Calcinosis Cutis and Calciphylaxis in Autoimmune Connective Tissue Diseases

Abstract

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.

Calcinosis in Rheumatic Disease Is Still an Unmet Need: A Retrospective Single-Center Study

“…In our study involving 872 patients with immune-mediated rheumatic diseases, calcinosis was observed in 86 individuals (9.7%), aligning with a previously reported prevalence ranging from 8.45% to 19.54% [ 2 , 15 , 26 ]. Calcinosis was more common in SSc, affecting 20% of patients (64/310).…”

“…Similar to Mexican and Canadian cohorts [ 3 , 27 ], our patient population mainly had diffuse cutaneous systemic sclerosis, accounting for 59.37%. The most commonly involved sites were the hands and feet, especially the fingers, consistent with findings in prior studies [ 15 , 28 ]. Additionally, SSc patients often exhibited ulcers (55%), impacting 81% of dcSSc and 56% of lcSSc patients.…”

“…Common clinical symptoms of calcinosis include pain, recurrent local inflammation, ulcers with infections, and deformities, causing functional impairment. Calcinosis can also lead to compression neuropathies, with motor and sensory deficits, with a higher likelihood of skin ulceration in areas like the forearm, elbows, fingers (especially the volar aspect of fingertips), and metacarpophalangeal and interphalangeal joints [ 15 ]. Radiological investigations can contribute to early diagnosis, treatment guidance, and prognosis assessment [ 16 ].…”

Calcinosis universalis accompanied by symptomatic systemic sclerosis

“…6 Dystrophic calcifications are most commonly seen in patients with autoimmune connective tissue diseases such as SSc, dermatomyositis and SLE. 7 Serum testing for calcium, phosphorus and parathyroid hormone levels usually does not reveal any significant results in patients with dystrophic calcifications. 6 Calcinosis cutis lesions often appear as subcutaneous nodules in individuals with SSc.…”

“…Generally, there are five different subtypes of soft tissue calcifications based on their causes: metastatic, tumoural, idiopathic, calciphylactic and dystrophic 6 . Dystrophic calcifications are most commonly seen in patients with autoimmune connective tissue diseases such as SSc, dermatomyositis and SLE 7 . Serum testing for calcium, phosphorus and parathyroid hormone levels usually does not reveal any significant results in patients with dystrophic calcifications 6 .…”