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Abstract：
UNASSIGNED: Idiopathic inflammatory myopathies (IIMs) encompass a diverse group of diseases characterized by considerable variability in clinical manifestations, antibody profiles, and responsiveness to immunosuppressive therapies. This study aimed to investigate the association between organ involvement and distinct myositis autoantibodies in individuals with IIM in a single-center cohort.
UNASSIGNED: Patients with ICD diagnoses M33.1, M33.2, M33.9, or M609 who (1) had been tested with Euroline blot assay for myositis autoantibodies and (2) met the classification criteria of definite/probable polymyositis (PM) or dermatomyositis (DM), anti-synthetase syndrome (ASS), or inclusion body myositis (IBM) were included. Medical journals were retrospectively examined with respect to clinical disease features.
UNASSIGNED: Seventy patients (median age 58 years; 66% females) were included and represented the following diagnosis: PM (n = 23), DM (n = 21), ASS (n = 23), and IBM (n = 3). Most of the patients (87%) presented a muscle biopsy indicative of myositis. The presence of autoantibodies was as follows: myositis-specific antibodies, MSA (n = 53), myositis-associated antibodies, MAA (n = 33), both MSA + MAA (n = 24), MSA only (n = 29), MAA only (n = 9), no MSA, or MAA (n = 8). Anti-Jo-1 was the most common MSA (19%), whereas the most common MAA was anti-Ro/SSA52 (31%). We observed a significant association between antibody patterns and lung disease. In our cohort, 47% of the patients in the whole study group, 86% of patients with anti-SSA52, and 100% with anti-Jo-1 had pulmonary involvement. Patients with both MSA and MAA had a higher incidence of lung disease and decreased CO-diffusion capacity. This was especially prominent in anti-Ro/SSA52-positive patients. Interestingly, none of the patients suffered from lung disease if only antibodies against Mi-2α, Mi-2β, NXP2, HMGCR, and TIF1γ were present or no MSA/MAA were detected.
UNASSIGNED: The simultaneous presence of both MAA and MSA indicates an increased risk of lung involvement in patients with inflammatory myopathies. The presence of any MAA, and especially anti-Ro/SSA52, is associated with more severe pulmonary disease. Our data suggest that MAA antibodies might be relevant markers for early detection and treatment of lung involvement in IIM.
UNASSIGNED: Patients with ICD diagnoses M33.1, M33.2, M33.9, or M609 who (1) had been tested with Euroline blot assay for myositis autoantibodies and (2) met the classification criteria of definite/probable polymyositis (PM) or dermatomyositis (DM), anti-synthetase syndrome (ASS), or inclusion body myositis (IBM) were included. Medical journals were retrospectively examined with respect to clinical disease features.
UNASSIGNED: Seventy patients (median age 58 years; 66% females) were included and represented the following diagnosis: PM (n = 23), DM (n = 21), ASS (n = 23), and IBM (n = 3). Most of the patients (87%) presented a muscle biopsy indicative of myositis. The presence of autoantibodies was as follows: myositis-specific antibodies, MSA (n = 53), myositis-associated antibodies, MAA (n = 33), both MSA + MAA (n = 24), MSA only (n = 29), MAA only (n = 9), no MSA, or MAA (n = 8). Anti-Jo-1 was the most common MSA (19%), whereas the most common MAA was anti-Ro/SSA52 (31%). We observed a significant association between antibody patterns and lung disease. In our cohort, 47% of the patients in the whole study group, 86% of patients with anti-SSA52, and 100% with anti-Jo-1 had pulmonary involvement. Patients with both MSA and MAA had a higher incidence of lung disease and decreased CO-diffusion capacity. This was especially prominent in anti-Ro/SSA52-positive patients. Interestingly, none of the patients suffered from lung disease if only antibodies against Mi-2α, Mi-2β, NXP2, HMGCR, and TIF1γ were present or no MSA/MAA were detected.
