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

Tilanus A, Van der Niepen P, Geers C, Wissing KM - Case Rep Nephrol (2015)

Bottom Line: 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.

View Article: PubMed Central - PubMed

Affiliation: Departamento de Medicina Interna/Infectologia, Hospital General de Medellin Luz Castro de Gutiérrez, Carrera 48 # 32- 102, Medellin, Colombia.

ABSTRACT
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.

No MeSH data available.


Related in: MedlinePlus

The methenamine silver stain shows fibrinoid necrosis with early crescent formation in the urinary space (Jones methenamine silver, ×400).
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Related In: Results  -  Collection


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fig2: The methenamine silver stain shows fibrinoid necrosis with early crescent formation in the urinary space (Jones methenamine silver, ×400).

Mentions: Renal biopsy (2007) showed necrotizing crescentic glomerulonephritis (Figures 1 and 2) with significant IgA deposits (not shown in figure).


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

Tilanus A, Van der Niepen P, Geers C, Wissing KM - Case Rep Nephrol (2015)

The methenamine silver stain shows fibrinoid necrosis with early crescent formation in the urinary space (Jones methenamine silver, ×400).
© Copyright Policy - open-access
Related In: Results  -  Collection

Show All Figures
getmorefigures.php?uid=PMC4525752&req=5

fig2: The methenamine silver stain shows fibrinoid necrosis with early crescent formation in the urinary space (Jones methenamine silver, ×400).
Mentions: Renal biopsy (2007) showed necrotizing crescentic glomerulonephritis (Figures 1 and 2) with significant IgA deposits (not shown in figure).

Bottom Line: 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.

View Article: PubMed Central - PubMed

Affiliation: Departamento de Medicina Interna/Infectologia, Hospital General de Medellin Luz Castro de Gutiérrez, Carrera 48 # 32- 102, Medellin, Colombia.

ABSTRACT
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.

No MeSH data available.


Related in: MedlinePlus