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Case Reports in Nephrology
Volume 2015, Article ID 378170, 5 pages
Case Report

Pulmonary Limited MPO-ANCA Microscopic Polyangiitis and Idiopathic Lung Fibrosis in a Patient with a Diagnosis of IgA Nephropathy

1Departamento de Medicina Interna/Infectologia, Hospital General de Medellin Luz Castro de Gutiérrez, Carrera 48 # 32- 102, Medellin, Colombia
2Departement Interne Geneeskunde/Nefrologie, Universitair Ziekenhuis Brussel, Laarbeeklaan 101, 1090 Brussels, Belgium
3Departement Anatomo-Pathologie, Universitair Ziekenhuis Brussel, Laarbeeklaan 101, 1090 Brussels, Belgium

Received 31 March 2015; Revised 22 June 2015; Accepted 28 June 2015

Academic Editor: Mahzuz Karim

Copyright © 2015 Alwin Tilanus et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.


We present a case of a male patient with chronic renal insufficiency, due to crescentic glomerulonephritis with IgA deposits, who successively developed (idiopathic) thrombocytopenic purpura (ITP) and MPO-ANCA microscopic polyangiitis (MPA) with pulmonary fibrosis. The patient presented with cough, weight loss, and dyspnea on exertion. CT imaging and pulmonary function tests were compatible with interstitial pneumonitis with pulmonary fibrosis. Laboratory results showed high MPO-ANCA titers; the urinary sediment was bland. The patient was treated successfully with cyclophosphamide and methyl-prednisolone. This unique case illustrates the diagnostic and therapeutic challenges of an unusual presentation of microscopic polyangiitis presenting first as isolated kidney disease with recurrence in the form of pneumonitis without renal involvement, in association with renal IgA deposits and ITP as coexisting autoimmune conditions.