Abstract

Immunoglobulin A nephropathy (IgAN) is the commonest glomerulopathy worldwide. It has a wide spectrum of clinical presentation, ranging from isolated hematuria to rapidly progressive glomerulonephritis.  Diffuse alveolar haemorrhage (DAH) is a life-threatening medical emergency and commonly presents with hemoptysis, anemia, hypoxemia and diffuse alveolar infiltrates. The most common cause for DAH is pulmonary capillaritis secondary to systemic autoimmune disorders. Association of DAH with IgAN is uncommon and it often goes undiagnosed. This is because the symptoms mimic pneumonia and fluid overload, which are much more commonly encountered in patients with end stage kidney disease. The diagnosis requires early bronchoscopy and bronchoalveolar lavage. A high index of clinical suspicion, early recognition and prompt treatment is the key to reduce mortality. Here we present a 21-year-old male who presented one year back with fluid overload and advanced azotemia. The kidney biopsy showed IgA nephropathy with significant chronicity. He was started on hemodialysis; 3 months in to hemodialysis, he developed recurrent episodes of treatment unresponsive DAH. The response to steroids were poor. The subsequent response to plasmapheresis and anti-CD 20 monoclonal antibodies was suboptimal and he finally responded to a combination of mycophenolate mofetil and oral steroids.

Keywords: IgA nephropathy, diffuse alveolar haemorrhage, pulmonary renal syndrome.