scholarly journals Újabb adatok a myositisspecifikus és -asszociált antitestekről juvenilis és felnőttkori myositisekben

2016 ◽  
Vol 157 (30) ◽  
pp. 1179-1184 ◽  
Author(s):  
Andrea Váncsa ◽  
Katalin Dankó
Keyword(s):  
Muscle Weakness ◽  
Immunosuppressive Treatment ◽  
Inflammatory Myopathy ◽  
Idiopathic Inflammatory Myopathy ◽  
General Term ◽  
Response To Treatment ◽  
Disease Course ◽  
Inflammatory Myopathies ◽  
Disease Response ◽  
Systemic Symptoms

Myositis, which means inflammation of the muscles, is a general term used for inflammatory myopathies. Myositis is a rare idiopathic autoimmune disease. It is believed that environmental factors such as virus, bacteria, parasites, direct injuries, drugs side effect can trigger the immune system of genetically susceptible individuals to act against muscle tissues. There are several types of myositis with the same systemic symptoms such as muscle weakness, fatigue, muscle pain and inflammation. These include dermatomyositis, juvenile dermatomyositis, inclusion-body myositis, polymyositis, orbital myositis and myositis ossificans. Juvenile and adult dermatomyositis are chronic, immune-mediated inflammatory myopathies characterized by progressive proximal muscle weakness and typical skin symptoms. The aim of the authors was to compare the symptoms, laboratory and serological findings and disease course in children and adult patients with idiopathic inflammatory myopathy. Early diagnosis and aggressive immunosuppressive treatment improve the mortality of these patients. Myositis-specific autoantibodies have predictive and prognostic values regarding the associated overlap disease, response to treatment and disease course. The authors intend to lighten the clinical and pathogenetic significance of the new target autoantigens. Orv. Hetil., 2016, 157(29), 1179–1184.

The association of colitis ulcerosa and coeliakia with idiopathic inflammatory myopathy

2014 ◽  
Vol 155 (26) ◽  
pp. 1033-1038 ◽  
Author(s):  
Levente Bodoki ◽  
Melinda Nagy-Vincze ◽  
Zoltán Griger ◽  
Andrea Péter ◽  
Katalin Dankó
Keyword(s):  
Ulcerative Colitis ◽  
Celiac Disease ◽  
General Population ◽  
Rare Case ◽  
Inflammatory Myopathy ◽  
Idiopathic Inflammatory Myopathy ◽  
Idiopathic Inflammatory Myopathies ◽  
International Literature ◽  
Inflammatory Myopathies ◽  
Immune Mediated

The authors discuss a rare case of a 25-year-old female patient having dermatomyositis associated with celiac disease and ulcerative colitis. The idiopathic inflammatory myopathies are systemic, chronic, immune-mediated diseases characterized by proximal, symmetrical muscle weakness. Many examples from the literature refer that celiac disease occurs more often in patients with myositis than in the general population, but its association with ulcerative colitis is a real rarity in the international literature. Orv. Hetil., 2014, 155(26), 1033–1038.

scholarly journals Multiple Skeletal Muscle Metastases from Colon Carcinoma Preceded by Paraneoplastic Dermatomyositis

2013 ◽  
Vol 2013 ◽  
pp. 1-4 ◽  
Author(s):  
Matteo Landriscina ◽  
Assunta Maria Teresa Gerardi ◽  
Alberto Fersini ◽  
Sergio Modoni ◽  
Luca Pio Stoppino ◽  
...  
Keyword(s):  
Skeletal Muscle ◽  
Skeletal Muscles ◽  
Inflammatory Myopathy ◽  
Idiopathic Inflammatory Myopathy ◽  
Skeletal Metastases ◽  
Metastatic Colon Cancer ◽  
Cutaneous Manifestations ◽  
Tumor Metastases ◽  
Systemic Symptoms ◽  
Skeletal Muscle Metastases

