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Felix S Chew - One of the best experts on this subject based on the ideXlab platform.
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Tumoral Calcinosis: Pearls, Polemics, and Alternative Possibilities
Radiographics, 2006Co-Authors: Kathryn M. Olsen, Felix S ChewAbstract:Massive periarticular Calcinosis of the soft tissues is a unique but not rare radiographic finding. On the contrary, tumoral Calcinosis is a rare familial disease. Unfortunately, the term tumoral Calcinosis has been liberally and imprecisely used to describe any massive collection of periarticular calcification, although this term actually refers to a hereditary condition associated with massive periarticular calcification. The inconsistent use of this term has created confusion throughout the literature. More important, if the radiologist is unfamiliar with tumoral Calcinosis or disease processes that mimic this condition, then diagnosis could be impeded, treatment could be delayed, and undue alarm could be raised, possibly leading to unwarranted surgical procedures. The soft-tissue lesions of tumoral Calcinosis are typically lobulated, well-demarcated calcifications that are most often distributed along the extensor surfaces of large joints. There are many conditions with similar appearances, including ...
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Tumoral Calcinosis: pearls, polemics, and alternative possibilities.
RadioGraphics, 2006Co-Authors: Kathryn M. Olsen, Felix S ChewAbstract:Massive periarticular Calcinosis of the soft tissues is a unique but not rare radiographic finding. On the contrary, tumoral Calcinosis is a rare familial disease. Unfortunately, the term tumoral Calcinosis has been liberally and imprecisely used to describe any massive collection of periarticular calcification, although this term actually refers to a hereditary condition associated with massive periarticular calcification. The inconsistent use of this term has created confusion throughout the literature. More important, if the radiologist is unfamiliar with tumoral Calcinosis or disease processes that mimic this condition, then diagnosis could be impeded, treatment could be delayed, and undue alarm could be raised, possibly leading to unwarranted surgical procedures. The soft-tissue lesions of tumoral Calcinosis are typically lobulated, well-demarcated calcifications that are most often distributed along the extensor surfaces of large joints. There are many conditions with similar appearances, including the Calcinosis of chronic renal failure, Calcinosis universalis, Calcinosis circumscripta, calcific tendonitis, synovial osteochondromatosis, synovial sarcoma, osteosarcoma, myositis ossificans, tophaceous gout, and calcific myonecrosis. The radiologist plays a critical role in avoiding unnecessary invasive procedures and in guiding the selection of appropriate tests that can result in a conclusive diagnosis of tumoral Calcinosis.
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progressive systemic sclerosis with massive paraspinal soft tissue Calcinosis
American Journal of Roentgenology, 2004Co-Authors: Kathryn M. Olsen, Eric J Pike, Felix S ChewAbstract:but normal calcium levels. Inclan et al. specified that this condition occurred in the absence of renal, metabolic, or collagen vascular disease. However, the term “tumoral Calcinosis” has gradually and imprecisely encompassed all tumorlike multilobulated calcific deposits in a paraarticular distribution in soft tissues. Because of the expanding definition in the literature, lesions now have a pathologically based classification: primary normophosphatemic tumoral Calcinosis, primary hyperphosphatemic tumoral Calcinosis, and secondary tumoral Calcinosis [2]. Primary forms of tumoral Calcinosis are less common than the secondary forms, which may be associated with renal failure, hemodialysis, collagen vascular disease, sarcoidosis, pseudoxanthoma elasticum, massive osteolysis, and other conditions. Patients present with a mass, regional compressive symptoms, or both, but pain and radiculopathy have also been described. Most of these lesions are related to the extensor surfaces of the hip, shoulder, elbows, wrists, and feet and likely originate in adjacent bursae. Rare locations also include dental and paraspinal regions [3]. Lesions of tumoral Calcinosis consist of macroscopic masses of amorphous calcified material, sometimes in aqueous suspension, surrounded by chronic fibrotic and inflammatory reaction. A paraarticular distribution is common. Conventional radiographs show a characteristic lobulated mass of several centimeters with amorphous calcific deposits. Although a cystic component may be present, evident as a fluid‐ sedimentation level, this feature is usually absent when tumoral Calcinosis is associated with connective tissue disease [4]. Bone scintigraphy shows increased radiotracer activity in the corresponding paraarticular distribution. CT shows soft-tissue Calcinosis without direct communication with adjacent bones or joints. Lesions with a cystic component may have high-attenuation septations as compared with the low-attenuation centers. Calcium layering, known as the “sedimentation” sign, may be seen. MRI may show fluid‐fluid levels with high signal layered over low signal on fluid-sensitive sequences [5]. If symptomatic, lesions of tumoral Calcinosis may be excised. Although recurrent lesions are common, their growth in secondary tumoral Calcinosis may be retarded with treatment of the underlying condition.
