- Antimelanoma Differentiation-associated Gene 5 Antibody: Expanding the Clinical Spectrum in North American Patients with Dermatomyositis. [Journal Article]
- JRJ Rheumatol 2017 Jan 15
- CONCLUSIONS: Anti-MDA5 positivity had a similar frequency in US patients with CADM and DM and is associated with ILD, RPILD, cutaneous ulcers, digital tip ulceration, and poor survival.
- Immune-array Analysis in Sporadic Inclusion Body Myositis Reveals HLA-DRB1 Amino Acid Heterogeneity across the Myositis Spectrum. [Journal Article]
- ARArthritis Rheumatol 2017 Jan 13
- CONCLUSIONS: This is the largest most comprehensive genetic association study to date in IBM. The data confirm that HLA is the most strongly associated region and identifies novel amino acid associations that may explain the risk in this locus; these amino acid associations differentiate IBM from polymyositis and dermatomyositis, and may determine properties of the peptide-binding groove allowing it to preferentially bind auto-antigenic peptides. A novel suggestive association within the chr3(p21.21) locus suggests a role for CCR5. This article is protected by copyright. All rights reserved.
- Intravenous Immunoglobulin in Pediatric Rheumatology: When to Use It and What Is the Evidence. [Journal Article]
- PAPediatr Ann 2017 Jan 01; 46(1):e19-e24
- Intravenous immunoglobulin (IVIG) is given to children with a variety of rheumatologic illnesses. The mechanism of action by which it exerts therapeutic effects is not well understood and likely diff...
Intravenous immunoglobulin (IVIG) is given to children with a variety of rheumatologic illnesses. The mechanism of action by which it exerts therapeutic effects is not well understood and likely differs in the medical conditions for which it is given. IVIG is approved by the US Food and Drug Administration and is the standard of care for Kawasaki disease, but most IVIG use in pediatric rheumatology is "off-label. " The literature supports the use of IVIG for juvenile dermatomyositis, although it is unclear whether its use should be limited to those children with more severe or refractory disease. It appears efficacious in the treatment of autoimmune thrombocytopenia secondary to lupus, but its use may be limited by transient responses. Treatment of other categories of pediatric rheumatologic diseases, such as juvenile idiopathic arthritis and non-Kawasaki vasculitides, is not well-established in the literature. This review focuses on current use of IVIG in the treatment of pediatric rheumatologic disorders. [Pediatr Ann. 2017;46(1):e19-e24.].
- Anti-MDA5 Antibody Dermatomyositis Overlap with Systemic Lupus Erythematosus: A Case Report and Review of the Literature. [Journal Article]
- OROpen Rheumatol J 2016; 10:122-128
- CONCLUSIONS: DM is known to overlap with other autoimmune diseases, including SLE, and coexistence can lead to a wide variety of clinical presentations. SLE overlapping with anti-MDA5 positive DM may present with distinct clinical features.
- Childhood Arthritis and Rheumatology Research Alliance consensus clinical treatment plans for juvenile dermatomyositis with skin predominant disease. [Journal Article]
- PRPediatr Rheumatol Online J 2017 Jan 11; 15(1):1
- CONCLUSIONS: Three CTPs were developed for use in children with skin predominant JDM, which reflect typical treatment approaches. These are not considered to be specific recommendations or standard of care. Using the CARRA network and prospective data collection, we will be able to apply statistical methods in the future to allow comparisons of JDM patients following these consensus treatment plans.
- Use of intravenous immunoglobulin therapy for myositis: an audit in South Australian patients. [Journal Article]
- IMIntern Med J 2017; 47(1):112-115
- In South Australia, between 2000 and 2014, 57 patients with idiopathic inflammatory myositis (IIM) were treated with intravenous immunoglobulin (IVIg). We reviewed disease characteristics to determin...
In South Australia, between 2000 and 2014, 57 patients with idiopathic inflammatory myositis (IIM) were treated with intravenous immunoglobulin (IVIg). We reviewed disease characteristics to determine predictors of response to therapy and IVIg dosing and duration to identify opportunities to rationalise IVIg use. Patients with dermatomyositis/polymyositis had a response rate of 77% and were more likely than inclusion body myositis to respond to therapy. Consideration should be given to the use of the lowest possible dose of IVIg and to the undertaking of trials of cessation of IVIg in patients with stable IIM.
