It is generally accepted that the combination of these MRI features provides the highest sensitivity and specificity.10 However, all MRI features are not always concomitantly found and the mere presence of just one of these indicators is associated with a good positive predictive value (above 2/3).10 In this case series, T2-weighted was positive only 3 times (25%). with an active myositis. When performed (n?=?11), cardiac MRI revealed a late hypersignal in the T1-images in 73% of the cases (n?=?8). Half of the patients required intensive care. Ten patients (83%) received dedicated cardiotropic drugs. Steroids and at least 1 immunosuppressive drug were given in all cases. After a median follow-up of 11 months (range 0C84) 9 (75%) patients recovered whereas 3 (25%) developed a chronic cardiac insufficiency. No patient died. The prevalence of myocarditis in aSS is similar to that of other inflammatory myopathies. Although the prognosis is relatively good, myocarditis is a severe condition and should be carefully considered as a possible manifestation in active aSS patients. INTRODUCTION Antisynthetase syndrome (aSS) belongs to the category of autoimmune inflammatory Rabbit Polyclonal to TOP2A myopathies and is characterized by the presence of various but mutually exclusive anti-tRNA-synthetase autoantibodies. Its spectrum often includes myositis with interstitial pneumonia, Raynaud’s phenomenon, inflammatory polyarthralgia/polyarthritis, and mechanic’s hands.1,2 Cardiac involvement in inflammatory myopathies is polymorphic and includes subclinical (electromyographic) changes or different features like congestive heart failure,3,4 pulmonary hypertension,5 pericarditis,6 and myocarditis. This latter manifestation seems to be particularly rare in DMT1 blocker 1 aSS, with only a few case reports published to date.7,8 Although potentially severe, this condition could be underestimated. Myocarditis can occur in contexts other than myopathies, including infectious or inflammatory diseases or hypereosinophilia.7,8 Diagnosing myocarditis remains difficult and the clinical classification criteria are still a matter of debate. Based on the WHO classification, myocarditis is an inflammatory myocardial injury and is defined based on an endomyocardial biopsy (EB) with specific histological and immunological features.9C11 However, performing an EB is risky DMT1 blocker 1 and is now only rarely performed, especially when cardiac-magnetic resonance imaging (MRI) is suggestive.9C13 Diagnosing myocarditis remains a challenge in patients with an autoimmune disease, particularly with inflammatory myopathies. This is because the clinical manifestations can be subclinical, nonspecific, or concealed by other clinical features, including pericarditis or lung involvement. It is however crucial to diagnose myocarditis, due to the need for specific treatments, and to DMT1 blocker 1 avoid a potentially fatal early evolution or the development of chronic heart failure at a later stage. We have conducted the largest retrospective series to date, with the objective of describing myocarditis in the context of aSS, specifying its prevalence and outcomes. PATIENTS AND METHODS The myocarditis cases occurring during aSS (n?=?12) were retrospectively selected from the French national registry (n?=?352, 10 university centers, 2000C2014). Myocarditis was defined by the occurrence of acute ( 24?hours) or subacute (24C72?hours) cardiac symptoms associated with either increased troponin I or T (which are highly specific for cardiomyocyte necrosis)14,15 and suggestive cardiac-MRI or EB (patient # 10) and in the absence of some other significant cause (including viral illness, coronaropathy, or valvulopathy). It should be mentioned that 2 qualified individuals who died before specific morphological examination were not included, despite a compatible myocarditis analysis. Demographic data, medical, biological and morphological findings, treatments, and results were retrospectively examined by CD,1 CD,2 and BH, using the same form. The study was authorized by local honest committees, and individuals were reported anonymously, in accordance with French law. RESULTS Of the entire cohort, 12 individuals (3.4%, woman/man percentage?=?3, median age 54, range 17C67) were identified as meeting the classification criteria. Cardiovascular risk factors were found in half of the instances (n?=?6) but rarely significant. Their distribution was smoking habit (n?=?2), systemic hypertension (n?=?2), dyslipidemia (n?=?2), obesity (n?=?2), and DMT1 blocker 1 mild diabetes mellitus (n?=?3), see Table ?Table11?. TABLE 1 DMT1 blocker 1 Patient Characteristics Open in a separate windowpane TABLE 1 (Continued) Patient Characteristics Open in a separate windowpane Among the medical features of aSS, myocarditis was one of the 1st aSS manifestations in 42% (n?=?5) of the cases. In the additional instances, myocarditis occurred during the follow-up period (median 36 months, range 8C156). In only 3 (43%) of these instances were the individuals treated with immunosuppressive medicines at the time of the myocarditis analysis. Myocarditis manifestations included remaining and/or right ventricular dysfunction (n?=?10), which was often severe (leading to congestive heart failure with New York heart association stage III: n?=?9). Rigorous care was indeed required 6 instances (50%). Chest pain was reported 3 times (25%)..