Lorinda Chung - One of the best experts on this subject based on the ideXlab platform.
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ultrasound detection of Calcinosis and association with ulnar artery occlusion in patients with systemic sclerosis
Arthritis Care and Research, 2020Co-Authors: Robert Fairchild, Melody P Chung, Lorinda Chung, Laurel SharplessAbstract:OBJECTIVE To investigate the ability of ultrasound (US) compared to radiographs in detecting Calcinosis in hands/wrists of systemic sclerosis (SSc) patients, and assess US markers of pathologic perfusion. METHODS SSc patients were evaluated for Calcinosis in the hands/wrists by X-ray and US. Presence or absence of Calcinosis was recorded by patient, hand, and anatomical zone; sensitivity and specificity for Calcinosis detection by US versus X-ray was determined. Bilateral US vascular measurements of ulnar artery occlusion (UAO) and finger pulp blood flow (FPBF) were obtained. For each hand, associations between markers of pathologic blood flow (UAO, FPBF, and a composite severity score of UAO and FPBF) and presence of Calcinosis were assessed using generalized estimating equations. RESULTS Of 43 SSc patients (19 diffuse, 24 limited), 39.5% had Calcinosis on X-ray compared to 30.2% on US. Sensitivity and specificity for US was 61% and 95% by zone, 78% and 98% by hand, and 76% and 100% by patient, respectively. UAO was seen in 30% and 28% of left and right hands, respectively; FPBF was absent in ≥1 digit of the left and right hands in 49% and 44%, respectively. UAO was associated with X-ray identified Calcinosis by hand (OR 8.08, 95% CI 2.45-26.60, p<0.001), whereas FPBF and the composite severity score were not significant. UAO was associated with Calcinosis even in the absence of digital ulcers (OR 33.00, 95% CI 3.39-321.09, p=0.003). CONCLUSION US was sensitive and highly specific in detecting Calcinosis in SSc. UAO was strongly associated with X-ray identified Calcinosis.
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Calcinosis biomarkers in adult and juvenile dermatomyositis
Autoimmunity Reviews, 2020Co-Authors: Melody P Chung, Carrie Richardson, David Kirakossian, Amir B Orandi, Lesley Ann Saketkoo, Lisa G Rider, Adam Schiffenbauer, Carlos A Von Muhlen, Lorinda ChungAbstract:Dermatomyositis (DM) is a rare idiopathic inflammatory myopathy characterized by muscle weakness and cutaneous manifestations in adults and children. Calcinosis, a complication of DM, is the abnormal deposition of insoluble calcium salts in tissues, including skin, subcutaneous tissue, tendons, fascia, and muscle. Calcinosis is more commonly seen in juvenile DM (JDM), but also develops in adult DM. Although the mechanism of Calcinosis remains unclear, several pathogenic hypotheses have been proposed, including intracellular accumulation of calcium secondary to an alteration of the cellular membrane by trauma and inflammation, local vascular ischemia, dysregulation of mechanisms controlling the deposition and solubility of calcium and phosphate, and mitochondrial damage of muscle cells. Identifying Calcinosis biomarkers is important for early disease detection and risk assessment, and may lead to novel therapeutic targets for the prevention and treatment of DM-associated Calcinosis. In this review, we summarize myositis autoantibodies associated with Calcinosis in DM, histopathology and chemical composition of Calcinosis, genetic and inflammatory markers that have been studied in adult DM and JDM-associated Calcinosis, as well as potential novel biomarkers.
