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

[抗合成酶综合征:加蓬利伯维尔大学医院内科的七例观察]

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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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