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Rheumatology AND Dermatomyositis [keywords]
- Correlation between serum levels of IL-15 and IL-17 in patients with idiopathic inflammatory myopathies. [JOURNAL ARTICLE]
- Scand J Rheumatol 2014 Nov 7.:1-5.
Objectives: To assess the serum levels of interleukin (IL)-15 and IL-17 in patients with idiopathic inflammatory myopathies (IIM) and correlate them with levels of IL-1 receptor antagonist (IL-1ra), IL-6, IL-10, interferon (IFN)-γ, monocyte chemoattractant protein (MCP)-1, macrophage inflammatory protein (MIP)-1α, and MIP-1β. Possible correlations with disease activity parameters were also evaluated. Method: Sera from 14 patients with new-onset polymyositis (PM), 10 with dermatomyositis (DM), seven with anti-synthetase syndrome (ASS) and 19 healthy controls (HC) were analysed by multiplex immunoassay. Sera from 19 patients were analysed after a median follow-up of 5 months. All patients underwent physical examination, manual muscle testing (MMT) using the five-point MMT scales, the Health Assessment Questionnaire (HAQ), and serum creatine kinase (CK) measurement. All patients received glucocorticoids, and 13 were taking immunosuppressive therapy. Results: At baseline, serum levels of IL-15, IL-17, MCP-1, and MIP-1β were significantly higher in IIM patients than in HC. IL-17 serum levels were directly correlated (r = 0.39, p = 0.02) with disease duration while a significant inverse correlation was detected between IL-17 levels and MMT scores (r = -0.4, p = 0.02). The highest IL-15 levels were present in DM patients (p = 0.02 vs. PM). The most striking finding was the strong correlation between IL-15 and IL-17 levels (r = 0.60, p = 0.0001), and this correlation was even stronger in DM patients (r = 0.82, p = 0.006). Conclusions: The strong correlation between IL-15 and IL-17 in IIM patients, and especially in DM, suggests that there may be an interplay between the two cytokines in the pathogenesis of myositis. Further studies of larger patient cohorts and of muscle biopsies are needed to confirm these preliminary data.
- Acute hemorrhagic myositis in inflammatory myopathy and review of the literature. [Journal Article]
- Case Rep Rheumatol 2014.:639756.
We describe two patients with dermatomyositis that presented with interstitial lung disease, positive V and Shawl sign who developed acute spontaneous abdominal/retroperitoneal bleed. Both patients expired despite aggressive treatment and resuscitation. Hemorrhagic myositis in these two patients with inflammatory myopathy is a very rare complication. The association of anti-Ro52 with this potentially very serious complication remains unclear. This potential relationship should be further evaluated in future studies.
- Possible interplay between interleukin-15 and interleukin-17 into the pathogenesis of idiopathic inflammatory myopathies. [Journal Article]
- Reumatismo 2014; 66(3):215-23.
The aim of this study was to assess the serum levels of interleukin (IL)-15 and IL-17 in patients with idiopathic inflammatory myopathies (IIM) and correlate them with IL-6, IL-10, monocyte chemoattractant protein-1 (MCP-1), macrophage inflammatory protein-1α (MIP-1α), MIP-1β levels. Possible correlations with disease activity parameters were also evaluated. Sera from 14 polymyositis (PM), 10 dermatomyositis (DM), 7 anti-synthetase syndrome new onset patients and 19 healthy controls (HCs) were analyzed by multiplex immunoassay. Sera from 19 patients were analyzed after 5 months median follow-up. All patients underwent physical examination, the 5-points manual muscle test (MMT), the health assessment questionnaire and serum creatine kinase measurement. All patients received glucocorticoids, and 13 were taking also immunosuppressive therapy. At baseline, serum levels of IL-15, IL-17, MCP-1 and MIP-1β were significantly higher in IIM patients than in HCs. IL-17 serum levels were directly correlated with disease duration (r=0.39, P=0.02), while a significant inverse correlation was detected between IL-17 levels and MMT scores (r=-0.4, P=0.02). The highest IL-15 levels were present in DM patients (P=0.02 vs PM). The most striking finding was the strong correlation between IL-15 and IL-17 levels (r=0.60, P=0.0001), and this correlation was even stronger in DM patients (r=0.82, P=0.006). The strong correlation between IL-15 and IL-17 in IIM patients, and especially in DM, suggests that there may be a interplay between the two cytokines in the pathogenesis of myositis. Further studies of larger patient cohorts and muscle biopsies are needed to confirm these preliminary data.
- The presentation, assessment, pathogenesis, and treatment of calcinosis in juvenile dermatomyositis. [Journal Article]
- Curr Rheumatol Rep 2014 Dec; 16(12):467.