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Management of Calcinosis Associated with Dermatomyositis
Current Treatment Options in Rheumatology, 2019Co-Authors: Melody Chung, Lorinda ChungAbstract:Purpose of review Calcinosis cutis, or dystrophic soft-tissue calcification, is a common and debilitating complication of adult and juvenile dermatomyositis. Dermatomyositis-associated Calcinosis is difficult to treat and is associated with significant morbidity. The purpose of this review is to provide an update of treatment modalities for Calcinosis in dermatomyositis based on published studies. Recent findings Specific guidelines are lacking for Calcinosis cutis management. Based on previous case reports, case series, cohort studies, and limited controlled studies, medications including diltiazem, bisphosphonates, sodium thiosulfate, aluminum hydroxide, warfarin, probenecid, colchicine, minocycline, and intravenous immunoglobulins have been used to control Calcinosis progression in dermatomyositis, but no treatment has convincingly prevented or reduced Calcinosis. Surgical excision of large or symptomatic calcium deposits remains the mainstay of treatment. Biologic therapies including infliximab, abatacept, rituximab, anakinra, and the oral JAK inhibitor tofacitinib have been used to control dermatomyositis-associated Calcinosis in case reports and series. Summary Pharmacological treatment aimed to reduce Calcinosis is challenging given that no therapies have consistently been found to be effective and few studies have a high level of evidence. Randomized controlled trials using novel outcome measures are necessary to evaluate the efficacy of proposed and emerging therapies. Trial registration: clinicaltrials.gov NCT03639779 and NCT03267277
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Management of Calcinosis Associated with Dermatomyositis
Current Treatment Options in Rheumatology, 2019Co-Authors: Melody P Chung, Lorinda ChungAbstract:Calcinosis cutis, or dystrophic soft-tissue calcification, is a common and debilitating complication of adult and juvenile dermatomyositis. Dermatomyositis-associated Calcinosis is difficult to treat and is associated with significant morbidity. The purpose of this review is to provide an update of treatment modalities for Calcinosis in dermatomyositis based on published studies. Specific guidelines are lacking for Calcinosis cutis management. Based on previous case reports, case series, cohort studies, and limited controlled studies, medications including diltiazem, bisphosphonates, sodium thiosulfate, aluminum hydroxide, warfarin, probenecid, colchicine, minocycline, and intravenous immunoglobulins have been used to control Calcinosis progression in dermatomyositis, but no treatment has convincingly prevented or reduced Calcinosis. Surgical excision of large or symptomatic calcium deposits remains the mainstay of treatment. Biologic therapies including infliximab, abatacept, rituximab, anakinra, and the oral JAK inhibitor tofacitinib have been used to control dermatomyositis-associated Calcinosis in case reports and series. Pharmacological treatment aimed to reduce Calcinosis is challenging given that no therapies have consistently been found to be effective and few studies have a high level of evidence. Randomized controlled trials using novel outcome measures are necessary to evaluate the efficacy of proposed and emerging therapies. Trial registration: clinicaltrials.gov NCT03639779 and NCT03267277
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identification of clinical features and autoantibodies associated with Calcinosis in dermatomyositis
JAMA Dermatology, 2014Co-Authors: Antonia Valenzuela, Lorinda Chung, Livia Casciolarosen, David FiorentinoAbstract:Importance Prior studies have estimated that up to 20% of adults with dermatomyositis (DM) have Calcinosis, which can lead to significant morbidity. Identification of risk factors may provide a better understanding of the pathogenesis and ultimately therapy for this difficult clinical problem. Risk factors for Calcinosis in adults with DM have not been extensively studied. Objectives To determine the prevalence of Calcinosis and to identify associated clinical features in a cohort of extensively phenotyped adults with DM. Design, Setting, and Participants A cross-sectional study of 126 patients diagnosed as having DM from January 1, 2006, through January 1, 2013, was performed. Patients were adults (≥18 years of age) attending the Stanford University Medical Center clinic. Main Outcomes and Measures Calcinosis, defined as the presence of calcium deposition in the skin and subcutaneous tissues on physical examination. Results Fourteen patients (11.1%) had Calcinosis, with the extremities most commonly involved. Patients with vs those without Calcinosis had a longer disease duration (median, 6.9 years; range, 2.4–18.1; vs median, 3.9 years; range, 0.2-19.2 years; P = .003) and more fingertip ulcers (50.0% vs 9.3%, P Conclusions and Relevance Calcinosis was a relatively uncommon clinical feature in our cohort of adults with DM. Our data suggest that Calcinosis is positively associated with longer disease duration, fingertip ulcers, and NXP-2 autoantibodies and negatively associated with transcriptional intermediary factor 1-γ antibodies. A common vascular mechanism may underlie the development of both Calcinosis and fingertip ulcers in patients with DM.
