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Query: UMLS:C0027121 (myositis)
 4,538 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The pathogenesis of the inflammatory myopathies is still unclear, making their treatment largely empirical. Improved understanding of the molecular mechanisms of inflammatory muscle injury may, however, lead to the development of more specific immunotherapies. To elucidate a possible pathogenic contribution of calcium-binding proteins such as the annexins, we immunohistochemically investigated muscle biopsy specimens from patients with dermatomyositis (10 cases), polymyositis (9 cases), and inclusion-body myositis (4 cases), compared to control cases comprising sarcoid myopathy (3 cases), Duchenne muscular dystrophy (DMD; 4 cases), and normal muscle (3 cases). We found expression of annexins A1, A2, A4, and A6 in the vascular endothelium of all cases. Myofibers expressed annexins A5, A6, and A7 diffusely and weakly in the cytosol, whereas annexins A5 and A7 were also particularly localized to the sarcolemma. In the inflammatory myopathies, in areas of myonecrosis in DMD, and in granulomatous lesions of sarcoid myopathy, reactivity of annexins A1, A2, A4, A5, and A6 was observed in macrophages and T-lymphocytes. Whereas the latter annexins appear to be nonspecific indicators of activation, annexin A1 upregulation may represent endogenous anti-inflammatory mechanisms that merit further investigation.
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PMID:Annexin expression in inflammatory myopathies. 1522 85

Although sarcoidosis can affect virtually any organ, musculoskeletal involvement is infrequent. Early in the course of the disease, acute polyarthritis may be observed in up to 40% of patients, but this is often self-limited. Chronic or recurrent sarcoid arthritis is rare, affecting only 1 to 4% of patients. Asymptomatic involvement of muscles has been reported in 25 to 75% of patients in small series, but symptomatic involvement of muscles is rare. Although prospective studies assessing the prevalence of muscle involvement in sarcoidosis have not been done, several large retrospective studies suggest that symptomatic muscle involvement occurs in < 0.5% of patients with sarcoidosis. Patterns of sarcoid muscle involvement include chronic, progressive myopathy; nodular or tumorous sarcoidosis affecting muscles; and an acute polymyositis-like syndrome. Corticosteroid therapy is usually efficacious for the acute myositis variant but is less consistently helpful in the other forms (e.g., chronic myopathy or nodules). Similarly, clinically significant involvement of bone is rare (< 2-5%), and is often associated with chronic multisystemic disease, lupus pernio, and chronic uveitis. Unfortunately, osseous sarcoidosis is often unresponsive to therapy. In this review, we discuss the prevalence, clinical features, and management of sarcoidosis involving muscle, joints, and bones.
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PMID:Sarcoidosis involving the musculoskeletal system. 1608 51

B7 family members regulate T cell activation and tolerance. Although butyrophilin proteins share sequence homology with the B7 molecules, it is unclear whether they have any function in immune responses. In the present study, we characterize an MHC class II gene-linked butyrophilin family member, butyrophilin-like 2 (BTNL2), the mutation of which has been recently associated with the inflammatory autoimmune diseases sarcoidosis and myositis. Mouse BTNL2 is a type I transmembrane protein with two pairs of Ig-like domains separated by a heptad peptide sequence. BTNL2 mRNA is highly expressed in lymphoid tissues as well as in intestine. To characterize the function of BTNL2, we produced a BTNL2-Ig fusion protein. It recognized a putative receptor whose expression on B and T cells was significantly enhanced after activation. BTNL2-Ig inhibited T cell proliferation and TCR activation of NFAT, NF-kappaB, and AP-1 signaling pathways. BTNL2 is thus the first member of the butyrophilin family that regulates T cell activation, which has implications in immune diseases and immunotherapy.
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PMID:BTNL2, a butyrophilin-like molecule that functions to inhibit T cell activation. 1675 79

A 30-year-old man complained of polyarthralgia and fatigue. The clinical findings and laboratory data included myositis, polyarthritis, interstitial pneumonia, Raynaud's phenomenon, mechanic's hand, and anti PL-7 antibody (threonyl-tRNA synthetase antibody). All of these signs were consistent with antisynthetase syndrome. His chest radiograph revealed bilateral hilar lymphadenopathy. Biopsy specimens from his mediastinal lymph node and muscle showed noncaseating epithelioid cell granulomas. Lung histology revealed nonspecific interstitial pneumonia. Antisynthetase syndrome associated with sarcoidosis was diagnosed. Interstitial pneumonia in this patient responded well to high-dose corticosteroid therapy.
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PMID:Antisynthetase syndrome associated with sarcoidosis. 1704 79

Granulomatous myositis (GM) is a rare condition that has generally been described in association with sarcoidosis. In the absence of sarcoidosis or other underlying disease, a diagnosis of isolated GM is considered. Only one study has focused on the clinical difference between isolated GM and sarcoid myopathy (SM). We report 13 cases of symptomatic GM; 8 had sarcoidosis. All patients with sarcoidosis had predominantly proximal, symmetrical lower-limb weakness, and 3 subsequently developed upper-limb or distal involvement. Three of the five patients with isolated GM had predominantly distal muscle involvement, and two had dysphagia. Corticosteroid treatment was followed by prolonged improvement in only one patient with sarcoidosis. One patient had acute sarcoid myositis and benefited from methotrexate; other immunosuppressants and etanercept proved ineffective in chronic sarcoid myopathy. Three of the five patients with isolated GM responded to corticosteroid treatment. When last examined, three patients with sarcoidosis had severe disability, whereas patients with isolated GM showed milder weakness. Thus, SM was frequently associated with severe disability and rarely improved after corticosteroid treatment, whereas most patients with isolated GM improved.
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PMID:Granulomatous myositis: a clinical study of thirteen cases. 1706 67

