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Microscopic polyangiitis, as its name implies, is the term given to nongranulomatous vasculitis involving small blood vessels It is often associated with ANCAs that produce a P-ANCA pattern on immunofluorescence testing and are directed against MPO, a constituent of neutrophil granules Because microscopic polyangiitis may involve medium-sized as well as small blood vessels and because it tends to affect capillaries within the lungs and kidneys, its spectrum overlaps those of both polyarteritis nodosa and Wegener s granulomatosis In a minority of cases, microscopic polyangiitis appears to be induced by reactions to medications, particularly propylthiouracil, hydralazine, allopurinol, penicillamine, minocycline, and sulfasalazine In rare instances, these drugs induce a systemic vasculitis associated with MPO-ANCA Such syndromes usually resolve with discontinuation of the offending drug
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Treatment
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In microscopic polyangiitis, patients are likely to require prednisone and cyclophosphamide because of the urgency in treating pulmonary hemorrhage and glomerulonephritis Cyclophosphamide may be administered either in an oral daily regimen or via intermittent (usually monthly) intravenous pulses The typical oral dose is 2 mg/kg/d, but this may need to be decreased substantially for patients with significant renal dysfunction (for example, the dose for patients receiving dialysis is 08 10 mg/kg/d) For intermittent administration, cyclophosphamide is usually dosed at 750 mg/m2 (500 mg/m2 for those with renal failure) Whenever cyclophosphamide is used, Pneumocystis jiroveci prophylaxis with either singlestrength trimethoprim-sulfamethoxazole or dapsone 100 mg daily is essential Following the induction of remission, azathioprine is a reasonable choice to replace cyclophosphamide
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Clinical Findings
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A wide variety of findings suggesting vasculitis of small blood vessels may develop in microscopic polyangiitis These include palpable purpura and numerous other signs of cutaneous vasculitis; hematuria, proteinuria, and red blood cell casts in the urine; and pulmonary hemorrhage The renal lesion is a segmental, necrotizing glomerulonephritis, often with localized intravascular coagulation and the observation of intraglomerular thrombi upon renal biopsy The pathologic findings in the lung are typically those of capillaritis Vasculitic neuropathy (mononeuritis multiplex) is also common in microscopic polyangiitis Microscopic polyangiitis is the most common cause of pulmonary-renal syndromes, being several times more common than Goodpasture s (antiglomerular basement membrane) syndrome In a subset of patients with microscopic polyangiitis, interstitial lung fibrosis that mimics usual interstitial pneumonitis is the presenting condition Microscopic polyangiitis is defined as a pauci-immune necrotizing vasculitis with few or no immune deposits that (1) affects small blood vessels (capillaries, venules, or arterioles); (2) often includes glomerulonephritis and pul-
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Prognosis
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The key to effecting good outcomes is early diagnosis Compared with patients who have Wegener s granulomatosis, those who have microscopic polyangiitis are more likely to have significant fibrosis on renal biopsy because of later diagnosis The likelihood of disease recurrence following remission in microscopic polyangiitis is about 33%
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Bonaci-Nikolic B et al Antineutrophil cytoplasmic antibody (ANCA)-associated autoimmune diseases induced by antithyroid drugs: comparison with idiopathic ANCA vasculitides Arthritis Res Ther 2005;7(5):R1072 81 [PMID: 16207324] Hogan SL et al Predictors of relapse and treatment resistance in antineutrophil cytoplasmic antibody-associated small-vessel vasculitis Ann Intern Med 2005 Nov 1;143(9):621 31 [PMID: 16263884] Molloy ES et al Advances in the treatment of small vessel vasculitis Rheum Dis Clin North Am 2006 Feb;32(1):157 72 [PMID: 16504828] Seo P et al The antineutrophil cytoplasmic antibody-associated vasculitides Am J Med 2004 Jul 1;117(1):39 50 [PMID: 15210387]
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CMDT 2008
The disease is usually self-limited, lasting 1 6 weeks, and subsides without sequelae if renal involvement is not severe Chronic courses with persistent or intermittent skin disease are more likely to occur in adults than in children The value of corticosteroids has been controversial In children, prednisone (1 mg/kg/d orally) may benefit those with severe extrarenal manifestations and with evidence of renal disease The incremental efficacy of steroid-sparing drugs such as azathioprine and mycophenolate mofetil often used in the setting of renal disease is not known
Coppo R et al Predictors of outcome in Henoch-Schonlein nephritis in children and adults Am J Kidney Dis 2006 Jun; 47(6):993 1003 [PMID: 16731294] Ronkainen J et al Early prednisone therapy in Henoch-Schonlein purpura: a randomized, double-blind, placebo-controlled trial J Pediatr 2006 Aug;149(2):241 7 [PMID: 16887443]
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