Database : MEDLINE
Search on : Dermatomyositis [Words]
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[PMID]: 29524269
[Au] Autor:Santos Briz A; Calle A; Linos K; Semans B; Carlson A; Sangüeza OP; Metze D; Cerroni L; Díaz-Recuero JL; Alegría-Landa V; Mascaró JM; Moreno C; Rodríguez-Peralto JL; Requena L
[Ad] Address:Department of Pathology, Hospital Clínico Universitario, Salamanca, Spain.
[Ti] Title:Dermatomyositis panniculitis: A clinico-pathologic and immunohistochemical study of 18 cases.
[So] Source:J Eur Acad Dermatol Venereol;, 2018 Mar 10.
[Is] ISSN:1468-3083
[Cp] Country of publication:England
[La] Language:eng
[Ab] Abstract:BACKGROUND: Panniculitis occurring in dermatomyositis is uncommon, with only a few cases described in the literature, most of them as case reports. OBJECTIVE: This report describes the clinico-pathological and immunohistochemical findings in a series of 18 patients with panniculitis associated to dermatomyositis. METHODS: In each patient we collected the clinical data of the cutaneous lesions as well as the characteristic clinical and laboratory findings. A series of histopathologic findings was recorded in the biopsy of each patient. A panel of antibodies was used in some cases to investigate the immunophenotype of the infiltrate. Data of treatment and follow-up were also collected. RESULTS: Of the 18 patients, 13 were female and 5 were male, ranging in age from 13 to 74 years (median, 46,4 years). In addition to panniculitis, all patients presented pathognomonic cutaneous findings of DM and reported proximal muscle weakness prior to the diagnosis of panniculitis. Muscle biopsy was performed in 17 patients and MRI in one, all with the diagnosis of inflammatory myopathy. None of the patients presented any associated neoplasia. Panniculitis lesions were located in the upper or lower limbs. Histopathology showed a mostly lobular panniculitis with lymphocytes as the main component of the infiltrate. Most cases showed also numerous plasma cells and lymphocytes surrounding necrotic adipocytes (rimming) were frequently seen. Lymphocytic vasculitis and abundant mucin interstitially deposited between collagen bundles of the dermis were also frequent findings. Late stage lesions showed hyaline necrosis of the fat lobule and calcification. Immunohistochemistry demonstrated that most lymphocytes of the infiltrate were T-helper lymphocytes, with some B-lymphocytes in the lymphoid aggregates and small clusters of CD-123 positive plasmacytoid dendritic cells in the involved fat lobule. CONCLUSION: Panniculitis in dermatomyositis is rare. Histopathologic findings of panniculitis dermatomysositis are identical to those of lupus panniculitis. Therefore, the final diagnosis requires clinic-pathologic correlation. This article is protected by copyright. All rights reserved.
[Pt] Publication type:JOURNAL ARTICLE
[Em] Entry month:1803
[Cu] Class update date: 180310
[Lr] Last revision date:180310
[St] Status:Publisher
[do] DOI:10.1111/jdv.14932

