BACKGROUND Dermatomyositis is an autoimmune disease characterized by distinctive skin changes, muscle involvement, and, in many cases, damage to various organs, such as interstitial lung disease, arthritis, and myocarditis. The association between dermatomyositis and malignancies is well established. The most common cancers that manifest with a dermatomyositis are neoplasms of the breast, lungs, cervix, and gastrointestinal tract. Primary biliary cirrhosis is a slowly progressive autoimmune disease characterized by a triad of chronic cholestasis, positive specific autoantibodies, and characteristic imaging or pathomorphological findings. Regardless of etiology, cirrhosis is considered as a major risk factor for development of hepatocellular carcinoma. This type of liver neoplasm is rarely associated with dermatomyositis. CASE REPORT We report the case of a 64-year-old woman with comorbid primary biliary cirrhosis, hepatocellular carcinoma, and dermatomyositis as a paraneoplastic syndrome. Pathognomonic skin lesions of dermatomyositis, such as heliotrope rash, Gottron papules, Gottron sign, V-sign, and proximal muscle weakness preceded the diagnosis of biliary cholangitis and hepatocellular carcinoma by more than a year but were not initially recognized. The failure to identify the symptoms of dermatomyositis, combined with the lack of pronounced and specific signs of primary biliary cirrhosis and hepatocellular carcinoma, contributed to delayed diagnoses. The patient died due to severe, decompensated heart failure. CONCLUSIONS This case report emphasized the importance of precise interpretation of pathogenetic findings by all specialists involved. In patients with multiple comorbidities, close collaboration between specialists and an interdisciplinary approach are essential for timely diagnosis and the selection of appropriate treatment, which can significantly improve prognosis and outcome.

A revision of the 2017 EULAR-ACR myositis classification criteria, namely EULAR-ACR funded Myositis Revision of Classification (MyoROC) project, is currently underway involving a large international group of experts. In the first phase of this project, we identified additional items to be tested in the criteria. We distributed an electronic survey to International Myositis Assessment and Clinical Studies (IMACS) members to identify new items. The identified items were discussed within the Steering Committee and a multi-step Delphi consensus process consisting of an open discussion and three rounds of e-voting were conducted to reach the final item list. The IMACS survey results revealed 24 new items. After an open discussion with Steering Committee members, 14 items were dropped and five new items were added, resulting in a total of 15 items. After three rounds of e-voting, the following variables were agreed to be tested in addition to the original items: finger flexion, knee extension ≥ hip flexion weakness, myonecrosis pattern on biopsy, magnetic resonance imaging and electromyography findings of myositis, additional rashes, skin biopsy, capillaroscopy, interstitial lung disease, arthritis, Raynaud's phenomenon, myositis-specific (MSA) and -associated autoantibodies, enzyme elevation at ≥2 time points, and aldolase. The new items that will be tested in the revised criteria were generated with input from a wide range of stakeholders and included, most importantly, MSA, pattern of weakness, skin changes, and additional diagnostic modalities. The next steps of the project are data collection followed by statistical analysis for development and validation of the revised criteria.

Among the idiopathic inflammatory myopathies, patients harbouring an Antisynthetase syndrome exhibit a unique clinical picture, with characteristic signs such as myositis, interstitial lung disease, arthritis, rash, and/or fever. Characteristic morphological features on skeletal muscle biopsies differentiate Antisynthetase syndrome from other forms of myositis. Autoantibodies typically recognizing one of the members of the aminoacyl-tRNA synthetase family of proteins can be detected in the serum of such patients, with anti-Jo1 being most frequent. Until now, an international consensus definition of the Antisynthetase syndrome is lacking, hence this workshop has undertaken the task to inform about the clinical, morphological and autoantibody profiles of Antisynthetase syndrome. The authors also expand their aims by giving management and therapeutic strategies, and finally provide precise classification criteria for Antisynthetase syndrome.

Anti-synthetase syndrome (AS) is a rare autoimmune disorder classified among the idiopathic inflammatory myopathies and is characterized by antibodies directed against aminoacyl-transfer RNA synthetases and the presence of myositis, interstitial lung disease, ±arthritis. Here, we report, for the first time, immune thrombocytopenia (ITP) in a patient with AS. This case reports a new association of AS with ITP and highlights the utility of identifying the underlying driver in secondary ITP to guide therapy.

Antisynthetase syndrome (anti-SS) is a rare systemic autoimmune disease characterised by autoantibodies against aminoacyl-tRNA synthetases manifesting as one or more components of the classic triad: interstitial lung disease, arthritis and myositis. While it is well-recognised that autoimmune rheumatological disorders in general can contribute to multiple pregnancy complications, very little is known about how anti-SS itself affects pregnancy outcomes. Described here is the case of a 26-year-old pregnant woman with anti-SS whose pregnancy course was complicated by placental dysfunction and subsequent extremely premature delivery at 24 weeks' gestation. This report presents a review of the literature to date and discusses potential pregnancy complications associated with anti-SS and their subsequent targeted management.