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)

A 27-year-old male patient with calcinosis universalis resulting from dermatomyositis was successfully treated with low-dose warfarin. On his trunk and extremities, there were many subcutaneous calcified nodules, and knee flexion was difficult. After oral warfarin therapy for three years, the calcified nodules became smaller, and the knee mobility improved. His serum vitamin K level was abnormally high, decreased just after starting warfarin therapy, and then remained within the normal range. Since vitamin K has been known to play an important role in the Ca2+ binding process in bones or tissues, we suggest that this therapy is effective in reducing subcutaneous calcification through the vitamin K cycle.
 ...
PMID:A case of calcinosis universalis successfully treated with low-dose warfarin. 986 84

A case of calcinosis cutis, appeared since childhood in a woman 73-years-old, affected by diabetes mellitus with complications, is described. This uncommon disorder is discussed on the basis of data from recent literature. Calcinosis cutis is a condition characterized by the deposition of crystals of calcium phosphate (hydroxyapatite) in the skin. Calcinosis cutis may be idiopathic or secondary. The idiopathic calcinosis cutis is uncommon, may be solitary or multiple, sporadic or associated with Down syndrome (MICC or "milialike idiopathic calcinosis cutis") and appears more often in childhood or adolescence. Secondary calcinosis cutis may appear in the course of juvenile dermatomyositis or in the form of systemic scleroderma named CREST syndrome (calcinosis cutis, Raynaud's phenomenon, esophageal dysfunction, sclerodactyly and telangectasia). Calcinosis cutis may also be seen later in the course of renal failure, associated with hyperphosphatemia and secondary hyperparathyroidism. In this case report, calcinosis cutis appeared early in life and the laboratory data showed normal erythrocyte sedimentation rate and leukocyte count, negative LE test and absence of rheumatoid factor and non-organ-specific auto-antibodies, and multiple localizations. On these grounds, the diagnosis of idiopathic multiple calcinosis cutis was made. This is a rare and benign syndrome, which does not cause any late complication and whose prognosis is therefore favourable.
 ...
PMID:[A case of idiopathic multiple calcinosis cutis]. 1018 2

Children with dermatomyositis may have extensive subcutaneous and intermuscular calcium-laden fluid collections referred to as "milk of calcium." The distinctive MR appearance of such collections in an upper extremity of a 16-year-old girl is presented. MR can differentiate these collections from abscesses and guide appropriate therapy.
 ...
PMID:Milk of calcium fluid collections in juvenile dermatomyositis: MR characteristics. 1066 4

Milk of calcium developed in 2 children with juvenile dermatomyositis. The fluid of the collection contained macrophages, interleukin-6, IL-1, and tumor necrosis factor. The patient who had dystrophic calcinosis had a dramatic improvement with the introduction of alendronate. These findings suggest that calcinosis of juvenile dermatomyositis may be mediated by activated macrophages and that alendronate can be an effective treatment for this condition.
 ...
PMID:New insight into calcinosis of juvenile dermatomyositis: a study of composition and treatment. 1134 59

Dermatomyositis is the connective tissue disease with the least renal involvement. Although some renal findings like proteinuria, hematuria, pyuria, progressive renal insufficiency, and glomerular and tubular calcium deposits with arteriolar fibrosis have been described, glomerulonephritides have rarely been associated with dermatomyositis, especially in childhood cases. We describe a 10-year old boy with the clinical picture of dermatomyositis who underwent renal biopsy due to microscopic hematuria demonstrating membranous glomerulonephritis with Clq deposition. Children with "full-house" membranous glomerulonephritis with deposition of Clq and the other immunoglobulins have been reported to present in the future with the clinical findings of systemic lupus erythematosus. However, laboratory evaluation of our patient for systemic lupus erythematosus was negative at the present time. Thus, we think this case should be followed up closely with special attention to the possible clinical and laboratory findings of systemic lupus erythematosus.
 ...
PMID:Dermatomyositis with membranous nephropathy. 1143 94

A case of pseudoxanthoma elasticum (PXE)-like calcification in adult dermatomyositis (DM) is described. The patient was a 38-year-old woman with a history of dermatomyositis for 3 months. Yellowish, hard, papulo-plaque lesions, which looked like those of pseudoxanthoma elasticum, were noted on her left axilla. Calcium deposition was confirmed by X-ray, histopathological, and electron microscopic examinations. Histopathological and histochemical examinations showed acicular calcium deposition in the middle and deep dermis surrounded by mucin. Electron microscopic examination revealed that the calcium deposition was not on collagen fibers. These morphological features were distinct from those of PXE. We proposed the possibility that degenerated mucin or degenerated elastic fiber might result in subsequent calcium deposition in reticular calcinosis in adult DM. The calcification clinically disappeared without any specific treatment except for prednisolone and cyclophosphamide.
 ...
PMID:Pseudoxanthoma elasticum (PXE)-like calcification in adult dermatomyositis. 1218 40

