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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 GutiƩrrez, 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

CT scan (prior to treatment) showing diffuse pulmonary fibrosis (honeycombing) and bilateral pleural fluid collection.
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fig3: CT scan (prior to treatment) showing diffuse pulmonary fibrosis (honeycombing) and bilateral pleural fluid collection.

Mentions: CT scan of the thorax was classified as usual interstitial pneumonia (UIP) with reticular infiltrates and honeycombing predominantly in the subpleural areas of the lower lobes, without possibility to differentiate between idiopathic pulmonary fibrosis and fibrosis secondary to vasculitis on radiological grounds (Figure 3). Giant vessel vasculitis was excluded by PET-CT scan (though performed under corticosteroid treatment). A biopsy of the nasal mucosa showed nonspecific inflammation without signs of vasculitis or granulomatous lesions. There were no signs of active renal disease with a bland urinary sediment and absence of proteinuria as well as a stable but decreased renal function (CKD stage 3B).


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)

CT scan (prior to treatment) showing diffuse pulmonary fibrosis (honeycombing) and bilateral pleural fluid collection.
© Copyright Policy - open-access
Related In: Results  -  Collection

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

fig3: CT scan (prior to treatment) showing diffuse pulmonary fibrosis (honeycombing) and bilateral pleural fluid collection.
Mentions: CT scan of the thorax was classified as usual interstitial pneumonia (UIP) with reticular infiltrates and honeycombing predominantly in the subpleural areas of the lower lobes, without possibility to differentiate between idiopathic pulmonary fibrosis and fibrosis secondary to vasculitis on radiological grounds (Figure 3). Giant vessel vasculitis was excluded by PET-CT scan (though performed under corticosteroid treatment). A biopsy of the nasal mucosa showed nonspecific inflammation without signs of vasculitis or granulomatous lesions. There were no signs of active renal disease with a bland urinary sediment and absence of proteinuria as well as a stable but decreased renal function (CKD stage 3B).

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 GutiƩrrez, 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