Clinical and autoantibody phenotypes of juvenile dermatomyositis.
Oksana BoyarchukAnna KukaIryna YurykPublished in: Reumatologia (2022)
Juvenile dermatomyositis (JDM) is a heterogeneous autoimmune inflammatory myositis with symmetrical proximal muscle weakness and a characteristic rash. Juvenile dermatomyositis is characterized by variable presentation and phenotypes. Detection of myositis autoantibodies is useful in improving JDM diagnosis and predicting the prognosis. In this literature review based on case series we analyze clinical and autoantibody phenotypes of JDM in four patients who were hospitalized in one regional center in Ukraine during the last 3 years and three of them presented in the time of the COVID-19 pandemic. The reviewed literature showed the last updates for the JDM diagnosis and the role of myositis autoantibodies in the prediction of disease course, systemic involvement, and malignancy risk. The presence of anti-synthetase syndrome in all presented patients, mainly due to anti-PL-7 autoantibodies, encourages further study with more patients and with detection of other myositis-specific autoantibodies to identify or refute certain regional features.
Keyphrases
- interstitial lung disease
- end stage renal disease
- systemic lupus erythematosus
- systemic sclerosis
- ejection fraction
- newly diagnosed
- chronic kidney disease
- peritoneal dialysis
- rheumatoid arthritis
- prognostic factors
- case report
- systematic review
- idiopathic pulmonary fibrosis
- myasthenia gravis
- patient reported outcomes
- loop mediated isothermal amplification