Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0011633 (dermatomyositis)
 4,181 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Intravenous immunoglobulin (IVIg) is a new modality used to help treat conditions associated with immune dysregulation. The inflammatory myopathies are a group of complex diseases including dermatomyositis, polymyositis, and inclusion-body myositis. Overall evaluation of IVIg in myopathy has been hampered by difficulty in accurately diagnosing and assessing disease activity. The lack of large, well controlled, double-blind trials has precluded clear evaluation of the effectiveness of IVIg in these diseases. However, from the data presented in published reports, it appears that IVIg may be useful in the treatment of inflammatory myopathies, particularly dermatomyositis.
 ...
PMID:Treatment of inflammatory myopathy with intravenous gamma globulin. 857 71

Amyopathic dermatomyositis is a disease of unknown origin characterized by the specific skin lesions of dermatomyositis but without clinical or laboratory evidence of myopathy. During the past 15 years, a great controversy between the different reports concerning a possible association of dermatomyositis with malignancy has been noted. In this report, the authors describe a patient with amyopathic dermatomyositis who presented first with a benign hyperplasia of the lymph node, which finally transformed into frank malignant lymphoma. In addition to follow-up care, screening tests to search for occult malignancy in patients with amyopathic dermatomyositis (or dermatomyositis) are recommended.
 ...
PMID:Case Report: amyopathic dermatomyositis associated with transformed malignant lymphoma. 861

Systemic lupus erythematosus, chronic cutaneous lupus erythematosus and subacute and acute cutaneous lupus erythematosus are associated with distinct cutaneous manifestations, and each disease has a particular clinical course and prognosis. Dermatomyositis, an immune-mediated disorder of unknown etiology that is often associated with a malignancy, consists of an inflammatory myopathy combined with characteristic cutaneous findings. Some patients with dermatomyositis also have clinical features that overlap with those of systemic lupus erythematosus or scleroderma. Manifestations of scleroderma range from cutaneous involvement alone to multisystem internal disease. Morphea, progressive systemic sclerosis, eosinophilic fascitis, eosinophilia myalgia syndrome and mixed connective tissue disease are all within the spectrum of scleroderma.
 ...
PMID:Cutaneous manifestations of selected rheumatologic diseases. 862 90

In 1991, Emslie-Smith and Engel described a distinct form of idiopathic inflammatory myopathy they called "necrotizing myopathy with pipestem capillaries, microvascular deposition of the complement membrane attack complex (MAC) and minimal cellular infiltration." We describe a patient with exercise-dependent painful myopathy related to a necrotizing myopathy with pipestem capillaries in whom mild cutaneous signs of dermatomyositis were detected 7 years after onset and who subsequently developed multiple cerebral infarcts.
 ...
PMID:Necrotizing myopathy with pipestem capillaries and minimal cellular infiltration: a case associated with cutaneous signs of dermatomyositis. 862 98

We describe two patients with sustained myoglobinuria that did not resolve with supportive treatment. After we established a diagnosis of dermatomyositis and started treatment with corticosteroids, there was rapid resolution of the myoglobinuria. Well-documented cases of inflammatory myopathy causing myoglobinuria are rare, but their prompt recognition has important therapeutic implications.
 ...
PMID:Sustained myoglobinuria: the presenting manifestation of dermatomyositis. 871 64

The aim of the current study was to analyze the frequency and characteristics of symptomatic myopathies occurring in rheumatoid arthritis (RA) patients, to correlate these findings with clinical data, and to evaluate their therapeutic implications. All RA patients from a cohort of 350 RA patients from a single institution who developed muscular symptomatology during an 8-year period were included in the study (n = 21). Clinical and laboratory data and electromyographic results were recorded in all cases, and an open muscle biopsy was performed. Weakness and muscle atrophy were the most common symptoms. Serum creatine kinase was increased in 8 cases (38%). Histopathologic study showed type 2 atrophy in 12 cases. In 13 cases, a treatable disease was diagnosed: dermatomyositis (n = 2), d-penicillamine-related dermatomyositis (n = 2), polymyositis (n = 1), muscular mononuclear cell infiltration (n = 3), polyarteritis nodosa (n = 1), glucocorticoid myopathy (n = 3), and toxic chloroquine myopathy (n = 1). In all but 1 patient, muscular clinical response to new therapy and/or drug withdrawal was satisfactory. Although symptomatic muscular involvement in RA is low (6% in the current series), we have found that nearly two thirds of cases were caused by potentially treatable conditions, mainly myositis or toxic myopathies.
 ...
PMID:Muscle involvement in rheumatoid arthritis: clinicopathological study of 21 symptomatic cases. 879 14

