Refractory isolated pulmonary capillaritis rescued by rituximab

Abstract

Isolated pulmonary capillaritis (IPC) is a rare autoimmune cause of diffuse alveolar haemorrhage (DAH), diagnosed by histopathological evidence of lung capillaritis in the absence of systemic vasculitis. Only 17 cases of IPC are described in the literature to date. Given its rarity, treatments reported for these cases are largely extrapolated from anti-neutrophil cytoplasmic antibodies (ANCA) associated vasculitis (AAV). Here we describe an additional case of IPC refractory to cyclophosphamide and azathioprine, which was rescued by rituximab.

Keywords:

• Isolated pulmonary capillaritis
• diffuse alveolar haemorrhage
• rituximab
• ANCA negative

Acknowledgements

The authors would like to thank pathologist Dr. Julia Flint (Department of Pathology and Laboratory Medicine, University of British Columbia) for contribution of the pathology figure.

Conflict of interest

None.

Ethics approval

According to the policy of the University of British Columbia Research Ethics Board, ethics approval for this case report is not required.

Patient consent

The patient described in this case has given written consent to this publication.