Pneumocystis Pneumonia and the Rheumatologist: Which Patients Are At Risk and How Can PCP Be Prevented?
- 906 Downloads
Purpose of Review
Immunosuppressive therapy for connective tissue diseases (CTDs) is steadily becoming more intense. The resultant impairment in cell-mediated immunity has been accompanied by an increasing risk for opportunistic infection (OI). Pneumocystis pneumonia (PCP) has been recognized as an OI in patients with CTDs, but specific risk factors and precise indications for PCP prophylaxis remain poorly defined. This review was undertaken to update information on the risk of PCP in patients with CTDs and to examine current guidelines for PCP prophylaxis in this population.
Data on the occurrence of PCP and indications for prophylaxis in patients with CTDs is sparse. Large systematic reviews did not incorporate patients with CTD secondary to the lack of randomized control trials. Upon reviewing guidelines published since 2015, prophylaxis for PCP is recommended only for patients with ANCA-positive vasculitis, specifically granulomatosis with polyangiitis (GPA), who are undergoing intense induction therapy.
Evidence-based recommendations for the prophylaxis of PCP in patients with CTDs cannot be provided. There is expert consensus that PCP prophylaxis is warranted in patients with GPA undergoing induction therapy. Prophylaxis should perhaps also be considered for other CTD patients who are receiving similar intense immunosuppressive therapy especially if they are lymphopenic or have a low CD4 count.
KeywordsPneumocystis pneumonia Granulomatosis with polyangiitis Systemic lupus erythematosus Inflammatory myopathy Trimethoprim-sulfamethoxazole Prophylaxis
Compliance with Ethical Standards
Conflict of Interest
Rachel M. Wolfe and James E. Peacock, Jr. declare that they have no conflict of interest.
Human and Animal Rights and Informed Consent
This article does not contain any studies with human or animal subjects performed by any of the authors.
Papers of particular interest, published recently, have been highlighted as: • Of importance •• Of major importance
- 3.Centers for Disease Control and Prevention: Pneumoycstis pneumonia. https://www.cdc.gov/fungal/diseases/pneumocystis-pneumonia (2014). Accessed 17 Feb 2017.
- 5.•• Green H, Paul M, Vidal L, et al. Prophylaxis of pneumocystis pneumonia in immunocompromised non-HIV-infected patients: systematic review and meta-analysis of randomized controlled trials. Mayo Clin Proc. 2007;82(9):1052–9. This study reviews data for PCP prophylaxis with special attention to CTD. CrossRefPubMedGoogle Scholar
- 12.•• Falagas ME, Manta KG, Betsi GI, et al. Infection-related morbidity and mortality in patients with connective tissue diseases: a systemic review. Clin Rheumatol. 2007;26:663–70. This study details infectious complications, including PCP, for the CTDs with large numbers of patients included. CrossRefPubMedGoogle Scholar
- 15.• Tadros S, Teichtahl AJ, Ciciriello S, et al. Pneumocystis jirovecii in systemic autoimmune rheumatic disease: a case-control study. Semin Arthritis Rheum. 2016; doi: 10.1016/j.semarthrit.2016.09.009. Case series examining risk factors for PCP while controlling for the specific rheumatic disease. PubMedGoogle Scholar
- 28.Mecoli CA, Saylor D, Gelber AC, et al. Pneumocystis jiroveci pneumonia in rheumatic disease: a 20-year single-centre experience [abstract]. Clin Exp Rheumatol 2017; Accessed 2/17/17.Google Scholar
- 39.UpToDate: Drug information. https://www.uptodate.com (2017). Accessed 7 Mar 2017.
- 57.• Grubbs JA, Baddley JW. Pneumocystis jirovecii pneumonia in patients receiving tumor-necrosis-factor-inhibitor therapy: implications for chemoprophylaxis. Curr Rheumatol Rep. 2014;16:445. Large meta-analysis of PCP in rheumatoid arthritis with a specific focus on biologic therapy. CrossRefPubMedGoogle Scholar