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Calcinosis

Calcinoses


Presentation

  • We believe this is the first presentation in the literature of Mönckeberg medial calcinosis visualized in the infraorbital arteries.[ncbi.nlm.nih.gov]
  • Presently, rituximab cannot be recommended for this indication in the absence of successful controlled trials.[ncbi.nlm.nih.gov]
  • Several classifications based on the etiology, the location of brain calcifications and the clinical presentation have been proposed.[ncbi.nlm.nih.gov]
  • These nodules usually present as painless, small, firm, mobile solitary cutaneous lesions with a predilection for the upper eyelid. Diagnosis is confirmed by histopathology, and treatment is with surgical excision.[ncbi.nlm.nih.gov]
  • We present a unique case of progressive acro-osteolysis of the distal phalanges and articular calcifications in a patient with scleroderma. The calcified deposit in a proximal interphalangeal joint was excised under local anesthesia.[ncbi.nlm.nih.gov]
Soft Tissue Swelling
  • Calcinosis symptoms included painful deposits (88 %), soft tissue swelling (66 %), and recurrent infections (44 %) from these deposits. On DECT, calcinosis deposits had the same color and density as the bone and no MSU was found.[ncbi.nlm.nih.gov]
  • If there is an associated underlying collagen vascular disease findings of soft tissue swelling and joint arthropathy may be present. Promoted articles (advertising)[radiopaedia.org]
Turkish
  • Copyright 2016 Turkish Association of Orthopaedics and Traumatology. Production and hosting by Elsevier B.V. All rights reserved.[ncbi.nlm.nih.gov]
Flexion Contracture
  • However, their CT images showed better details of the calcinosis locations in the soft tissues as well as the bone destruction, especially if there was overlying bulky deposits or flexion contractures.[ncbi.nlm.nih.gov]
  • On physical examination the patient appeared cachectic, with normal cardiac, pulmonary and abdominal examination, and characteristic sclerodermatous skin changes, including diffuse thickening, taut fingers with flexion contractures, the typical microstomia[ard.bmj.com]
Hand Deformity
  • However, CT imaging was superior to plain radiographs in locating these deposits within the soft tissues and may be a useful tool to study SSc-calcinosis affecting the hands, particularly in the setting of progressive hand deformities.[ncbi.nlm.nih.gov]
Leg Pain
  • Three months before consultation, he experienced bilateral leg pain accompanied by progressive hardening of muscles and the presence of small nodules around the elbows and submandibular region.[ncbi.nlm.nih.gov]
Scrotal Mass
  • Ultrasound scanning of the scrotum revealed calcified scrotal mass [Figure 1] b. A tentative diagnosis of right scrotal mass was made. Patient was prepared for surgical exploration with biopsy.[archintsurg.org]

Workup

  • A hormonal workup, including calcitonin, parathyroid hormone, and thyroid hormone levels, was also within normal limits.[karger.com]
  • Laboratory workup revealed normal blood counts, serum calcium, phosphorous, and alkaline phosphate levels.[jcnonweb.com]
Soft Tissue Calcification
  • Radiographically, idiopathic tumoral calcinosis demonstrates lobulated, cloudy soft-tissue calcifications near a joint (A).[jaocr.org]
  • Soft tissue calcifications may be easily demonstrated on plain radiographs, but CT scan is more effective in demonstrating the location and extent of calcifications.[sajr.org.za]
  • Click here to view Figure 2: Elbow X-ray: soft tissue calcification and vascular calcification. Click here to view Figure 3: a and b: Hands and knee X-ray showing vascular calcifications.[sjkdt.org]
  • Scintigraphic research showed no pathology in the thyroid and parathyroid glands but revealed soft-tissue calcification. A chest roentgenogram, blood tests, and testing of stools for occult blood showed no indication of internal malignancy.[mdedge.com]
  • There is also diffuse soft tissue calcification present (red arrows. Tumoral Calcinosis: Pearls, Polemics and Alternative Possibilities. KM Olsen and FS Chew. RadioGraphics. May-June, 2006, Vol. 26, 3.[learningradiology.com]
Muscle Calcification
  • Pathology: Calcinosis may affect the skin, subcutaneous layers including the fascia and muscle. Calcific lesions are rich in hydroxyapatite but may also contain calcium oxalate and uric acid in crystalline form.[rheumaknowledgy.com]

Treatment

  • Experienced physicians are more likely to use non-immunosuppressive treatments.[ncbi.nlm.nih.gov]
  • Progression of calcinosis was still evident 6 and 12 months after anti-CD20 treatment. Many treatments have been tried to treat calcinosis without demonstrated effectiveness.[ncbi.nlm.nih.gov]
  • Case 6 had a long duration of severe active disease before treatment initiation. No important adverse event was observed.[ncbi.nlm.nih.gov]
  • Less frequent, but severe if present, were psychotic symptoms, with different grade of structure and emotional involvement, and always resistant to treatment.[ncbi.nlm.nih.gov]
  • With a lower response to medication, surgical treatment is considered the only option for treating symptomatic lesions of calcinosis in order to improve quality of life.[ncbi.nlm.nih.gov]

