Characterization of the biomarkers related to the clinical course and outcomes of juvenile dermatomyositis

ObjectiveThis study aimed to evaluate the clinical characteristics of children diagnosed with juvenile dermatomyositis (JDM) in a tertiary medical centre in Taiwan and to identify important biomarkers for predicting the disease course and outcomes of JDM.MethodsWe retrospectively reviewed patients with JDM diagnosed at the National Taiwan University Hospital between 1 January 2001 and 31 December 2021. The endpoints for disease assessment included complete clinical response or remission. The JDM courses were divided into monocyclic, polycyclic, and chronic continuous statuses. The significant relationship between the predictors and outcomes was further analysed.ResultsA total of 47 patients were included in this study. The mean age at disease onset was 7.5 years. The female-to-male ratio was 1.35. The most common initial presentations were Gottron's sign (74%), followed by muscle weakness (66%) and facial rash (66%). Among all included patients, 35 (74.5%) patients achieved complete clinical remission, 15 (31.9%) had a monocyclic course, six (12.7%) had a polycyclic course, and 24 (51.1%) had a chronic continuous course. Negative facial rash and arthralgia were favourable factors for achieving complete clinical remission. Muscle weakness, higher lactate dehydrogenase (LDH), and higher erythrocyte sedimentation rate (ESR) at disease onset were related to the chronic continuous course. The most common long-term complication was calcinosis (29.8%).ConclusionJuvenile dermatomyositis is a rare disease, and only a few studies have been conducted in Asia. Our results identified the important predictors of the disease course and outcomes. The chronic continuous course requires more attention and aggressive treatment.     

Serum uric acid is independently associated with hypertension in patients with polymyositis and dermatomyositis

Serum uric acid (UA), as an antioxidant, has been associated with hypertension in the general population. Hypertension is highly prevalent in patients with polymyositis and dermatomyositis (PM/DM). Owning elevated levels of reactive oxygen species, patients with PM/DM have lower concentrations of UA in comparison with healthy people. We explored a potential association between UA levels and hypertension in PM/DM and evaluated whether this association is independent of hypertension risk factors, PM/DM characteristics and relevant drugs. A total of 472 PM/DM patients were assessed. UA and related laboratory data were measured. Demographic, hypertension-related factors, PM/DM characteristics and drug use were assessed as potential covariates. Results were analyzed using logistic models to test the independence of the association between UA and hypertension. UA levels were higher in hypertension subjects compared to non-hypertensive PM/DM patients [284.70 (239.93-357.38) vs 264.00(222.50-322.75), p = .017]. When adjusted for hypertension risk factors, PM/DM characteristics and drugs, the odds of being a hypertensive PM/DM patient per 1 μmol/L UA increase were significantly increased: odds ratio = 1.473 (95% confidence interval:1.063-2.042, p = .020). This cross-sectional study suggests that UA levels are independently associated with hypertension in PM/DM patients.     

Adult and juvenile dermatomyositis treatment

Introduction

Dermatomyositis is a rare autoimmune inflammatory condition affecting skin and muscles. The disease can be seen in both adults and children. It can be associated with malignancy. Considering involvement of skin in the disease, many patients consult dermatologists for its treatment. Hence, knowledge about its presentation, complications, prognosis, and treatment is necessary.

Objective

The objective of this review article is to provide comprehensive information about treatment of dermatomyositis.

Methods

In this review article, we reviewed the published literature on adult and juvenile dermatomyositis to highlight the treatment. Articles published in peer-reviewed journals including reviews, clinical trials, case series, and case reports published in electronic database (MEDLINE/PubMed) through January 2021, cross references of respective articles and trials from clinicaltrials.gov were included for qualitative analysis of the literature.

Results

Treatment options for dermatomyositis include traditional immunosuppressive agents and immunomodulatory therapy. High-dose corticosteroids represent the first line of treatment while other immunosuppressive agents are also used, either in combination with or as an alternative to corticosteroids, after initial treatment failure. Some biological agents have been used for the treatment of dermatomyositis with variable responses.

Conclusion

Although several treatment options are available, several questions remain unanswered about the optimal treatment of dermatomyositis.     

Using imaging methods to assess severe calcinosis in juvenile dermatomyositis: A case report

Calcinosis and lipodystrophy are severe complications of juvenile dermatomyositis (JDM). Up to 20% of patients have calcinosis, and the onset of calcinosis usually occurs 1 to 3 years after that of the illness. We report a case of JDM with severe complications of calcinosis and lipodystrophy, and we assess calcinosis using a variety of imaging methods. To evaluate the patient's inflammatory state, bone scintigraphy was performed, which demonstrated increased uptake in the right scapula, in addition, multiple calcifications are present subcutaneously on the shoulder and back, and inflammatory imaging features are also present in the right knee joint.