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Rheumatology AND Dermatomyositis [keywords]
- 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]
- 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.
- Adult dermatomyositis with bleeding ulcer in the pharynx. [Journal Article]
- Case Rep Otolaryngol 2014.:854841.
Dermatomyositis (DM) is one of the idiopathic inflammatory myopathies caused by complement-mediated vasculopathy or vasculitis in the muscle. Although the gastrointestinal (GI) mucosa has been reported to be involved as a result of vasculitis or vasculopathy, ulceration in the pharynx is a rare manifestation of DM. A 54-year-old woman complaining of muscle weakness in the extremities, low-grade fever, and dysphagia was diagnosed as having DM. Despite medical treatment with corticosteroids and immunosuppressive agents, her DM progressed rapidly, leading to exacerbation of the dysphagia. About 3 weeks after undergoing tracheostomy as a preventive measure against aspiration, the patient developed intractable respiratory tract hemorrhage. Repeated laryngoendoscopy revealed a bleeding ulceration in the pharynx that required hemostasis with electric cautery under general anesthesia. No bleeding recurred thereafter. Histopathologically, the pharynx exhibited nonspecific inflammatory cell infiltration in the muscle tissue. This rare manifestation may be considered in cases of DM with unexplainable airway bleeding.
- Drug-Associated Dermatomyositis Following Ipilimumab Therapy: A Novel Immune-Mediated Adverse Event Associated With Cytotoxic T-Lymphocyte Antigen 4 Blockade. [JOURNAL ARTICLE]
- JAMA Dermatol 2014 Oct 15.
Ipilimumab, a human monoclonal antibody targeted against cytotoxic T-lymphocyte antigen 4, has shown promise in the treatment of metastatic melanoma. However, given its mechanism of action, immune-related adverse effects have been reported with this therapy. Despite increasing reports of immune-related adverse effects related to ipilimumab therapy, dermatomyositis associated with this agent has not previously been reported.We describe a woman undergoing treatment with ipilimumab for metastatic melanoma who developed classic cutaneous findings of dermatomyositis along with proximal muscle weakness and elevated muscle enzymes.This case adds to the expanding literature regarding immune-related adverse events associated with ipilimumab. To our knowledge, drug-induced dermatomyositis from ipilimumab has not previously been reported. Physicians should be aware of these potential immune-related adverse events and consider drug-associated dermatomyositis in the differential diagnosis in patients receiving ipilimumab who present with a cutaneous eruption or muscle weakness.
- Nailfold capillaroscopic changes in dermatomyositis and polymyositis. [JOURNAL ARTICLE]
- Clin Rheumatol 2014 Oct 17.
Inflammatory myopathies (IM) are a group of muscle diseases occurring both in children and adults. Nailfold videocapillaroscopy (NVC) alterations are described in IM, but available data are discordant, including differences between polymyositis (PM) and dermatomyositis (DM). The aim of this study was to describe the capillaroscopic differences between PM and DM patients and possible correlation with clinical and serological features. We analyzed 52 unselected patients with IM in a cross-sectional study in a 6-month period. NVC findings of 29 DM and 23 PM patients were compared with those of 52 patients with primary Raynaud's phenomenon. Tortuosities, capillary loss, enlarged and giant capillaries, microhemorrhages, and ramified capillaries were scored by a semiquantitative rating; disorganization of the vascular array, avascular areas, and scleroderma pattern were scored as presence/absence. Sex, mean age, and mean disease duration were similar in both groups. Disorganization of the vascular array, enlarged and giant capillaries, capillary loss, and scleroderma-like pattern were observed almost only in IM patients. Significant differences were observed between PM and DM with higher frequency and mean score of NVC changes in DM. In DM patients with disease duration ≤6 months (14/29 patients), capillary density was significantly reduced (P = 0.039) and giant capillaries more frequent (P = 0.027), compared with patients with longer disease duration, while a scleroderma pattern tended to be more frequent in patients with a disease duration of less than 6 months. On the contrary, no differences were observed for ramified capillaries with regard to disease duration. Capillaroscopic alterations are identified only in DM patients as expression of diffuse microangiopathy; surprisingly, more severe changes were associated with shorter disease duration, while persistence of ramified capillaries with long-standing disease.
- A crucial role of anti-RPLP0 and anti-galectin-3 antibodies in the development of skin lesions in systemic lupus erythematosus. [JOURNAL ARTICLE]
- Arthritis Rheumatol 2014 Oct 10.
Objective: The specific autoantibodies and antigens which mediate systemic lupus erythematosus (SLE)-related organ injuries remain largely unknown. This study aimed to investigate the antibody-mediated immune response that leads to SLE skin lesions. Methods: The study involved 85 SLE patients with specific lupus skin lesions and 31 without skin lesions. The reactivity of serum antibody to skin antigens was determined by immunoblot using human foreskin as the substrate. Skin antigens were identified using mass spectrometry. Serum antibody was isolated by affinity purification and was injected intracutaneously into mouse skin to determine pathogenicity. The antibody serum levels were monitored by enzyme-linked immunoabsorbent assay. Results: We determined that 78% of patients with skin lesions presented serum antibodies reactive to 35kD and/or 25kD skin antigens, which was significantly higher than patients without skin lesions (p < 0.0001), suggesting a correlation between immune response and skin lesions. Acidic ribosomal P protein 0 (RPLP0) and galectin-3 were two target antigens identified from 35kD and 25kD proteins, respectively. Purified serum anti-RPLP0 and anti-galectin-3 antibodies induced lupus-like histological changes after intracutaneous injection. Anti-RPLP0 and anti-galectin-3 antibodies were significantly higher in SLE patients than in healthy controls and decreased with skin recovery. Anti-galectin-3 antibody was also significantly higher in SLE patients than in patients with dermatomyositis and systemic scleroderma, and strongly related to lupus cutaneous vasculitis. Additionally, the two antibodies were positively correlated with leucopenia and C3 deficiency, and anti-RPLP0 antibody was also positively correlated with arthritis and SLE disease activity. Conclusion: The immune response mediated by serum anti-RPLP0 and anti-galectin-3 antibodies play a key role in the pathogenesis of SLE skin lesions. These findings provide new insights into the mechanism of SLE-related organ disorders. © 2014 American College of Rheumatology.