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Rheumatology AND Dermatomyositis [keywords]
- Pulmonary function and autoantibodies in a long-term follow-up of juvenile dermatomyositis patients. [JOURNAL ARTICLE]
- Rheumatology (Oxford) 2013 Dec 5.
Objectives. Pulmonary disease is a rare complication in JDM, described in only a few studies. This long-term follow-up study aimed to (i) describe pulmonary involvement in a national cohort of JDM patients estimated by conventional spirometry, (ii) compare pulmonary impairment with overall JDM outcome, and (iii) identify possible associations between pulmonary impairment and myositis-specific autoantibodies (MSAs).Methods. Fifty-one JDM patients performed conventional spirometry in a cross-sectional follow-up study. The scores of the Myositis Damage Index (MDI), Myositis Damage by visual analogue scale (MYODAM-VAS) and physician's global damage assessment were used to estimate JDM outcome. ANAs, MSAs and myositis-associated autoantibodies were analysed in all patients.Results. Forty-two patients (82%) (mean follow-up time 14.3 years) had normal lung function. Four patients (8%) were diagnosed with JDM-related restrictive interstitial lung disease. No patients reported pulmonary symptoms. Patients with restrictive pulmonary function had increased long-term damage estimated by MDI (P = 0.008), MYODAM-VAS (P = 0.04), global assessment (P = 0.03) and number of organ systems involved (P = 0.009). We found significant correlation between the restrictive pulmonary function test and damage by the MDI (r = 0.43, P = 0.003), MYODAM-VAS (r = 0.44, P = 0.002), and global damage assessment (r = 0.43, P = 0.003). No association was found between the restrictive pulmonary function test and autoantibodies.Conclusion. In a long-term follow-up study of JDM patients, the majority of patients demonstrated normal lung function. However, restrictive pulmonary impairment was identified in 8% of patients, indicating a need for repetitive pulmonary follow-up in JDM patients. Restrictive pulmonary involvement was associated with increased long-term JDM damage.
- Subscapular bursitis and calcinosis in a dermatomyositis patient. [JOURNAL ARTICLE]
- Arthritis Rheum 2013 Nov 27.
- Inhibition of myogenic MicroRNAs-1, 133, and 206 by inflammatory cytokines links inflammation and muscle degeneration in adult inflammatory myopathies. [JOURNAL ARTICLE]
- Arthritis Rheum 2013 Nov 27.
Objective: The molecular basis of inflammatory myopathies such as dermatomyositis, polymyositis, and inclusion body myositis, which share the characteristics of chronic muscle inflammation and skeletal muscle wasting, are poorly understood. As such, effective targeted treatments for these diseases are lacking, resulting in critical unmet medical needs for these devastating diseases. In order to identify possible new targets for drug development, this study explored the mechanism by which inflammation may play a role in inflammatory myopathy pathology. Methods: We compared expression levels of inflammatory cytokines and microRNAs (miRs) between muscle biopsies from inflammatory myopathy patients and non-myositic muscle donors. In vitro human and mouse model systems where then used to characterize the role of these cytokines and miRs on myoblast-to-myocyte differentiation. Results: We observed increased expression of inflammatory cytokines including TNFα, IFNα, IFNβ, and IL1β in different subtypes of inflammatory myopathies. In all subtypes evaluated, we observed decreased expression of miRs miR-1, 133a, and 133b, as well as decreased miR-206 in DM; these miRs are essential for adult skeletal muscle differentiation and maintenance. TNFα was significantly inversely correlated with decreased myogenic miR expression in inflammatory myopathy subtypes. In mechanistic studies, TNFα inhibited expression of myogenic miRs and suppressed differentiation of C2C12 myoblasts to myocytes/myotubes in an NF-ĸB dependent manner. This block in differentiation by TNFα was relieved by overexpression of miR-1, miR-206, or miR-133a/b. Conclusions: Taken together, these results provide a new mechanistic link between pro-inflammatory cytokine action and the degenerative pathology of inflammatory myopathies, and suggest therapeutic approaches for these diseases. © 2013 American College of Rheumatology.
- Gemcitabine-induced radiation recall myositis in a patient with dermatomyositis. [LETTER]
- Int J Rheum Dis 2013 Nov 28.
- The heart and pediatric rheumatology. [Journal Article]
- Rheum Dis Clin North Am 2014 Feb; 40(1):61-85.
Recent advances in Kawasaki disease have included attempts to define genes involved in its pathogenesis. There have been recent advances in the studies of rheumatic carditis, leading to a better understanding of the mechanism of the disease. Histologic evaluation of patients with neonatal lupus erythematosus has revealed fibrosis with collagen deposition and calcification of the atrioventricular node. Therapy for cardiac involvement in systemic juvenile idiopathic arthritis should involve treatment of the underlying disease and systemic inflammatory state, and typically includes nonsteroidal antiinflammatory drugs, corticosteroids, disease-modifying drugs, and biologic therapies targeting tumor necrosis factor-alpha, interleukin-1, and interleukin-6.