Kathryn M. Olsen - One of the best experts on this subject based on the ideXlab platform.
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Tumoral Calcinosis: Pearls, Polemics, and Alternative Possibilities
Radiographics, 2006Co-Authors: Kathryn M. Olsen, Felix S ChewAbstract:Massive periarticular Calcinosis of the soft tissues is a unique but not rare radiographic finding. On the contrary, tumoral Calcinosis is a rare familial disease. Unfortunately, the term tumoral Calcinosis has been liberally and imprecisely used to describe any massive collection of periarticular calcification, although this term actually refers to a hereditary condition associated with massive periarticular calcification. The inconsistent use of this term has created confusion throughout the literature. More important, if the radiologist is unfamiliar with tumoral Calcinosis or disease processes that mimic this condition, then diagnosis could be impeded, treatment could be delayed, and undue alarm could be raised, possibly leading to unwarranted surgical procedures. The soft-tissue lesions of tumoral Calcinosis are typically lobulated, well-demarcated calcifications that are most often distributed along the extensor surfaces of large joints. There are many conditions with similar appearances, including ...
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Tumoral Calcinosis: pearls, polemics, and alternative possibilities.
RadioGraphics, 2006Co-Authors: Kathryn M. Olsen, Felix S ChewAbstract:Massive periarticular Calcinosis of the soft tissues is a unique but not rare radiographic finding. On the contrary, tumoral Calcinosis is a rare familial disease. Unfortunately, the term tumoral Calcinosis has been liberally and imprecisely used to describe any massive collection of periarticular calcification, although this term actually refers to a hereditary condition associated with massive periarticular calcification. The inconsistent use of this term has created confusion throughout the literature. More important, if the radiologist is unfamiliar with tumoral Calcinosis or disease processes that mimic this condition, then diagnosis could be impeded, treatment could be delayed, and undue alarm could be raised, possibly leading to unwarranted surgical procedures. The soft-tissue lesions of tumoral Calcinosis are typically lobulated, well-demarcated calcifications that are most often distributed along the extensor surfaces of large joints. There are many conditions with similar appearances, including the Calcinosis of chronic renal failure, Calcinosis universalis, Calcinosis circumscripta, calcific tendonitis, synovial osteochondromatosis, synovial sarcoma, osteosarcoma, myositis ossificans, tophaceous gout, and calcific myonecrosis. The radiologist plays a critical role in avoiding unnecessary invasive procedures and in guiding the selection of appropriate tests that can result in a conclusive diagnosis of tumoral Calcinosis.
