Search results for "DERMATOMYOSITIS"
showing 10 items of 23 documents
Ultrastructural myopathology in the molecular era.
2013
Electron microscopy is an essential component of myopathology, both in diagnostics and research of neuromuscular diseases. Although recently reduced in the diagnostic armamentarium, it has greatly been expanded to mouse models in research. Mostly it is descriptive, but a few additional techniques in combination with transmission electron microscopy have been employed. Foremost among them is immunoelectron microscopy, which assists in guiding molecular analysis in hereditary conditions, but may be vital in diagnostics of certain acquired entities, e.g., undulating tubules in dermatomyositis and in those congenital myopathies where genes and mutations remain to be identified, as in cylindrica…
Review: Immune-mediated necrotizing myopathies - a heterogeneous group of diseases with specific myopathological features
2012
Immune-mediated necrotizing myopathies (IMNMs) are now well recognized among the so-called idiopathic inflammatory myopathies (IIMs), which also comprise dermatomyositis (DM), polymyositis (PM), sporadic inclusion body myositis (sIBM) and non-specific myositis. All of these conditions are defined on the basis of distinct clinical symptoms, in combination with results derived from muscle biopsy and additional data, such as measurement of the serum creatine kinase (CK) level as well as myositis-associated and myositis-specific autoantibodies, electromyography (EMG) and modern imaging techniques. Importantly, diagnosis of one of the above mentioned myositis forms implies a specific clinical sy…
Chapter 13 Antiphospholipid Antibodies and Vasculitis
2009
Abstract Besides the large group of primary vasculitis diseases, vasculitis in autoimmune diseases is most commonly associated with systemic lupus erythematosus (SLE), scleroderma, rheumatoid arthritis, insulin-dependent diabetes mellitus, and dermatomyositis. The antiphospholipid syndrome (APS), as a relatively recently recognized autoimmune disorder, may also present with a variety of vasculitis features. These non-typical vasculitis syndromes have to be considered very carefully in the APS, and vasculitis manifestations presenting as systemic involvement in APS patients have to be identified very early to prevent patients from rapidly developing organ damage. The aim of this review artic…
Value of whole-body magnetic resonance imaging for screening multifocal osteonecrosis in patients with polymyositis/dermatomyositis
2017
To assess the value of coronal short-tau inversion recovery whole-body MRI (STIR-WBMRI) for screening osteonecrosis in patients with polymyositis (PM)/dermatomyositis (DM).The imaging and medical records of 129 patients with PM/DM who met the Bohan and Peter diagnostic criteria were retrospectively analyzed. STIR-WBMRI was performed in all patients. 18 patients had follow-up STIR-WBMRI. 12 patients underwent regional knee and/or hip MRI while 25 patients underwent radiography of the lower extremities.STIR-WBMRI detected osteonecrosis in 15 (11.6%) patients. 38 joints were affected (mean, 2.5 per patient; range, 1-5 joints). Of the 38 joints affected by osteonecrosis, 33 had no clinical symp…
Monocyte/macrophage differentiation in dermatomyositis and polymyositis.
2004
Recent advances have revealed significant differences in the pathogenesis of inflammatory myopathies. To determine whether different patterns of macrophage differentiation are a useful tool to delineate the major groups of inflammatory myopathies, the muscle biopsies of 11 patients with dermatomyositis and 12 patients with polymyositis were studied using different macrophage markers. In polymyositis, the early-activation markers MRP14 and 27E10 stained the majority of macrophages, which were recognized by the pan-macrophage marker Ki-M1P and which were located primarily in the endomysium. In dermatomyositis, macrophages predominantly expressed the late-activation marker 25F9 and were found …
Immune-mediated necrotizing myopathy is characterized by a specific Th1-M1 polarized immune profile.
2012
Immune-mediated necrotizing myopathy (IMNM) is considered one of the idiopathic inflammatory myopathies, comprising dermatomyositis, polymyositis, and inclusion body myositis. The heterogeneous group of necrotizing myopathies shows a varying amount of necrotic muscle fibers, myophagocytosis, and a sparse inflammatory infiltrate. The underlying immune response in necrotizing myopathy has not yet been addressed in detail. Affected muscle tissue, obtained from 16 patients with IMNM, was analyzed compared with eight non-IMNM (nIMNM) tissues. Inflammatory cells were characterized by IHC, and immune mediators were assessed by quantitative real-time PCR. We demonstrate that immune- and non–immune-…
Nuclear actin aggregation is a hallmark of anti-synthetase syndrome-induced dysimmune myopathy
2015
Objective: To analyze antisynthetase syndrome–associated myositis by modern myopathologic methods and to define its place in the spectrum of idiopathic inflammatory myopathies (IIMs). Methods: Skeletal muscle biopsies from antisynthetase syndrome–associated myositis and other IIMs from different institutions worldwide were analyzed by histopathology, quantitative PCR, and electron microscopy. Results: Myonuclear actin filament inclusions were identified as a unique morphologic hallmark of antisynthetase syndrome–associated myositis. Nuclear actin inclusions were never found in dermatomyositis, polymyositis, sporadic inclusion body myositis, autoimmune necrotizing myopathy associated with si…
Enzymatic Determination in Serum of Lathyritic and Prednisone-treated Lathyritic Rats
1966
WHILE a remarkably raised serum-level of transaminases and aldolase is found in dermatomyositis, such augmentation, as a rule, is not found in cases of severe muscular involvement of scleroderma and disseminated lupus erythematosus (acutus)1–3.
Cell Death and Oxidative Damage in Inflammatory Myopathies
1998
There is evidence that muscle fibers in denervating disorders and muscular dystrophies undergo apoptosis. In 21 patients with autoimmune inflammatory myopathies, we found no features of muscle fiber apoptosis such as DNA fragmentation or expression of apoptosis-related proteins. However, muscle fibers in myositis displayed distinct up-regulation of inducible and neuronal nitric oxide synthase (NOS). While inducible NOS was distinctly up-regulated on the sarcolemma of all kinds of muscle fibers neuronal NOS displayed increased expression in the sarcoplasm of damaged as well as atrophic muscle fibers. There were no disease-specific patterns in the different myositis subtypes. Enhanced express…
Successful “Salvage” Therapy of Intravenous Cyclophosphamide for Refractory Polymyositis in an Elderly Patient: Case Report
2006
Dermatomyositis and polymyositis may affect children and adults and are now widely recognized as major causes of disability which, thanks to the introduction of immunosuppressive drugs, is often treatable, at least to some extent. Few data exist regarding polymyositis in elderly patients. We describe a case of refractory life-threatening polymyositis in an elderly patient, successfully treated with intravenous cyclophosphamide.