OBJECTIVES: To compare clinical characteristics, including the frequency of cutaneous, extramuscular manifestations and malignancy, between adults with anti-synthetase syndrome (ASyS) and DM. METHODS: Using data regarding adults from the MYONET registry, a cohort of DM patients with anti-Mi2/-TIF1γ/-NXP2/-SAE/-MDA5 autoantibodies, and a cohort of ASyS patients with anti-tRNA synthetase autoantibodies (anti-Jo1/-PL7/-PL12/-OJ/-EJ/-Zo/-KS) were identified. Patients with DM sine dermatitis or with discordant dual autoantibody specificities were excluded. Sub-cohorts of patients with ASyS with or without skin involvement were defined based on presence of DM-type rashes (heliotrope rash, Gottron's papules/sign, violaceous rash, shawl sign, V-sign, erythroderma, and/or periorbital rash). RESULTS: In total 1054 patients were included (DM, n = 405; ASyS, n = 649). In the ASyS cohort, 31% (n = 203) had DM-type skin involvement (ASyS-DMskin). A higher frequency of extramuscular manifestations, including Mechanic's hands, Raynaud's phenomenon, arthritis, interstitial lung disease and cardiac involvement differentiated ASyS-DMskin from DM (all P < 0.001), whereas higher frequency of any of four DM-type rashes-heliotrope rash (n = 248, 61% vs n = 90, 44%), violaceous rash (n = 166, 41% vs n = 57, 9%), V-sign (n = 124, 31% vs n = 28, 4%), and shawl sign (n = 133, 33% vs n = 18, 3%)-differentiated DM from ASyS-DMskin (all P < 0.005). Cancer-associated myositis (CAM) was more frequent in DM (n = 67, 17%) compared with ASyS (n = 21, 3%) and ASyS-DMskin (n = 7, 3%) cohorts (both P < 0.001). CONCLUSION: DM-type rashes are frequent in patients with ASyS; however, distinct clinical manifestations differentiate these patients from classical DM. Skin involvement in ASyS does not necessitate increased malignancy surveillance. These findings will inform future ASyS classification criteria and patient management.

• MeSH
• Amino Acyl-tRNA Synthetases immunology   MeSH
• Autoantibodies * blood   immunology   MeSH
• Dermatomyositis * immunology   complications   MeSH
• Adult MeSH
• Exanthema etiology   MeSH
• Lung Diseases, Interstitial immunology   etiology   MeSH
• Middle Aged MeSH
• Humans MeSH
• Myositis * immunology   complications   MeSH
• Neoplasms complications   MeSH
• Registries * MeSH
• Aged MeSH
• Check Tag
• Adult MeSH
• Middle Aged MeSH
• Humans MeSH
• Male MeSH
• Aged MeSH
• Female MeSH
• Publication type
• Journal Article MeSH
• Comparative Study MeSH

Anti-melanoma differentiation-associated gene-5 (MDA-5) antibody is an autoantibody found in patients with dermatomyositis. These antibody-positive patients are clinically characterized by complications of rapidly progressive interstitial pneumonia resistant to treatment and with poor prognosis. We describe herein a patient with MDA-5 antibody-positive interstitial lung disease, which progressed rapidly to death after a period of slow progress. Recently, attention has been paid to the similarities in clinical courses and CT images between MDA-5 antibody-positive interstitial lung disease and coronavirus disease 2019 (COVID-19)-associated pneumonia. Patients with MDA-5 antibody do not always have diffuse and evenly distributed bilateral opacities at the time of first presentation. This patient had significant laterality of such opacities. It should be considered that MDA-5 antibody-positive patients with such laterality in opacities might progress rapidly. Chest physicians, dermatologists, and dermatologists need to be aware of the characteristics of the disease for optimal treatment choices.