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progressive systemic sclerosis with massive paraspinal soft tissue Calcinosis
American Journal of Roentgenology, 2004Co-Authors: Kathryn M. Olsen, Eric J Pike, Felix S ChewAbstract:but normal calcium levels. Inclan et al. specified that this condition occurred in the absence of renal, metabolic, or collagen vascular disease. However, the term “tumoral Calcinosis” has gradually and imprecisely encompassed all tumorlike multilobulated calcific deposits in a paraarticular distribution in soft tissues. Because of the expanding definition in the literature, lesions now have a pathologically based classification: primary normophosphatemic tumoral Calcinosis, primary hyperphosphatemic tumoral Calcinosis, and secondary tumoral Calcinosis [2]. Primary forms of tumoral Calcinosis are less common than the secondary forms, which may be associated with renal failure, hemodialysis, collagen vascular disease, sarcoidosis, pseudoxanthoma elasticum, massive osteolysis, and other conditions. Patients present with a mass, regional compressive symptoms, or both, but pain and radiculopathy have also been described. Most of these lesions are related to the extensor surfaces of the hip, shoulder, elbows, wrists, and feet and likely originate in adjacent bursae. Rare locations also include dental and paraspinal regions [3]. Lesions of tumoral Calcinosis consist of macroscopic masses of amorphous calcified material, sometimes in aqueous suspension, surrounded by chronic fibrotic and inflammatory reaction. A paraarticular distribution is common. Conventional radiographs show a characteristic lobulated mass of several centimeters with amorphous calcific deposits. Although a cystic component may be present, evident as a fluid‐ sedimentation level, this feature is usually absent when tumoral Calcinosis is associated with connective tissue disease [4]. Bone scintigraphy shows increased radiotracer activity in the corresponding paraarticular distribution. CT shows soft-tissue Calcinosis without direct communication with adjacent bones or joints. Lesions with a cystic component may have high-attenuation septations as compared with the low-attenuation centers. Calcium layering, known as the “sedimentation” sign, may be seen. MRI may show fluid‐fluid levels with high signal layered over low signal on fluid-sensitive sequences [5]. If symptomatic, lesions of tumoral Calcinosis may be excised. Although recurrent lesions are common, their growth in secondary tumoral Calcinosis may be retarded with treatment of the underlying condition.
M S Seshadri - One of the best experts on this subject based on the ideXlab platform.
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tumoral Calcinosis of the scalp an unusual site for a rare tumor
Laryngoscope, 2007Co-Authors: Jubbin J. Jacob, Nihal Thomas, M S SeshadriAbstract:Tumoral Calcinosis is a rare calcifying disorder that is associated with deposition of calcium crystals in the periarticular tissues. The mass is most often around the hips, elbows, shoulders, and feet but may be occasionally found elsewhere. We report a case of multiple sporadic tumoral calcinoses in an adult male over the scalp. The scalp as a site of tumoral Calcinosis has not been previously reported in adults. Previous surgical excisions done on two occasions had resulted in recurrence of the tumors. This report highlights the need to include tumoral Calcinosis in the differential diagnosis of tumors of the scalp.
Mark D P Davis - One of the best experts on this subject based on the ideXlab platform.
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plain radiography is effective for the detection of Calcinosis cutis occurring in association with autoimmune connective tissue disease
British Journal of Dermatology, 2014Co-Authors: Varun Shahi, David A Wetter, Benjamin M Howe, Michael D Ringler, Mark D P DavisAbstract:Summary Background Studies on the radiological findings of Calcinosis cutis in patients with autoimmune connective tissue disease (ACTD) are limited. Objectives To describe imaging findings and radiological patterns of Calcinosis cutis occurring with ACTD. Methods We retrospectively reviewed the cases of 37 patients with imaging studies assessing for Calcinosis cutis associated with ACTD at our institution between 1996 and 2009. A formal blinded review of available images (i.e. radiographs, computed tomography scans) for all 37 patients was performed by radiologists. Results Of 37 patients, 30 (81%) were female. The mean age at diagnosis of Calcinosis cutis was 434 years. Patients had the following ACTDs: dermatomyositis (n = 17) with classic (n = 9), juvenile (n = 7) and amyopathic (n = 1) subtypes; systemic sclerosis with limited cutaneous scleroderma (n = 8); undifferentiated connective tissue disease (n = 4); mixed connective tissue disease (n = 3); systemic lupus erythematosus (n = 2); lupus panniculitis (n = 1); overlap connective tissue disease (n = 1); and polymyositis (n = 1). Plain radiographs detected Calcinosis in all 37 patients; a nodular pattern of calcification was most commonly observed (31/37; 84%). Two or more morphological patterns of calcification were present in 19 patients (51%). In general, individual patterns were not specific to a particular ACTD subtype. Conclusions Radiography detected Calcinosis in all patients and is recommended for initial imaging of Calcinosis. Multiple morphological patterns of calcification were observed across ACTD subtypes. