Vieujean Sophie, Moens Annick, Hassid Deborah, Rothfuss Katja, Savarino Edoardo Vincenzo, Vavricka Stephan R, Reenaers Catherine, Jacobsen Bent Ascanius, Allez Matthieu, Ferrante Marc, Rahier Jean-Francois
Department of Gastroenterology, University Hospital of Liège, Liège, Belgium.
Department of Gastroenterology and Hepatology, University Hospitals Leuven, KU Leuven, Leuven, Belgium.
J Crohns Colitis. 2023 Apr 19;17(4):472-479. doi: 10.1093/ecco-jcc/jjac153.
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 Organisation. Clinical data were provided through a case report form.
In all, 18 PJP episodes were reported in 17 IBD patients [10 ulcerative colitis and seven Crohn's disease]. The median age at PJP diagnosis was 55 years (interquartile range [IQR], 40-68 years]. Two PJP [11.1%] occurred in patients on triple immunosuppression, 10 patients [55.6%] had double immunosuppressive treatment, four patients [22.2%] had monotherapy and two 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/sulphamethoxazole or atovaquone and an intensive care unit [ICU] stay was required in seven 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.
耶氏肺孢子菌肺炎（PJP）是一种非常罕见的、可能危及生命的肺部真菌感染，发生于包括炎症性肠病（IBD）患者在内的免疫功能低下个体中。我们的目的是描述IBD合并PJP患者的免疫抑制治疗暴露情况及预后。
通过欧洲克罗恩病和结肠炎组织罕见病例协作网络回顾性收集PJP病例。临床数据通过病例报告表提供。
17例IBD患者（10例溃疡性结肠炎和7例克罗恩病）共报告了18次PJP发作。PJP诊断时的中位年龄为55岁（四分位间距[IQR]，40 - 68岁）。2例PJP（11.1%）发生在接受三联免疫抑制治疗的患者中，10例患者（55.6%）接受双重免疫抑制治疗，4例患者（22.2%）接受单一疗法，2例PJP发生在未进行免疫抑制治疗的情况下（1例为人类免疫缺陷病毒患者，1例有自体干细胞移植史）。免疫抑制治疗包括类固醇（n = 12）、硫唑嘌呤（n = 10）、英夫利昔单抗（n = 4）、环孢素（n = 2）、甲氨蝶呤（n = 1）和他克莫司（n = 1）。所有诊断为PJP的患者均未接受过预防治疗。所有患者均接受甲氧苄啶/磺胺甲恶唑或阿托伐醌治疗，7例患者需要入住重症监护病房（ICU）。2例患者（分别为71岁和32岁）死亡，1例患者在初始治疗16个月后复发。其他患者病情好转。
本病例系列报告了可能致命的PJP，强调了对接受免疫抑制治疗的IBD患者进行调整后的预防治疗的必要性。
