Iba Ba Josaphat, Mfoumou Annick, Nseng-Nseng Ondo Ingrid, Kanganga Ekomy Arthur, Ledaga Lentombo Léonie Esther, Igala Marielle, Kombila Ulrich Davy, Boguikouma Jean Bruno
Service de médecine interne, CHU de Libreville, BP 2228, Libreville, Gabon.
Service de rhumatologie, CHU de Libreville, BP 2228, Libreville, Gabon.
Med Trop Sante Int. 2025 Jan 6;5(1). doi: 10.48327/mtsi.v5i1.2025.629. eCollection 2025 Mar 31.
Introduction. Still's Disease (SD) is a rare systemic inflammatory disorder that is most common in children. In the adult form, it can be primary or the resurgence of an infantile form. We report 10 observations of Still's disease in the Gabonese population, with the aim of confirming its existence in this country an studying its specific features.
This was a retrospective, descriptive and analytical study carried out in the Internal Medicine Department of the CHU of Libreville from 1.12.2003 to 31.12.2021, using the records of patients hospitalized in this department and/or followed up on an outpatient basis. The selected patients fulfilled the Yamaguchi and Fautrel criteria. Epidemiologic, socioeconomic, clinical, biological, morphologic, immunologic, therapeutic, evolutionary and seasonal data were detailed.
Ten patients (4 men and 6 women), mostly students with a mean age of 22 years, were included. The time to diagnosis of SD was 31.3 months, with fever in 100% of cases, joint and skin involvement in 80%, and ENT involvement in 70%. An inflammatory syndrome, predominantly neutrophilic hyperleukocytosis, hepatic signs, and hyperferritinemia coexisted with an immunologic work-up that was always negative. Corticosteroid therapy (n=10) combined with per os methotrexate from the beginning (n=1) or in cases of corticoresistance (n=4) was the rule. One patient died and one was lost to follow-up.
The clinical and biological presentation of SD is comparable to that of the Caucasian population. However, our study confirms in the low incidence of liver, lymph node and heart damage, which needs to be confirmed by other studies in a larger cohort. The high cost of this disease, which is a diagnosis of exclusion, may partly explain the difficulties in diagnosing SD.
斯蒂尔病（SD）是一种罕见的全身性炎症性疾病，在儿童中最为常见。在成人形式中，它可以是原发性的，也可以是婴儿型的复发。我们报告了加蓬人群中10例斯蒂尔病的观察结果，目的是确认其在该国的存在并研究其具体特征。
这是一项回顾性、描述性和分析性研究，于2003年12月1日至2021年12月31日在利伯维尔CHU内科进行，使用该科室住院患者和/或门诊随访患者的记录。所选患者符合山口和富特雷尔标准。详细记录了流行病学、社会经济、临床、生物学、形态学、免疫学、治疗、演变和季节数据。
纳入了10名患者（4名男性和6名女性），大多数是学生，平均年龄22岁。SD的诊断时间为31.3个月，100%的病例有发热，80%有关节和皮肤受累，70%有耳鼻喉受累。炎症综合征，主要是中性粒细胞增多性白细胞增多、肝脏体征和高铁蛋白血症，与免疫检查结果始终为阴性并存。皮质类固醇治疗（n = 10）从一开始就联合口服甲氨蝶呤（n = 1）或在皮质类固醇抵抗的情况下（n = 4）联合使用是常规治疗方法。1例患者死亡，1例失访。
SD的临床和生物学表现与白种人群相似。然而，我们的研究证实了肝脏、淋巴结和心脏损伤的低发生率，这需要在更大的队列中通过其他研究来证实。这种疾病作为一种排除性诊断，成本高昂，这可能部分解释了SD诊断的困难。
