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

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,...

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Main Authors: Alwin Tilanus, Patricia Van der Niepen, Caroline Geers, Karl Martin Wissing
Format: Article
Language:English
Published: Wiley 2015-01-01
Series:Case Reports in Nephrology
Online Access:http://dx.doi.org/10.1155/2015/378170
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author Alwin Tilanus
Patricia Van der Niepen
Caroline Geers
Karl Martin Wissing
author_facet Alwin Tilanus
Patricia Van der Niepen
Caroline Geers
Karl Martin Wissing
author_sort Alwin Tilanus
collection DOAJ
description 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.
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institution Kabale University
issn 2090-6641
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series Case Reports in Nephrology
spelling doaj-art-8f7897cc238e4476a8757ba389025cd42025-02-03T07:24:39ZengWileyCase Reports in Nephrology2090-66412090-665X2015-01-01201510.1155/2015/378170378170Pulmonary Limited MPO-ANCA Microscopic Polyangiitis and Idiopathic Lung Fibrosis in a Patient with a Diagnosis of IgA NephropathyAlwin Tilanus0Patricia Van der Niepen1Caroline Geers2Karl Martin Wissing3Departamento de Medicina Interna/Infectologia, Hospital General de Medellin Luz Castro de Gutiérrez, Carrera 48 # 32- 102, Medellin, ColombiaDepartement Interne Geneeskunde/Nefrologie, Universitair Ziekenhuis Brussel, Laarbeeklaan 101, 1090 Brussels, BelgiumDepartement Anatomo-Pathologie, Universitair Ziekenhuis Brussel, Laarbeeklaan 101, 1090 Brussels, BelgiumDepartement Interne Geneeskunde/Nefrologie, Universitair Ziekenhuis Brussel, Laarbeeklaan 101, 1090 Brussels, BelgiumWe 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.http://dx.doi.org/10.1155/2015/378170
spellingShingle Alwin Tilanus
Patricia Van der Niepen
Caroline Geers
Karl Martin Wissing
Pulmonary Limited MPO-ANCA Microscopic Polyangiitis and Idiopathic Lung Fibrosis in a Patient with a Diagnosis of IgA Nephropathy
Case Reports in Nephrology
title Pulmonary Limited MPO-ANCA Microscopic Polyangiitis and Idiopathic Lung Fibrosis in a Patient with a Diagnosis of IgA Nephropathy
title_full Pulmonary Limited MPO-ANCA Microscopic Polyangiitis and Idiopathic Lung Fibrosis in a Patient with a Diagnosis of IgA Nephropathy
title_fullStr Pulmonary Limited MPO-ANCA Microscopic Polyangiitis and Idiopathic Lung Fibrosis in a Patient with a Diagnosis of IgA Nephropathy
title_full_unstemmed Pulmonary Limited MPO-ANCA Microscopic Polyangiitis and Idiopathic Lung Fibrosis in a Patient with a Diagnosis of IgA Nephropathy
title_short Pulmonary Limited MPO-ANCA Microscopic Polyangiitis and Idiopathic Lung Fibrosis in a Patient with a Diagnosis of IgA Nephropathy
title_sort pulmonary limited mpo anca microscopic polyangiitis and idiopathic lung fibrosis in a patient with a diagnosis of iga nephropathy
url http://dx.doi.org/10.1155/2015/378170
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AT carolinegeers pulmonarylimitedmpoancamicroscopicpolyangiitisandidiopathiclungfibrosisinapatientwithadiagnosisofiganephropathy
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