Skeletal muscle metastases are very rare events in colorectal carcinoma. By contrast, dermatomyositis is an idiopathic inflammatory myopathy with characteristic cutaneous manifestations and a well-recognized association with several human malignancies and, among others, colorectal cancer. Here, we report the case of a 71-year-old woman with paraneoplastic dermatomyositis followed by the development of a metastatic colon cancer. Interestingly, this patient developed multiple skeletal metastases which were preceded by the worsening of systemic symptoms of dermatomyositis. This observation suggests that, while muscle tissue is usually resistant to the development of tumor metastases, the inflammatory and immune response which characterizes and boosts paraneoplastic myopathy may represent a favorable soil for tumor cell invasion and metastasization to skeletal muscles.

scholarly journals Role of antifibrotics in the management of idiopathic inflammatory myopathy associated interstitial lung disease

2021 ◽  
Vol 13 ◽  
pp. 1759720X2110609
Author(s):  
Erin M. Wilfong ◽  
Rohit Aggarwal
Keyword(s):  
Interstitial Lung Disease ◽  
Lung Disease ◽  
Inflammatory Myopathy ◽  
Idiopathic Inflammatory Myopathy ◽  
The United States ◽  
Inflammatory Myopathies ◽  
Mortality And Morbidity ◽  
Randomized Controlled ◽  
Fda Approval

The antifibrotic therapies nintedanib and pirfenidone were first approved by the United States for the treatment of idiopathic pulmonary fibrosis in 2014. In 2020, nintedanib received U.S. Food and Drug Administration (FDA) approval for the treatment of all progressive fibrosing interstitial lung disease (ILD). Given that a major cause of mortality and morbidity in the idiopathic inflammatory myopathies (IIM) is progressive interstitial lung disease and respiratory failure, antifibrotic therapies may be useful as adjuvant to traditional immunosuppression. However, randomized controlled trials of antifibrotic therapies in IIM are lacking. The purpose of this review is to (1) summarize the mechanism of action of nintedanib and pirfenidone in ILD with possible role in IIM-ILD, (2) review the clinical data supporting their use in interstitial lung disease in general, and more specifically in connective tissue disease associated ILD, and (3) discuss the evidence and remaining challenges for using antifibrotic therapies in IIM-ILD.

scholarly journals Thrombospondin-1 mediates muscle damage in brachio-cervical inflammatory myopathy and systemic sclerosis

2020 ◽  
Vol 7 (3) ◽  
pp. e694 ◽  
Author(s):  
Xavier Suárez-Calvet ◽  
Jorge Alonso-Pérez ◽  
Ivan Castellví ◽  
Ana Carrasco-Rozas ◽  
Esther Fernández-Simón ◽  
...  
Keyword(s):  
Systemic Sclerosis ◽  
Muscle Biopsy ◽  
Transforming Growth Factor ◽  
Immunosuppressive Treatment ◽  
Inflammatory Myopathy ◽  
Response To Treatment ◽  
Muscle Involvement ◽  
Thrombospondin 1

ObjectiveTo describe the clinical, serologic and histologic features of a cohort of patients with brachio-cervical inflammatory myopathy (BCIM) associated with systemic sclerosis (SSc) and unravel disease-specific pathophysiologic mechanisms occurring in these patients.MethodsWe reviewed clinical, immunologic, muscle MRI, nailfold videocapillaroscopy, muscle biopsy, and response to treatment data from 8 patients with BCIM-SSc. We compared cytokine profiles between patients with BCIM-SSc and SSc without muscle involvement and controls. We analyzed the effect of the deregulated cytokines in vitro (fibroblasts, endothelial cells, and muscle cells) and in vivo.ResultsAll patients with BCIM-SSc presented with muscle weakness involving cervical and proximal muscles of the upper limbs plus Raynaud syndrome, telangiectasia and/or sclerodactilia, hypotonia of the esophagus, and interstitial lung disease. Immunosuppressive treatment stopped the progression of the disease. Muscle biopsy showed pathologic changes including the presence of necrotic fibers, fibrosis, and reduced capillary number and size. Cytokines involved in inflammation, angiogenesis, and fibrosis were deregulated. Thrombospondin-1 (TSP-1), which participates in all these 3 processes, was upregulated in patients with BCIM-SSc. In vitro, TSP-1 and serum of patients with BCIM-SSc promoted proliferation and upregulation of collagen, fibronectin, and transforming growth factor beta in fibroblasts. TSP-1 disrupted vascular network, decreased muscle differentiation, and promoted hypotrophic myotubes. In vivo, TSP-1 increased fibrotic tissue and profibrotic macrophage infiltration in the muscle.ConclusionsPatients with SSc may present with a clinically and pathologically distinct myopathy. A prompt and correct diagnosis has important implications for treatment. Finally, TSP-1 may participate in the pathologic changes observed in muscle.