Calcinosis is one of the hallmark sequelae of juvenile dermatomyositis (JDM), and despite recent progress in the therapy of JDM, dystrophic calcification still occurs in approximately one third of patients. This review discusses our current, albeit limited, understanding of risk factors for the development of calcinosis in JDM, as well as approaches to assessment, and current views on its pathogenesis. Anecdotal approaches to treating calcinosis associated with JDM, including both anti-inflammatory therapies and agents aimed at inhibiting the deposition of calcium hydroxyapatite, are reviewed. An improved understanding of the pathogenesis of calcinosis, the establishment of standardized measurement tools to assess calcinosis, and randomized controlled trials employing more sensitive outcome measures are needed to develop efficacious therapies for this often disabling complication.
- Risk of ischemic stroke in patients with polymyositis and dermatomyositis: a systematic review and meta-analysis. [JOURNAL ARTICLE]
- Rheumatol Int 2014 Oct 30.
Several chronic inflammatory disorders, such as rheumatoid arthritis and systemic lupus erythematosus, have been demonstrated to increase ischemic stroke risk, but the data on polymyositis (PM) and dermatomyositis (DM) remain unclear. We conducted a systematic review and meta-analysis of observational studies that reported odds ratio, relative risk, hazard ratio or standardized incidence ratio comparing ischemic risk in patients with PM/DM versus non-PM/DM participants. Pooled risk ratio and 95 % confidence intervals were calculated using a random-effect, generic inverse variance method of DerSimonian and Laird. Three cohort studies were identified and included in our data analysis. The pooled risk ratio of ischemic stroke in patients with PM/DM was 1.61 (95 % CI 1.28-2.02). The statistical heterogeneity of this meta-analysis was insignificant with an I (2) of 0 %. Our study demonstrated a statistically significant increased ischemic stroke risk among patients with PM/DM.
- The lymphoid follicle variant of dermatomyositis. [Journal Article]
- Neurol Neuroimmunol Neuroinflamm 2014 Aug; 1(2):e19.
To investigate the clinical and morphologic spectrum of early adult-onset dermatomyositis (DM), an inflammatory disease that affects small vessels of the muscle and the skin.Histologic evaluation of frozen muscle samples was employed to visualize the cellular organization of ectopic lymphoid structures in muscle biopsies obtained from 2 patients diagnosed with DM. Clinical presentation and morphologic features, as well as treatment and follow-up, were assessed and documented. Electron microscopy was used to confirm the light microscopic diagnosis of DM. Clonality analysis of B-cell populations using PCR was performed.Muscle biopsy of both patients fulfilled the morphologic European Neuromuscular Centre criteria of DM. Analyses of muscle biopsy samples revealed ectopic lymphoid follicle-like structures that showed a remarkable similarity to secondary lymphoid organs (SLOs) with distinct T- and B-cell compartmentalization. Our 2 patients exhibited an atypical and mild clinical presentation and responded favorably to therapy.The clinical and histopathologic features of DM can be atypical, and the presence of SLOs is not inevitably linked to an unfavorable prognosis.
- Genetic Association Study of TNFAIP3, IFIH1, IRF5 Polymorphisms with Polymyositis/Dermatomyositis in Chinese Han Population. [Journal Article]
- PLoS One 2014; 9(10):e110044.
Single-nucleotide polymorphisms (SNPs) in the TNFAIP3, IFIH1, and IRF5 genes have been associated with several auto-inflammation diseases, while the susceptibility between these genes and idiopathic inflammatory myopathies (IIMs) were not reported. This study aimed to investigate whether TNFAIP3, IFIH1, and IRF5 gene polymorphisms confer susceptibility for the IIMs in Chinese Han population.A large case-control study of Chinese subjects with polymyositis (PM) (n = 298) and dermatomyositis (DM) (n = 530) was accomplished. 968 healthy and ethnically matched controls were available for comparison. Six SNPs in the TNFAIP3 region (rs2230926 and rs5029939), the IFIH1 gene (rs1990760 and rs3747517) and the IRF5 region (rs4728142 and rs729302) were assessed and genotyped using the Sequenom MassArray iPLEX platform.Our study indicated a strong allele association was observed in PM/DM and PM patients for rs2230926 (OR: 1.61, 95%CI: 1.20-2.16, Pc = 7.5×10-3; OR: 1.88, 95%CI: 1.30-2.74, Pc = 4.0×10-3, respectively) and rs5029939 (OR: 1.64, 95%CI: 1.21-2.21, Pc = 6.0×10-3; OR: 1.88, 95%CI: 1.28-2.76, Pc = 5.5×10-3,respectively). And rs2230926 and rs5029939 were significantly associated with interstitial lung disease (ILD) in PM/DM and PM patients (Pc = 0.04 and Pc = 0.016; Pc = 0.02 and Pc = 0.03, respectively). In addition, rs4728142 allele and genotype had significant association with PM/DM patients (Pc = 0.026 and Pc = 0.048, respectively). Further analysis with three logistic regression genetic models revealed statistically significant difference in the genotypic distribution in the PM/DM, PM or DM patients when the additive and dominant models were used.This was the first study to reveal TNFAIP3 and IRF5 polymorphisms were associated with PM/DM patients or these patients with ILD, indicating that TNFAIP3 and IRF5 might be the susceptibility gene for PM/DM patients in Chinese Han population.
