A rare case of isolated pauci-immune pulmonary capillaritis.

Sir,

A 66-year-old male presented with complaints of dry cough and dyspnea with bilateral crackles on examination. Chest imaging showed bilateral opacification of the lungs [Figures 1 and 2]. Due to progressive respiratory failure, mechanical ventilation and antibiotics were started. Bronchoalveolar lavage was consistent with alveolar hemorrhage. Perinuclear antineutrophil cytoplasmic antibodies (P-ANCAs) returned as positive, but other autoimmune markers were negative. Methylprednisolone and later on cyclophosphamide was started, which initially improved oxygen requirements, but patient developed multiorgan failure and subsequently expired. Autopsy confirmed diffuse alveolar hemorrhage associated with isolated pauci-immune pulmonary capillaritis (IPIPC).

Figure 1

Chest radiography showing diffuse white opaque bilateral pulmonary infiltrates

Figure 2

Non-contrast computed tomography of the chest showing extensive patchy ground-glass lung infiltrates with bilateral pleural effusions

In the absence of identifiable systemic disease, pulmonary vasculitis is recognized as IPIPC.[1] Corticosteroids and cyclophosphamide remains the conventional therapy. Jennings et al., reported eight cases of IPIPC without any associated illness.[2] We present a rare case of IPIPC, who initially responded well to cyclophosphamide, but subsequently failed the treatment.