Unit of Immunology, Rheumatology, Allergy and Rare Diseases, IRCCS San Raffaele Hospital, Milan, Italy.
School of Medicine, Vita-Salute San Raffaele University, Milan, Italy.
Rheumatology (Oxford). 2024 Jul 1;63(7):1902-1910. doi: 10.1093/rheumatology/kead541.
Myocarditis is an overlooked manifestation of anti-synthetase syndrome (ASS). Our study describes the clinical and instrumental features of ASS myocarditis and evaluates the performance of cardiac MRI (CMRI) with mapping techniques in assisting diagnosis of ASS myocarditis.
Data from patients with ASS were retrospectively analysed. CMRI data for patients diagnosed with myocarditis, including late gadolinium enhancement (LGE), T2 ratio, T1 mapping, extracellular volume (ECV) and T2 mapping, were reviewed. Myocarditis was defined by the presence of symptoms and/or signs suggestive for heart involvement, including increased high-sensitive troponin T (hs-TnT) and/or N-terminal pro-brain natriuretic peptide (NT-proBNP), and at least an instrumental abnormality. The clinical features of patients with ASS with and without myocarditis were compared. A P-value of <0.05 was considered statistically significant.
Among a cohort of 43 patients with ASS [median age 58 (48.0-66.0) years; females 74.4%; anti-Jo1 53.5%], 13 (30%) were diagnosed with myocarditis. In 54% of those 13 patients, myocarditis was diagnosed at clinical onset. All patients with ASS with myocarditis had at least one CMRI abnormality: increased ECV in all cases, presence of LGE in 91%, and increased T1 and T2 mapping in 91%. The 2009 Lake Louise criteria (LLC) were satisfied by 6 patients, and the 2018 LLC by 10 patients. With the updated LLC, the sensitivity for myocarditis improved from 54.6% to 91.0%. Patients with ASS with myocarditis were more frequently males (53% vs 13%; P = 0.009) with fever (69% vs 17%; P = 0.001), and had higher hs-TnT [88.0 (23.55-311.5) vs 9.80 (5.0-23.0) ng/l; P < 0.001], NT-proBNP [525.5 (243.5-1575.25) vs 59.0 (32.0-165.5; P = 0.013) pg/ml; P = 0.013] and CRP [7.0 (1.7-15.75) vs 1.85 (0.5-2.86) mg/l; P = 0.011] compared with those without myocarditis.
In ASS, myocarditis is frequent, even at clinical onset. Patients with ASS with myocarditis frequently presented with fever and increased CRP, suggesting the existence of an inflammatory phenotype. The use of novel CMRI mapping techniques may increase diagnostic sensitivity for myocarditis in ASS.
心肌炎是抗合成酶综合征（ASS）的一种被忽视的表现。我们的研究描述了 ASS 心肌炎的临床和仪器特征，并评估了心脏 MRI（CMRI）与映射技术在协助 ASS 心肌炎诊断中的性能。
回顾性分析了 ASS 患者的数据。对诊断为心肌炎的患者的 CMRI 数据（包括晚期钆增强（LGE）、T2 比值、T1 映射、细胞外体积（ECV）和 T2 映射）进行了回顾。心肌炎的定义是存在提示心脏受累的症状和/或体征，包括升高的高敏肌钙蛋白 T（hs-TnT）和/或 N 末端脑钠肽前体（NT-proBNP），以及至少存在一种仪器异常。比较了 ASS 患者有无心肌炎的临床特征。P 值<0.05 被认为具有统计学意义。
在一组 43 名 ASS 患者[中位年龄 58（48.0-66.0）岁；女性占 74.4%；抗 Jo1 占 53.5%]中，13 名（30%）被诊断为心肌炎。在这 13 名患者中，有 54%在临床发病时被诊断为心肌炎。所有 ASS 合并心肌炎的患者均至少存在一项 CMRI 异常：所有患者的 ECV 均增加，91%的患者存在 LGE，91%的患者存在 T1 和 T2 映射增加。6 名患者符合 2009 年路易斯湖标准（LLC），10 名患者符合 2018 年 LLC。采用更新后的 LLC，心肌炎的灵敏度从 54.6%提高到 91.0%。ASS 合并心肌炎的患者更常为男性（53%比 13%；P=0.009），伴有发热（69%比 17%；P=0.001），hs-TnT 更高[88.0（23.55-311.5）比 9.80（5.0-23.0）ng/l；P<0.001]，NT-proBNP 更高[525.5（243.5-1575.25）比 59.0（32.0-165.5；P=0.013）pg/ml；P=0.013]，C 反应蛋白（CRP）更高[7.0（1.7-15.75）比 1.85（0.5-2.86）mg/l；P=0.011]。
在 ASS 中，心肌炎很常见，甚至在临床发病时也很常见。ASS 合并心肌炎的患者常伴有发热和 CRP 升高，提示存在炎症表型。新型 CMRI 映射技术的使用可能会提高 ASS 中心肌炎的诊断敏感性。
