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

We report a 14-year-old girl with juvenile dermatomyositis (JDM) complicated by severe inflammatory calcinosis successfully treated with thalidomide. She was diagnosed as JDM when she was 4 years old after a few months of increasing lethargy, muscle pain, muscle weakness, and rash. During three months, clinical manifestations and abnormal laboratory findings were effectively treated with oral prednisolone. However, calcinosis was recognized 18 months after disease onset. Generalized calcinosis rapidly progressed with high fever, multiple skin/subcutaneous inflammatory lesions, and increased level of CRP. Fifty mg/day (1.3 mg/kg day) of oral thalidomide was given for the first four weeks, and then the dose was increased to 75 mg/day. Clinical manifestations subsided, and inflammatory markers had clearly improved. Frequent high fever and local severe pain with calcinosis were suppressed. The levels of FDP-E, IgG, and tryglyceride, which were all elevated before the thalidomide treatment, were gradually returned to the normal range. Over the 18 months of observation up to the present, she has had no inflammatory calcinosis, or needed any hospitalization, although established calcium deposits still remain. Her condition became painless, less extensive and less inflammatory with the CRP level below 3.08 mg/dL. Recent examination by whole-body 18F-FDG-PET-CT over the 15 months of thalidomide treatment demonstrated fewer hot spots around the subcutaneous calcified lesions.
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PMID:Efficacy of thalidomide in a girl with inflammatory calcinosis, a severe complication of juvenile dermatomyositis. 2018 Oct 85

Calcinosis cutis is a common clinical feature of dermatomyositis and scleroderma but rarely reported in association with systemic lupus erythematosus (SLE). Calcinosis cutis in SLE occurs without calcium and phosphorus metabolic abnormalities and may be localized or generalized. The pathophysiology remains unclear and no effective therapy is currently available. We report a 30-year-old woman with a 13-year history of SLE who developed multiple calcinosis cutis around both knees and we review the relevant published work.
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PMID:Calcinosis cutis in systemic lupus erythematosus: a case report and review of the published work. 2088 67

Calcinosis cutis is an uncommon disease of unclear pathophysiology that is often disabling. It is characterized by the formation of calcium deposits in the skin or subcutaneous tissue. It is classified into four subtypes: dystrophic, metastatic, idiopathic or iatrogenic. It may be seen in a variety of systemic diseases such as hyperparathyroidism and hypervitaminosis D, but is most commonly found in dermatomyositis, scleroderma and overlap syndromes and is a rare complication of systemic lupus erythematosus. The management of secondary complications and the success of therapy are constant challenges in the follow-up of these cases.
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PMID:Calcinosis cutis universalis associated with systemic lupus erythematosus: an exuberant case. 2130 14

Many rheumatic diseases show changes and are visible in the hands. The pattern of distribution in the relevant joints, soft-tissue changes, skin manifestations, neurological and vascular symptoms and clinical findings provide fundamental information. Imaging and lab results provide diagnostic support. In this review, common diseases are presented in terms of their clinical expressions in the hands: osteoarthritis, rheumatoid arthritis, gout, calcium pyrophosphate dihydrate deposition disease, psoriatic arthritis, reactive arthritis, systemic sclerosis, dermatomyositis/polymyositis and systemic lupus erythematosus. Furthermore, we discuss pathological findings of the hands as a result of diabetic cheiroarthropathia, endocarditis, secondary hypertrophic osteo-arthropathy and chronic regional pain syndrom.
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PMID:[The rheumatic hand - diagnostics in practice]. 2193 98

Calcinosis cutis is a condition characterized by the deposition of calcium salts in the skin and subcutaneous tissues, and patients suffering from it encounter various connective tissue disorders, such as dermatomyositis (DM), scleroderma, and systemic lupus erythematosus. Although calcinosis cutis is frequently accompanied by juvenile dermatomyositis, rare cases have been reported in adult patients with DM. On the other hand, lichen sclerosus (LS) is a chronic inflammatory disease of the skin and mucosal surfaces. In the present report, we present a rare case of a 71-year-old patient with DM accompanied by ulcerated calcinosis cutis and vulvar LS.
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PMID:The co-existence of vulvar lichen sclerosus, ulcerated calcinosis cutis, and dermatomyositis: coincidence or immunological mechanism? 2234 82

Calcinosis is a connective tissue disorder classified into the following four types: metastatic; idiopathic; iatrogenic and dystrophic. Dystrophic calcinosis can occur, for example, in dermatomyositis, mainly in juvenile dermatomyositis, and is characterized by an abnormal deposition of calcium salts in affected skin, subcutaneous tissues, and muscles or tendons, with normal serum levels of calcium and phosphate. The treatment of calcinosis in dermatomyositis remains a challenge, with few descriptions in the literature of low scientific evidence. So far, no therapy has proved to be highly effective in the combat and resolution of that comorbidity. The present study discusses the concept of calcinosis, particularly in dermatomyositis, as well as its treatment described in the literature.
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PMID:Update on the treatment of calcinosis in dermatomyositis. 2385 99

