Background/Purpose: Potent immunosuppressive therapy for connective tissue diseases (CTDs) imparts an increased risk for opportunistic infections including Pneumocystis pneumonia (PCP). High mortality rates have been reported in CTD patients with PCP which raises the question as to if and when prophylaxis for PCP is indicated. Unfortunately, the specific risk factors and precise indications for PCP prophylaxis in CTD patients remain poorly defined and are guided only by expert opinion and personal experience. Wide variations in PCP prophylaxis patterns among rheumatologists have been previously established. This study was aimed to evaluate patterns among infectious disease physicians.

Methods: An electronic survey on PCP prophylaxis in CTD patients was emailed to 1,264 adult infectious disease physicians who are members of the Infectious Diseases Society of America Emerging Infections Network.

Results: 631 (50%) physicians responded to the survey. Respondents were significantly more likely to work in an academic/university system (p = 0.02) and either be early (<5 years) or late (≥25 years) in their careers (p = 0.0002). Almost half (43%) reported that they did not make recommendations for PCP prophylaxis in non-HIV patients. Of those making recommendations for prophylaxis, there was little agreement on indications for prophylaxis. The greatest consensus for specific CTDs was for granulomatosis with polyangiitis (GPA) with 53% advocating for PCP prophylaxis. Only about a third recommended prophylaxis for other vasculitides, lupus, lupus nephritis, inflammatory myositis and rheumatoid arthritis on high dose corticosteroids. Interestingly, the most frequent single response was “Not Sure” (35%) but this option was also often selected along with other specific CTDs (41% of total respondents). When questioned about specific therapies, corticosteroids ≥20 mg/day was the most frequently cited indication for PCP prophylaxis (87%). Few recommended PCP prophylaxis with DMARD therapy (both biologic and non-biologic) without concurrent high dose corticosteroids (2-12% versus 69-77%). Surrogate laboratory markers were not routinely used to guide decisions about prophylaxis (21%). There was no consensus for the specific indices although CD4 alone was the most frequent response. While the majority recommended discontinuation of PCP prophylaxis with tapering of corticosteroids (65%), there was considerable variability in the specific dose at which it should be discontinued. The most frequent response was below 16-20 mg/daily. 89% of respondents felt that guidelines about PCP prophylaxis would be helpful in their practice.

Conclusion: There is little consensus about PCP prophylaxis in CTDs among infectious disease physicians although certain diseases (GPA) and immunosuppressants (higher dose corticosteroids) are common indications to recommend prophylaxis. There remains much uncertainty in clinical practice with respect to the role for PCP prophylaxis in patients with CTD, both by infectious disease physicians and rheumatologists. Guidelines for PCP prophylaxis would be an important adjunct in caring for these complex patients.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/practice-patterns-of-pneumocystis-pneumonia-prophylaxis-in-connective-tissue-diseases-a-survey-of-infectious-disease-physicians/