Dystrophic calcinosis with both a huge calcified mass in the cervical spine and calcification in the chest wall in a patient with rheumatoid overlap syndrome

Abstract

Dystrophic calcinosis in soft tissue occurs in damaged or devitalized tissues in the presence of normal calcium and phosphorous metabolism. It is often noted in subcutaneous tissues in patients with collagen vascular diseases and may involve a relatively localized area or be widespread. A 74-year-old Japanese woman with an overlap of rheumatoid arthritis, Sjögren's syndrome, and systemic sclerosis developed a huge tumor-like mass at the atlanto-axial vertebral joint region that caused severe cervical pain and difficulty in activities of daily living. She also had subcutaneous dystrophic calcification in the soft tissue of the chest wall. Calcinosis associated with systemic sclerosis is a well-recognized phenomenon, but a destructive paraspinal tumor in the cervical spine associated with overlap syndrome is extremely unique. Because calcinosis in spinal locations can be complicated by neurological involvement, patients with progressive symptoms may require surgical intervention. Surgical resection and biological therapy improved this patient's life and activities of daily living. Calcinosis is common in the conditions reviewed here, and different agents have been used for treatment. However, calcinosis management is poorly organized and lacks an accepted classification, systematic studies, and clinical therapeutic trials. The association of calcinosis and collagen vascular diseases is clinically and etiologically important. Although a combination of calcinosis and rheumatoid overlap syndrome is rare, various collagen vascular diseases may occur simultaneously. A perceptive diagnostic approach toward these diseases is critical, and early diagnosis and treatment are needed to prevent dystrophic calcinosis.

Intraspinal calcinosis mimicking intervertebral disc extrusion: A clinical and surgical case report

“…[ 4 5 6 ] Serum levels of calcium and phosphorus in patients with dystrophic calcinosis, including our patient, are usually within normal limits. There is a fifth type of calcinosis called calciphylaxis because of the presence of a vitamin D or parathyroid hormone playing the role of a “calcifer.”[ 5 ]…”

“…Subcutaneous calcinosis occurs in both types of systemic sclerosis, and is more common in patients with limited scleroderma as part of the CREST syndrome. [ 2 5 ] Paraspinal and intraspinal calcifications (calcinosis) rarely occur in the cervical followed by the thoracic and even more rarely the lumbar spine where they can lead to severe neurological deficits warranting operative decompression. [ 3 5 11 ] Here, we present a patient with severe systemic sclerosis who exhibited marked left-sided L4-L5 lumbar calcinosis, mimicking a disc herniation, that warranted such operative decompression.…”

Parathyroidectomy May Cause Remission of Uraemic Tumoral Calcinosis in Haemodialysis Patients

“…The pathogenesis of UTC has not been elucidated, and some researchers believe that the disease is caused by autosomal dominant genetic malformation [3,11]. Other researchers consider that a primary defect of the collagen tissue causes a stress response of the collagen fibres around the diseased joint [12]. Some scholars believe that the abnormal metabolism of calcium, phosphorus, and cholesterol results in the ectopic distribution of calcium [13].…”

Systemic sclerosis-associated compressive cervical calcinosis: intersection of degenerative spine instability, lesional spinal cord compression, and traumatic spinal cord injury. Illustrative case

“…We limited our search to patients with a confirmed diagnosis of scleroderma or systemic sclerosis; cases in which individual patients, their symptoms, imaging, and treatment courses, could be identified and reviewed; and English language articles. This returned 19 cases [10][11][12][13][14][15][21][22][23][24][25][26][27][28][29][30][31] (Table 1). Fifteen of 19 (78.9%) affected patients were women, suggesting a female predominance.…”