- Evaluation of the Reliability of the Cutaneous Dermatomyositis Disease Area and Severity Index (CDASI) and the Cutaneous Assessment Tool Binary Method (CAT-BM) in Juvenile Dermatomyositis Among Pediatric Dermatologists, Rheumatologists, and Neurologists. [Journal Article]
- BJBr J Dermatol 2017 Apr 19
- CONCLUSIONS: Our data confirm the reliability of the CDASI activity and damage scores and the CAT-BM activity scores when used by pediatric dermatologists and rheumatologists in assessing JDM. Significant variation existed in the pediatric neurologists' scores. This article is protected by copyright. All rights reserved.
- Detection of interferon alpha protein reveals differential levels and cellular sources in disease. [Journal Article]
- JEJ Exp Med 2017 Apr 18
- Type I interferons (IFNs) are essential mediators of antiviral responses. These cytokines have been implicated in the pathogenesis of autoimmunity, most notably systemic lupus erythematosus (SLE), di...
Type I interferons (IFNs) are essential mediators of antiviral responses. These cytokines have been implicated in the pathogenesis of autoimmunity, most notably systemic lupus erythematosus (SLE), diabetes mellitus, and dermatomyositis, as well as monogenic type I interferonopathies. Despite a fundamental role in health and disease, the direct quantification of type I IFNs has been challenging. Using single-molecule array (Simoa) digital ELISA technology, we recorded attomolar concentrations of IFNα in healthy donors, viral infection, and complex and monogenic interferonopathies. IFNα protein correlated well with functional activity and IFN-stimulated gene expression. High circulating IFNα levels were associated with increased clinical severity in SLE patients, and a study of the cellular source of IFNα protein indicated disease-specific mechanisms. Measurement of IFNα attomolar concentrations by digital ELISA will enhance our understanding of IFN biology and potentially improve the diagnosis and stratification of pathologies associated with IFN dysregulation.
- Original article: Varicella vaccination elicits a humoral and cellular response in children with rheumatic diseases using immune suppressive treatment. [Journal Article]
- VVaccine 2017 Apr 12
- CONCLUSIONS: The humoral response to VZV vaccination in patients with pediatric rheumatic diseases (PRD) is similar to that of HC. Generally, patients are able to mount a VZV-specific cellular response. This study has been registered in the Brazilian Clinical Trials Registry under number U1111-1189-9837.
- The epigenetic mechanism for discordance of autoimmunity in monozygotic twins. [Review]
- JAJ Autoimmun 2017 Apr 12
- Monozygotic twins share an identical DNA sequence but are not truly "identical". In fact, when it comes to health and disease, they may often display some level of phenotypic discordance. The cause o...
Monozygotic twins share an identical DNA sequence but are not truly "identical". In fact, when it comes to health and disease, they may often display some level of phenotypic discordance. The cause of this discordance is often unknown. Epigenetic modifications such as DNA methylation, histone modification, and microRNAs-mediated regulation regulate gene expression and are sensitive to external stimuli. These modifications may be seen to bridge the gap between genetics and the environment. Over the years, the importance of epigenetics as a primary mechanism for the role that the environment plays in defining phenotype has been increasingly appreciated. Mechanisms of epigenetics include DNA methylation, histone modifications and microRNAs. Discordance rates in monozygotic twins vary depending on the specific condition, from 11% in SLE to 64% in psoriasis and 77% in PBC. Other autoimmune diseases in which discordance is found among monozygotic twins has also been studied include type 1 diabetes, multiple sclerosis, rheumatoid arthritis, dermatomyositis and systemic sclerosis. In some cases, the differences in various epigenetic modifications is slight, even though the concordance rate is low, suggesting that epigenetics is not the only factor that needs to be considered. Nonetheless, the study of phenotypic discordance in monozygotic twins may shed light on the pathogenesis of autoimmune diseases and contribute to the development of new methodologies for the diagnosis and treatment of these diseases.
- Race, Income, and Disease Outcomes in Juvenile Dermatomyositis. [Journal Article]
- JPedJ Pediatr 2017; 184:38-44.e1
- CONCLUSIONS: Minority race and lower family income are associated with worse morbidity and outcomes in subjects with JDM. Calcinosis was more common in black subjects. Further studies are needed to examine these associations in more detail, to support efforts to address health disparities in subjects with JDM and improve disease outcomes.
- Accidental hydroxychloroquine overdose resulting in neurotoxic vestibulopathy. [Journal Article]
- BCBMJ Case Rep 2017 Apr 12; 2017
- Hydroxychloroquine is an oral antimalarial medication commonly used off-label for a variety of rheumatological conditions, including systemic lupus erythematosus, rheumatoid arthritis, Sjögren's synd...