• MeSH
• Autoantibodies MeSH
• COVID-19 * complications   MeSH
• Dermatomyositis * complications   MeSH
• Lung Diseases, Interstitial * complications   MeSH
• Humans MeSH
• Check Tag
• Humans MeSH
• Publication type
• Journal Article MeSH

• MeSH
• Cisplatin adverse effects   MeSH
• Cytostatic Agents * adverse effects   MeSH
• Dermatomyositis etiology   complications   MeSH
• Lambert-Eaton Myasthenic Syndrome etiology   complications   MeSH
• Humans MeSH
• Myasthenia Gravis etiology   complications   MeSH
• Oxaliplatin adverse effects   MeSH
• Paraneoplastic Polyneuropathy diagnosis   etiology   MeSH
• Paraneoplastic Syndromes, Nervous System * diagnosis   etiology   MeSH
• Polyneuropathies chemically induced   MeSH
• Taxoids adverse effects   MeSH
• Urogenital Neoplasms complications   MeSH
• Vinca Alkaloids adverse effects   MeSH
• Check Tag
• Humans MeSH

• MeSH
• Spondylitis, Ankylosing complications   MeSH
• Dermatomyositis complications   MeSH
• Cardiovascular Diseases * etiology   MeSH
• Humans MeSH
• Polymyositis complications   MeSH
• Arthritis, Rheumatoid complications   MeSH
• Scleroderma, Systemic complications   MeSH
• Lupus Erythematosus, Systemic complications   MeSH
• Takayasu Arteritis complications   MeSH
• Check Tag
• Humans MeSH
• Publication type
• Review MeSH

• MeSH
• Spondylitis, Ankylosing complications   MeSH
• Anti-Glomerular Basement Membrane Disease complications   MeSH
• Dermatomyositis complications   MeSH
• Diagnosis, Differential MeSH
• Collagen Diseases complications   MeSH
• Hemorrhage etiology   MeSH
• Humans MeSH
• Connective Tissue Diseases complications   MeSH
• Pulmonary Alveoli MeSH
• Lung Diseases etiology   therapy   MeSH
• Polymyositis classification   MeSH
• Prognosis MeSH
• Arthritis, Rheumatoid complications   MeSH
• Sjogren's Syndrome complications   MeSH
• Scleroderma, Systemic complications   MeSH
• Systemic Vasculitis complications   MeSH
• Lupus Erythematosus, Systemic complications   MeSH
• Check Tag
• Humans MeSH

• Keywords
• imunopatogeneze, netóza,
• MeSH
• Apoptosis immunology   MeSH
• Biological Therapy MeSH
• Dermatomyositis immunology   complications   physiopathology   MeSH
• Extracellular Matrix immunology   pathology   MeSH
• Humans MeSH
• Connective Tissue * immunology   physiopathology   MeSH
• Polymyositis immunology   complications   physiopathology   MeSH
• Arthritis, Rheumatoid diagnosis   drug therapy   genetics   immunology   physiopathology   therapy   MeSH
• Ribonucleoproteins analysis   immunology   MeSH
• Mixed Connective Tissue Disease immunology   physiopathology   MeSH
• Scleroderma, Systemic etiology   immunology   physiopathology   pathology   MeSH
• Lupus Erythematosus, Systemic diagnosis   genetics   immunology   physiopathology   therapy   MeSH
• Inflammation * immunology   classification   physiopathology   MeSH
• Check Tag
• Humans MeSH
• Publication type
• Review MeSH

Zánětlivé myopatie představují heterogenní skupinu získaných myopatií, jejichž podkladem je zánět. Nejčastěji se vyskytují idiopatické zánětlivé myopatie, které se liší průběhem, výskytem přidružených chorob, efektem léčby a nálezem ve svalové biopsii. Zánětlivý původ mají rovněž myopatie u systémových chorob, vaskulitid, infekčních chorob virových, bakteriálních, mykotických i parazitárních. V předložené práci je podán přehled základních zánětlivých myopatií s důrazem na klinické charakteristiky a nálezy svalové biopsie.

