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