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Rheumatology AND Dermatomyositis [keywords]
- Redefining dermatomyositis: a description of new diagnostic criteria that differentiate pure dermatomyositis from overlap myositis with dermatomyositis features. [Journal Article]
- Medicine (Baltimore) 2014 Nov; 93(24):318-32.
Dermatomyositis (DM) is a major clinical subset of autoimmune myositis (AIM). The characteristic DM rash (Gottron papules, heliotrope rash) and perifascicular atrophy at skeletal muscle biopsy are regarded as specific features for this diagnosis. However, new concepts are challenging the current definition of DM. A modified Bohan and Peter classification of AIM was proposed in which the core concept was the inclusion of the diagnostic significance of overlap connective tissue disease features. In this clinical classification, a DM rash in association with myositis in the absence of overlap features indicates a diagnosis of pure DM. However, overlap features in association with myositis allow a diagnosis of overlap myositis (OM), irrespective of the presence or absence of the DM rash. Perifascicular atrophy may be present in both pure DM and OM. Recently, the presence of perifascicular atrophy in myositis without a DM rash was proposed as diagnostic of a novel entity, adermatopathic DM. We conducted the present study to evaluate these new concepts to further differentiate pure DM from OM.Using the modified Bohan and Peter classification, we performed a follow-up study of a longitudinal cohort of 100 consecutive adult French Canadian patients with AIM, including 44 patients with a DM phenotype, defined as a DM rash, and/or DM-type calcinosis, and/or the presence of perifascicular atrophy on muscle biopsy. A detailed evaluation was performed for overlap features, the extent and natural history of the DM rash, adermatopathic DM, DM-specific and overlap autoantibodies by protein A immunoprecipitation on coded serum samples, and associations with cancer and survival.Two distinct subsets were identified in patients with a DM phenotype: pure DM (n = 24) and OM with DM features, or OMDM (n = 20). In pure DM, the DM rash was a dominant finding. It was the first disease manifestation, was always present at the time of myositis diagnosis, and was associated with a high cutaneous score and chronicity. Concurrent heliotrope rash and Gottron papules (positive predictive value [PPV] 91%), as well as the V-sign and/or shawl sign (PPV 100%), were diagnostic of pure DM. Anti-Mi-2, anti-MJ, and anti-p155 autoantibodies were present in 50% of pure DM patients and were restricted to this subset (PPV 100%). Cancer was present in 21% of pure DM patients. The 15-year survival was excellent (92%).In contrast, in patients with OMDM, the first manifestation was proximal muscle weakness or other skeletal muscle-related complaints. The DM rash appeared at diagnosis or at follow-up, was associated with a low cutaneous extent score and was transient. Adermatopathic DM, which was absent in pure DM, was highly predictive (PPV 100%) of OMDM. Overlap autoantibodies (including anti-Jo-1, anti-PL-7, anti-PM-Scl, anti-U1RNP, and/or anti-U5-RNP) were found in 70% of OMDM patients. OMDM was not associated with cancer, but the 15-year survival was significantly decreased (65%).Perifascicular atrophy occurred as commonly in OMDM (n = 6/20, 30%) as in pure DM (n = 4/24, 17%) patients. These 6 OMDM patients had adermatopathic DM at myositis diagnosis, and only 1 of them developed a DM rash at follow-up, emphasizing the lack of specificity of perifascicular atrophy for pure DM.In conclusion, using the modified Bohan and Peter classification of AIM allowed identification of OMDM, a new clinical subset of OM. Furthermore, identification of OMDM allowed recognition of pure DM as a new entity that was distinct from OMDM or from OM without DM features. However, the absolute specificity of a DM rash and perifascicular muscle atrophy for the diagnosis of pure DM was lost. The distinctive clinical manifestations and autoantibody profiles presented are proposed as diagnostic criteria to differentiate pure DM from OMDM.
- Cricothyroid perichondritis in a patient with dermatomyositis. [Journal Article]
- Intern Med 2014; 53(24):2833.
- Diffuse cutaneous mucinosis in dermatomyositis: a case report and review of the literature. [Journal Article]
- Case Rep Dermatol Med 2014.:938414.
We present the case of a patient with dermatomyositis and diffuse cutaneous mucinosis and give an up-to-date detailed review of all the published cases in the English literature describing the demographics, clinical picture, pathology management, and outcomes of this unique group of patients.
- Two simultaneous autoimmune processes in a patient presenting with respiratory insufficiency. [Journal Article]
- Respirol Case Rep 2014 Mar; 2(1):21-3.
The idiopathic inflammatory myopathies, including dermatomyositis, are uncommon acquired autoimmune diseases, sometimes associated with interstitial lung disease. Myasthenia gravis, a separate autoimmune disorder involving the neuromuscular junction, has some overlapping clinical features but has only rarely been reported to occur simultaneously within the same patient. Here we present the first reported case of concomitant dermatomyositis, myasthenia gravis, and interstitial lung disease.
- Benign pneumatosis intestinalis in a pediatric patient with multiple risk factors including granulomatosis with polyangiitis: A case report and review of the literature. [JOURNAL ARTICLE]
- Semin Arthritis Rheum 2014 Oct 19.
To describe the first reported case of pneumatosis intestinalis (PI) in a pediatric patient with granulomatosis with polyangiitis (GPA) and multiple other risk factors and review the literature for PI in adult and pediatric rheumatologic conditions.A PubMed search was completed using the search phrase "pneumatosis intestinalis." Searches were limited to humans and the English language, and remaining articles involving patients with rheumatologic diagnoses were identified and included in our discussion.This is the first reported of case of PI in a patient with underlying GPA or antineutrophil cytoplasmic antibody-associated vasculitides. Out of 90 previously reported cases of PI in patients with rheumatologic conditions, 79 cases were in adults and 11 in children. There were 30 patients with systemic sclerosis, 18 with MCTD/overlap syndrome, 18 with dermatomyositis or polymyositis, 16 with SLE, and 8 with other diagnoses. Overall, 81% of the patients were on corticosteroids or other immunosuppressants prior to development of PI. The most common presenting symptom was abdominal pain, and 51% of patients had associated pneumoperitoneum.PI can be associated with a broad spectrum of rheumatic diseases, including GPA, and should be included in the differential diagnosis of patients with rheumatologic conditions and nonspecific gastrointestinal symptoms.
- Proteomic profiling of antigens in circulating immune complexes associated with each of seven autoimmune diseases. [JOURNAL ARTICLE]
- Clin Biochem 2014 Nov 29.
Immune complexes (ICs) trigger humoral immune responses. Therefore, the identification of constituent antigens within ICs would have very different clinical significance than identification of free antigens.Here, we applied immune complexome analysis of serum to the study of seven major autoimmune diseases-anti-neutrophil cytoplasmic antibody-associated vasculitis, Takayasu's arteritis, mixed connective tissue disease, dermatomyositis, Sjögren's syndrome, systemic scleroderma, and systemic lupus erythematosus-and healthy donors to comprehensively identify antigens incorporated into circulating ICs and to find disease-specific antigens.We identified 468 distinct IC-associated antigens using this method. Importantly, 62 of those antigens were disease-specific antigens, and there were at least three disease-specific antigens for each of the seven autoimmune diseases. Of the disease-specific antigens identified, coiled-coil domain-containing protein 158 and spectrin were identified as potential autoantigens important to SSc and SS pathogenesis, respectively; notable titin and spectrin autoantibodies are reportedly found in SSc and SS patients, respectively.Immune complexome analysis may be generally applicable to the study of the relationship between ICs and autoimmune diseases in animals and humans.
- Severe calcinosis cutis with cutaneous ulceration in juvenile dermatomyositis. [Journal Article]
- Indian Pediatr 2014 Nov 8; 51(11):925-7.
Calcinosis cutis is usually seen in long standing and untreated cases of juvenile dermatomyositis.7-year-old girl with severe calcinosis cutis who developed cutaneous ulceration, rash and myopathy.Myopathic changes in EMG, muscle edema in MRI, elevated muscle enzymes and Jo-1 positive antibodies.Treatment with prednisolone and methotrexate resulted in improvement of the lesion.Calcinosis cutis may be a presenting feature of juvenile dermatomyositis even in the absence of characteristic findings of rash and weakness.
- A case of sarcoidosis with interstitial lung disease mimicking clinically amyopathic dermatomyositis and rapidly progressive interstitial lung disease. [Journal Article]
- Case Rep Rheumatol 2014.:195617.
Here, we report a patient with sarcoidosis who developed edematous erythema and interstitial lung disease. At the initial visit, clinically amyopathic dermatomyositis (CADM) with rapidly progressive interstitial lung disease (RP-ILD) was suspected because he had progressive dyspnea but no muscle weakness. The presence of anti-CADM-140/MDA5 autoantibodies was immediately assessed to facilitate a precise diagnosis, with negative results. Thereafter, skin and transbronchial lung biopsies revealed noncaseating granuloma with Langhans giant cells in both specimens, leading to a diagnosis of sarcoidosis. In this case, clinical features of skin and lung were unable to distinguish DM (including CADM) from sarcoidosis, but the lack of anti-CADM-140/MDA5 antibody was useful for differentiating CADM with RP-ILD mimicking sarcoidosis from bona fide sarcoidosis.
- Traditional cardiovascular risk factors and coronary artery calcification in adults with polymyositis and dermatomyositis - a Danish multicentre study. [JOURNAL ARTICLE]
- Arthritis Care Res (Hoboken) 2014 Nov 21.
Objectives To determine the occurrence of traditional cardiovascular (CV) risk factors and coronary artery calcification (CAC) in adults with polymyositis (PM) or dermatomyositis (DM) compared to healthy controls and to assess the association between CV risk factors, PM/DM and CAC score. Methods In a cross-sectional, observational study of 76 patients with PM/DM and of 48 gender- and age- matched healthy controls traditional CV risk factors were assessed. CAC was quantified by means of cardiac CT scan and expressed in Agatston units (U). The associations between CV risk factors, PM/DM and CAC were studied by multivariate analyses. Results Thirty-three percent of the patients were obese compared to 11% of the controls (P = 0.005). Hypertension and diabetes were more frequent in patients (71 % vs. 42%, P = 0.002 and 13% vs. 0%, P = 0.007) and patients had higher levels of triglycerides (P = 0.0009). High CAC score occurred more frequently in patients (20% vs. 4%, P = 0.04). In multivariate analysis of patients factors associated with CAC were age (P = 0.02) and smoking (P = 0.02). Conclusion In this study, traditional CV risk factors and severe CAC were commonly found in patients with PM/DM. However, severe CAC was not associated with PM/DM per se, but rather with age and smoking in these patients. This article is protected by copyright. All rights reserved.
- Correlation between serum levels of IL-15 and IL-17 in patients with idiopathic inflammatory myopathies. [JOURNAL ARTICLE]
- Scand J Rheumatol 2014 Nov 7.:1-5.