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Abstract：
OBJECTIVE: To investigate the correlation factors of complete clinical response in idiopathic inflammatory myopathies (IIMs) patients receiving conventional treatment.
METHODS: Patients diagnosed with IIMs hospitalized in Peking University People\'s Hospital from January 2000 to June 2023 were included. The correlation factors of complete clinical response to conventional treatment were identified by analyzing the clinical characteristics, laboratory features, peripheral blood lymphocytes, immunological indicators, and therapeutic drugs.
RESULTS: Among the 635 patients included, 518 patients finished the follow-up, with an average time of 36.8 months. The total complete clinical response rate of IIMs was 50.0% (259/518). The complete clinical response rate of dermatomyositis (DM), anti-synthetase syndrome (ASS) and immune-mediated necrotizing myopathy (IMNM) were 53.5%, 48.9% and 39.0%, respectively. Fever (P=0.002) and rapid progressive interstitial lung disease (RP-ILD) (P=0.014) were observed much more frequently in non-complete clinical response group than in complete clinical response group. The aspartate transaminase (AST), lactate dehydrogenase (LDH), D-dimer, erythrocyte sedimentation rate (ESR), C-reaction protein (CRP) and serum ferritin were significantly higher in non-complete clinical response group as compared with complete clinical response group. As for the treatment, the percentage of glucocorticoid received and intravenous immunoglobin (IVIG) were significantly higher in non-complete clinical response group than in complete clinical response group. Risk factor analysis showed that IMNM subtype (P=0.007), interstitial lung disease (ILD) (P=0.001), eleva-ted AST (P=0.012), elevated serum ferritin (P=0.016) and decreased count of CD4+T cells in peripheral blood (P=0.004) might be the risk factors for IIMs non-complete clinical response.
CONCLUSIONS: The total complete clinical response rate of IIMs is low, especially for IMNM subtype. More effective intervention should be administered to patients with ILD, elevated AST, elevated serum ferritin or decreased count of CD4+T cells at disease onset.
METHODS: Patients diagnosed with IIMs hospitalized in Peking University People\'s Hospital from January 2000 to June 2023 were included. The correlation factors of complete clinical response to conventional treatment were identified by analyzing the clinical characteristics, laboratory features, peripheral blood lymphocytes, immunological indicators, and therapeutic drugs.
RESULTS: Among the 635 patients included, 518 patients finished the follow-up, with an average time of 36.8 months. The total complete clinical response rate of IIMs was 50.0% (259/518). The complete clinical response rate of dermatomyositis (DM), anti-synthetase syndrome (ASS) and immune-mediated necrotizing myopathy (IMNM) were 53.5%, 48.9% and 39.0%, respectively. Fever (P=0.002) and rapid progressive interstitial lung disease (RP-ILD) (P=0.014) were observed much more frequently in non-complete clinical response group than in complete clinical response group. The aspartate transaminase (AST), lactate dehydrogenase (LDH), D-dimer, erythrocyte sedimentation rate (ESR), C-reaction protein (CRP) and serum ferritin were significantly higher in non-complete clinical response group as compared with complete clinical response group. As for the treatment, the percentage of glucocorticoid received and intravenous immunoglobin (IVIG) were significantly higher in non-complete clinical response group than in complete clinical response group. Risk factor analysis showed that IMNM subtype (P=0.007), interstitial lung disease (ILD) (P=0.001), eleva-ted AST (P=0.012), elevated serum ferritin (P=0.016) and decreased count of CD4+T cells in peripheral blood (P=0.004) might be the risk factors for IIMs non-complete clinical response.
CONCLUSIONS: The total complete clinical response rate of IIMs is low, especially for IMNM subtype. More effective intervention should be administered to patients with ILD, elevated AST, elevated serum ferritin or decreased count of CD4+T cells at disease onset.
摘要：
目的：探讨特发性炎症性肌病(IIMs)患者接受常规治疗后临床完全缓解的相关因素。
方法：纳入2000年1月至2023年6月北京大学人民医院住院诊断为IIMs的患者。通过分析临床特点,找出影响常规治疗完全缓解的相关因素,实验室特点,外周血淋巴细胞，免疫学指标，和治疗药物。
结果：在635名患者中，518名患者完成了随访，平均时间为36.8个月。IIMs的总临床完全缓解率为50.0%(259/518)。皮肌炎（DM）的临床完全缓解率，抗合成酶综合征（ASS）和免疫介导的坏死性肌病（IMNM）占53.5％，48.9%和39.0%,分别。发热（P=0.002）和快速进展性间质性肺病（RP-ILD）（P=0.014）在非完全临床反应组中观察到的频率高于完全临床反应组。天冬氨酸转氨酶(AST),乳酸脱氢酶（LDH），D-二聚体,红细胞沉降率（ESR），非完全临床反应组C-反应蛋白(CRP)和血清铁蛋白明显高于完全临床反应组。至于治疗,非完全临床缓解组患者接受糖皮质激素和静脉注射免疫球蛋白(IVIG)的百分比显著高于完全临床缓解组.危险因素分析显示IMNM亚型(P=0.007),间质性肺病（ILD）（P=0.001），抬高的AST（P=0.012），血清铁蛋白升高(P=0.016)和外周血CD4+T细胞计数减少(P=0.004)可能是IIMs非完全临床应答的危险因素.
结论：IIMs的总临床完全缓解率较低，特别是对于IMNM子类型。应该对ILD患者进行更有效的干预，AST升高,疾病发作时血清铁蛋白升高或CD4+T细胞计数降低。
方法：纳入2000年1月至2023年6月北京大学人民医院住院诊断为IIMs的患者。通过分析临床特点,找出影响常规治疗完全缓解的相关因素,实验室特点,外周血淋巴细胞，免疫学指标，和治疗药物。
结果：在635名患者中，518名患者完成了随访，平均时间为36.8个月。IIMs的总临床完全缓解率为50.0%(259/518)。皮肌炎（DM）的临床完全缓解率，抗合成酶综合征（ASS）和免疫介导的坏死性肌病（IMNM）占53.5％，48.9%和39.0%,分别。发热（P=0.002）和快速进展性间质性肺病（RP-ILD）（P=0.014）在非完全临床反应组中观察到的频率高于完全临床反应组。天冬氨酸转氨酶(AST),乳酸脱氢酶（LDH），D-二聚体,红细胞沉降率（ESR），非完全临床反应组C-反应蛋白(CRP)和血清铁蛋白明显高于完全临床反应组。至于治疗,非完全临床缓解组患者接受糖皮质激素和静脉注射免疫球蛋白(IVIG)的百分比显著高于完全临床缓解组.危险因素分析显示IMNM亚型(P=0.007),间质性肺病（ILD）（P=0.001），抬高的AST（P=0.012），血清铁蛋白升高(P=0.016)和外周血CD4+T细胞计数减少(P=0.004)可能是IIMs非完全临床应答的危险因素.
结论：IIMs的总临床完全缓解率较低，特别是对于IMNM子类型。应该对ILD患者进行更有效的干预，AST升高,疾病发作时血清铁蛋白升高或CD4+T细胞计数降低。
