Background/Purpose: Pneumocystis jiroveci pneumonia (PJP) is rare, but can be fatal among immunocompromised. There is no consensus on indications for PJP prophylaxis in rheumatologic patients. The challenge involves the benefit of prophylaxis with antimicrobials versus the risk of adverse events caused by the prophylactic medication.

Methods: A retrospective chart review was conducted to identify patients with PJP and a rheumatologic disease including rheumatoid arthritis (RA), Sjogren’s syndrome, systemic lupus erythematosus (SLE), granulomatosis with polyangiitis (GPA), eosinophilic granulomatosis with polyangiitis (EGPA), microscopic polyangiitis (MPA), giant cell arteritis, polyarteritis nodosa, Takayasu’s arteritis, dermatomyositis (DM), polymyositis, and systemic sclerosis (SSc) at a single, tertiary academic medical center between 1/1/2007 and 1/1/2019. Electronic medical records were reviewed to confirm diagnoses. PJP was based on clinical symptoms, laboratory studies (bronchoalveolar lavage PCR, Fungitell, beta 1,3 glucan, lactate dehydrogenase), radiographic findings, and infectious disease assessment. Data regarding immunosuppressants, prednisone use, lymphocyte count, interstitial lung disease (ILD), and use of prophylaxis was abstracted. PJP prophylaxis recommendations commonly used in rheumatologic patients were reviewed and indications for prophylaxis were determined.

Results: Fifteen patients were confirmed with a diagnosis of a rheumatologic disease and PJP. Diseases noted were RA in 5 patients, GPA in 3 patients, MPA in 2 patients, SLE in 1 patient, DM in 1 patient, psoriatic arthritis in 1 patient, antiphospholipid syndrome and autoimmune hemolytic anemia in 1 patient, and limited cutaneous SSc and necrotizing myopathy in 1 patient. Of these patients, 1 with SLE developed PJP despite prophylaxis with atovaquone. The other 14 patients did not receive prophylaxis. Fourteen of the 15 patients were on prednisone and 13 of these patients were on at least one additional immunosuppressant. Twelve of the 15 patients had lymphopenia at the time of PJP diagnosis, and 5 had ILD.
Four of the 15 patients who developed PJP were on low dose prednisone with at least one immunosuppressant. One of the 15 patients was on 3 immunosuppressants and no steroids. Three of these 5 patients had RA without ILD, 1 had PsA, and 1 had SSc with ILD and necrotizing myopathy. These 5 patients are considered low risk and prophylaxis would not be indicated due to current recommendations.

The remaining 10 patients would have been candidates for prophylaxis due to their dose and duration of prednisone, concurrent immunosuppression, or underlying disease.

Conclusion: Based on recommendations commonly used for PJP prophylaxis in patients with rheumatologic diseases, 10 of the 15 patients would have been candidates for prophylaxis and PJP may have been prevented, but 5 patients were considered low risk with no prophylactic indication. Although rheumatologists need to improve on utilizing PJP prophylaxis for high risk patients, this study indicates that further research is needed to determine individualized risk for initiating prophylaxis in rheumatologic patients who are not considered high risk for developing PJP.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/pneumocystis-jiroveci-pneumonia-in-immunocompromised-patients-with-rheumatologic-disease-in-a-single-tertiary-medical-center/