Systemic sclerosis and calcinosis cutis: response to rituximab

Poormoghim, Hadi; Amini, Elham; Almasi, Alireza; Hadibigi, E.

doi:10.1111/jcpt.12340

Cited by 13 publications

(3 citation statements)

References 6 publications

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“…For example, Moazedi-Fuerst et al [164] observed the resolution of calcinosis cutis in three patients with SSc after six months of rituximab 500 mg on day 0 and day 14, and then twice every three months, with no relapse after 1-2 years follow-up, suggesting the efficacy of B-cell depletion therapy as an option in the treatment in these patients. Similarly, other authors reported the efficacy of low-dose rituximab (four weekly infusions of 375 mg/m 2 ) on calcinosis cutis in SSc patients [160,161,165,171]. On the contrary, evidence on DM/JM-related calcinosis cutis is limited and non-conclusive.…”

Section: The Potential Role Of Immunosuppressive Therapiesmentioning

confidence: 93%

Calcinosis Cutis and Calciphylaxis in Autoimmune Connective Tissue Diseases

et al. 2023

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Calcinosis represents a severe complication of several autoimmune disorders. Soft-tissue calcifications have been classified into five major types: dystrophic, metastatic, idiopathic, iatrogenic, and calciphylaxis. Autoimmune diseases are usually associated with dystrophic calcifications, including calcinosis cutis, occurring in damaged or devitalized tissues in the presence of normal serum levels of calcium and phosphate. In particular, calcinosis cutis has been described in dermatomyositis, polymyositis, juvenile dermatomyositis, systemic sclerosis, systemic lupus erythematosus, primary Sjögren’s syndrome, overlap syndrome, mixed connective tissue disease, and rheumatoid arthritis. Calciphylaxis, a severe and life-threatening syndrome presenting with vascular calcifications and thrombosis, has also been associated with some autoimmune conditions. Due to the potentially disabling character of calcinosis cutis and calciphylaxis, physicians’ awareness about the clinical presentation and management of these diseases should be increased to select the most appropriate treatment option and avoid long-term complications. In this review, we aim to analyze the clinical features of calcinosis cutis and calciphylaxis associated with autoimmune diseases, and the main treatment strategies evaluated up to now for treating this potentially disabling disease.

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Section: The Potential Role Of Immunosuppressive Therapiesmentioning

confidence: 93%

Calcinosis Cutis and Calciphylaxis in Autoimmune Connective Tissue Diseases

et al. 2023

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“…A recent case report of a 16-year-old JDM patient with calcinosis and skin ulcerations showed complete resolution without radiologic progression or new lesions after 3 months of abatacept. 107…”

Section: Abataceptmentioning

confidence: 99%

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

Valenzuela

Chung

2021

Journal of Scleroderma and Related Disorders

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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.

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“…Among them, conservative treatments such as medication and follow-up observation are ineffective, and laser or cryotherapy are prone to recurrence [ 23 ]. Therefore, surgical treatment is more often used to excise the diseased tissue intact, and direct suturing, transfer of tipped flaps, and free skin pieces can be chosen according to the skin defect [ 15 , 19 , 24 ]. In this case, the lesion was large and multiple hard nodes were fused into a block, but the lesion was located in the middle and upper part of the scrotum, and the lower part of the skin was intact and the scrotal skin was more extensible, so direct suture was used to close the skin.…”

Section: Discussionmentioning

confidence: 99%