"Calcinosis"

Patient Experience of Systemic Sclerosis-Related Calcinosis: An International Study Informing Clinical Trials, Practice, and the Development of the Mawdsley Calcinosis Questionnaire. Systemic sclerosis (SSc) -related calcinosis can be a debilitating, constantly painful, poorly understood vascular complication of calcium hydroxyapatite deposition in soft tissue structures that affects approximately 40% of both limited and diffuse cutaneous SSc subtypes. This publication describes the iterative and multitiered international qualitative investigations that yielded remarkable insights into natural history, daily experience, and complications of SSc-calcinosis providing pivotal information for health management. Patient-driven question development and field testing, according to Food and Drug

Spatial Atlas for Mapping Vascular Microcalcification Using 18F-NaF PET/CT: Application in Hyperphosphatemic Familial Tumoral Calcinosis. Vascular calcification causes significant morbidity and occurs frequently in diseases of calcium/phosphate imbalance. Radiolabeled sodium fluoride positron emission tomography/computed tomography has emerged as a sensitive and specific method for detecting and quantifying active microcalcifications. We developed a novel technique to quantify and map total vasculature microcalcification to a common space, allowing simultaneous assessment of global disease burden and precise tracking of site-specific microcalcifications across time and individuals. To develop this technique, 4 patients with hyperphosphatemic familial tumoral calcinosis, a monogenic disorder

Local injection of infliximab into calcinosis lesions in patients with juvenile dermatomyositis (JDM): a clinical trial. Juvenile Dermatomyositis (JDM) is a rare autoimmune disorder that primarily affects muscles and skin. One of the severe complications associated with JDM is calcinosis, and treating this condition presents significant challenges. This study aimed to evaluate the efficacy and safety of local injection of infliximab into calcinosis lesions in patients with JDM. In this clinical trial, five patients diagnosed with JDM and calcinosis lesions were enrolled. The primary treatment consisted of weekly infliximab injections for 16 weeks, targeting all four sides of each lesion. Lesion dimensions, including length and width, were documented and monitored weekly. Before

Structural and Molecular Imaging-based Characterization of Soft Tissue and Vascular Calcification in Hyperphosphatemic Familial Tumoral Calcinosis. Hyperphosphatemic Familial Tumoral Calcinosis (HFTC) is a rare disorder caused by deficient FGF23 signaling and resultant ectopic calcification. In this study, we systematically characterized and quantified macro- and micro-calcification in an HFTC

Calcinosis in dermatomyositis. To provide the most recent literature on our understanding behind the pathogenesis and the treatment of calcinosis in dermatomyositis. Early diagnosis and controlling the overall disease activity are cornerstones to prevent calcinosis in juvenile dermatomyositis. Observational cohort studies showed that prolonged state of inflammation and features of vascular dysfunction like digital ulcers and abnormal nailfold capillaries are associated with calcinosis. Neutrophil activation and mitochondrial dysfunction have recently emerged as potential mechanistic pathways involved in calcinosis pathogenesis. Few recent case series have alluded to the efficacy of topical and intralesional sodium thiosulfate, while JAK inhibitors appear to be newer promising therapy

Mining for Gems: Early Detection of Calcinosis in Juvenile Dermatomyositis Using Low-Dose Computed Tomography. Calcinosis, a common complication in juvenile dermatomyositis (JDM), affects up to 40% of patients and can be associated with uncontrolled disease activity and morbidity.A 9-year-old female with a 17-month history of JDM presented with worsened bilateral knee pain.

Excessive collagen fiber deposition in idiopathic scrotal calcinosis: a case report. Idiopathic scrotal calcinosis (ISC) is a manifestation of idiopathic calcinosis cutis, and its etiology is still unknown. We report a 36-year-old patient manifested multiple gradually increasing yellowish-white scrotal nodules with occasional itching and stinging in the past 6 years and was successfully cured via surgical excision. The laboratory test combined with pathological analysis confirmed the diagnosis of ISC. Like pathological calcinosis in other soft tissues, a large amount of collagen fiber deposition was observed around the calcification nodule, suggesting that abnormal collagen fiber deposition might be an important factor leading to idiopathic calcinosis in the scrotum. Moreover, koilocytes

Infected Tumoral Calcinosis Associated With Rheumatoid Arthritis. Tumoral calcinosis (TC) is a syndrome of unknown etiology characterized by calcium salt deposition in soft tissues. A case of infected TC with rheumatoid arthritis (RA) is described.

Scrotal Calcinosis in Juvenile Systemic Sclerosis. Calcinosis cutis is the deposition of insoluble calcium in the skin and subcutaneous tissues, frequently associated with autoimmune connective tissue diseases, including systemic sclerosis (SSc). Calcinosis in SSc presents as subcutaneous nodules of variable size and shape, most frequently on the fingers, but can also affect other locations..

Dermatomyositis-associated Calcinosis Cutis on Digital Breast Tomosynthesis. .