scholarly journals Acute heart failure and rhabdomyolysis: a clue for the diagnosis of polymyositis with cardiac involvement

Reumatismo ◽  
2017 ◽  
Vol 69 (2) ◽  
pp. 78 ◽  
Author(s):  
P. Pinto-Lopes ◽  
L. Carneiro-Leão ◽  
R. Morais ◽  
J. Pinheiro ◽  
A. Vieira Lopes ◽  
...  
Keyword(s):  
Heart Failure ◽  
Acute Heart Failure ◽  
Muscle Weakness ◽  
Cardiac Involvement ◽  
Diuretic Therapy ◽  
Inflammatory Myopathy ◽  
Pacemaker Implantation ◽  
Idiopathic Inflammatory Myopathy ◽  
Mitral Valve Regurgitation ◽  
Immunological Study

Polymyositis is an idiopathic inflammatory myopathy, characterized by proximal muscle weakness and sometimes extramuscular manifestations. We report the case of a 51-year-old male, with history of complete heart block, which required pacemaker implantation, and subsequently heart failure, presenting to the emergency department with worsening of dyspnea and peripheral edema. He was admitted to the Internal Medicine ward with acute heart failure and started on diuretic therapy. During hospitalization, he was discovered to have marked rhabdomyolysis. Examination revealed proximal symmetrical muscle weakness and arthralgia. The immunological study, electromyography and muscle biopsy confirmed polymyositis. The patient was started on prednisolone with clinical improvement and resolution of rhabdomyolysis. The presence of conduction defect, ventricular dysfunction, mitral valve regurgitation, segmental hypokinesia (myocardial scintigraphy without perfusion defects) and pulmonary hypertension, as well as elevated troponin with improvement after specific therapy, points to cardiac involvement. Polymyositis is a rare entity, with an insidious evolution and a myriad of extramuscular features that can mimic other conditions. In particular, cardiac involvement may be the first and only recognized manifestation. The key point for the diagnosis is to contemplate the possibility of polymyositis.

scholarly journals Statin-associated anti-HMGCR immune-mediated necrotizing myopathy with dermatomyositis-like features: A case report

2020 ◽  
Vol 8 ◽  
pp. 2050313X2098412
Author(s):  
Darosa Lim ◽  
Océane Landon-Cardinal ◽  
Benjamin Ellezam ◽  
Annie Belisle ◽  
Annie Genois ◽  
...  
Keyword(s):  
Creatine Kinase ◽  
Muscle Weakness ◽  
Inflammatory Myopathy ◽  
Idiopathic Inflammatory Myopathy ◽  
Intravenous Immunoglobulins ◽  
Proximal Muscle Weakness ◽  
Proximal Muscle ◽  
Therapeutic Implications ◽  
Necrotizing Myopathy ◽  
Immune Mediated

Anti-3-hydroxy-3-methylglutaryl-coenzyme A reductase (HMGCR) immune-mediated necrotizing myopathy is a subtype of idiopathic inflammatory myopathy which may be associated with statin exposure. It presents with severe proximal muscle weakness, high creatine kinase levels and muscle fiber necrosis. Treatment with intravenous immunoglobulins and immunosuppressants is often necessary. This entity is not commonly known among dermatologists as there are usually no extramuscular manifestations. We report a rare case of statin-associated anti-HMGCR immune-mediated necrotizing myopathy with dermatomyositis-like cutaneous features. The possibility of anti-HMGCR immune-mediated necrotizing myopathy should be considered in patients with cutaneous dermatomyositis-like features associated with severe proximal muscle weakness, highly elevated creatine kinase levels and possible statin exposure. This indicates the importance of muscle biopsy and specific autoantibody testing for accurate diagnosis, as well as significant therapeutic implications.

scholarly journals Clinical manfestation of dermatomyositis and difficulties in its differential diagnosis

