Pneumocystis Pneumonia and the Rheumatologist: Which Patients Are At Risk and How Can PCP Be Prevented?

  • Rachel M. Wolfe
  • James E. PeacockJr
Infection and Arthritis (K Winthrop, Section Editor)
Part of the following topical collections:
  1. Topical Collection on Infections and Arthritis


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.

Recent Findings

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.


Pneumocystis 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

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Copyright information

© Springer Science+Business Media New York 2017

Authors and Affiliations

  1. 1.Wake Forest Health Sciences, Department of Internal Medicine, Section on Rheumatology and ImmunologyWinston SalemUSA
  2. 2.Wake Forest Health Sciences, Department of Internal Medicine, Section on Infectious DiseaseWinston SalemUSA

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