Calcinosis Biomarkers in Adult and Juvenile Dermatomyositis

Chung, Mo; Richardson, Carrie; Kirakossian, David; Orandi, Amir B.; Saketkoo, Lesley Ann; Rider, Lisa G.; Schiffenbauer, Adam; Mühlen, Carlos Alberto von; Chung, Lorinda

doi:10.1016/j.autrev.2020.102533

Cited by 45 publications

(28 citation statements)

References 107 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…There is an urgent need for accessible serum biomarkers which can alert the physician to ongoing disease activity-despite the fact that the JDM appears to be "inactive"-both to guide therapy and to explore more effective interventions. A series of recent studies have emerged, ranging from a review of biomarkers of calcinosis [81] to studies of possible novel mechanisms propelling their formation [82]. A comprehensive series of investigations have identified serological indicators reflecting endothelial and vascular disruption [83][84][85].…”

Section: Accessible Serum Biomarkersmentioning

confidence: 99%

Juvenile Dermatomyositis: New Clues to Diagnosis and Therapy

Pachman

Nolan

DeRanieri

et al. 2021

Curr Treat Options in Rheum

View full text Add to dashboard Cite

Purpose of review To identify clues to disease activity and discuss therapy options. Recent findings The diagnostic evaluation includes documenting symmetrical proximal muscle damage by exam and MRI, as well as elevated muscle enzymes—aldolase, creatine phosphokinase, LDH, and SGOT—which often normalize with a longer duration of untreated disease. Ultrasound identifies persistent, occult muscle inflammation. The myositis-specific antibodies (MSA) and myositis-associated antibodies (MAA) are associated with specific disease course variations. Anti-NXP-2 is found in younger children and is associated with calcinosis; anti-TIF-1γ+ juvenile dermatomyositis has a longer disease course. The diagnostic rash—involving the eyelids, hands, knees, face, and upper chest—is the most persistent symptom and is associated with microvascular compromise, reflected by loss of nailfold (periungual) end row capillaries. This loss is associated with decreased bioavailability of oral prednisone; the bioavailability of other orally administered medications should also be considered. At diagnosis, at least 3 days of intravenous methyl prednisolone may help control the HLA-restricted and type 1/2 interferon–driven inflammatory process. The requirement for avoidance of ultraviolet light exposure mandates vitamin D supplementation. Summary This often chronic illness targets the cardiovascular system; mortality has decreased from 30 to 1–2% with corticosteroids. New serological biomarkers indicate occult inflammation: ↑CXCL-10 predicts a longer disease course. Some biologic therapies appear promising.

show abstract

Section: Accessible Serum Biomarkersmentioning

confidence: 99%

Juvenile Dermatomyositis: New Clues to Diagnosis and Therapy

Pachman

Nolan

DeRanieri

et al. 2021

Curr Treat Options in Rheum

View full text Add to dashboard Cite

show abstract

“…Calcinosis presents in up to 40% of patients with JDM; 9,10 however, a frequency of 71% was described in a retrospective study of 21 JDM children in a tertiary hospital in South Africa. 11 Similarly, calcinosis in DM has been described in up to 20% of patients, 12 but in a cohort of 627 adult DM patients from China, only 35 (5.6%) were found to have calcinosis. 13…”

Section: Epidemiologymentioning

confidence: 99%

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

Valenzuela

Chung

2021

Journal of Scleroderma and Related Disorders

Self Cite

View full text Add to dashboard Cite

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.

show abstract

“…However, it is only about half as common in adults, with a prevalence of about 20% and tends to occur later in the disease course (59,60). DM-associated calcinosis can be categorized into four overlapping types-calcinosis circumscripta, which appears as superficial, discrete papules or nodules on the skin and around joints; tumoral calcinosis, which occurs deeper in the dermis, subcutaneous tissue, muscle, and/or fascia; deposits along the myofascial planes known a calcinosis universalis; or an extensive, generalized form known as exoskeletal calcinosis (56,57,(61)(62)(63). The lesions may be associated with significant pain and discomfort, particularly if concomitant ulceration, panniculitis, and secondary infection occur (56).…”

Section: Calcinosismentioning

confidence: 99%

Covert clues: the non-hallmark cutaneous manifestations of dermatomyositis

Castillo¹,

Femia²

2021

Ann Transl Med

View full text Add to dashboard Cite

Dermatomyositis (DM) is a strikingly heterogenous disease characterized by a broad and everevolving spectrum of cutaneous manifestations that transcend the classic "hallmarks" defined by Peter and Bohan in 1975. Despite the increasing preponderance and ubiquity of autoantibody, radiologic, and electrophysiologic testing, the diagnosis of DM still hinges largely on prompt detection of cutaneous manifestations of this condition. While pathognomonic cutaneous features of DM are more readily recognizable, many patients present with subtle and/or atypical skin manifestations, and diagnosis of DM may require clinician identification of these cutaneous clues. In this review, we highlight several of the lesserknown skin manifestations of DM, specifically, panniculitis, diffuse subcutaneous edema, erythroderma, calcinosis, ulceration, flagellate erythema, Wong-type DM, gingival telangiectasias, and the ovoid palatal patch. We describe the clinical and histopathologic presentation of these cutaneous findings. While manifesting less frequently than the heliotrope rash, Gottron's papules, and Gottron's sign, these cutaneous clues are equally important for clinicians to recognize in order to facilitate timely diagnosis and early intervention.

show abstract

Calcinosis Biomarkers in Adult and Juvenile Dermatomyositis

Cited by 45 publications

References 107 publications

Juvenile Dermatomyositis: New Clues to Diagnosis and Therapy

Juvenile Dermatomyositis: New Clues to Diagnosis and Therapy

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

Covert clues: the non-hallmark cutaneous manifestations of dermatomyositis

Contact Info

Product

Resources

About