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Rheumatology AND Dermatomyositis [keywords]
- Leishmaniasis and autoimmune diseases in pediatric age. [REVIEW]
- Cell Immunol 2014 Aug 27; 292(1-2):9-13.
Leishmaniasis is a group of diseases caused by the protozoa Leishmania, endemic in the Mediterranean countries. Clinical manifestations can be divided into three different forms: cutaneous leishmaniasis, mucosal leishmaniasis and the visceral leishmaniasis, the most severe form which is potentially lethal if untreated. Immunology and pathogenesis are complex: many different aspects of immune response, resistance and susceptibility to Leishmania have been studied but many others remain to be clarified. The gold standard in diagnosis of visceral Leishmaniasis is the presence of amastigotes in bone marrow or tissue sections. Patients can be initially misdiagnosed as having an autoimmune disease because it may mimic diseases like systemic lupus erythematosus, autoimmune hepatitis, dermatomyositis or others disorders. As in pediatric age the risk of life-threatening complications is very high, leishmaniasis, must be kept in mind to the clinician, in order to avoid wrong diagnosis and an inappropriate immunosuppressive therapy.
- Acute acalculous cholecystitis in a patient with juvenile dermatomyositis. [Journal Article]
- BMJ Case Rep 2014.
Juvenile dermatomyositis (JDM) is a rare autoimmune disease, characterised by a systemic capillary vasculopathy that typically affects skin and muscle. Gastrointestinal involvement is relatively rare. We report the case of an 11-year-old girl admitted for investigation of skin rash, progressive symmetric proximal muscle weakness, dysphagia and weight loss. The diagnosis of JDM was confirmed and during hospitalisation the patient developed abrupt and intense right hypocondrium pain associated with nausea and vomiting. Abdominal ultrasound revealed a thick gallbladder wall (8 mm) with pericholecystic fluid and no evidence of gallstones. An acute acalculous cholecystitis was assumed and the patient was started on intravenous fluids, prednisolone and analgaesic therapy. Clinical resolution was verified after 48 h. We hypothesised that the vasculitic process of JDM could have been the basis for this complication as described in other autoimmune diseases.
- Republished: New age of biological therapies in paediatric rheumatology. [REVIEW]
- Postgrad Med J 2014 Oct; 90(1068):590-596.
Many paediatric rheumatic diseases result from the abnormal activation or control of the immune system. Biologic drugs, which are synthesised within a biological system, have been designed to target specific molecules involved in cytokine signalling or cell-cell interactions. The past 15 years have seen a revolution in the range of effective treatments for rheumatic diseases, particularly juvenile idiopathic arthritis (JIA). As a result, the target of inactive disease and minimal long-term disease-associated damage is increasingly becoming achievable. In this article we review evidence from recent trials of the use of biologic drugs in the treatment of systemic JIA, juvenile dermatomyositis and juvenile systemic lupus erythematosus. We also highlight novel agents currently undergoing investigation which may broaden our therapeutic armamentarium over the coming decade. Key to these developments are well-designed multicentre controlled clinical trials and long-term safety monitoring as part of international drug registries.
- Biologics in polymyositis and dermatomyositis-associated interstitial lung disease. [Journal Article]
- Curr Pharm Biotechnol 2014; 15(6):521-4.
Interstitial lung disease (ILD) is one of the most common complications of polymyositis (PM) and dermatomyositis (DM). It is always progressive and does not respond to conventional immunosuppressive agent treatment. Biologics are commonly used in treatment of rheumatic diseases. They are also used in polymyositis and dermatomyositis associated interstitial lung disease. This review will focus on the updated use of biologics in PM/DM-ILD.
- Editorial: biologics in autoimmune diseases. [Journal Article]
- Curr Pharm Biotechnol 2014; 15(6):509.
Autoimmune diseases are a large group of diseases with diverse clinical manifestations. They occur due to uncontrolled abnormal immune responses to self tissue and organs. Biologic therapy as a new weapon in the war to against autoimmune diseases is rapidly expanding owning to their specificity, efficacy and safety profiles compared with the traditional non-biologic disease modifying anti-rheumatic drugs (DMARDs). The major targets of these biologic therapies include cytokines, immune cells and some co-stimulation molecules. Cytokines as the targets of biologic therapies are mainly the three classic inflammatory cytokines include tumor necrosis factor (TNF)-α, interleukin (IL)-6 and IL-1. Immune cells targets include B cells and T cells. Though these agents are useful in most patients, the adverse effects accompanied with biologic therapy such as infection and tumor incidence make it quite important to decide appropriately when and how to use these agents. Research in order to define more candidate targets of biologic therapy in autoimmune disease is ongoing. Based on this background, we assembled this special issue for a better understanding of the use of biologics in autoimmune diseases, on aspect of various biologics used in rheumatoid arthritis (RA), lupus, Primary Sjögren's Syndrome (pSS), autoimmune liver diseases, vasculities, Systemic Sclerosis and polymyositis and dermatomyositis-associated interstitial lung disease. In this special issue, Dashan Wang summarized the biologic agents currently available to treat RA, and the prospective biologic therapies that might be used in the management of RA the in future. Manisha Relan gave an update on the use of biologics in lupus, with a focus on Rituximab, Abatacept, Belimumab, and Epratuzumab. The use of biologics in psoriatic arthritis was summarized by Yan Li. Shiju Chen reviewed the biologics therapy in pSS, focused on the Rituximab in the treatment of pSS to explicate pathogenic function of B cells and assesse its efficacy in sicca symptoms, systemic manifestations and laboratory parameters in pSS patients. Mei Liu provided an overview of emerging biotherapy for autoimmune liver diseases, which gave us a new insight into the treatment strategy in autoimmune liver diseases. Sahana Vishwanath presented a review on biologic agents used in various vasculities. Jingxiu Xuan made a review on the use of biologics in treatment of Systemic Sclerosis. The use of biologics in polymyositis and dermatomyositis was discussed by Yuechi Sun, with a focus on the polymyositis and dermatomyositis- associated interstitial lung disease. This special issue covers many important aspects in the use of biologics in treatment autoimmune diseases, which will surely provide us a better understanding about the role of various biologics in treatment of autoimmune diseases.
- A case of multicentric reticulohistiocytosis. [JOURNAL ARTICLE]
- Mod Rheumatol 2014 Sep 11.:1-4.
Multicentric reticulohistiocytosis (MRH) is a rare non-Langerhans histiocytosis of unknown etiology with a predilection for joint and skin. The characteristic clinical features are papulonodular skin eruptions and inflammatory polyarthritis, sometimes progressive to arthritis mutilans, a severe destructive arthropathy. Although these manifestations can present at the same time, it is more common that one feature precedes the others. Notably, these features are similar to those found in some rheumatic diseases, such as rheumatoid arthritis or dermatomyositis, and this can lead to a misdiagnosis, especially during periods where only one feature is present. Herein, we report a female patient with polyarthralgia and subsequent skin eruptions, who was eventually diagnosed with MRH. Her symptoms seemed to resemble those of some rheumatic diseases, but several features such as affected joints and the characteristic shape of the skin lesions did not correspond to that. The histological result of infiltration of histiocytes and multinucleated giant cells in the skin ultimately facilitated the correct diagnosis. In this paper, we review MRH briefly and highlight several differential points which enable us to increase the likelihood of correctly diagnosing MRH.
- Therapy of myositis: biological and physical. [JOURNAL ARTICLE]
- Curr Opin Rheumatol 2014 Sep 5.
To give an update on reported use and effects of biological and physical therapies in patients with myositis.The most promising biological treatment in polymyositis, dermatomyositis and juvenile dermatomyositis is B-cell blockade by rituximab. Anti-Jo or anti-Mi-2 antibodies were predictors of response suggesting different molecular pathways in different subsets of myositis. T-cell blockade with abatacept is a new possibility, as is blockade of interleukin-1, interleukin-6 or type I interferon, but controlled studies are needed. Metabolic abnormalities may contribute to muscle impairment, lending support to combine pharmacological therapy with exercise in patients with polymyositis and dermatomyositis. Exercise improved the aerobic milieu in the muscle, along with improved aerobic capacity, and reduced disability. Support is also provided for the safety of exercise in patients with recent-onset polymyositis and dermatomyositis and exercise is well tolerated in patients with juvenile dermatomyositis.There is a strong need to develop new therapies in patients with myositis. To achieve this, more knowledge is needed on the molecular pathogenesis. Targeted therapies using biologics or exercise can be employed to achieve an improved understanding of molecular pathways, provided that clinical outcome measures are combined with molecular studies on muscle and blood.
- Risk of deep venous thrombosis and pulmonary embolism in individuals with polymyositis and dermatomyositis: a general population-based study. [JOURNAL ARTICLE]
- Ann Rheum Dis 2014 Sep 5.
Patients with polymyositis (PM) and dermatomyositis (DM) may have an increased risk of venous thromboembolism (VTE); however, no general population data are available to date. The purpose of this study was to estimate the future risk and time trends of new VTE (deep venous thrombosis (DVT) or pulmonary embolism (PE)) in individuals with incident PM/DM at the general population level.We assembled a retrospective cohort of all patients with incident PM/DM in British Columbia and a corresponding comparison cohort of up to 10 age-matched, sex-matched and entry-time-matched individuals from the general population. We calculated incidence rate ratios (IRR) for VTE, DVT and PE and stratified by disease duration. We calculated HRs adjusting for relevant confounders.Among 752 cases with inflammatory myopathies, 443 had PM (58% female, mean age 60 years) and 355 had DM (65% female, mean age 56 years); 46 subjects developed both diseases. The corresponding IRRs (95% CI) for VTE, DVT and PE in PM were 8.14 (4.62 to 13.99), 6.16 (2.50 to 13.92) and 9.42 (4.59 to 18.70), respectively. Overall, the highest IRRs for VTE, DVT and PE were observed in the first year after PM diagnosis (25.25, 9.19 and 38.74, respectively). Fully adjusted HRs for VTE, DVT and PE remained statistically significant (7.0 (3.34 to 14.64), 6.16 (2.07 to 18.35), 7.23 (2.86 to 18.29), respectively). Similar trends were seen in DM.These findings provide the first general population-based evidence that patients with PM/DM have an increased risk of VTE. Increased vigilance of this serious but preventable complication is recommended.
- Autoantibodies to DNA mismatch repair enzymes in polymyositis/dermatomyositis and other autoimmune diseases: A possible marker of favorable prognosis. [JOURNAL ARTICLE]
- Arthritis Rheumatol 2014 Sep 3.
Objective. Myositis-specific autoantibodies (MSAs) are useful tools for identifying clinical subsets of patients with idiopathic inflammatory myopathies (IIMs). There have been few reports on antibodies (Abs) to some DNA mismatch repair enzymes (MMREs) in patients with IMM. In this study, the frequencies and clinical associations of antibodies to 7 kinds of MMREs (MLH1, MLH3, MSH2, MSH3, MSH6, PMS1 and PMS2) were evaluated in patients with IIMs and other systemic autoimmune diseases. Methods. Clinical data and serum samples were collected from 239 Japanese patients with IIMs (147 with adult dermatomyositis, 13 with juvenile dermatomyositis, 57 with polymyositis and 22 with myositis overlap syndrome). One hundred patients with other diseases, including 40 patients with systemic lupus erythematosus (SLE), were assessed as disease controls. Serum was examined for anti-MMRE Abs by immunoprecipitation, ELISA and immunoprecipitation-Western blotting. Results. Anti-MMRE Abs were found in 15 patients with IIMs and 3 patients with SLE. They were restricted to MLH1, PMS1, MSH2 and PMS2, and were sometimes present in individual patients simultaneously. Although 9 IIM patients with anti-MMREs had other MSAs and their associated clinical features, all patients with anti-MMREs were alive during follow-up. Conclusion. Anti-MMRE Abs, which often coexist with other MSAs, may be serological markers of good prognosis in IIMs. © 2014 American College of Rheumatology.
- Expression of the Dermatomyositis Autoantigen TIF1γ in Regenerating Muscle. [JOURNAL ARTICLE]
- Arthritis Rheumatol 2014 Sep 3.
Objective. Autoantibodies against TIF1γ are found in many patients with dermatomyositis (DM). Although TIF1γ is known to play a role in the differentiation of other tissues, its functional role in muscle regeneration has not been elucidated. This study was undertaken to explore the regulation and functional role of this protein during muscle differentiation and regeneration. Methods. TIF1γ expression was analyzed in human muscle biopsy specimens using immunofluorescence microscopy. Immunofluorescence microscopy and immunoblotting analyses were used to study TIF1γ expression in a mouse model of muscle injury and repair. The effect of premature TIF1γ silencing on muscle differentiation was studied in cultured mouse myoblasts. Results. In muscle biopsy specimens from DM patients, TIF1γ was expressed at low levels in the nuclei of histologically normal muscle cells but at high levels in the centralized nuclei of atrophic, perifascicular myofibers expressing markers of regeneration. TIF1γ levels were also increased in regenerating myonuclei following muscle injury in mice. Premature silencing of TIF1γ in vitro using siRNA did not accelerate the expression of myogenin, a transcription factor that plays a central role in regulating relatively early stages of muscle differentiation. However, premature silencing of TIF1γ did accelerate myotube fusion and the expression of myosin heavy chain (MyHC), a later marker of muscle differentiation. Conclusion. The DM autoantigen TIF1γ is markedly upregulated during muscle regeneration in human and mouse muscle cells. Premature silencing of this protein in cultured myoblasts accelerates MyHC expression and myoblast fusion. However, TIF1γ may function independently of, or downstream from myogenin. © 2014 American College of Rheumatology.