  2 / 8752 MEDLINE  
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[PMID]: 29457995
[Au] Autor:Anelli CG; Len CA; Terreri MTRA; Russo GCS; Reiff AO
[Ad] Address:Universidade Federal de São Paulo (UNIFESP), Escola Paulista de Medicina, Departamento de Pediatria, Disciplina de Alergia, Imunologia Clínica e Reumatologia, Setor de Reumatologia, São Paulo, SP, Brazil.
[Ti] Title:Translation and validation of the Transition Readiness Assessment Questionnaire (TRAQ).
[So] Source:J Pediatr (Rio J);, 2018 Feb 16.
[Is] ISSN:1678-4782
[Cp] Country of publication:Brazil
[La] Language:eng
[Ab] Abstract:OBJECTIVE: To translate and validate the Brazilian Portuguese version of the Transition Readiness Assessment Questionnaire in a population of adolescents and young adults with chronic rheumatologic disorders. This questionnaire evaluates the patient's readiness for making the transition from the pediatric health service to adult care. METHODS: The four-phase methodology for the translation and validation of generic questionnaires was followed, including translation, back-translation, pilot testing and clinical validation of the final tool. The confirmatory factor analysis was used for clinical validation and the Cronbach's alpha coefficient was used to assess the overall internal consistency of the final tool. RESULTS: A total of 150 patients with a mean age of 17.0 years (SD=2.2 years, range 14-21 years) were enrolled for the final tool validation. Of those, 71 patients had juvenile systemic lupus erythematosus (47.3%), 64 had juvenile idiopathic arthritis (42.7%), and 15 had juvenile dermatomyositis (10%). During the confirmatory factor analysis, the dimension "Talking with providers" consisting of two questions, was considered as not fitting the translated questionnaire due to a very high ceiling effect and was therefore excluded. All other translated items favorably contributed to the overall consistency of the final tool; removing that dimension did not result in a substantial increase in Cronbach's alpha, which was 0.776. CONCLUSIONS: The Brazilian Portuguese version of the Transition Readiness Assessment Questionnaire was validated in a population of transitional patients with chronic rheumatologic disorders, after one dimension from the original questionnaire was excluded. It is a non-specific disease questionnaire; thus, it can be used to evaluate the transition readiness of Brazilian patients with other chronic diseases.
[Pt] Publication type:JOURNAL ARTICLE
[Em] Entry month:1802
[Cu] Class update date: 180310
[Lr] Last revision date:180310
[St] Status:Publisher

  3 / 8752 MEDLINE  
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[PMID]: 29522204
[Au] Autor:Hou Y; Luo YB; Dai T; Shao K; Li W; Zhao Y; Lu JQ; Yan C
[Ad] Address:Research Institute of Neuromuscular and Neurodegenerative Diseases and Department of Neurology, Qilu Hospital, Shandong University, Jinan, Shandong, China.
[Ti] Title:Revisiting Pathological Classification Criteria for Adult Idiopathic Inflammatory Myopathies: In-Depth Analysis of Muscle Biopsies and Correlation Between Pathological Diagnosis and Clinical Manifestations.
[So] Source:J Neuropathol Exp Neurol;, 2018 Mar 07.
[Is] ISSN:1554-6578
[Cp] Country of publication:England
[La] Language:eng
[Ab] Abstract:The European Neuromuscular Centre (ENMC) pathological classification criteria of idiopathic inflammatory myopathies (IIMs) are debatable. The aim of this study was to explore their practicability and reproducibility. We conducted a retrospective analysis of 57 cases of IIMs excluding dermatomyositis (DM) and sporadic inclusion body myositis (sIBM) by in-depth analysis of muscle biopsies and comparisons of the clinical characteristics among polymyositis (PM), non-specific myositis (NSM) and necrotizing autoimmune myopathy (NAM). In 57 non-DM/sIBM-IIM cases, 25 were classified as PM, 15 as NSM, and 17 as NAM. Among them, 51 underwent multilevel sectioning examination of biopsies, with pathological changes at different levels warranting diagnostic rectification in 11 patients (21.57%): 4 PM were reclassified as NSM, and 7 NSM as NAM. Applying atypical CD8+ T cells surrounding non-necrotic muscle fibers resulted in diagnostic rectification from NSM to PM in 2 patients; using 20 T cells (instead of 10) as the threshold for the perivascular infiltration led to diagnostic rectification from NSM to NAM in 9 patients. There were no differences in disease duration or treatment outcomes among the subgroups. The strict pathological criteria to distinguish non-DM/sIBM-IIMs are of limited practicability and reproducibility, and may be of limited clinical significance.
[Pt] Publication type:JOURNAL ARTICLE
[Em] Entry month:1803
[Cu] Class update date: 180309
[Lr] Last revision date:180309
[St] Status:Publisher
[do] DOI:10.1093/jnen/nly017

  4 / 8752 MEDLINE  
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[PMID]: 29521630
[Au] Autor:Adachi R; Muro Y; Kono M; Akiyama M
[Ad] Address:Department of Dermatology, Nagoya University Graduate School of Medicine, Nagoya, 466-8550, Japan.
[Ti] Title:Intramuscular haemorrhage in a patient with dermatomyositis and anti-TIF1γ antibodies.
[So] Source:Eur J Dermatol;28(1):116-118, 2018 Feb 01.
[Is] ISSN:1952-4013
[Cp] Country of publication:France
[La] Language:eng
[Pt] Publication type:JOURNAL ARTICLE
[Em] Entry month:1803
[Cu] Class update date: 180309
[Lr] Last revision date:180309
[St] Status:In-Data-Review
[do] DOI:10.1684/ejd.2017.3168

