 

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® Isolated polymyalgia rheumatica is a clinical diagnosis made by the presence of typical symptoms of stiffness, aching, and pain in the muscles of the hip and shoulder girdle, an increased ESR, the absence of clinical features suggestive of giant cell arteritis, and a prompt therapeutic response to low-dose prednisone. 

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سس Treatment: Giant Cell Arteritis and Polymyalgia ۵ © Giant cell arteritis and its associated symptoms are exquisitely sensitive to glucocorticoid therapy © The ESR can serve as a useful indicator of inflammatory disease activity in monitoring and tapering therapy © Patients with isolated polymyalgia rheumatica respond promptly to prednisone 

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Takayasu's arteritis ® aortic arch and its branches. ® Pulses are commonly absent in the involved vessels, particularly the subclavian artery © Hypertension occurs in 32-93% of patients and contributes to renal, cardiac, and cerebral injury. ® Characteristic laboratory findings include an elevated ESR, mild anemia, and elevated immunoglobulin levels. 

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FIGURE386e-13 _Arteriogram of the aortic arch demonstrating complete occlusion of the left common carotid artery just af origin from the aorta. This 20-year-old female presented with syncope and was subsequently diagnosed with Takayasu arteritis. 

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خب ۱7 © The diagnosis of Takayasu's arteritis should be suspected strongly in a young woman who develops a decrease or absence of peripheral pulses, discrepancies in blood pressure, and arterial bruits. 

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خب ۱7 ® the characteristic pattern on arteriography, which includes irregular vessel walls, stenosis, poststenotic dilation, aneurysm. formation, occlusion, and evidence of increased collateral circulation 

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خب ۱7 © The combination of glucocorticoid therapy for acute signs and symptoms and an aggressive surgical and/or arterioplastic approach to stenosed vessels has markedly improved outcome and decreased morbidity by lessening the risk of stroke, correcting hypertension due to renal artery stenosis, and improving blood flow to ischemic viscera and limbs. 

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Purpura ® also referred to as anaphylactoid purpura ® palpable purpura (most commonly distributed over the buttocks and lower extremities), arthralgias, gastrointestinal signs and symptoms, and glomerulonephritis 

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© most patients range in age from 4 to 7 years; however, the disease may also be seen in infants and adults ® by colicky abdominal pain usually associated with nausea, vomiting, diarrhea, or constipation and is frequently accompanied by the passage of blood and mucus per rectum; bowel intussusception 

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Petechiae in Henoch-Schénlein purpura (IgA vascu ‏تحص رن‎ Clusters of palpable, pruritic petechiae on the thigh of a patient with Henoch-Schénlein purpura (IgA vasculitis). These lesions could be mistaken for thrombocytopenic petechiae. 

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Skin manifestations of Henoch- vasculitis) hénlein purpura (IgA This picture shows the classic skin manifestations of Henoch-Schoniein purpure (IgA vasculitis), with clusters of typical ecchymoses, petechiae, and palpable lesions on the legs in a typical distribution (gravity/pressure-dependent areas), Courtesy of Susan Kim, MD. 

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خب ۱7 © proteinuria and microscopic hematuria, with red blood cell casts © Serum complement components are normal, and IgA levels are elevated in about one-half of patients. 

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® The diagnosis of Henoch-Schénlein purpura is based on clinical signs and symptoms. © Skin biopsy specimen can be useful in confirming leukocytoclastic vasculitis with IgA and C3 deposition by immunofluorescence 

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خب ۱7 © The prognosis of Henoch-Schonlein purpura is excellent. © glucocorticoid therapy ® plasma exchange combined with cytotoxic drugs 

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هت 6 ryoglobulinemic Vasculitis ® systemic vasculitis characterized by palpable purpura, arthralgias, weakness, neuropathy, and glomerulonephritis ® in association with a variety of underlying disorders including multiple myeloma, lymphoproliferative disorders, connective tissue diseases, infection, and liver disease 

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Leucocytoclastic vasc Leukocytoclastic vasculitis appearing as raised purpura. This lesion can occur with any vasculitic syndrome and in the collagen vascular diseases. Courtesy of Marvin I Schwarz, MD. 

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خب ۱7 ® chronic hepatitis C © The most common clinical manifestations of cryoglobulinemic vasculitis are cutaneous vasculitis, arthritis, peripheral neuropathy, and glomerulonephritis 

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FIGURE 386e-15 Arteriogram of the hand der kip lesion v na patient with 

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خب ۱7 © Rheumatoid factor is almost always found and may be a useful clue to the disease when cryoglobulins are not detected © Hypocomplementemia occurs in 90% ® elevated ESR and anemia 

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خب ۱7 ® the majority of cases are associated with hepatitis C infection. In such patients, treatment with IFN- and ribavirin ® Glucocorticoids © Plasmapheresis and cytotoxic agents 

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Isolated Vasculitis 

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Isolated Vasculitis of the Central Nervous System ® restricted to the vessels of the CNS without other apparent systemic vasculitis © altered mental function, and focal neurologic defects ® Systemic symptoms are generally absent 

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® abnormal MRI of the brain, an abnormal lumbar puncture, and/or demonstration of characteristic vessel abnormalities on arteriography © The prognosis of granulomatous PACNS is poor ® glucocorticoid therapy, alone or together with cyclophosphamide 

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FIGURE 386e-10 Cerebral arteriogram demonstrating beading along branches of the internal carotid artery v 

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9 Disease a ® also referred to as mucocutaneous lymph node syndrome ® an acute, febrile, multisystem disease of children © Some 80% of cases occur prior to the age of 5 

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® nonsuppurative cervical adenitis and changes in the skin and mucous membranes such as edema; congested conjunctivae; erythema of the oral cavity, lips, and palms; and desquamation of the skin of the fingertips. 

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® coronary artery aneurysms © These complications usually occur between the third and fourth weeks of illness during the convalescent stage © Other manifestations include pericarditis, myocarditis, myocardial ischemia and infarction, and cardiomegaly. 

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Courtesy of Robert Sundel, MD. 

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. Copyright Logical Images, Inc. 

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_ Strawberry tongue Reproduced with permission from: vwa.visualdx.com. Copyright Logical images, Inc 

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Indurated edema of the dorsum of the hands as seen a Kawasaki disease (acute phase) Reproduced with permission from: ww jaldx.com. Copyright Logical Images, Inc. 

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iste perungual desquamation of the hands: a and feet seen in Kawasaki disease 

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® High-dose IV globulin (2 g/kg as a single infusion over 10 h) together with aspirin (100 mg/kg per day for 14 days followed by 3-5 mg/kg per day for several weeks) ® Surgery may be necessary for Kawasaki disease patients that have giant coronary artery aneurysms or other coronary complications 

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