Background/Purpose:   Pneumocystis jirovecii pneumonia (PJP) is most frequently associated with AIDS, however, this infection also occurs in a variety of rheumatologic conditions in the context of immunosuppressive therapy. The mechanisms of immune compromise in patients without AIDS who have PCP are usually multifactorial, related to underlying cytotoxic therapy and malnutrition. PCP infection has been extensively studied among AIDS patients, however, not many studies have evaluated its occurrence in the setting of underlying rheumatologic diseases.

Methods:   The study cohort included a consecutive series of patients without AIDS and malignancy or organ transplantation who were assessed at a single institution for a first episode of PCP infection. Medical records were examined to determine underlying immunosuppressive disorders, corticosteroid dosage, associated infections, and subsequent respiratory failure and in-hospital mortality.

Results:   The study included 54 patients (mean age at presentation 62.8 years; 54% male) with mean follow up of 2.5 years. Conditions associated with a first episode of PCP were rheumatoid arthritis (30%), inflammatory bowel disease (17%), systemic lupus erythematosus, polymyalgia rheumatic (7%) and giant cell arteritis (6%). Regardless of the underlying condition, corticosteroids had been administered systematically in 45 patients (83%) within 3 months before the diagnosis of PCP. The median daily corticosteroid dose was 27.5 mg. Predominant symptoms on presentation were cough (78%) and shortness of breath (76%). The majority of the patients (87%) on immunosuppressive therapy did not receive any prophylaxis for PCP infection. There were 25 patients (46%) that improved upon institution of therapy while 14 patients (26%) died as a result of infection.

Conclusion:   In this large consecutive series, systemic administration of corticosteroid therapy, even in the moderate doses may increase susceptibility to PCP infections. Consideration should be given to instituting PCP prophylaxis (when not contraindicated) in patients for whom prolonged systemic corticosteroid therapy is prescribed.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/pneumocystis-jirovecii-pneumonia-in-systemic-autoimmune-inflammatory-diseases/