SESSION TITLE: Interstitial Lung Disease Global Case Reports
SESSION TYPE: Global Case Report
PRESENTED ON: Tuesday, October 29, 2013 at 01:30 PM - 02:30 PM
INTRODUCTION: Pulmonary alveolitis presenting as diffuse alveolar haemorrhage is a rare manifestation in patients with IgA nephropathy, and the association between these conditions is controversial in the medical literature, with only a few case reports. We present a case of pulmonary alveolitis with biopsy proven IgA deposits in the alveoli.
CASE PRESENTATION: A 24-year-old caucasian male presented with a five days history of fever, chills, malaise and mild exertional dyspnoea. On second day he developed dyspnoea, tachypnea, hypoxia and pulmonary rales. Six years earlier the patient had been hospitalized for glomerulonephritis ans IgA nephropathy was diagnosed based on glomerular IgA deposits. Radiological evaluation revealed bilateral consolidations on both conventional and CT images. The patient was admitted to the intensive care unit for supportive care and empirical antimicrobial therapy. Sputum examinations were negative for bacteria, acid-fast bacilli but showed abundant haemosiderin-laden macrophages. With worsening infiltrates and clinical condition, and with negative cultures on every specimen, the decision to initiate intravenous corticosteroids to presumed alveolitis was taken, and clinical improvement noticed. On hospital day 4, a lung biopsy and a skin biopsy were performed. Lung tissue showed alveoli fullfilled with macrophages and giant multinucleated cell with inflammatory edema, hyperplasia of alveoli epithelium and signs of active and previous haemorrhage. Immunofluorescense microscopy showed marked diffuse alveolar deposits of IgA.
DISCUSSION: Pulmonary haemorrhage has been very uncommonly associated with IgA nephropathy, although it is frequently seen in other glomerulonephritides. Renal and pulmonary compromise usually occur concurrently, reflecting the same underlying immune process. Although isolated sporadic pulmonary haemorrhage due to alveolitis is a possible differential diagnosis that cannot be excluded, there are a number of mechanisms that can attribute the pulmonary disease to IgA nephropathy, and the findings on biopsy and favorable response to steroids in reality suggest that this is the case.
CONCLUSIONS: We describe a case of a life-threatening pulmonary haemorrhage due to alveolar injury with intense deposits of IgA seen on immunofluorescence, without signs of active glomeruli involvement. To our knowledge, this is the first description with well defined IgA deposits with predominantly alveolar damage.
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Reference #2: Anantham D, Chan KP, Chuah KL, Vathsala A, Eng P. Pulmonary capillaritis in IgA nephropathy. South Med J 2007;100(6):605-7.
Reference #3: Barratt J, Feehally J. IgA nephropathy. J Am Soc Neph 2005;16:2088-2097.
DISCLOSURE: The following authors have nothing to disclose: Fabricio Fortuna, Bernardo Marson, Aluisio Maciel
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