A case report of uremic tumoral calcinosis in the head and neck and literature review of calcified lesions of the head and neck. Soft-tissue calcifications in the head and neck are relatively common and are the result of a wide variety of benign and malignant pathologies. They can present a diagnostic challenge given the broad range of underlying etiologies. Uremic tumoral calcinosis (UTC prolapse, and ESRD secondary to diabetes mellitus type II (DMII) on peritoneal dialysis who was found to have large, heterogeneous, bilateral calcified neck masses. Given her clinical history, laboratory results, and imaging findings, she was diagnosed with UTC. In addition to this case, we provide an overview of tumoral calcinosis (TC) and a differential diagnosis for calcified lesions of the head

Different outcomes following parathyroidectomy in patients with uremic tumoral calcinosis: two case reports. Uremic tumoral calcinosis (UTC) is a rare complication in hemodialysis patients, whose mechanism remains incompletely understood. We report two cases with UTC who experienced completely different patterns of regression following parathyroidectomy, although there were no significant

Calcinosis universalis accompanied by symptomatic systemic sclerosis. Calcinosis cutis is represented by the deposition of insoluble calcium salts in the skin and subcutaneous tissue. Calcinosis can lead to repeated episodes of local inflammation and repeated infections, resulting in pain and functional disability, and even death. Here, we present a case of a patient with SSc who experienced calcinosis universalis and eventually died from recurrent infections at the sacrococcygeal calcification.

Calcinosis in systemic sclerosis. To provide updated information on the prevalence, pathogenesis, diagnostics, and therapeutics of calcinosis cutis associated with systemic sclerosis (SSc). Observational studies show ethnic and geographical differences in the prevalence of calcinosis. In addition to clinical and serological associations, biochemical studies and in-vivo models have attempted to explain theories behind its pathogenesis, including prolonged state of inflammation, mechanical stress, hypoxia, and dysregulation in bone and phosphate metabolism. Long-term use of proton pump inhibitors may increase the risk for calcinosis in SSc. Few single center observational studies have shown mild benefit with minocycline and topical sodium thiosulfate. Calcinosis cutis is the deposition

Therapeutic success of sodium thiosulfate in treating cutaneous calciphylaxis in a patient with hyperphosphataemic familial tumoral calcinosis. Calciphylaxis is a potencially disorder in patients with hyperphosphatemic familial tumoral calcinosis (HFTC). Patients commonly present livedo racemosa and retiform purpura, which may progress to necrosis and very painful ulcers. Treatment with sodium

Early Abnormal Nailfold Capillary Changes Are Predictive of Calcinosis Development in Juvenile Dermatomyositis. The long-term outcomes of juvenile dermatomyositis (JDM) are more favorable in recent years. However, calcinosis is still among the complications that can cause serious functional impairment. Little is known about the pathogenesis and risk factors of calcinosis. The aim of this study is to determine risk factors for the development of calcinosis in JDM. This was a single-center, retrospective cohort study. All patients were diagnosed and followed at the multidisciplinary JDM clinic of The Hospital for Sick Children, from January 1, 1989, until May 31, 2018. To investigate predictors of incident calcinosis, Cox regression analysis was performed. A total of 172 patients met inclusion criteria

Management of Calcinosis Cutis in Rheumatic Diseases. Calcinosis (hydroxyapatite and calcium phosphate crystal deposition) within the extracellular matrix of the dermis and subcutaneous tissue is a frequent manifestation of adult and pediatric systemic autoimmune rheumatic diseases, specifically systemic sclerosis, dermatomyositis, mixed connective tissue disease, and systemic lupus erythematosus . In this article, we review classification of calcinosis, highlight mechanisms that may contribute to the pathogenesis of calcinosis, and summarize the evidence evaluating nonpharmacologic and pharmacologic interventions for the treatment of calcinosis.

PTH and FGF23 Exert Interdependent Effects on Renal Phosphate Handling: Evidence From Patients With Hypoparathyroidism and Hyperphosphatemic Familial Tumoral Calcinosis Treated With Synthetic Human PTH 1-34. Parathyroid hormone (PTH) and fibroblast growth factor 23 (FGF23) both influence blood phosphate levels by regulating urinary phosphate reabsorption. Clinical data suggest that adequate renal phosphate handling requires the presence of both FGF23 and PTH, but robust evidence is lacking. To investigate whether the phosphaturic effects of PTH and FGF23 are interdependent, 11 patients with hypoparathyroidism, which features high blood phosphate in spite of concomitant FGF23 elevation, and 1 patient with hyperphosphatemic familial tumoral calcinosis (HFTC), characterized by deficient

Calcinosis in systemic sclerosis. The aim of this study was to provide updated information on the prevalence, pathogenesis, diagnostics and therapeutics of calcinosis cutis associated with systemic sclerosis (SSc). Observational studies show ethnic and geographical differences in the prevalence of calcinosis. In addition to clinical and serological associations, biochemical studies and in-vivo models have attempted to explain theories behind its pathogenesis, including prolonged state of inflammation, mechanical stress, hypoxia and dysregulation in bone and phosphate metabolism. Long-term use of proton pump inhibitors may increase the risk for calcinosis in SSc. Few single-centre observational studies have shown mild benefit with minocycline and topical sodium thiosulfate. Calcinosis cutis

Carpal tunnel syndrome secondary to tumoral calcinosis: a case report and review of the literature. Carpal Tunnel Syndrome (CTS) is the most prevalent peripheral nerve entrapment disease. Its pathophysiology is multifactorial and defined as idiopathic in most cases. We present a rare case of CTS secondary to tumoral calcinosis and then searched the English literature to present the details in the carpal tunnel. Subsequently, carpal tunnel release and mass excision were successfully performed with no recurrence at a 3-month interval. CTS secondary to tumoral calcinosis is a rare benign condition. Physicians should remain vigilant and include it in their differential diagnosis when facing a previously healthy patient presenting for chronic CTS symptoms.