Missounga Landry, Ba Josaphat Iba, Nseng Nseng Ondo Ingrid Rosalie, Nziengui Madjinou Maria Ines Carine, Malekou Doris, Mouendou Mouloungui Emeline Gracia, Nzengue Emmanuel Ecke, Boguikouma Jean Bruno, Kombila Moussavou
Département de Médecine Interne et Spécialités Médicales, Université des Sciences de la Santé, BP 4009 Libreville Gabon.
Service de Cardiologie, Centre Hospitalier Universitaire de Libreville, BP 2228 Libreville, Gabon.
Pan Afr Med J. 2017 Jun 30;27:162. doi: 10.11604/pamj.2017.27.162.12572. eCollection 2017.
The literature reports that mixed connective tissue disease seems more frequent in the black population and among Asians. This study aims to determine the prevalence of mixed connective tissue disease (MCTD) among connective tissue disorders and all rheumatologic pathologies in a hospital population in Gabon as well as to describe the clinical features of this disease. We conducted a retrospective study by reviewing the medical records of patients treated for mixed connective tissue disease (Kasukawa criteria) and other entities of connective tissue disorders (ACR criteria) in the Division of Rheumatology at the University Hospital in Libreville between January 2010 and December 2015. For each case of MCTD the parameters studied were articular and extra-articular manifestations, anti-U1RNP antibodies levels, patient's evolution. Over a period of 6 years, data were collected by medical records of 7 patients out of 6050 patients and 67 cases of connective tissue disorders, reflecting a prevalence of 0.11% and 10.44% respectively. the 7 patients were women (100%), with an average age of 39.5 years. Articular manifestations included: polyarthritis, myalgias, chubby fingers and Raynaud's phenomenon in 87.5%, 87.5%, 28.6% and 14% respectively. The 7 patients had high anti-U1RNP antibodies levels, ranging between 5 and 35N (N≤ 7 IU). A case of death due to pulmonary arterial hypertension (PAH) was certified. This is the largest case series of MCTD reported in Black Africa. The disease seems to be rare among the black Africans; the reason could be genetic. The demographic and clinical aspects appear similar to those in Caucasians, Asians and Blacks except for a low frequency of Raynaud?s phenomenon among Blacks.
文献报道混合性结缔组织病在黑人人群和亚洲人中似乎更为常见。本研究旨在确定加蓬一家医院人群中混合性结缔组织病（MCTD）在结缔组织疾病和所有风湿性疾病中的患病率，并描述该疾病的临床特征。我们通过回顾2010年1月至2015年12月期间在利伯维尔大学医院风湿病科接受混合性结缔组织病（Kasukawa标准）和其他结缔组织疾病实体（美国风湿病学会标准）治疗的患者的病历进行了一项回顾性研究。对于每例MCTD，研究的参数包括关节和关节外表现、抗U1RNP抗体水平、患者的病情演变。在6年的时间里，从6050例患者的病历中收集到7例患者的数据，结缔组织疾病有67例，患病率分别为0.11%和10.44%。这7例患者均为女性（100%），平均年龄39.5岁。关节表现包括：多关节炎、肌痛、腊肠样手指和雷诺现象，分别占87.5%、87.5%、28.6%和14%。这7例患者抗U1RNP抗体水平较高，范围在5至35N（N≤7 IU）之间。有1例因肺动脉高压（PAH）死亡得到证实。这是在黑非洲报道的最大系列MCTD病例。该疾病在非洲黑人中似乎很罕见；原因可能是遗传因素。除黑人中雷诺现象发生率较低外，其人口统计学和临床方面与白种人、亚洲人和黑人相似。
