Zhao Bing, Hou Ying, Shao Kai, Ma XiaoTian, Yan YaPing, Lu Jian-Qiang, Li Wei, Yan ChuanZhu, Zhang LiNing, Dai TingJun
Department of Neurology, Qilu Hospital (Qingdao), Cheeloo College of Medicine, Shandong University, Qingdao, Shandong, China.
Department of Neurology, Shandong Key Laboratory of Mitochondrial Medicine and Rare Diseases, Research Institute of Neuromuscular and Neurodegenerative Disease, Qilu Hospital of Shandong University, Jinan, Shandong, China.
Brain Pathol. 2025 May;35(3):e13319. doi: 10.1111/bpa.13319. Epub 2024 Nov 18.
To define the clinical, serological, and muscle histopathological characteristics, as well as treatment outcomes, of patients with anti-Ha antibody. We performed a retrospective analysis of clinical, serological, and pathological data and long-term treatment outcomes of anti-Ha patients between January 2005 and July 2023 at our center. Anti-Ha antibody was identified by immunoblot and reconfirmed by immunoprecipitation. Of the 570 patients with idiopathic inflammatory myopathies, 17 (3.0%) were found to be anti-Ha positive, of whom 5 (29.4%) were also positive for another myositis-specific antibody (MSA). All patients with anti-Ha antibody as the single MSA (12/17, 70.6%) had clinical and histopathological evidence of muscle damage. Skin lesions were identified in nine of them (75%), while both interstitial lung disease and Raynaud's phenomenon were only seen in four patients. A necrotizing myopathy without a perifascicular pattern was the most common pathological manifestation (50%). Perifascicular necrosis (PFN) and myofiber major histocompatibility complex class-II expression were observed only in one and four patients, respectively. Muscle weakness relapse was reported in five patients, and skin rashes worsening were observed in one patient. Most of the anti-Ha patients (66.7%) finally achieved a favorable outcome at last follow-up. Anti-Ha antibody might not be as rare as previously thought and may coexist with other MSAs. Muscle damage is the most common manifestation in anti-Ha patients, while extra-muscular symptoms except for the cutaneous manifestations are unusual. The histopathological features varied with a predominance of necrotizing myopathy without PFN. These patients often finally had favorable outcomes, although relapses often occur.
为明确抗Ha抗体患者的临床、血清学及肌肉组织病理学特征以及治疗效果。我们对2005年1月至2023年7月在本中心就诊的抗Ha患者的临床、血清学、病理学数据及长期治疗效果进行了回顾性分析。通过免疫印迹法鉴定抗Ha抗体，并通过免疫沉淀法再次确认。在570例特发性炎性肌病患者中，发现17例（3.0%）抗Ha抗体阳性，其中5例（29.4%）同时还存在另一种肌炎特异性抗体（MSA）阳性。所有以抗Ha抗体作为单一MSA的患者（12/17，70.6%）均有肌肉损伤的临床和组织病理学证据。其中9例（75%）出现皮肤病变，而间质性肺疾病和雷诺现象仅在4例患者中出现。无束周型的坏死性肌病是最常见的病理表现（50%）。仅1例患者观察到束周坏死（PFN），4例患者观察到肌纤维主要组织相容性复合体II类表达。5例患者报告有肌无力复发，1例患者观察到皮疹加重。大多数抗Ha患者（66.7%）在最后一次随访时最终取得了良好的治疗效果。抗Ha抗体可能不像之前认为的那么罕见，并且可能与其他MSA共存。肌肉损伤是抗Ha患者最常见的表现，而除皮肤表现外的肌肉外症状并不常见。组织病理学特征各不相同，以无PFN的坏死性肌病为主。这些患者虽然常出现复发，但最终往往有良好的治疗效果。
