International Journal of Critical Illness and Injury Science

: 2014  |  Volume : 4  |  Issue : 4  |  Page : 319--320

A rare case of isolated pauci-immune pulmonary capillaritis

Raghav Gupta1, Wisam Naji1, Aditi Jindal2, Bhavin Sureshbhai Patel3, Garima Mittal4, Andrew Labelle1,  
1 Department of Internal Medicine, Pulmonary and Critical Care Medicine, St. Luke's Hospital, Chesterfield, Missouri, USA
2 Department of Pediatric Dentistry, Tufts University School of Dental Medicine, Boston, Massachusetts, USA
3 Department of Internal Medicine, Odessa State Medical University, India
4 Department of Medicine, Government Medical College, Patiala, Punjab, India

Correspondence Address:
Dr. Raghav Gupta
St. Luke«SQ»s Hospital, Chesterfield, Missouri, 63017

How to cite this article:
Gupta R, Naji W, Jindal A, Patel BS, Mittal G, Labelle A. A rare case of isolated pauci-immune pulmonary capillaritis.Int J Crit Illn Inj Sci 2014;4:319-320

How to cite this URL:
Gupta R, Naji W, Jindal A, Patel BS, Mittal G, Labelle A. A rare case of isolated pauci-immune pulmonary capillaritis. Int J Crit Illn Inj Sci [serial online] 2014 [cited 2022 Sep 24 ];4:319-320
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Full Text


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 [Figure 1] and [Figure 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).

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.{Figure 1}{Figure 2}


1sSeo JB, Im JG, Chung JW, Song JW, Goo JM, Park JH, et al. Pulmonary vasculitis: The spectrum of radiological findings. Br J Radiol 2000;73:1224-31.
2Jennings CA, King TE Jr, Tuder R, Cherniack RM, Schwarz MI. Diffuse alveolar hemorrhage with underlying isolated, pauciimmune pulmonary capillaritis. Am J Respir Crit Care Med 1997;155:1101-9.