Inflammatory myopathies represent a heterogennic group of acquired myopathies, which have an inflammatory cause. Idiopathic inflammatory myopathies have the most frequent occurrence and they are differentiated by a course, occurrence of associated diseases, by treatment effect and by findings in muscle biopsy. Myopathies in systemic diseases, vasculitides, viral, bacterial, fungal and parasitary infectious diseases have an inflammatory cause too. In the following paper we present overview of basic inflammatory myopathies with emphasis on the clinical characteristics and muscle biopsy findings.

• Keywords
• zánětlivé neuropatie, přidružené choroby, biopsie svalu, nekrotizující myopatie,
• MeSH
• Biopsy MeSH
• Dermatomyositis diagnosis   drug therapy   complications   MeSH
• Diagnosis, Differential MeSH
• Electromyography MeSH
• Immunohistochemistry MeSH
• Immunosuppressive Agents therapeutic use   MeSH
• Muscle, Skeletal pathology   MeSH
• Humans MeSH
• Myositis, Inclusion Body diagnosis   drug therapy   complications   MeSH
• Myositis classification   MeSH
• Polymyositis diagnosis   drug therapy   complications   MeSH
• Prednisone administration & dosage   adverse effects   therapeutic use   MeSH
• Signs and Symptoms MeSH
• Antibodies analysis   MeSH
• Check Tag
• Humans MeSH

• MeSH
• Biomarkers blood   metabolism   MeSH
• Densitometry methods   utilization   MeSH
• Dermatomyositis drug therapy   complications   MeSH
• Glucocorticoids administration & dosage   adverse effects   therapeutic use   MeSH
• Granulomatosis with Polyangiitis drug therapy   complications   MeSH
• Bone Density physiology   immunology   drug effects   MeSH
• Middle Aged MeSH
• Humans MeSH
• Young Adult MeSH
• Drug-Related Side Effects and Adverse Reactions drug therapy   complications   MeSH
• Osteoporosis epidemiology   etiology   complications   MeSH
• Calcium, Dietary therapeutic use   MeSH
• Vertebroplasty methods   utilization   MeSH
• Vitamin D therapeutic use   MeSH
• Check Tag
• Middle Aged MeSH
• Humans MeSH
• Young Adult MeSH
• Male MeSH
• Female MeSH
• Publication type
• Case Reports MeSH

• MeSH
• Spondylitis, Ankylosing complications   MeSH
• Anti-Glomerular Basement Membrane Disease complications   MeSH
• Dermatomyositis complications   MeSH
• Diagnosis, Differential MeSH
• Collagen Diseases complications   MeSH
• Hemorrhage etiology   MeSH
• Humans MeSH
• Connective Tissue Diseases complications   MeSH
• Pulmonary Alveoli MeSH
• Lung Diseases etiology   therapy   MeSH
• Polymyositis classification   MeSH
• Prognosis MeSH
• Arthritis, Rheumatoid complications   MeSH
• Sjogren's Syndrome complications   MeSH
• Scleroderma, Systemic complications   MeSH
• Systemic Vasculitis complications   MeSH
• Lupus Erythematosus, Systemic complications   MeSH
• Check Tag
• Humans MeSH

• Keywords
• myozitida s inkluzními tělísky (IBM),
• MeSH
• Azathioprine therapeutic use   MeSH
• Dermatomyositis diagnosis   drug therapy   complications   MeSH
• Diagnostic Techniques and Procedures MeSH
• Diagnosis, Differential MeSH
• Adult MeSH
• Disease Attributes MeSH
• Drug Therapy, Combination MeSH
• Drug Resistance immunology   drug effects   MeSH
• Humans MeSH
• Methotrexate therapeutic use   MeSH
• Myositis, Inclusion Body diagnosis   drug therapy   complications   MeSH
• Myositis history   drug therapy   classification   MeSH
• Polymyositis diagnosis   drug therapy   complications   MeSH
• Check Tag
• Adult MeSH
• Humans MeSH
• Male MeSH
• Publication type
• Case Reports MeSH
• Review MeSH