- Juvenile dermatomyositis: a tertiary center experience. [Journal Article]
- CRClin Rheumatol 2017 Jan 05
- Juvenile dermatomyositis (JDM) is a rare chronic inflammatory disease of unknown etiology and primarily involves muscle and skin. It is the most common idiopathic inflammatory myopathy of childhood. ...
Juvenile dermatomyositis (JDM) is a rare chronic inflammatory disease of unknown etiology and primarily involves muscle and skin. It is the most common idiopathic inflammatory myopathy of childhood. This study aimed to evaluate demographic and clinical features, laboratory data, treatment modalities, and outcome of patients with JDM at a referral pediatric rheumatology center in Turkey. We retrospectively reviewed medical records of patients diagnosed with JDM between the years 2003-2016 at the Pediatric Rheumatology Department Cerrahpasa Medical Faculty. A total of 50 patients (35 females), median age at the onset 6.1 ± 4.1 years, were identified. Mean follow-up period was 74.5 ± 49.7 months. Presenting clinical symptoms included heliotrope rash (100%), Gottron papule (96%), muscle weakness (90%), erythroderma (88%), and calcinosis (38%). All patients had elevated muscle enzymes at the disease onset. Sixty-eight percent of the patients had anti-nuclear antibody positivity. Electromyography on 27 patients and muscle biopsy on 14 patients were performed, and all of them showed signs of juvenile dermatomyositis. Early aggressive treatment with corticosteroids mostly in combination with methotrexate was used. Cyclosporine was added to 48% of the patients' treatment regimen in case of severe or refractory disease. All patients except two cases, who were referred to our clinic after long disease duration with widespread calcinosis, achieved remission. Early diagnosis and early initiation of intensive therapy are important in reducing JDM complications. International collaboration is needed in order to better understanding and management of the disease.
- Recurrence of juvenile dermatomyositis 8 years after remission. [Journal Article]
- JCJAAD Case Rep 2017; 3(1):29-32
- Serum level of DNase1l3 in patients with dermatomyositis/polymyositis, systemic lupus erythematosus and rheumatoid arthritis, and its association with disease activity. [Journal Article]
- CEClin Exp Med 2016 Dec 30
- DNase1l3 is an endonuclease to degrade the chromatin of apoptotic or necrotic cells. Serum DNase1l3 may fulfill the function of clearance of chromatin released into the circulation by dying cells, wh...
DNase1l3 is an endonuclease to degrade the chromatin of apoptotic or necrotic cells. Serum DNase1l3 may fulfill the function of clearance of chromatin released into the circulation by dying cells, which can trigger autoimmune responses. To date, it remains unclear whether serum DNase1l3 level associates with the pathogenesis of autoimmune diseases. Sixty-eight patients with dermatomyositis/polymyositis (DM/PM, n = 30), systemic lupus erythematosus (SLE, n = 20) and rheumatoid arthritis (RA, n = 18), as well as 26 healthy blood donors were enrolled in the present study. Serum levels of DNase1l3 were quantified by enzyme-linked immunosorbent assay. DNASE1L3 activity in serum was estimated by the capability of serum to digest nucleosomal DNA. Clinical, biochemical, serological and other markers of disease activity (CRP, ESR, C3, C4, anti-Jo-1 and anti-dsDNA, etc.) were measured by standard laboratory procedure. We found a decrease in DNase1l3 level in the DM/PM and SLE patients, resulting in the reduction in serum activity to digest nucleosome DNA. In contrast, the level and activity of DNase1l3 remained unchanged in the RA patients. The DNase1l3 level was relatively lower in the DM/PM patients with anti-Jo-1 antibody and interstitial lung disease, and in the SLE patients with SLE disease activity index higher than 6, renal involvement and anti-dsDNA antibody. DNase1l3 level negatively correlated with CRP and IgG in the PM/DM patients and correlated with ESR in the SLE patients. We found a significant reduction in serum DNase1l3 level in DM/PM and SLE, which may associate with clinic features and disease activity.
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- Sarcoplasmic MxA expression: A valuable marker of dermatomyositis. [Journal Article]
- NeurNeurology 2016 Dec 30
- CONCLUSIONS: Sarcoplasmic MxA expression detected by immunohistochemistry is a more sensitive marker of DM than the conventional hallmarks, indicating its practical utility in the diagnosis of DM. It may well be included in the routine immunohistochemistry panel for myositis.