Calcinosis In Dermatomyositis Research Articles

Improvement of Calcinosis in Dermatomyositis With Intravenous Immunoglobulin.

Improvement of Calcinosis in Dermatomyositis With Intravenous Immunoglobulin.

Subcutaneous calcinosis: Is it different between systemic sclerosis and dermatomyositis?

Calcinosis cutis is the deposition of insoluble calcium in the skin and subcutaneous tissues. It is a manifestation of several autoimmune connective tissue diseases, most frequently with systemic sclerosis and juvenile dermatomyositis, followed by adult dermatomyositis. Autoimmune connective tissue disease-associated calcinosis is of the dystrophic subtype, which occurs at sites of damaged tissue in the setting of normal serum calcium and phosphate levels. In juvenile dermatomyositis, calcinosis is considered a marker of ongoing disease activity and possibly inadequate treatment, while in adult dermatomyositis, it is a hallmark of skin damage due to chronic rather than active disease. Calcinosis is associated with long disease duration in systemic sclerosis and dermatomyositis, anti-polymyositis/sclerosis autoantibodies in systemic sclerosis and NXP-2 and melanoma differentiation-associated gene 5 in dermatomyositis. Calcinosis in systemic sclerosis occurs most frequently in the hands, particularly the fingers, whereas in dermatomyositis, it affects mainly the trunk and extremities. The primary mineral component of calcinosis is hydroxyapatite in systemic sclerosis and carbonate apatite in dermatomyositis. Calcinosis in dermatomyositis and systemic sclerosis share some pathogenic mechanisms, but vascular hypoxia seems to play a more important role in systemic sclerosis, whereas the release of calcium from mitochondria in muscle cells damaged by myopathy may be a primary mechanism contributing to dermatomyositis-related calcinosis. Multiple treatment strategies for dermatomyositis and systemic sclerosis-related calcinosis have been used with variable results. Early aggressive treatment of underlying myositis in patients with dermatomyositis may improve long-term outcomes of calcinosis. A better understanding of the pathogenesis of calcinosis is needed to improve treatment options.

Giant Calcinosis in Dermatomyositis and Scleroderma Overlap

Giant Calcinosis in Dermatomyositis and Scleroderma Overlap

Calcinosis Biomarkers in Adult and Juvenile Dermatomyositis.

Dermatomyositis (DM) is a rare idiopathic inflammatory myopathy characterized by muscle weakness and cutaneous manifestations in adults and children. Calcinosis, a complication of DM, is the abnormal deposition of insoluble calcium salts in tissues, including skin, subcutaneous tissue, tendons, fascia, and muscle. Calcinosis is more commonly seen in juvenile DM (JDM), but also develops in adult DM. Although the mechanism of calcinosis remains unclear, several pathogenic hypotheses have been proposed, including intracellular accumulation of calcium secondary to an alteration of the cellular membrane by trauma and inflammation, local vascular ischemia, dysregulation of mechanisms controlling the deposition and solubility of calcium and phosphate, and mitochondrial damage of muscle cells. Identifying calcinosis biomarkers is important for early disease detection and risk assessment, and may lead to novel therapeutic targets for the prevention and treatment of DM-associated calcinosis. In this review, we summarize myositis autoantibodies associated with calcinosis in DM, histopathology and chemical composition of calcinosis, genetic and inflammatory markers that have been studied in adult DM and JDM-associated calcinosis, as well as potential novel biomarkers.

Management of Calcinosis Associated with Dermatomyositis

Calcinosis cutis, or dystrophic soft-tissue calcification, is a common and debilitating complication of adult and juvenile dermatomyositis. Dermatomyositis-associated calcinosis is difficult to treat and is associated with significant morbidity. The purpose of this review is to provide an update of treatment modalities for calcinosis in dermatomyositis based on published studies. Specific guidelines are lacking for calcinosis cutis management. Based on previous case reports, case series, cohort studies, and limited controlled studies, medications including diltiazem, bisphosphonates, sodium thiosulfate, aluminum hydroxide, warfarin, probenecid, colchicine, minocycline, and intravenous immunoglobulins have been used to control calcinosis progression in dermatomyositis, but no treatment has convincingly prevented or reduced calcinosis. Surgical excision of large or symptomatic calcium deposits remains the mainstay of treatment. Biologic therapies including infliximab, abatacept, rituximab, anakinra, and the oral JAK inhibitor tofacitinib have been used to control dermatomyositis-associated calcinosis in case reports and series. Pharmacological treatment aimed to reduce calcinosis is challenging given that no therapies have consistently been found to be effective and few studies have a high level of evidence. Randomized controlled trials using novel outcome measures are necessary to evaluate the efficacy of proposed and emerging therapies. Trial registration: clinicaltrials.gov NCT03639779 and NCT03267277

Calcinosis Universalis of the Elbow: A Rare Case with Classical Presentation.

