Pneumocystis jirovecii pneumonia in patients with inflammatory bowel disease - A case series.

Pneumocystis jirovecii pneumonia (PJP) is a very rare, potentially life-threatening pulmonary fungal infection that occurs in immunocompromised individuals including patients with inflammatory bowel disease (IBD). Our aim was to describe immunosuppressive treatment exposure as well as the outcome in IBD patients with PJP. PJP cases were retrospectively collected through the COllaborative Network For Exceptionally Rare case reports of the European Crohn's and Colitis Organization. Clinical data were provided through a case report form. 18 PJP episodes were reported in 17 IBD patients (10 ulcerative colitis and 7 Crohn's disease). The median age on PJP diagnosis was 55 years (IQR, 40-68 years). Two PJP (11.1%) occurred in patients on triple immunosuppression, 10 patients (55.6%) had double immunosuppressive treatment, 4 patients (22.2%) had monotherapy and 2 PJP occurred in absence of immunosuppressive treatment (one in a human immunodeficiency virus patient and one in a patient with a history of autologous stem cell transplantation). Immunosuppressive therapies included steroids (n=12), thiopurines (n=10), infliximab (n=4), ciclosporin (n=2), methotrexate (n=1) and tacrolimus (n=1). None of the patients diagnosed with PJP had received prophylaxis. All patients were treated by trimethoprim/sulfamethoxazole or atovaquone and an ICU stay was required in 7 cases. Two patients (aged 71 and 32 years) died, and one patient had a recurrent episode 16 months after initial treatment. Evolution was favourable for the others. This case series reporting potentially fatal PJP highlights the need for adjusted prophylactic therapy in patients with IBD on immunosuppressive therapy.