  5 / 8752 MEDLINE  
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[PMID]: 29516280
[Au] Autor:Gupta L; Chaurasia S; Srivastava P; Dwivedi S; Lawrence A; Misra R
[Ad] Address:Department of Clinical Immunology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, 226014, India.
[Ti] Title:Serum BAFF in Indian patients with IIM: a retrospective study reveals novel clinico-phenotypic associations in children and adults.
[So] Source:Clin Rheumatol;, 2018 Mar 07.
[Is] ISSN:1434-9949
[Cp] Country of publication:Germany
[La] Language:eng
[Ab] Abstract:We studied the serum levels of B cell survival factors BAFF and APRIL in patients with idiopathic inflammatory myositis (IIM) and their relation with clinical and autoantibodies. Seventy-five patients (51 females and 24 males) with IIM (Bohan and Peter's criteria 1975) and 25 healthy adults were analyzed for BAFF, APRIL and IL-17 by ELISA, and myositis-specific and associated antibodies (MSA and MAA) using line immunoblot assay. Of the 75 patients, 59 were adults, 42 had Dermatomyositis (DM), and 17 had Polymyositis. Median disease duration was 5 (3-12) months. BAFF levels were higher in IIM than healthy controls [p = 0.001], and in children with jDM than adults [p = 0.026]. BAFF levels were higher in adults with arthritis [p = 0.018], weight loss [p = 0.007], and PAH [p = 0.004]. Among the various MSAs, lowest levels were seen in those with anti-SRP [p = 0.043]. Median follow-up duration was 145 patient years. Twelve patients relapsed, while nine were in drug-free remission. BAFF were similar between these groups. Serum APRIL levels were elevated in limited number of patients with myositis, and the levels did not differ amongst the clinico-serologic phenotypes. IL-17 levels were higher in individuals positive for anti-SRP [p = 0.028]. Serum BAFF levels are elevated in IIM, more so in children. BAFF levels may be useful as biomarker for PAH and arthritis. Anti-SRP positivity is associated with elevated IL-17 levels suggesting role in pathogenesis.
[Pt] Publication type:JOURNAL ARTICLE
[Em] Entry month:1803
[Cu] Class update date: 180308
[Lr] Last revision date:180308
[St] Status:Publisher
[do] DOI:10.1007/s10067-018-4046-x

  6 / 8752 MEDLINE  
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[PMID]: 29447389
[Au] Autor:Bernstein EJ; Bathon JM; Lederer DJ
[Ad] Address:Department of Medicine, Columbia University Medical Center, New York, NY.
[Ti] Title:Survival of adults with systemic autoimmune rheumatic diseases and pulmonary arterial hypertension after lung transplantation.
[So] Source:Rheumatology (Oxford);, 2018 Feb 13.
[Is] ISSN:1462-0332
[Cp] Country of publication:England
[La] Language:eng
[Ab] Abstract:Objectives: Pulmonary arterial hypertension (PAH) is a major cause of morbidity and mortality in adults with systemic autoimmune rheumatic diseases (ARDs). The aim of this study was to determine whether adults with ARDs and PAH on right-sided heart catheterization (ARD-PAH) have increased mortality following lung transplantation compared with those with PAH not due to an ARD. Methods: We conducted a retrospective cohort study of 93 adults with ARD-PAH and 222 adults with PAH who underwent lung transplantation in the USA between 4 May 2005 and 9 March 2015 using data from the United Network for Organ Sharing. We examined associations between diagnosis and survival after lung transplantation using stratified Cox models adjusted for potential confounding recipient factors. Results: Among adults undergoing lung transplantation in the USA, we did not detect a difference in the multivariable-adjusted mortality rate between those with ARD-PAH and those with PAH [hazard ratio 0.75 (95% CI 0.47, 1.19)]. Conclusion: The presence of an ARD was not associated with increased mortality after lung transplantation in adults with PAH.
[Pt] Publication type:JOURNAL ARTICLE
[Em] Entry month:1802
[Cu] Class update date: 180308
[Lr] Last revision date:180308
[St] Status:Publisher
[do] DOI:10.1093/rheumatology/kex527