Members of the synaptotagmin family have been proposed to function as Ca2+ sensors in membrane fusion. Syt VII is a ubiquitously expressed synaptotagmin previously implicated in plasma membrane repair and Trypanosoma cruzi invasion, events which are mediated by the Ca2+-regulated exocytosis of lysosomes. Here, we show that embryonic fibroblasts from Syt VII-deficient mice are less susceptible to trypanosome invasion, and defective in lysosomal exocytosis and resealing after wounding. Examination of mutant mouse tissues revealed extensive fibrosis in the skin and skeletal muscle. Inflammatory myopathy, with muscle fiber invasion by leukocytes and endomysial collagen deposition, was associated with elevated creatine kinase release and progressive muscle weakness. Interestingly, similar to what is observed in human polymyositis/dermatomyositis, the mice developed a strong antinuclear antibody response, characteristic of autoimmune disorders. Thus, defective plasma membrane repair in tissues under mechanical stress may favor the development of inflammatory autoimmune disease.
 ...
PMID:Impaired membrane resealing and autoimmune myositis in synaptotagmin VII-deficient mice. 1292 4

The pathophysiological role of infiltrating macrophages and their subtypes in idiopathic inflammatory myopathies such as dermatomyositis, polymyositis, and inclusion body myositis is not fully clear. Monocytes exhibit various phenotypes with different functional properties such as release of pro- or anti-inflammatory mediators. Expression of myeloid-related proteins MRP8 and MRP14, two calcium-binding S100-proteins, characterizes a proinflammatory subtype of macrophages. We immunohistochemically investigated expression of MRP8 and MRP14 in muscle biopsies of 33 patients with dermatomyositis, polymyositis, and inclusion body myositis. We found a clear association of expression of MRP8 and MRP14 by infiltrating macrophages with degeneration of myofibers. Because MRP8 and MRP14 are secreted by activated macrophages we investigated if these proteins would have direct extracellular effects on myocytes. We found that the purified MRP8/MRP14 complex inhibited proliferation and differentiation of C2C12 myoblasts and that it induced apoptosis via activation of caspase-3 in a time- and dose-dependent manner. These results indicate that in the course of inflammatory myopathies, activated macrophages can promote destruction and impair regeneration of myocytes via secretion of MRP8/MRP14.
 ...
PMID:Expression of calcium-binding proteins MRP8 and MRP14 in inflammatory muscle diseases. 1293 35

The aim of this study was to evaluate strategies to halt the progression of severe dystrophic calcification in a patient with progressive systemic sclerosis (PSS) and to monitor serial changes in biochemical markers of bone resorption and indices of calcium (Ca) metabolism in response to therapy. The relationship of bone turnover to the extent of dystrophic calcification was also investigated in a number of additional patients with varying degrees of calcinosis. Serial markers of bone turnover and indices of Ca metabolism were measured over a 3-year period in one patient with PSS and severe dystrophic calcification. Bone mineral density (BMD) was assessed by dual-energy x-ray absorptiometry (DXA). Bone turnover in this patient and two additional patients with PSS or dermatomyositis (DM) and severe dystrophic calcification (Group A, n = 3) was compared with that in patients with connective tissue disease with little or no evidence of dystrophic calcification (Group B, n = 13). Serial data on one patient with severe progressive calcinosis showed increased bone resorption markers, which remained high over a 3-year period despite antiresorptive therapy. BMD was low. Patients with PSS/DM with severe dystrophic calcification had higher bone resorption than those with minimal or no evidence of calcification. Mean serum ionized Ca and urinary Ca excretion were both lower in those with severe calcinosis. Bone resorption was increased in patients with connective tissue disease and severe dystrophic calcification. Several antiresorptive agents were shown to be ineffective in limiting either bone turnover or clinical progression in one patient.
 ...
PMID:Increased bone resorption and failure to respond to antiresorptive therapy in progressive dystrophic calcification. 1295 96

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.
 ...
PMID:Annexin expression in inflammatory myopathies. 1522 85


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