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We performed a retrospective analysis of clinical, serological, and pathological data and long\u2010term treatment outcomes of anti\u2010Ha patients between January 2005 and July 2023 at our center. Anti\u2010Ha 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\u2010Ha positive, of whom 5 (29.4%) were also positive for another myositis\u2010specific antibody (MSA). All patients with anti\u2010Ha 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\u2010II 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\u2010Ha patients (66.7%) finally achieved a favorable outcome at last follow\u2010up. Anti\u2010Ha antibody might not be as rare as previously thought and may coexist with other MSAs. Muscle damage is the most common manifestation in anti\u2010Ha patients, while extra\u2010muscular 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.<\/jats:p>","DOI":"10.1111\/bpa.13319","type":"journal-article","created":{"date-parts":[[2024,11,19]],"date-time":"2024-11-19T02:53:56Z","timestamp":1731984836000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":3,"title":["Clinico\u2010sero\u2010pathological characteristics of anti\u2010Ha antisynthetase syndrome"],"prefix":"10.1111","volume":"35","author":[{"given":"Bing","family":"Zhao","sequence":"first","affiliation":[{"name":"Department of Neurology Qilu Hospital (Qingdao), Cheeloo College of Medicine, Shandong University  Qingdao Shandong China"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ying","family":"Hou","sequence":"additional","affiliation":[{"name":"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"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Kai","family":"Shao","sequence":"additional","affiliation":[{"name":"Department of Medicine Experimental Center Qilu Hospital (Qingdao), Cheeloo College of Medicine, Shandong University  Qingdao Shandong China"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"XiaoTian","family":"Ma","sequence":"additional","affiliation":[{"name":"Department of Medicine Experimental Center Qilu Hospital (Qingdao), Cheeloo College of Medicine, Shandong University  Qingdao Shandong China"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"YaPing","family":"Yan","sequence":"additional","affiliation":[{"name":"Key Laboratory of the Ministry of Education for Medicinal Resources and Natural Pharmaceutical Chemistry College of Life Sciences, Shanxi Normal University  Xi'an 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