  7 / 8752 MEDLINE  
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[PMID]: 29373707
[Au] Autor:Silverberg JI; Kwa L; Kwa MC; Laumann AE; Ardalan K
[Ad] Address:Departments of Dermatology, Preventive Medicine and Medical Social Sciences, Northwestern University Feinberg School of Medicine, Chicago, IL, USA.
[Ti] Title:Cardiovascular and cerebrovascular comorbidities of juvenile dermatomyositis in US children: an analysis of the National Inpatient Sample.
[So] Source:Rheumatology (Oxford);, 2018 Jan 24.
[Is] ISSN:1462-0332
[Cp] Country of publication:England
[La] Language:eng
[Ab] Abstract:Objective: JDM is associated with multiple potential risk factors for cardiovascular disease, including reduced heart rate variability, systolic/diastolic cardiac dysfunction, abnormal brachial artery reactivity and metabolic syndrome. However, little is known about cardiovascular risk in JDM. We sought to examine the association between JDM and cardiovascular risk factors and disease in US children. Methods: Data from the 2002-12 National Inpatient Sample was analysed, including ∼20% of all US hospitalizations (n = 14 535 620 paediatric hospitalizations). Results: JDM was significantly associated with 12 of 13 comorbidities, including hypertension [survey logistic regression; crude odds ratio (95% CI): 22.25 (15.51, 31.92)], obesity [5.87 (3.44, 10.02)], uncomplicated diabetes [7.95 (4.21, 15.00)], lipid abnormalities [5.84 (2.77, 12.31)], particularly lipodystrophy [151.08 (38.24, 596.86)], peripheral and visceral atherosclerosis [10.09 (3.70, 27.56)], late effects of cerebrovascular disease [15.49 (2.37, 101.43)], personal history of transient ischaemic attack and cerebral infarction [10.82 (2.46, 47.65)], pulmonary circulatory disorder [12.23 (2.59, 57.73)], arrhythmia [3.93 (2.80, 5.52)], bradycardia [4.22 (2.65, 6.74)] and hypotension [2.62 (1.27, 5.39)]. Conclusions: There are significantly higher odds of cardiovascular and cerebrovascular comorbidities among inpatients with JDM, with adolescents, girls and racial/ethnic minorities being at highest risk.
[Pt] Publication type:JOURNAL ARTICLE
[Em] Entry month:1801
[Cu] Class update date: 180308
[Lr] Last revision date:180308
[St] Status:Publisher
[do] DOI:10.1093/rheumatology/kex465

  8 / 8752 MEDLINE  
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[PMID]: 29361142
[Au] Autor:Karasawa R; Tamaki M; Sato T; Tanaka M; Nawa M; Yudoh K; Jarvis JN
[Ad] Address:Department of Frontier Medicine, St Marianna University School of Medicine, Kawasaki, Japan.
[Ti] Title:Multiple target autoantigens on endothelial cells identified in juvenile dermatomyositis using proteomics.
[So] Source:Rheumatology (Oxford);, 2018 Jan 18.
[Is] ISSN:1462-0332
[Cp] Country of publication:England
[La] Language:eng
[Ab] Abstract:Objective: Although generally classified within the group of inflammatory myopathies, JDM displays many pathological features of vasculitis. Previous work has shown that AECA are abundant in other forms of vasculitis. We therefore investigated whether such antibodies might also be detected in JDM. Methods: We screened plasma from children with JDM for the presence of AECA by western blotting and 2D gel electrophoresis (2DE) using proteins extracted from human aortic endothelial cells as the substrate. We performed mass spectrometry to identify candidate antigens from 2DE gels and used ELISA to confirm the presence of specific antibodies. Results: We identified 22 candidate target autoantigens for AECA probed with JDM plasma. Interestingly, 17 of these 22 target antigens were proteins associated with antigen processing and protein trafficking. ELISA confirmed the presence of antibodies to heat shock cognate 71 kDa protein in JDM plasma, particularly in children with active, untreated disease. Conclusion: Children with JDM express antibodies to autoantigens in endothelial cells. The clinical and pathological significance of such autoantibodies require further investigation.
[Pt] Publication type:JOURNAL ARTICLE
[Em] Entry month:1801
[Cu] Class update date: 180308
[Lr] Last revision date:180308
[St] Status:Publisher
[do] DOI:10.1093/rheumatology/kex468