Prognosis

  • Treatment and prognosis No established therapy is recognized, but calcium chelates (EDTA) bisphosphonates, and steroids have been used with little consistent benefit.[radiopaedia.org]
  • Prognosis : Lesions tend to be chronic and less commonly are relapsing or remittent. Calciphylaxis has a significant mortality rate of 60% to 80%. BIBLIOGRAPHY Khafif RA, DeLima C, Silverberg A, et al. Calciphylaxis and systemic calcinosis.[rheumaknowledgy.com]
  • Surgical excision is the treatment of choice and has an excellent prognosis. This case series attempts to create an awareness of this rare pathology, which is often missed out on routine investigations and misdiagnosed. References 1.[ijdpdd.com]
  • Infectious complications of these lesions are associated with poor prognosis. [4] Tumoral calcinosis is a rare inherited metabolic disorder characterized by massive calcium and phosphate deposits. [5] It is rare in dialyzed patients, [6] and its pathogenesis[sjkdt.org]
  • It is also important to follow-up these patients as the cardiovascular prognosis is poor due to accelerated vascular calcification.[elsevier.es]

Etiology

  • Several classifications based on the etiology, the location of brain calcifications and the clinical presentation have been proposed.[ncbi.nlm.nih.gov]
  • Abstract Idiopathic scrotal calcinosis (ISC) is characterised by the presence of multiple firm scrotal nodules of unknown or controversial etiology. It is a rare, yet benign, entity of the scrotal skin.[ncbi.nlm.nih.gov]
  • It is classified into 4 major types according to etiology: dystrophic, metastatic, iatrogenic, and idiopathic.[ajnr.org]
  • Etiology Although most cases are idiopathic there is an association with Raynaud syndrome, scleroderma or dermatomyositis in up to 40% of cases. Fine clustered calcifications are seen, usually around the finger and toe tips.[radiopaedia.org]

Epidemiology

  • PURPOSE OF REVIEW: This article reviews the most updated literature regarding the epidemiology, pathophysiology, diagnosis, and treatment of calcinosis cutis in patients with systemic sclerosis (SSc).[ncbi.nlm.nih.gov]
  • 10.1097/BOR.0000000000000539 RAYNAUD PHENOMENON, SCLERODERMA, OVERLAP SYNDROMES AND OTHER FIBROSING SYNDROMES: Edited by John Varga Abstract Author Information Authors Article Metrics Metrics To provide an update on the available literature regarding the epidemiology[journals.lww.com]
  • This article reviews the most updated literature regarding the epidemiology, pathophysiology, diagnosis, and treatment of calcinosis cutis in patients with systemic sclerosis (SSc). PubMed, Curr Opin Rheumatol, 2015 Nov;27(6):542–8.[sclero.org]
  • Epidemiology Calcinosis cutis commonly occurs in patients with systemic sclerosis, especially the limited form (CREST).[ncbi.nlm.nih.gov]
  • Epidemiologically, most ISC presentations occur in the third to fourth decade of life, following years of insidious onset without any systemic disorder of the calcium/phosphorus metabolism. 1 To the best of our knowledge, 10 cases of ISC have been reported[dovepress.com]
Sex distribution
Age distribution

Pathophysiology

  • PURPOSE OF REVIEW: This article reviews the most updated literature regarding the epidemiology, pathophysiology, diagnosis, and treatment of calcinosis cutis in patients with systemic sclerosis (SSc).[ncbi.nlm.nih.gov]
  • .0000000000000539 RAYNAUD PHENOMENON, SCLERODERMA, OVERLAP SYNDROMES AND OTHER FIBROSING SYNDROMES: Edited by John Varga Abstract Author Information Authors Article Metrics Metrics To provide an update on the available literature regarding the epidemiology, pathophysiology[journals.lww.com]
  • The pathophysiology of calcinosis cutis is not well understood, and there is a broad range of severity seen, from benign localized, small nodules to large, severely debilitating lesions (2).[clinicaltrials.gov]
  • Pathophysiologically, MPC consists of calcium deposition in the epithelial and vascular basement membrane of alveoli, bronchial walls, and media layer of pulmonary arteries, resulting in a lymphoproliferative interstitial response and pulmonary fibrosis[revistanefrologia.com]
  • Fibroblast growth factor 23 and Klotho: physiology and pathophysiology of an endocrine network of mineral metabolism. Annu Rev Physiol, 75 (2013), pp. 503-533 [5] C. Bergwitz, H. Jüppner.[revistanefrologia.com]

Prevention

  • With a long evolution, the aim of the treatment is to control the symptoms and prevent complications. In this article, we present the challenging management of a profuse lesion of calcinosis in a patient with systemic sclerosis.[ncbi.nlm.nih.gov]
  • A complete excision prevents recurrence. Psychological support is required in association with surgery because the lesions are benign and concern an intimate part of the body.[ncbi.nlm.nih.gov]
  • This classification awaits validation for a possible use in clinical practice and to support early treatment and prevention of complications. The Author 2016. Published by Oxford University Press on behalf of the British Society for Rheumatology.[ncbi.nlm.nih.gov]
  • Your skin Holds body fluids in, preventing dehydration Keeps harmful microbes out, preventing infections Helps you feel things like heat, cold, and pain Keeps your body temperature even Makes vitamin D when the sun shines on it Anything that irritates[icdlist.com]
  • Rapid and intensive treatment escalation in patients with scleroderma and refractory Raynaud's phenomenon is one of the most effective preventive action available in order to avoid the development of digital ulcers and tissue loss.[reumatologiaclinica.org]

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