A 54-year-old woman complaining of muscle weakness and weight loss was admitted to our hospital with suspected polymyositis. Muscle biopsy revealed Langhans-type giant cells and noncaseating granulomas. Therefore, sarcoid myositis was diagnosed. The patient was treated with prednisolone, and the symptoms improved gradually. Generally, sarcoidosis is identified clinically in patients with foggy vision or mediastinal lymphadenopathy, but muscular weakness may be an infrequently observed initial symptom. Sarcoidosis should be considered in the differential diagnosis of polymyositis.
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PMID:Sarcoid myositis with muscle weakness as a presenting symptom. 1756 82

Granulomatous myositis is a rare neuromuscular disorder histologically characterized by the development of endomyseal and/or perimyseal granulomas. Clinical hallmarks are generalized muscle weakness, myalgias, and bulbar symptoms. The association of granulomatous myositis with sarcoidosis is well known; less recognized is the association with several infectious diseases, inflammatory bowel diseases, malignancy, thymoma, graft-vs-host disease, and myasthenia gravis. In absence of sarcoidosis or other underlying disorders, the diagnosis of isolated or primary granulomatous myositis must be considered. Therapeutic strategies focus on immunosuppression, whereas the therapy response is unpredictable. Here we discuss the clinical features, diagnosis, and differential diagnosis and therapeutic strategies of primary and secondary granulomatous myositis.
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PMID:[Diagnosis and differential diagnosis of granulomatous myositis]. 1821 45

Many rheumatic diseases have been observed in HIV-infected persons. We, therefore, conducted a comprehensive literature search in order to review the prevalence, presentation and pathogenesis of rheumatic manifestations in HIV-infected subjects. Articular conditions (arthralgia, arthritis and SpAs) are either caused by the HIV infection itself, triggered by adaptive changes in the immune system, or secondary to microbial infections. Muscular symptoms may result from rhabdomyolysis, myositis or from side-effects of highly active anti-retroviral therapy (HAART). Osseous complications include osteonecrosis, osteoporosis and osteomyelitis. Some conditions such as the diffuse infiltrative lymphocytosis syndrome and sarcoidosis affect multiple organ systems. SLE may be observed but may be difficult to differentiate from HIV infection. Some anti-retroviral agents can precipitate hyperuricaemia and are associated with arthralgia. When indicated, immunosuppressants and even anti-TNF-alpha agents can be used in the carefully monitored HIV patient. Thus, rheumatic diseases and asymptomatic immune phenomena remain prevalent in HIV-infected persons even after the widespread implementation of highly active anti-retroviral therapy.
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PMID:Rheumatic conditions in human immunodeficiency virus infection. 1841 46

Besides the classical inflammatory myopathies (IM), dermatomyositis (DM), polymyositis, and inclusion body myositis, the much larger spectrum of IM includes focal and nodular myositis, granulomatous myositis, macrophagic myofasciitis, graft vs. host myositis, eosinophilic myositis, and other immune-associated conditions, some of them only recently described. In addition, paraneoplastic, statin-induced and critical illness myopathies have been considered immune-associated IM. Infectious, i.e., bacterial, viral, and parasitic IM are much less frequent in the northern hemisphere. In IM, muscle biopsy is an essential diagnostic procedure to initiate therapy. The myopathological spectrum encompasses disease-specific histopathological features, such as perifascicular atrophy in DM, non-necrotizing granulomas in sarcoid myopathy, autophagic vacuoles with tubulofilamentous inclusions in inclusion body myositis, rarely electron microscopic criteria, such as undulating tubules in endothelial cells of DM specimens, and, foremost, immunohistochemical findings. These latter features concern inflammatory infiltrates, the muscle parenchyma, the interstitial compartment, and the vasculature with varying involvement of each component in the different IM. Differences in immunohistochemical parameters among the IM, such as major histocompatibility complexes I and II, cytokines, cell adhesion molecules, different types of inflammatory cells, metalloproteinases, and complement factors procure a large gamut of data, the individual patterns of which characterize the myopathology of individual IM.
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PMID:Myopathology of non-infectious inflammatory myopathies - the current status. 1851 33

Orbital myositis is an inflammatory process that primarily involves the extraocular muscles and most commonly affects young adults in the third decade of life, with a female predilection. Clinical characteristics of orbital myositis include orbital and periorbital pain, ocular movement impairment, diplopia, proptosis, swollen eyelids, and conjunctival hyperemia. The most common presentation is acute and unilateral, which initially responds to systemic corticosteroid therapy. However, chronic and recurrent cases may involve both orbits. Many inflammatory, vascular, neoplastic, and infectious conditions that affect the extraocular muscles and other orbital tissue can mimic orbital myositis. The most important differential diagnoses include thyroid-related eye disease, other orbital inflammatory processes (unspecific idiopathic inflammation, vasculitis, and sarcoidosis), orbital cellulitis, and orbital tumors. In refractory, chronic, or recurrent cases, steroid-sparing agents, inmmunosuppressants, or radiation therapy may be indicated.
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PMID:Orbital myositis: diagnosis and management. 1965 80


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