Humans ; Dermatomyositis/therapy*

Humans ; Dermatomyositis/therapy* ; Asian People ; China

Acupuncture Points ; Acupuncture Therapy ; Child ; Dermatomyositis ; therapy ; Humans ; Male

Sweet's syndrome (SS) has been reported as an association with malignant neoplasms and autoimmune diseases, e.g., Behcet's disease, Sjogren's syndrome, and rheumatoid arthritis. But dermatomyositis (DM), one of the rare autoimmune diseases, was not reported as an associated disease of SS. We describe an interesting case of SS associated with DM. Diagnosis was made by skin biopsy, and subsequent clinical resolution occurred after institution of prednisolone.
Case Report ; Dermatomyositis/pathology ; Dermatomyositis/drug therapy ; Dermatomyositis/complications* ; Human ; Male ; Middle Age ; Prednisolone/therapeutic use ; Sweet's Syndrome/pathology ; Sweet's Syndrome/drug therapy ; Sweet's Syndrome/complications*

Dermatomyositis ; complications ; Humans ; Lung Diseases, Interstitial ; diagnosis ; etiology ; therapy

A patient with skin rash, skin denudation, anuria, general dropsy and dyspnea for unknown etiology underwent continuous renal replacement therapy (CRRT) for 3 consecutive days. The biochemical indexes were monitored during the therapy and biopsy was performed on the right thigh. Pathological examination of the biopsy sample established the diagnosis of polymyositis(PM) and dermatomyositis(DM). After the start of CRRT, the patient's heart, liver, kidney and lung injuries showed obvious improvement, and the urine volume (UV) increased and serum creatinine (Cr), urea, total bilirubin (TBIL), alanine aminotransferase (ALT), aspartate aminotransferase (AST), creatine kinase (CK), creatine kinase isoenzyme (CK-MB) and lactate dehydrogenase (LDH) levels all decreased promptly. The patient showed progressive improvement of the physiological condition even after CRRT, and was discharged 10 days later. This case suggests the efficacy of CRRT in the management of severe PM/DM and its value as a good option for treatment of severe autoimmune disease, especially systemic inflammatory response syndrome.
Adult ; Dermatomyositis ; therapy ; Humans ; Male ; Polymyositis ; therapy ; Renal Replacement Therapy ; Treatment Outcome

Amyopathic dermatomyositis is diagnosed when the biopsy-confirmed cutaneous lesions of dermatomyositis are present for longer than 2 years in the absence of muscle weakness, elevated muscle enzymes, and the history of immunosuppressive drug therapy and ingestion of drugs such as hydroxyurea that can produce dermatomyositis-like cutaneous hypersensitivity changes. We report a 36-year-old woman with a 3-year history of typical skin features of dermatomyositis with no evidences of muscle involvement.
Adult ; Dermatomyositis* ; Drug Therapy ; Eating ; Female ; Humans ; Hydroxyurea ; Hypersensitivity ; Muscle Weakness ; Skin

Antibodies, Monoclonal, Humanized/therapeutic use* ; Autoantibodies ; Dermatomyositis ; Disease Progression ; Humans ; Lung Diseases, Interstitial/drug therapy*

Objective: To elucidate the clinical features of patients with refractory juvenile dermatomyositis (JDM), and to explore the efficacy and safety of tofacitinib in the treatment of refractory JDM. Methods: A total of 75 JDM patients admitted to the Department of Rheumatology and Immunology in Shenzhen Children's Hospital from January 2012 to January 2021 were retrospectively analyzed, and to analyze the clinical manifestations, efficacy and safety of tofacitinib in the treatment of refractory JDM. Patients were divided into refractory group with using of glucocorticoids in combination with two or more anti-rheumatic drugs for treatment, and the presence of disease activity or steroid dependence after a one-year follow-up. The non-refractory group is defined as clinical symptoms disappeared, laboratory indicators were normal, and clinical remission was achieved after initial treatment, and the clinical manifestations and laboratory indexes of the two groups were compared. The Mann-Whitney U test, Fisher's precision probability test was used for intergroup comparison. Binary Logistic multivariate regression analysis was used to identify risk factors for refractory JDM. Results: Among the 75 children with JDM, 41 were males and 34 were females with a age of onset of 5.3 (2.3, 7.8) years. The refractory group consisted of 27 cases with a age of onset of 4.4 (1.5, 6.8) years, while the non-refractory group consisted of 48 cases with a age of onset of 5.9 (2.5, 8.0) years. Compared with 48 cases in the non-refractory group, the proportion of interstitial lesions and calcinosis in the refractory group was higher than that in the non-refractory group (6 cases (22%) vs. 2 cases (4%), 8 cases (30%) vs. 4 cases (8%), both P<0.05). Binary Logistic regression analysis showed that observation group were more likely to be associated with to interstitial lung disease (OR=6.57, 95%CI 1.22-35.31, P=0.028) and calcinosis (OR=4.63, 95%CI 1.24-17.25, P=0.022). Among the 27 patients in the refractory group, 22 cases were treated with tofacitinib, after treatment with tofacitinib, 15 of 19 cases (86%) children with rashes showed improvement, and 6 cases (27%) with myositis evaluation table score less than 48 score both were improved, 3 of 6 cases (27%) had calcinosis were relieved, and 2 cases (9%) had glucocorticoid-dependence children were successfully weaned off. During the tofacitinib treatment, there was no increase in recurrent infection, blood lipids, liver enzymes, and creatinine were all normal in the 22 cases. Conclusions: Children with JDM with calcinosis and interstitial lung disease are more likely to develop refractory JDM. Tofacitinib is safe and effective for refractory JDM.
Child ; Female ; Male ; Humans ; Dermatomyositis/drug therapy* ; Retrospective Studies ; Risk Factors ; Calcinosis ; Glucocorticoids/therapeutic use*

A case of dermatomyositis presented as bronchiolitis obliterans organizing pneumonia has been rarely reported. We describe a 46-year-old female patient with dermatomyositis without elevation of creatine kinase presented as bronchiolitis obliterans organizing pneumonia. She was treated with prednisolone and azathioprine. Over a 2-year follow-up she has had no elevation of creatine kinase. The patient remains asymptomatic and has no medication for dermatomyositis and bronchiolitis obliterans organizing pneumonia two years after initial treatment. It has been suggested that the prognosis of dermatomyositis without creatine kinase elevation may be poor. Because the prognosis of bronchiolitis obliterans organizing pneumonia is generally believed to be good, we tentatively suggest that the normal value of creatine kinase in dermatomyositis does not always seem to herald a poor prognosis, an associated malignancy or severe interstitial lung disease.
Azathioprine/administration +ACY- dosage ; Biopsy, Needle ; Bronchiolitis Obliterans Organizing Pneumonia/pathology ; Bronchiolitis Obliterans Organizing Pneumonia/diagnosis+ACo- ; Case Report ; Creatine Kinase/blood+ACo- ; Dermatomyositis/pathology ; Dermatomyositis/enzymology ; Dermatomyositis/drug therapy ; Dermatomyositis/diagnosis+ACo- ; Diagnosis, Differential ; Female ; Follow-Up Studies ; Human ; Middle Age ; Prednisone/administration +ACY- dosage ; Tomography, X-Ray Computed