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CMDT 2008
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Table 20 13 Classification scheme of vasculitides according to size of predominant blood vessels involved
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Primary vasculitides Predominantly large-vessel vasculitides Takayasu s arteritis Giant cell arteritis (temporal arteritis) Beh et s disease1 Predominantly medium-vessel vasculitides Polyarteritis nodosa Buerger s disease Predominantly small-vessel vasculitides Immune-complex mediated Cutaneous leukocytoclastic angiitis ( hypersensitivity vasculitis ) Henoch-Sch nlein purpura Essential cryoglobulinemia2 ANCA-associated disorders3 Wegener s granulomatosis2 Microscopic polyangiitis2 Churg-Strauss syndrome2
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May involve small, medium, and large-sized blood vessels Frequent overlap of small and medium-sized blood vessel involvement 3 Not all forms of these disorders are always associated with ANCA ANCA, antineutrophil cytoplasmic antibodies
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Vasculitis is a heterogeneous group of disorders characterized by inflammation within the walls of affected blood vessels The major forms of primary systemic vasculitis are listed in Table 20 13 The first consideration in classifying cases of vasculitis is the size of the major vessels involved: large, medium, or small The presence of the clinical signs and symptoms shown in Table 20 14 help distinguish among these three groups After determining the size of the
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Table 20 14 Typical clinical manifestations of large-, medium-, and small-vessel involvement by vasculitis
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Vigorous fluid resuscitation (4 6 L/d, with careful monitoring for fluid overload), mannitol (100 mg/d), and urine alkalinization are suggested early in the course, but definitive evidence for the efficacy of these measures is lacking On occasion, oliguric tubular necrosis may be converted to a nonoliguric variety, and though the prognosis for recovery of renal function and mortality is the same many clinicians believe it is easier to care for nonoliguric disease because hyperkalemia and pulmonary edema are less important concerns Myopathic complications of statins usually resolve within several weeks of discontinuing the drug
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Kashani A et al Risks associated with statin therapy: a systematic overview of randomized clinical trials Circulation 2006 Dec 19;114(25):2788 97 [PMID: 17159064] Thompson PD et al Statin-associated myopathy JAMA 2003 Apr 2;289(13):1681 90 [PMID: 12672737]
Constitutional symptoms: fever, weight loss, malaise, arthralgias/arthritis Limb claudication Asymmetric blood pressures Absence of pulses Bruits Aortic dilation Cutaneous nodules Ulcers Livedo reticularis Digital gangrene Mononeuritis multiplex Microaneurysms Purpura Vesiculobullous lesions Urticaria Glomerulonephritis Alveolar hemorrhage Cutaneous extravascular necrotizing granulomas Splinter hemorrhages Uveitis Episcleritis Scleritis
CMDT 2008
major vessels involved, other issues that contribute to the classification include the following: Does the process involve arteries, veins, or both What are the patient s demographic characteristics (age, gender, ethnicity, smoking status) Which organs are involved Is there evidence of immune complex deposition Is there granulomatous in ammation on tissue biopsy Are antineutrophil cytoplasmic antibodies (ANCA) present In addition to the disorders considered to be primary vasculitides, there are also multiple forms of vasculitis that are associated with other known underlying conditions These secondary forms of vasculitis occur in the setting of infections (eg, hepatitis B or C), connective tissue disorders, inflammatory bowel disease, malignancies, and reactions to medications Only the major primary forms of vasculitis are discussed here
Clinical Findings
A Symptoms and Signs
The clinical onset is usually insidious, with fever, malaise, weight loss, and other symptoms developing over weeks to months Pain in the extremities is often a prominent early feature caused by arthralgia, myalgia (particularly affecting the calves), or neuropathy The combination of mononeuritis multiplex (with the most common finding being footdrop) and features of a systemic illness is one of the earliest specific clues to the presence of an underlying vasculitis Polyarteritis nodosa is among the forms of vasculitis most commonly associated with vasculitic neuropathy In polyarteritis nodosa, the typical skin findings livedo reticularis, subcutaneous nodules, and skin ulcers reflect the involvement of deeper, medium-sized blood vessels Digital gangrene is not an unusual occurrence The most common cutaneous presentation is lower extremity ulcerations, usually occurring near the malleoli Involvement of the renal arteries leads to a renin-mediated hypertension (much less characteristic of vasculitides involving smaller blood vessels) For unclear reasons, classic polyarteritis nodosa seldom (if ever) involves the lung, with the occasional exception of the bronchial arteries Abdominal pain particularly diffuse periumbilical pain precipitated by eating is common but often difficult to attribute to mesenteric vasculitis in the early stages Nausea and vomiting are common symptoms Infarction compromises the function of major viscera and may lead to acalculous cholecystitis or appendicitis Some patients present dramatically with an acute abdomen caused by mesenteric vasculitis and gut perforation or with hypotension resulting from rupture of a microaneurysm in the liver, kidney, or bowel Subclinical cardiac involvement is common in polyarteritis nodosa, and overt cardiac dysfunction occasionally occurs (eg, myocardial infarction secondary to coronary vasculitis, or myocarditis)
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