- Infection is the leading cause of hospital mortality in patients with dermatomyositis/polymyositis: Data from a population-based study. [JOURNAL ARTICLE]
- Arthritis Care Res (Hoboken) 2014 Oct 20.
Dermatomyositis (DM) and polymyositis (PM) are debilitating inflammatory myopathies associated with significant mortality. We evaluated the relative contribution of infection to hospital mortality in a large population-based study of individuals with DM/PM.Data derive from the 2007 to 2011 Healthcare Cost and Utilization Project National Inpatient Samples and included all hospital discharges that met a validated administrative definition of DM/PM. The primary outcome was hospital mortality. Variables for infections and comorbidities were generated from discharge diagnoses using validated administrative definitions. Logistic regression was used to investigate the relationship between infection and mortality in individuals with DM/PM, adjusting for sociodemographics, utilization variables, and comorbidities. Relative risks were calculated to compare the overall prevalence of specific infections and associated mortality in DM/PM hospitalizations with those seen in the general hospitalized population.15,407 hospitalizations with DM/PM met inclusion criteria for this study and inpatient mortality was 4.5% (700 deaths). In adjusted logistic regression analyses, infection (OR 3.4, 95% CI 2.9-4.0) was the strongest predictor of hospital mortality among individuals with DM/PM. Bacterial infection (OR 3.5, 95% CI 3.0-4.1), comprised primarily of pneumonia and bacteremia, and opportunistic fungal infections (OR 2.5, 95% CI 1.5-4.0) were independently associated with hospital mortality. The overall burden of infection in hospitalizations with DM/PM was significantly increased in comparison with the general hospitalized population (RR 1.5, 95% CI 1.4-1.6).Among hospitalized individuals with DM/PM, infection is the leading cause of mortality. Strategies to mitigate infection risk in both the clinic and hospital settings should be evaluated to improve disease outcomes. © 2014 American College of Rheumatology.
- Cutaneous ulceration in dermatomyositis: Association with anti-melanoma differentiation-associated gene 5 antibodies and interstitial lung disease. [JOURNAL ARTICLE]
- Arthritis Care Res (Hoboken) 2014 Oct 20.
Objective: To identify clinical and serologic correlates of cutaneous ulcers in dermatomyositis (DM). Methods: We retrospectively examined a cohort of 152 DM patients. We compared the features of patients with ulcers to those without ulcers using chi-squared or Fisher's exact tests and used univariate and multivariate logistic regression models to assess the association between ulcers and clinical features such as malignancy, interstitial lung disease (ILD), and amyopathic disease. Results: 43 (28%) patients had cutaneous ulcers. Nearly half the patients had ulcers present in more than one location: 24 (56%) had ulcers over the extensor surfaces of joints, 18 (42%) at the digital pulp or periungual areas, and 25 (58%) had ulcers located elsewhere. In univariate analysis ulcers were associated with Asian race, but not with other clinical and demographic features, including malignancy or ILD. In multivariate analysis ulcers were significantly associated with anti-melanoma differentiation-gene 5 (MDA5) antibodies (OR=10.14, 95%CI 1.95-52.78, p=0.0059) and this was greatest for ulcers located at the digital pulp. In patients with cutaneous ulcers, ILD risk was specifically increased only in patients with anti-MDA5+ antibodies. Conclusion: We confirmed the strong association between anti-MDA5 antibodies and cutaneous ulcers, with the novel finding that the association of cutaneous ulcers with ILD depends upon the presence of anti-MDA5 antibodies. DM patients who display this cutaneous phenotype should undergo appropriate evaluation for ILD. © 2014 American College of Rheumatology.
- [A case of clinical overlap syndrome of rheumatoid arthritis and amyopathic dermatomyositis with multiple pulmonary injuries]. [English Abstract, Journal Article, Research Support, Non-U.S. Gov't]
- Beijing Da Xue Xue Bao 2014 Oct 18; 46(5):805-8.
Autoimmune diseases can cause various kinds of lung injuries. Clinical features of a case of overlap syndrome with multiple pulmonary injuries were investigated, and the treatment experiences discussed. The patient suffered from rheumatoid arthritis (RA) at first, and then had a lobectomy surgery due to the rheumatoid nodules in her right lung. A year later her disease was diagnosed as amyopathic dermatomyositis (ADM) with typical Gottron's sign, craftsmen hands and rapid-progressive organizing pneumonia (OP). After a combined treatment with glucocorticoids (GCs) and cyclophosphamide (CTX), her OP became better but lung infections progressed. Her lung infections eventually were cured after we used antibiotics and antifungal treatment while we ceased to use CTX and reduced the dosage of GCs. The clinical feature of the patient was overlap syndrome with a variety of lung injuries, such as pulmonary rheumatoid nodules, OP, secondary bronchopleural fistula and lung infections. Its diagnosis and treatment experiences could improve our understanding of pulmonary manifestations of connective tissue disease and improve our diagnosis and treatment level.