Hydroxychloroquine is an oral antimalarial medication commonly used off-label for a variety of rheumatological conditions, including systemic lupus erythematosus, rheumatoid arthritis, Sjögren's syndrome and dermatomyositis. We present a case of a 64-year-old woman who presented with acute onset headache, bilateral tinnitus, and left-sided facial numbness and tingling in the setting of accidentally overdosing on hydroxychloroquine. By the next morning, the patient began to experience worsening in the tingling sensation and it eventually spread to her left arm, thigh and distal extremities. The patient also complained of new onset blurring of her peripheral vision and feeling 'off balance.' Despite a complete neurological and ophthalmological work-up with unremarkable imaging and blood work, the patient has had no improvement in her tinnitus, left-sided paresthesias, visual disturbance or ataxia. This is a unique case of hydroxychloroquine overdose resulting in permanent neurotoxic vestibulopathy.
- Coexisting Juvenile Dermatomyositis and Sickle Cell Disease: Maintaining a High Degree of Suspicion. [Journal Article]
- JPedJ Pediatr 2017 Apr 07
- Juvenile dermatomyositis is an idiopathic inflammatory myopathy of childhood not previously described in a patient with sickle cell disease. We present a case of an 11-year-old girl with sickle cell ...
Juvenile dermatomyositis is an idiopathic inflammatory myopathy of childhood not previously described in a patient with sickle cell disease. We present a case of an 11-year-old girl with sickle cell disease who was diagnosed subsequently with juvenile dermatomyositis, and highlight the diagnostic and therapeutic challenges of these concurrent chronic diseases.
- "Hiker's feet": a novel cutaneous finding in the inflammatory myopathies. [Review]
- CRClin Rheumatol 2017 Apr 07
- Mechanic's hands is a well-characterized manifestation of select idiopathic inflammatory myopathy (IIM) syndromes. Less well characterized is the hyperkeratosis of the toes and plantar surface of the...
Mechanic's hands is a well-characterized manifestation of select idiopathic inflammatory myopathy (IIM) syndromes. Less well characterized is the hyperkeratosis of the toes and plantar surface of the feet that can also accompany these disorders. We aim to describe common pedal signs in the context of IIM, and suggest that it may be another key feature in the presentation of these syndromes. A cohort of 2145 myositis patient charts gathered since 2003 were retrospectively reviewed using the key search terms "mechanic's feet" and/or "mechanic's foot." Charts that included either phrase were further reviewed for clinical characteristics. Nine patients were identified with documentation describing "mechanic's feet" or "mechanic's foot." All nine affected individuals carried a diagnosis of DM, seven of whom also met criteria for antisynthetase syndrome. In eight patients (89%), it presented in conjunction with mechanic's hands. Six (67%) presented with anti-Jo-1 antibodies, and three (33%) were seronegative. Although the term "mechanic's feet" has been used to describe this clinical finding in patients in our myositis cohort, we propose the term "hiker's feet," given that the presentation resembles a callousing pattern more typical of avid hikers or long-distance walkers. Prevalence data are not yet known but should be considered for further study. If the presenting signs of IIM are expanded to include hiker's feet, it could aid in not only diagnosis and management but also provide insights into the pathophysiology of these diseases.
- Pregnancy in polymyositis or dermatomyositis: retrospective results from a tertiary centre in China. [Journal Article]
- RRheumatology (Oxford) 2017 Apr 05
- CONCLUSIONS: PM/DM, especially those less well controlled, might contribute to an increased risk of complicated pregnancy.
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- The epidemiology of dermatomyositis in northwestern Thrace region in Turkey: epidemiology of dermatomyositis in Turkey. [Journal Article]
- RIRheumatol Int 2017 Apr 06
- Dermatomyositis (DM) is a rare disease that may affect the skeletal muscles and the skin. Literature data on its incidence and prevalence are limited. There are no data on its incidence or prevalence...
Dermatomyositis (DM) is a rare disease that may affect the skeletal muscles and the skin. Literature data on its incidence and prevalence are limited. There are no data on its incidence or prevalence in Turkey. Patients diagnosed with DM at the Trakya University Medical Faculty, Department of Rheumatology from November 2004 to November 2014 were reviewed retrospectively. Patients' clinical and demographic features, laboratory data, treatment modalities, follow-up durations, disease courses, outcomes, and complications were evaluated. Our study included 23 patients with DM; 14 were females and 9 were males (female/male: 1.55). Over the course of the study, the annual incidence of DM was 3.7 per million (95% CI 0-18.8) person years, and the overall prevalence was 32.2 per million (95% CI 18.1-46.3). Incidence in women was higher (4.6/1,000,000 person years) compared to men (2.9/1,000,000 person years). The frequencies of most common findings were as follows: heliotrope rash (82.6%), Gottron papules (87%), proximal myopathy (78.3%), and facial erythema (60.9%). In our hospital-based study, the frequency of DM was lower than those reported in North America; however, they were similar to European countries.