Integrated therapy decreases the mortality of patients with polymyositis and dermatomyositis: A Taiwan-wide population-based retrospective study.

ETHNOPHARMACOLOGICAL RELEVANCE: The issue of whether integrated treatment with conventional medicine (CM) and herbal medicine (HM) can reduce mortality in patients with polymyositis/dermatomyositis (PM/DM) had not been addressed. AIM OF THE STUDY: In this study, we investigated the effect of integrated therapy on mortality in a retrospective PM/DM cohort in the Taiwan National Health Insurance Research Database (NHIRD). MATERIALS AND METHODS: Patients with PM/DM were retrospectively enrolled from the PM/DM Registry of Catastrophic Illnesses cohort in the Taiwan NHIRD between 1997 and 2011. The patients were divided into an integrated medicine (IM) group that received CM and HM and a non-IM group that received CM alone. The Cox proportional hazards regression model and Kaplan-Meier method were used to evaluate the hazard ratio (HR) for mortality. RESULTS: Three hundred and eighty-five of 2595 patients with newly diagnosed PM/DM had received IM and 99 had received non-IM. The adjusted HR for mortality was lower in the IM group than in the non-IM group (0.42, 95% confidence interval 0.26-0.68, p < 0.001). The adjusted HR for mortality was also lower in the IM group that had received CM plus HM than in the group that received CM alone (0.48, 95% confidence interval 0.28-0.84, p < 0.05). The core pattern of HM prescriptions integrated with methylprednisolone, methotrexate, azathioprine, or cyclophosphamide to decrease mortality included "San-Qi" (Panax notoginseng), "Bai-Ji" (Bletilla striata), "Chen-Pi" (Citrus reticulata), "Hou-Po" (Magnolia officinalis), and "Dan-Shan" (Salvia miltiorrhiza). CONCLUSION: Integrated therapy has reduced mortality in patients with PM/DM in Taiwan. Further investigation of the clinical effects and pharmaceutical mechanism involved is needed.

The long-term outcome of interstitial lung disease with anti-aminoacyl-tRNA synthetase antibodies.

RATIONALE: Anti-aminoacyl transfer RNA synthetase antibodies (anti-ARS) are a group of myositis-specific autoantibodies that are detected in the sera of patients with polymyositis and dermatomyositis (PM/DM) and also in those of patients with idiopathic interstitial pneumonias without any connective tissue disease (CTD), including PM/DM. Although we reported the clinical characteristics of interstitial lung disease with anti-ARS antibodies (ARS-ILD) with and without PM/DM, the long-term prognosis of ARS-ILD remains undetermined. As our previous studies revealed that ARS-ILD without PM/DM was similar to CTD-associated ILD, and that ARS-ILD with PM/DM was radiologically suggestive of a nonspecific interstitial pneumonia (NSIP) pathological pattern, we hypothesized that the prognosis of ARS-ILD might be distinct from that of idiopathic pulmonary fibrosis (IPF) without anti-ARS. OBJECTIVES: To elucidate the long-term outcome of ARS-ILD with and without PM/DM and compare it to that of IPF. METHODS: A two-center retrospective study was conducted. The study population comprised 36 patients with ARS-ILD (8 with PM, 12 with DM, and 16 without myositis throughout the course), 100 patients with IPF without anti-ARS, and 7 patients with NSIP without anti-ARS. The presence of anti-ARS was determined by RNA immunoprecipitation using the sera obtained at the time of diagnosis before specific treatment. MEASUREMENTS AND MAIN RESULTS: During the observational period (median 49 months; range, 1-114 months), 7 patients with ARS-ILD (19%; 3 with PM, 1 with DM, and 3 without PM/DM) and 51 patients with IPF (51%) died. Patients with ARS-ILD had better overall survival than those with IPF (log-rank test, P < 0.001) and similar survival compared to those with NSIP (log-rank test, P = 0.59). The prognosis for patients with ARS-ILD was similar between those with and without myositis (log-rank test, P = 0.91). At the median follow-up time of 76.5 months, 14 of the 36 patients with ARS-ILD had deteriorated. Both a decline in forced vital capacity or an initiation of long-term oxygen therapy during the course (odds ratio [OR], 5.34) and acute exacerbation (OR, 28.4) significantly increased the mortality risk. CONCLUSIONS: The long-term outcome of ARS-ILD was significantly better than that of IPF regardless of the presence or absence of myositis.

Clinical course and outcomes of Iranian children with juvenile dermatomyositis and polymyositis.

This study evaluated the clinical features, course, and outcomes of Iranian children with juvenile dermatomyositis (JDM), juvenile polymyositis (JPM), and other uncommon connective tissue disorders. A chart review of 85 Iranian children with JDM and JPM was performed during a 10-year period from 2003 to 2013. The patients' clinical signs and symptoms, laboratory data, and other factors affecting clinical outcomes were recorded using questionnaires. Statistical analysis was performed using SPSS software version 20. In all, 40 boys and 45 girls were included in the study (F/M, 1.1:1). Disease frequency was significantly higher in boys aged <5 years (F/M, 0.4:1) and girls aged >5 years (F/M, 1.6:1). The combined mean age at diagnosis was 7.5 years. Muscle weakness, particularly in the proximal muscles of lower extremities (96 %); fatigue (83 %); and heliotrope rash (71 %) were the most frequently recorded symptoms. Elevated lactate dehydrogenase level was the most common enzyme disturbance (98 %). Monocyclic course was seen in 60 % of patients. The mean treatment duration was 3 years. The incidence rate of complications such as calcinosis, lipodystrophy, and growth disturbances was 20, 9, and 30 %, respectively. The occurrence of these complications in patients with monocyclic disease was significantly lower. Vital organ involvement led to the death of four patients. The incidence of calcinosis was significantly lower in patients having a shorter interval between disease onset and treatment. Two important complications, failure to thrive and lipodystrophy, were significantly higher in patients having antinuclear antibodies. The incidence of the above three complications was higher in patients with polycyclic or continuous chronic disease. Respiratory failure was the most common cause of patient mortality.

Juvenile dermatomyositis at a tertiary care hospital: is there any change in the last decade?

INTRODUCTION: Juvenile dermatomyositis (JDM) is a rare multisystem disorder of childhood primarily involving the skeletal muscles and skin. PATIENTS AND METHODS: The case records of patients with JDM seen at our centre in the last 10 years were reviewed and data on clinical presentation, management, outcome and complications were retrieved. RESULTS: Eighteen patients (nine boys) were diagnosed as JDM with median age at presentation of 12.5 years, duration of illness of 9.25 months and follow-up duration of 24 months. At presentation, rash was seen in all patients, 17 had muscle weakness, fever in 11 and arthritis in six. Gottron's lesions and heliotrope rash were seen in 14 and 11 patients, respectively. Calcinosis was seen in five patients and lipoatrophy in two patients. Four patients had dysphagia, one each had dilated cardiomyopathy and respiratory failure. Electromyograph was abnormal in 15 patients and antinuclear antibodies were positive in nine patients. Prednisolone and methotrexate were used in 17 patients. Other disease-modifying anti-rheumatic drugs used were hydroxychloroquine, azathioprine, cyclophosphamide and cyclosporine. Sixteen patients achieved remission. Five patients had pyogenic infections and one patient died of this. In addition two patients had tuberculosis. CONCLUSION: Compared to our experience in the previous decade we saw more girls, used methotrexate upfront but the median duration of illness and prevalence of calcinosis (30%) was the same, suggesting that we need to improve awareness about JDM among paediatricians for early referral.