2021 ◽  
Vol 97 (3) ◽  
pp. 145-149
Author(s):  
Ágnes Kinyó ◽  
◽  
Anita Hanyecz ◽  
Rolland Gyulai
Keyword(s):  
Differential Diagnosis ◽  
Muscle Weakness ◽  
Early Recognition ◽  
Inflammatory Myopathy ◽  
Idiopathic Inflammatory Myopathy ◽  
Muscle Inflammation ◽  
Skin Symptoms ◽  
Limb Muscles

Dermatomyositis (DM) is an idiopathic inflammatory myopathy with muscle weakness and specific cutaneous findings. The proximal weakness of the limb muscles is present in the majority of the patients, in a proportion of them however the muscle inflammation is preceded by the skin symptoms with several months, and in 40%, skin symptoms are the only manifestations of the disease. While, there may have many difficulties in differential diagnosis of DM without myositis, dermatologists have a major role in the early recognition of the disorder and in the initiation of the proper therapy

scholarly journals Rituximab and intravenous immunoglobulin treatment in PM/Scl antibody-associated disease: case-based review

2022 ◽  
Author(s):  
Denesh Srikantharajah ◽  
Mark E. Lloyd ◽  
Patrick D. W. Kiely
Keyword(s):  
Intravenous Immunoglobulin ◽  
Clinical Features ◽  
Muscle Weakness ◽  
Inflammatory Myopathy ◽  
Idiopathic Inflammatory Myopathy ◽  
Immunoglobulin Therapy ◽  
Rapid Onset ◽  
Calcinosis Cutis ◽  
Intravenous Immunoglobulin Therapy ◽  
Disease Case

AbstractAutoantibodies to the 75-kDa and 100-kDa subunits of the PM/Scl nucleolar protein complex are associated with an overlap syndrome, manifesting with clinical features of systemic sclerosis and idiopathic inflammatory myopathy. We describe the diverse clinical features in a series of 4 cases with anti-PM/Scl-75 and/or anti-PM/Scl-100 antibodies, including severe proximal muscle weakness, oesophageal dysfunction, respiratory weakness requiring mechanical ventilation, Raynaud’s, calcinosis cutis, sclerodactyly and critical digital ischaemia. Despite the severity of striated and oesophageal muscle weakness, all patients responded very well to immune suppression, and calcinosis cutis in one case regressed substantially. We highlight the efficacy of Rituximab and intravenous immunoglobulin therapy (IVIg) in these cases, enabling return to normal muscle function within six months. Rituximab was preferentially chosen for cases with hyper-gammaglobulinemia and multiple autoantibodies in addition to anti-PM/Scl, and IVIg was utilised for cases where a rapid onset of effect was required, such as severe ventilator-dependent respiratory muscle weakness and oesophageal dysfunction.

scholarly journals Dermatomyositis as a paraneoplastic phenomenon in oesophageal cancer

2018 ◽  
Vol 11 (1) ◽  
pp. e227387
Author(s):  
Nicholas Kevin Laidler
Keyword(s):  
Muscle Weakness ◽  
Clinical Symptoms ◽  
Inflammatory Myopathy ◽  
Oral Prednisolone ◽  
Idiopathic Inflammatory Myopathy ◽  
Oesophageal Adenocarcinoma ◽  
Tertiary Institution ◽  
Multisystem Disease ◽  
Positron Emission ◽  
New Diagnosis

Dermatomyositis is a multisystem disease manifesting as an idiopathic inflammatory myopathy, characterised predominantly by cutaneous and muscular abnormalities. Patients with dermatomyositis display characteristic skin changes in addition to muscle weakness. Many consider dermatomyositis a paraneoplastic syndrome, as up to 32% of patients with dermatomyositis will develop cancer. Discussed here is the case of a 69-year-old man with a new diagnosis of dermatomyositis who was referred to a tertiary institution with an 8-month history of rash, proximal muscle weakness, fatigue and weight loss. In the absence of clinical symptoms, negative cancer markers and a negative CT chest, abdomen and pelvis, positron emission tomography–CT prompted a gastroscopy, which led to the discovery of a stage T1b oesophageal adenocarcinoma. The oesophageal adenocarcinoma was treated with a gastrointestinal endoscopic mucosal resection and subsequent oesophajectomy. The patient’s dermatomyositis was treated successfully with oral prednisolone and methotrexate.

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