Calprotectin (CP) is a calcium- and zinc-binding protein of the S100 family expressed mainly by neutrophils with important extracellular activity. The aim of the current review is to summarize the latest findings concerning the role of CP in a diverse range of inflammatory and noninflammatory conditions among children. Increasing evidence suggests the implication of CP in the diagnosis, followup, assessment of relapses, and response to treatment in pediatric pathological conditions, such as inflammatory bowel disease, necrotizing enterocolitis, celiac disease, intestinal cystic fibrosis, acute appendicitis, juvenile idiopathic arthritis, Kawasaki disease, polymyositis-dermatomyositis, glomerulonephritis, IgA nephropathy, malaria, HIV infection, hyperzincemia and hypercalprotectinemia, and cancer. Further studies are required to provide insights into the actual role of CP in these pathological processes in pediatrics.
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PMID:The role of calprotectin in pediatric disease. 2417 91

Abstract Autoantibodies to calpastatin (endogenous inhibitor of calpain, a calcium-dependent neutral proteinase) have been detected in sera of patients with rheumatoid arthritis (RA) and other diseases. We investigated the epitope reactivity of anticalpastatin autoantibodies in patients with rheumatic diseases. cDNAs encoding each calpastatin domain (L, I, II, III, and IV) were amplified by PCR and ligated into an expression vector. The fusion proteins were expressed in E. coli. The presence of autoantibodies specific for each calpastatin domain was assayed in sera of patients with various rheumatic diseases by immunoblotting the fusion proteins with these sera. Of the RA patient sera, 81% reacted with at least one calpastatin domain. This reaction was significantly greater than with sera from patients with systemic lupus erythematosus (46%), scleroderma (32%), polymyositis/dermatomyositis (43%), and normal controls (13%). Domains I and II were recognized by RA patient sera significantly more than by other patient sera, whereas domains III and IV reacted almost equally among all patient sera. Although, collectively, sera from RA and lupus patients reacted equally with all domains, scleroderma sera tended to react with only domains I and IV and myositis sera tended to recognize only domains III and IV. Patients with RA positive for anticalpastatin antibodies exhibited more active disease (i.e., a higher erythrocyte sedimentation rate and C-reative protein level) than antibody-negative patients. Our results suggest that anticalpastatin antibodies were detected in RA with the highest frequency and that different domain reactivity was shown among different diseases. The presence of these antibodies in sera may be related to the type of disease and, in RA, with disease activity, suggesting their importance in rheumatic disorders.
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PMID:Domain reactivity of autoantibodies to calpastatin in patients with systemic rheumatic diseases. 2438 32

Rheumatic diseases such as juvenile dermatomyositis (JDM), juvenile sistemic lupus erythematosus (JSLE) and sistemic sclerosis may have calcium deposits in the subcutaneous and muscle tissues known as calcinosis. Extensive calcium-laden fluid collections referred as "milk of calcium" are rare forms of calcinosis in JDM. We describe a 15-year old patient with overlap syndrome (sclerodermatomyositis and JSLE), whose magnetic resonance imaging (MRI) showed perimusculares fluid collections in the lower limbs. During surgery, we observed the presence of whitish fluid collection suggestive of "milk of calcium".
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PMID:"Milk of calcium": a rare presentation of calcinosis. 2487 95

Calcinosis cutis constitutes a heterogeneous group of chronic disorder. It can be associated with disturbance of calcium and/or phosphate metabolism (metastatic, tumor calcinosis, calciphylaxis) but may also develop without any metabolic disorder, in particular during the course of connective tissue diseases. Among these, the most common are dermatomyositis and the limited form of systemic sclerosis. The physiopathology of calcinosis cutis is poorly known. It can cause pain, chronic ulcerations, infections, which are sources of sometimes major disability. Treatment of calcinosis is challenging because no drug has been shown to be reliably effective in stopping the progression or decreasing dystrophic calcifications in controlled trials. Calcium blocker and colchicine are generally prescribed as the first line systemic therapy. In the localized forms of small lesions, surgical excision is often effective and sometimes preceded by local treatments (laser therapy, extracorporeal shock wave lithotripsy, topical sodium thiosulfate, etc.) or systemic treatment (minocycline, warfarine). When calcinosis is disseminated, it may require additional treatments (aluminium hydroxyde, bisphosphonates) possibly associated with surgery in case of large lesions. Time to response may be prolonged from weeks to months. The calcinosis cutis can lead to secondary infection, pain and functional disability that have to be prevented.
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PMID:[Treatment of subcutaneous calcinosis in systemic disorders]. 2488 Jul 41


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