Abstract

Optimal timing for Pneumocystis jirovecii pneumonia (PCP) prophylaxis among patients with vasculitis is not clear. We set out to characterize the clinical presentation and duration of prednisone use before the development of PCP among these patients. All patients with PCP at The Ottawa Hospital (TOH) between 2006 and 2017 were identified. Using TOH data repositories, the following data were extracted: prednisone dosage, treatment duration, other immunosuppressive medications, PCP prophylaxis, PCP treatment, and death. Data were reported as median and range or as mean and standard deviation. We identified seven patients (5 men, 2 women) with biopsy-proven vasculitis who developed PCP: six with anti-neutrophil cytoplasmic antibody-associated vasculitis and one with giant cell arteritis. None of the patients were on PCP prophylaxis. The most common symptoms on presentation were cough and dyspnea. At diagnosis, the median lymphocyte count was 0.30 × 109/L (range 0.03-2.10), creatinine was 186 µmol/L (range 78-359), and lactate dehydrogenase was 471 U/L (range 301-1032). All patients were on prednisone at time of PCP diagnosis, with six on doses of ≥20 mg/day for at least 12 weeks. All but one patient were on additional immunosuppressants, with cyclophosphamide being the most common agent for five of the seven patients. Four (57%) required intensive care unit admission, and two (29%) died secondary to complications of PCP. PCP is a severe and often fatal opportunistic infection among immunocompromised patients with vasculitis. Frequent evaluation of the need for prophylaxis is required for patients who remain on high-dose steroids and concomitant immunosuppressants.

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