[Antisynthetase syndrome: seven observations from the internal medicine department of the Libreville University Hospital, Gabon].

Josaphat Iba Ba, Annick Mfoumou, Ingrid Nseng-Nseng Ondo, Ulrich Davy Kombila, Jean-Bruno Boguikouma
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Abstract

Introduction: Antisynthetases syndrome (AS) is a subgroup of inflammatory myopathy where myositis is associated to polyarthritis, interstitial pneumopathy, Raynaud's phenomenon, "mechanic's hands" skin lesions, and positive anti-aminoacyl-transfer RNA (tRNA) synthetase antibodies. Here, we present seven cases from Gabon.

Materials and methods: This retrospective descriptive study was conducted in the Internal Medicine Department at Libreville University Hospital from January 1, 1984, to December 31, 2023. The study aimed to identify all patients with documented AS and clarify its characteristics.

Results: Seven female patients with a mean age of 42.5 years were identified. The cardinal signs of AS were predominantly muscular (n = 7) and pulmonary (n = 5) with diffuse interstitial pneumopathy (n = 5). The patients experienced stage 2 (n = 3) to stage 3 (n = 2) dyspnea and cutaneous manifestations, including "mechanic's hands" (n = 4). Two patients were positive for Jo-1 antibodies, one for PL7 antibodies, and four for PL12 antibodies.

Discussion: AS poses a significant challenge to clinicians in sub-Saharan Africa due to the diffuse interstitial pneumonitis that accompanies pulmonary involvement.

Conclusion: Our study confirms the predominance of anti-PL12 antibodies and the late diagnosis of this condition.

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[反合成酶综合征:来自加蓬利伯维尔大学医院内科的7项观察]。
简介:抗合成酶综合征(AS)是炎性肌病的一个亚群,其中肌炎与多发性关节炎、间质性肺炎、雷诺现象、“机械师之手”皮肤病变和抗氨基酰基转移RNA (tRNA)合成酶抗体阳性有关。在这里,我们介绍来自加蓬的七个病例。材料和方法:本回顾性描述性研究于1984年1月1日至2023年12月31日在利伯维尔大学医院内科进行。该研究旨在确定所有有AS记录的患者并阐明其特征。结果:7例女性患者,平均年龄42.5岁。AS的主要征象主要是肌肉(n = 7)和肺部(n = 5),并伴有弥漫性间质性肺病(n = 5)。患者经历第2期(n = 3)至第3期(n = 2)呼吸困难和皮肤表现,包括“机械师的手”(n = 4)。2例患者Jo-1抗体阳性,1例患者PL7抗体阳性,4例患者PL12抗体阳性。讨论:由于弥漫性间质性肺炎伴肺受累,AS对撒哈拉以南非洲的临床医生提出了重大挑战。结论:我们的研究证实了抗pl12抗体的优势和这种疾病的晚期诊断。
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