  9 / 8752 MEDLINE  
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[PMID]: 29512806
[Au] Autor:El Euch M; Bani W; Mahfoudhi M; Jaziri F; Ben Abdelghani K; Turki S; Ben Abdallah T
[Ti] Title:A severe anti MDA-5 antibodies associated amyopathic dermatomyositis.
[So] Source:Tunis Med;95(6):444-445, 2017 Jun.
[Is] ISSN:0041-4131
[Cp] Country of publication:Tunisia
[La] Language:eng
[Ab] Abstract:BACKGROUND: Lung damage during amyopathic dermatomyositis (ADM) associated with auto antibodies anti MDA-5 is serious. We report a rare observation of a severe ADM associated to anti MDA-5 antibodies complicated with diffuse interstitial lung disease (ILD) rapidly extensive and fatal. OBSERVATION: We report the observation of a Tunisian adult who was admitted for ADM. The anti MDA-5 antibodies were detected. He developed an ILD rapidly extensive to fibrosis. Corticosteroids were unefficient and he deceded of pneumomediastin and refractory respiratory distress. CONCLUSION: This observation illustrates the severity of ADM with anti MDA-5 antibodies. Every clinician should detect any ILD to avoid the progression to fibrosis.
[Pt] Publication type:JOURNAL ARTICLE
[Em] Entry month:1803
[Cu] Class update date: 180307
[Lr] Last revision date:180307
[St] Status:In-Data-Review

  10 / 8752 MEDLINE  
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[PMID]: 29510265
[Au] Autor:Uchida H; Ogawa Y; Tominaga T
[Ad] Address:Department of Neurosurgery, Tohoku University Graduate School of Medicine, Sendai, Miyagi, Japan; Department of Neurosurgery, Kohnan Hospital, Sendai, Miyagi, Japan.
[Ti] Title:Marked effectiveness of low-dose oral methotrexate for steroid-resistant idiopathic hypertrophic pachymeningitis: Case report.
[So] Source:Clin Neurol Neurosurg;168:30-33, 2018 Mar 03.
[Is] ISSN:1872-6968
[Cp] Country of publication:Netherlands
[La] Language:eng
[Ab] Abstract:Idiopathic hypertrophic pachymeningitis (HP) is a rare clinical entity characterized by thickening of the dura mater without obvious underlying disease. High-dose steroid therapy is considered to be the first line for idiopathic HP, but half of patients show resistance for steroid therapy and suffer progressive clinical course. We describe low-dose methotrexate (MTX) administration for recurrent and steroid-resistant idiopathic HP resulting in noticeable improvement without severe adverse effects. A 51-year-old Japanese woman with dermatomyositis first presented with right retro-orbital pain caused by dural thickening in the sella and upper clivus involving the right trigeminal nerve, which was diagnosed as idiopathic HP by transsphenoidal biopsy. High-dose methylprednisolone therapy led to remission, and she remained healthy with low-dose dexamethasone. Three years after the initial therapy she presented with right facial nerve and lower cranial nerve palsies caused by diffuse and significant dural thickening in the posterior cranial fossa. Second highdose methylprednisolone therapy was introduced, but the effect was transient and she suffered aspiration pneumonia. Low-dose oral MTX therapy was begun, and her symptoms were almost resolved and dural thickening was remarkably improved without severe adverse effects. Lowdose MTX may be a more appropriate choice for idiopathic HP than steroid administration. Randomized controlled clinical trials are now needed.
[Pt] Publication type:JOURNAL ARTICLE
[Em] Entry month:1803
[Cu] Class update date: 180306
[Lr] Last revision date:180306
[St] Status:Publisher


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