- The heart in inflammatory myopathies. [Journal Article]
- Rheum Dis Clin North Am 2014 Feb; 40(1):1-10.
Cardiac involvement in the idiopathic inflammatory myopathies (IIM) has been reported in 5% to 70% of cases. It is estimated that between 15% and 55% of deaths are related to heart disease in patients with dermatomyositis (DM) and polymyositis (PM). Increased atherosclerotic disease in IIM patients has also been reported and is associated with traditional risk factors such as hypertension and dyslipidemia. In the current article we review some of the more recent literature on clinical manifestations and outcomes of cardiovascular disease in IIM patients.
- [The clinical characteristics of 26 cases of amyopathic dermatomyositis]. [English Abstract, Journal Article]
- Zhonghua Nei Ke Za Zhi 2013 Jul; 52(7):578-80.
To analyze the clinical characteristics of amyopathic dermatomyositis (ADM).Twenty six patients diagnosed as ADM from January 2006 to January 2010 in PLA General Hospital were retrospectively analyzed. The clinical manifestation, laboratory findings, imaging manifestations, treatment and prognosis of the 26 patients were recorded.There were 18 females and 8 males with age of 30-68 years. Overall disease course after diagnosis was 2-18 months. All patients had Gottron rash and interstitial pneumonia. Fifteen patients had history of pulmonary infections. Three patients had comorbidity of tumor. Creatine phosphokinase, creatine phosphokinase isoenzyme, glutamic-oxaloacetic transaminase and lactate dehydrogenase were normal in all 26 patients. Four patients had positive anti-Jo-1 antibodies. Antinuclear antibodies were positive in nine patients. Electromyogram was slightly abnormal in 5 patients. Muscle biopsy was abnormal in 19 patients. Twenty patients had improved after receiving corticosteroids and immunosuppressive agents. Six patients died.It has been estimated that ADM represents approximately 20% of all cases of dermatomyositis. It seems that patients with ADM have greater incidence of lung involvement and combined cancer. ADM patients need to be treated positively to improve the prognosis.
- A case of dermatomyositis with rhabdomyolysis, rescued by intravenous immunoglobulin. [JOURNAL ARTICLE]
- Mod Rheumatol 2013 Nov 5.
We describe a case of severe dermatomyositis (DM) complicated by rhabdomyolysis, acute tubular necrosis, and hemophagocytosis. The case failed to respond to corticosteroids, but showed rapid and significant improvement after the addition of intravenous immunoglobulin (IVIG). While the prognosis of DM is poor when it is complicated by rhabdomyolysis, the early administration of IVIG has the potential to be the cornerstone of its management.
- Serum interleukin 6 levels as a useful prognostic predictor of clinically amyopathic dermatomyositis with rapidly progressive interstitial lung disease. [JOURNAL ARTICLE]
- Mod Rheumatol 2013 Nov 4.
Objectives. Rapidly progressive interstitial lung disease (RP-ILD) is life-threatening in patients with clinically amyopathic dermatomyositis (CADM). Useful prognostic markers are necessary for treatment selection. This study aimed to investigate differences in clinical and laboratory characteristics between surviving and non-surviving patients. Methods. Twelve CADM patients with RP-ILD were enrolled. Six patients lived (Group A) and six patients died (Group B) after immunosuppressive treatment for RP-ILD. Clinical manifestations and laboratory data before treatment were compared between the two groups. Results. Among the clinical manifestations and laboratory data examined, serum interleukin 6 (IL-6) levels in Group B were significantly higher than those in Group A (mean ± SD 28.5 ± 21.0 vs. 7.2 ± 1.6 pg/mL; p = 0.009). Simple regression analysis showed that serum IL-6 was the only significant prognostic factor (p = 0.032). Kaplan-Meier estimates showed that the cumulative survival rate was significantly lower in patients with serum IL-6 levels of ≥ 9 pg/mL than in patients with those of < 9 pg/mL (p = 0.04). Conclusions. Serum IL-6 levels may predict the prognosis of CADM patients with RP-ILD. The intensity of immunosuppressive treatment can be decided according to serum IL-6 levels at an early phase of the disease.
- Successful treatment of macrophage activation syndrome in a patient with dermatomyositis by combination with immunosuppressive therapy and plasmapheresis. [JOURNAL ARTICLE]
- Mod Rheumatol 2013 Oct 21.
Macrophage activation syndrome (MAS), also known as secondary hemophagocytic lymphohistiocytosis, is mediated by cytokine overproduction from excessive activation of T lymphocytes and macrophages. We present a dermatomyositis patient with MAS, caused by hypercytokinemia. The combination of tacrolimus and plasma exchange therapy was effective in this case for treating MAS. This combination therapy is especially useful for MAS refractory to steroids.