The complement system has been shown to play a pathogenic role in only a small number of muscle diseases. A complement-induced microangiopathy is important in dermatomyositis and in the rare disorder, necrotizing myopathy with pipestem capillaries. Recent studies demonstrate the reversibility of microvascular damage in dermatomyositis by intravenous immune globulin which appears to intercept the assembly and deposition of membrane attach complex. In myasthenia gravis, complement-mediated lysis directed at the acetylcholine receptor leads to a cascade of events reducing the number of receptors, simplifying the complex architecture of the junctional folds and decreasing the available surface for the insertion of new receptors. The newest condition in which a role for complement has been demonstrated is X-linked vacuolated myopathy. The condition is of interest because membrane attack complex deposits result in shedding of complement-laden membrane fragments in contrast to a cytolytic process as the consequence of assembly of the terminal complement components.
 ...
PMID:The immunopathogenic role of complement in human muscle disease. 883 17

Inclusion body myositis, polymyositis, and dermatomyositis are three distinct categories of inflammatory myopathy. Some authorities commented on the selective early weakness of the volar forearm muscles, quadriceps, and ankle dorsiflexors in inclusion body myositis. The most important feature distinguishing inclusion body myositis from the other two inflammatory myopathies is the lack of responsiveness to immunosuppressive treatment. Although most patients with inclusion body myositis have characteristic muscle biopsy findings, some cannot be distinguished histologically early from polymyositis. Predicting responsiveness to immunosuppressive medications, independent of muscle histology, would be valuable to clinicians. We retrospectively reviewed the pattern of weakness and other clinical features of 46 patients newly diagnosed with either inclusion body myositis, polymyositis, or dermatomyositis. Asymmetrical muscle weakness with prominent wrist flexor, finger flexor, and knee extensor involvement was specific for inclusion body myositis and unresponsive polymyositis. Male sex, lower creatine kinase levels, slower rate of progression, and peripheral neuropathy were also more common in inclusion body myositis and unresponsive polymyositis than in responsive polymyositis and dermatomyositis patients. Repeat muscle biopsy in 2 patients in the unresponsive polymyositis group demonstrated histological features of inclusion body myositis. We suspect that patients with clinical features of inclusion body myositis but lacking histological confirmation may nonetheless have inclusion body myositis. Our study supports the recently proposed criteria for definite and possible inclusion body myositis.
 ...
PMID:Inclusion body myositis: clinical and pathological boundaries. 887 77

Immunostaining of Bcl-2 protein which represses apoptosis was performed on 178 biopsied human pathologic muscles and 10 control muscles by the ABC method using two monoclonal anti-Bcl-2 antibodies. Bcl-2 in control muscles was positive mainly in nuclear membrane and cytoplasm in type 2 fibers (especially type 2B fibers), and negative in type 1 fibers. In myopathies, it was not expressed in type 2C (regenerating) fibers, and its expression in atrophic fibers such as forming pyknotic nuclear clumps was strong. In inflammatory myopathies, expression was observed in infiltrating lymphocytes, and especially in dermatomyositis in atrophic fibers facing perimysium. In mitochondrial myopathies, the positivity was observed only in type 2 ragged-red fibers. In muscles of neurogenic disorders, both small angulated fibers and atrophic grouped fibers were strongly positive. Western blot analysis using anti-Bcl-2 antibody showed a single band at 26 kDa in control and diseased skeletal muscles. Compared to immunostaining of Fas antigen in serial sections, both Bcl-2 and Fas were expressed in same atrophic fibers in distal myopathy with rimmed vacuoles. In myotonic dystrophy, they were often expressed in type 2 fibers containing internal nucleus. In carriers of Duchenne dystrophy, Fas-positive but Bcl-2 negative fibers were observed in same dystrophin-negative fibers. In conclusion, expression of Bcl-2 in skeletal muscles might suggest that Bcl-2 plays a role on surviving muscle fibers.
 ...
PMID:[Immunostaining of anti-Bcl-2 antibody in diseased human muscles]. 893 93

An 8-year-old girl with juvenile dermatomyositis (DM) developed dystrophic calcifications 26 months after diagnosis. She also had severe steroid induced bone loss (osteoporosis). The calcifications turned into generalized heterotopic calcinosis with an exoskeleton-like pattern, despite successful treatment of her myopathy with methylprednisolone and immunosuppressive drugs. She was subsequently treated with oral diltiazem (5 mg/kg/day) to control calcinosis and oral pamidronate (4 mg/kg/day) in addition to calcium and vitamin D supplementation, which she had been taking for 3 years. After 21 months of treatment, clinical and radiological examination revealed dramatic regression of the calcinosis. Bone mass reached normal levels, as determined by bone absorptiometry. Diltiazem alone or in combination with other drugs could be a useful therapy in patients with juvenile DM and pronounced calcifications.
 ...
PMID:Regression of calcinosis during diltiazem treatment in juvenile dermatomyositis. 897 55


<< Previous 1 2 3 4 5 6 7 8 9 10 Next >>