Pneumocystis jirovecii pneumonia in IBD patients treated with immunomodulator(s)

Background: Pneumocystis jirovecii Pneumonia (PJP) is an extremely rare life-threatening pulmonary fungal infection that occurs in immunocompromised individuals including patients with inflammatory bowel disease (IBD). Prophylaxis for PJP is recommended in IBD patients treated with triple immunomodulators where one agent is a calcineurin inhibitor or an anti-TNFα1 but there is no consistency in a preventive approach in patients with double or single immunomodulators. Our aim was to describe immunosuppressive treatment profile of IBD patients infected by PJP and the outcome of the disease.

Methods: Cases of PJP were retrospectively collected through the COllaborative Network For Exceptionally Rare case reports of the European Crohn’s and Colitis Organization (ECCO CONFER). All ECCO members were invited to report cases of PJP. Data were collected through a case report form.

Results: A total of 15 PJP infections were reported in 14 IBD patients (9 ulcerative colitis and 5 Crohn’s disease including 10 men and 4 women). The median age at PJP diagnosis was 55 years (IQR 44-80). Diagnosis was performed by a positive PJ polymerase chain reaction on the bronchoalveolar lavage in 87% of the cases and by a microscopic direct exam in 7% (unreported in 1 patient). One patient was co-infected by HIV and 57% were non-smokers. Immunosuppressive therapies at the time of diagnosis included steroids (n=11), thiopurines (n=9), infliximab (n=3), cyclosporin (n=2), methotrexate (n=1) and tacrolimus (n=1). Two PJP (13%) occurred in patients on triple immunosuppression, 9 patients (60 %) had a double immunosuppressive treatment, 3 patients (20%) were on monotherapy and PJP in the HIV patient occurred in absence of immunosuppressive treatment (Table 1). None of the patients diagnosed with PJP had received prophylaxis. All patients were treated by trimethoprim/sulfamethoxazole or atovaquone and 5 required an intensive care unit stay. Two patients (14%) died and 1 patient had a recurrent episode 16 months after initial treatment. Evolution was favourable for the others.
Conclusion: This small case series reports PJP in IBD patients while on single or double immunosuppression highlighting a significant risk in this population. Identifying risk factors for PJP infection in the IBD population is essential to provide a case-by-case prophylaxis.