UNASSIGNED: The simultaneous presence of both MAA and MSA indicates an increased risk of lung involvement in patients with inflammatory myopathies. The presence of any MAA, and especially anti-Ro/SSA52, is associated with more severe pulmonary disease. Our data suggest that MAA antibodies might be relevant markers for early detection and treatment of lung involvement in IIM.
摘要：
■特发性炎性肌病（IIMs）包括一组不同的疾病，其特征是临床表现具有相当大的变异性，抗体谱，以及对免疫抑制疗法的反应。本研究旨在调查单中心队列中IIM患者器官受累与不同肌炎自身抗体之间的关系。
■ICD诊断为M33.1，M33.2，M33.9或M609的患者（1）已通过Euroline印迹测定法检测肌炎自身抗体，并且（2）符合明确/可能的多发性肌炎（PM）或皮肌炎（DM）的分类标准，抗合成酶综合征（ASS），或包涵体肌炎（IBM）被包括在内。对医学期刊的临床疾病特征进行了回顾性检查。
■包括70例患者（中位年龄58岁；66％为女性），并代表以下诊断：PM（n=23），DM（n=21），ASS（n=23），和IBM(n=3)。大多数患者（87％）表现为肌炎的肌肉活检。自身抗体的存在如下：肌炎特异性抗体，MSA（n=53），肌炎相关抗体，MAA（n=33），MSA+MAA（n=24），仅MSA（n=29），仅MAA（n=9），没有MSA,或MAA(n=8)。Anti-Jo-1是最常见的MSA（19％），而最常见的MAA是抗Ro/SSA52（31％）。我们观察到抗体模式与肺部疾病之间存在显着关联。在我们的队列中，整个研究组中47%的患者，86％的抗SSA52患者和100％的抗Jo-1患者肺部受累。同时患有MSA和MAA的患者肺部疾病的发生率更高，CO扩散能力降低。这在抗Ro/SSA52阳性患者中尤其突出。有趣的是,如果仅针对Mi-2α的抗体，则没有患者患有肺部疾病，Mi-2β,NXP2,HMGCR,和TIF1γ存在或未检测到MSA/MAA。
■同时存在MAA和MSA表明炎性肌病患者肺部受累的风险增加。任何MAA的存在，尤其是抗Ro/SSA52与更严重的肺部疾病相关。我们的数据表明，MAA抗体可能是早期发现和治疗IIM肺部受累的相关标志物。
■ICD诊断为M33.1，M33.2，M33.9或M609的患者（1）已通过Euroline印迹测定法检测肌炎自身抗体，并且（2）符合明确/可能的多发性肌炎（PM）或皮肌炎（DM）的分类标准，抗合成酶综合征（ASS），或包涵体肌炎（IBM）被包括在内。对医学期刊的临床疾病特征进行了回顾性检查。
■包括70例患者（中位年龄58岁；66％为女性），并代表以下诊断：PM（n=23），DM（n=21），ASS（n=23），和IBM(n=3)。大多数患者（87％）表现为肌炎的肌肉活检。自身抗体的存在如下：肌炎特异性抗体，MSA（n=53），肌炎相关抗体，MAA（n=33），MSA+MAA（n=24），仅MSA（n=29），仅MAA（n=9），没有MSA,或MAA(n=8)。Anti-Jo-1是最常见的MSA（19％），而最常见的MAA是抗Ro/SSA52（31％）。我们观察到抗体模式与肺部疾病之间存在显着关联。在我们的队列中，整个研究组中47%的患者，86％的抗SSA52患者和100％的抗Jo-1患者肺部受累。同时患有MSA和MAA的患者肺部疾病的发生率更高，CO扩散能力降低。这在抗Ro/SSA52阳性患者中尤其突出。有趣的是,如果仅针对Mi-2α的抗体，则没有患者患有肺部疾病，Mi-2β,NXP2,HMGCR,和TIF1γ存在或未检测到MSA/MAA。
■同时存在MAA和MSA表明炎性肌病患者肺部受累的风险增加。任何MAA的存在，尤其是抗Ro/SSA52与更严重的肺部疾病相关。我们的数据表明，MAA抗体可能是早期发现和治疗IIM肺部受累的相关标志物。
