Case Reports
Microscopic Polyangiitis Presenting as Idiopathic Pulmonary Fibrosis: Is Anti-Neutrophilic Cytoplasmic Antibody Testing Indicated?

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ABSTRACT

We report a 55-year old woman with microscopic polyangiitis who presented with idiopathic pulmonary fibrosis and 1 year later developed hematuria and proteinuria. She had a high serum level of perinuclear anti-neutrophilic cytoplasmic antibodies. Renal angiogram was normal. The diagnosis of microscopic polyangiitis was confirmed by renal biopsy, which showed pauci-immune crescentic glomerulonephritis.The patient received immunosuppressive therapy and improved markedly. Consideration of small vessel vasculitis is important in the differential diagnosis of idiopathic pulmonary fibrosis.

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Case Report

A 55-year-old diabetic woman presented with fever, cough, and yellowish sputum. She had crackles bilaterally at the lung bases. Complete blood count revealed leukocytosis. Routine serum chemistries and urinalysis were normal. A chest radiograph showed increased interstitial markings at the bases bilaterallythat was considered to be atypical pneumonia. The patient was treated with intravenous antibiotics. Bronchoscopy with bronchoalveolar lavage was negative for Pneumocystis carinii, acid-fast

Discussion

ANCA-associated, small-vessel vasculitis is the most common primary systemic vasculitis in adults and commonly affects men and women between 50 and 60 years of age.1 Microscopic polyangiitis is a necrotizing vasculitis affecting small vessels (venules, capillaries, and arterioles) with few or no immune deposits. Small and medium arteries may also be affected.5 Wegener granulomatosis and Churg-Strauss syndrome also preferentially involve small vessels.5 Wegener granulomatosis is characterized by

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    Additional references quoted in these publications were checked. Data of > 200 patients included in case series [13–27] and case reports [28–42] constitute the basis of this review. Interstitial lung disease associated with ANCA is usually observed in patients older than 65 years old.

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    However there may be a selection bias in their study because ANCA was measured based only on the referring doctor's decision resulting in relatively high percentage of female patients.13 Previously reported series indicated that IPF precedes the development of vasculitis in the majority cases.9,14–17 The precise mechanism of MPO-ANCA production in IPF, however, remains unclear.

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    What is actually unusual in our patient is the clinical course, he developed first pulmonary fibrosis, seven-year later renal failure, soon afterwards haemoptysis followed by fulminant course of the vasculitis with alveolar haemorrhage and constitutional symptoms. In the literature, several cases of pulmonary fibrosis have been described that presented concomitantly or predated the other features of vasculitis for months or few years.5–10 The interval between fibrosis and the appearance of the other vasculitic symptoms almost never reached 7 years.

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    2008, Respiratory Medicine
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    The coexistence of pulmonary fibrosis and antineutrophil cytoplasmic autoantibodies (ANCA)-positive vasculitis, essentially microscopic polyangiitis (MPA) and rapidly progressive glomerulonephritis has been consistently reported in case reports or small series.1–8

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