Isolated pauci-immune pulmonary capillaritis

A young house wife presented with low grade fever, cough, haemoptysis and SOB of unknown aetiology for 40 days duration. Respiratory system examination revealed diffuse crepts and rhonchi. Other organ system examination did not reveal any abnormality. X-ray chest PA view and CT thorax showed diffuse bilateral necrotising nodular lesions of various sizes with small pleural effusion. She also had low resting oxygen saturation with falling haematocrit. Her Serum was week positive for p-ANCA and negative for MPO-ANCA. Bronchoscopy revealed continuous bloody aspirates. We could not isolate any organisms in any of the specimens from her and she was unresponsive to any of the antibiotics either. Based on the clinical, laboratory data, radiological features and positive outcome to pulse therapy of methylprednisolone and cyclophosphamide, she was diagnosed as a case of IPIPC.