Crescentic Glomerulonephritis Requiring Hemodialysis and Elevated MPO-ANCA Level and Vasculitis Allergica Cutis in a 21-Year-Old Girl

Ismail H. Kara, MD; M. Emin Yilmaz, MD; Yasin Sari, MD; Ali Gür, MD; Hüseyin Büyükbayram, MD


May 01, 2002

Abstract and Introduction

Microscopic polyangiitis (mPA) is a systemic necrotizing nongranulomatous vasculitis that affects small blood vessels. Clinical features include constitutional symptoms such as fever, anorexia, fatigue, and weight loss. Skin lesions include purpura and splinter hemorrhages, which occur in 50% of patients. Another characteristic feature is rapidly progressive glomerulonephritis, which often affects the kidneys in the early stages of the condition. Diagnosis is based on typical clinical features, biopsy, and presence of antineutrophil cytoplasmic antibodies (ANCA). This disorder tends to involve middle-aged and older persons, with a predilection for males; it is very rare in children. A 21-year-old female patient with mPA who did not respond well to treatment, required hemodialysis, developed vasculitis allergica cutis (VAC) later, and had a relatively short-term survival is reported herein.

Small vessel vasculitides are being recognized more frequently, mainly because of increased awareness. Estimates of incidence have increased from fewer than 5 cases per million in the early 1980s to more than 20 per million.[1] Davson introduced the term "microscopic polyangiitis" into the literature in 1948. The Chapel Hill Consensus Conference on the Nomenclature of Systemic Vasculitis held in 1992 officially adopted the term to connote a necrotizing vasculitis with few or no immune complexes (pauci-immune) affecting small vessels (capillaries, venules, or arterioles).[2] At presentation, the clinical features of patients with mPA, Wegener's granulomatosis (WG), and Churg-Strauss syndrome (CSS) may include glomerulonephritis, alveolar hemorrhage, tracheobronchitis, sinusitis, palpable purpura, arthritis, ocular inflammation, and neuropathy.[3]

Patients with connective tissue diseases may also display many of these features. However, patients with connective tissue diseases are known to develop multiple autoantibodies; positivity for ANCA in such patients is rare. Therefore, testing for ANCA, if highly specific, could be of great importance in the initial diagnostic evaluation of patients with a differential diagnosis that includes both connective tissue disease and vasculitis.[4] Two types of ANCA, antiproteinase 3 antibodies (PR3-ANCA) and antimyeloperoxidase antibodies (MPO-ANCA), are useful in the diagnosis of such types of vasculitis as mPA, WG, and CSS. Most patients with WG have the cytoplasmic form, whereas the perinuclear form predominates in mPA and idiopathic rapidly progressing glomerulonephritis (RPGN). The renal lesion is characteristically a focal segmental necrotizing glomerulonephritis, often with crescents, but with few or no deposits on immunostaining.[5,6,7]

The authors report herein a 21-year-old girl with MPO-ANCA-positive RPGN requiring hemodialysis, and association of various painful vasculitic skin lesions.


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