Juvenile Dermatomyositis (JDM) is a rare autoimmune disease in children and adolescents. In these patients calcinosis might be the most characteristic symptom. However there are only few reported cases of intramuscular calcinosis in Dermatomyositis. We report a case of calcinosis universalis (CU) of the elbow in JDM successfully treated with broaching. The patient, a 24-year-old woman, suffered from a long history of JDM. On examination she presented with a fistula lateral to the olecranon and pain of the right elbow joint. Plain X-rays displayed a diffuse pattern of multiple periarticular, subcutaneous, and intramuscular calcifications. The patient underwent surgery for histological and microbiological sampling as well as broaching. Intraoperatively sinus formation and subfascial hard calcium deposition were found. Due to the risk of collateral tissue damage, incomplete broaching was performed. A local infection with Staphylococcus was diagnosed and treated with antibiotics. On six-week and 30-month follow-up the patient was free of pain and had very good function. Calcifications on standard radiographs had almost resolved entirely. This case report gives a summary on calcinosis in Dermatomyositis and adds a new case of recalcitrant CU to the literature. Broaching surgery proved to be a reliable treatment option in symptomatic calcinosis.

Identification of clinical features and autoantibodies associated with calcinosis in dermatomyositis.

Prior studies have estimated that up to 20% of adults with dermatomyositis (DM) have calcinosis, which can lead to significant morbidity. Identification of risk factors may provide a better understanding of the pathogenesis and ultimately therapy for this difficult clinical problem. Risk factors for calcinosis in adults with DM have not been extensively studied. To determine the prevalence of calcinosis and to identify associated clinical features in a cohort of extensively phenotyped adults with DM. A cross-sectional study of 126 patients diagnosed as having DM from January 1, 2006, through January 1, 2013, was performed. Patients were adults (≥18 years of age) attending the Stanford University Medical Center clinic. Calcinosis, defined as the presence of calcium deposition in the skin and subcutaneous tissues on physical examination. Fourteen patients (11.1%) had calcinosis, with the extremities most commonly involved. Patients with vs those without calcinosis had a longer disease duration (median, 6.9 years; range, 2.4-18.1; vs median, 3.9 years; range, 0.2-19.2 years; P = .003) and more fingertip ulcers (50.0% vs 9.3%, P < .001). An association between calcinosis and both interstitial lung disease and anti-MDA-5 autoantibodies was identified, but this association did not persist in multivariate models that adjusted for fingertip ulcers. Fingertip ulcers and disease duration were strongly associated with calcinosis in all multivariate models, independent of the underlying autoantibody present. Autoantibodies to NXP-2 were associated with calcinosis (odds ratio, 15.52; 95% CI, 2.01-119.90), whereas anti-transcriptional intermediary factor 1-γ antibodies were protective (odds ratio, 0.2; 95% CI, 0.01-0.99) in multivariate analyses that adjusted for fingertip ulcers and other covariates. Calcinosis was a relatively uncommon clinical feature in our cohort of adults with DM. Our data suggest that calcinosis is positively associated with longer disease duration, fingertip ulcers, and NXP-2 autoantibodies and negatively associated with transcriptional intermediary factor 1-γ antibodies. A common vascular mechanism may underlie the development of both calcinosis and fingertip ulcers in patients with DM.

Update on the treatment of calcinosis in dermatomyositis

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.

Calcinosis in dermatomyositis; observations on course of disease in children and adults.

Dermatomyositis is a disease of various cutaneous manifestations associated with an unpredictable degree of nonsuppurative polymyositis. Involvement of the skeletal muscles is always a factor and is responsible for the varying degrees of disability. The characteristic and unusual features of this disease have been described elsewhere.1-4Important among these is calcinosis cutis. As a result of our recent review of calcinosis in scleroderma,5 we decided to extend these investigations to include dermatomyositis. It was our purpose to examine the relationship of calcinosis to prognosis in dermatomyositis as well as to contrast its pattern of occurrence to that in scleroderma. From the records of approximately 260 patients seen at the Mayo Clinic from 1945 to 1955 with the diagnosis of dermatomyositis, sclerodermatomyositis, poikilodermatomyositis, and polymyositis, we selected 182 as representative of classic dermatomyositis; we rejected any case in which clinical and laboratory features