Risk Factors and Prevention of Pneumocystis jirovecii Pneumonia in Patients With Autoimmune and Inflammatory Diseases

Patients with autoimmune and/or inflammatory diseases (AIIDs) are prone to serious infectious complications such as Pneumocystis jirovecii pneumonia (PJP). In non-HIV patients, the prognosis is poorer, and diagnostic tests are of lower sensitivity. Given the low incidence of PJP in AIIDs, with the exception of granulomatosis with polyangiitis, and the non-negligible side effects of chemoprophylaxis, routine prescription of primary prophylaxis is still debated. Absolute peripheral lymphopenia, high doses of corticosteroids, combination with other immunosuppressive agents, and concomitant lung disease are strong predictors for the development of PJP and thus should warrant primary prophylaxis. Trimethoprim-sulfamethoxazole is considered first-line therapy and is the most extensively used drug for PJP prophylaxis. Nevertheless, it may expose patients to side effects. Effective alternative drugs such as atovaquone or aerosolized pentamidine could be used when trimethoprim-sulfamethoxazole is not tolerated or contraindicated. No standard guidelines are available to guide PJP prophylaxis in patients with AIIDs. This review covers the epidemiology, risk factors, and prevention of pneumocystis in the context of AIIDs. Patients with autoimmune and/or inflammatory diseases (AIIDs) are prone to serious infectious complications such as Pneumocystis jirovecii pneumonia (PJP). In non-HIV patients, the prognosis is poorer, and diagnostic tests are of lower sensitivity. Given the low incidence of PJP in AIIDs, with the exception of granulomatosis with polyangiitis, and the non-negligible side effects of chemoprophylaxis, routine prescription of primary prophylaxis is still debated. Absolute peripheral lymphopenia, high doses of corticosteroids, combination with other immunosuppressive agents, and concomitant lung disease are strong predictors for the development of PJP and thus should warrant primary prophylaxis. Trimethoprim-sulfamethoxazole is considered first-line therapy and is the most extensively used drug for PJP prophylaxis. Nevertheless, it may expose patients to side effects. Effective alternative drugs such as atovaquone or aerosolized pentamidine could be used when trimethoprim-sulfamethoxazole is not tolerated or contraindicated. No standard guidelines are available to guide PJP prophylaxis in patients with AIIDs. This review covers the epidemiology, risk factors, and prevention of pneumocystis in the context of AIIDs. Disease-dependent Risk of Pneumocystis Pneumonia: The Case of Autoimmune Blistering DiseaseCHESTVol. 158Issue 6PreviewWe read with great interest the article by Ghembaza et al1 in this issue of CHEST. The study discusses an important consideration for Pneumocystis jirovecii pneumonia (PJP) chemoprophylaxis in patients with autoimmune diseases. We particularly applaud the discussion regarding the importance of the underlying disease rather than the status of being on immunosuppression as is propagated in older literature. We did, however, note the omission of autoimmune blistering diseases (AIBD). In these diseases, autoantibodies target epidermal and dermal structures resulting in blistering and other dermatologic manifestations. Full-Text PDF ResponseCHESTVol. 158Issue 6PreviewWe thank Dr Patel and colleagues for their interest in our review on risk factors and prevention of Pneumocystis jirovecii pneumonia (PJP) in patients with autoimmune and inflammatory diseases.1 In their letter, they highlighted the importance of disease-dependent risk of PJP rather than the status of being on immunosuppression. We do believe that underlying disease is really an independent risk factor. PJP in autoimmune and inflammatory diseases develops in patients with immunosuppression or immunomodulation resulting from the underlying disease or its treatment. Full-Text PDF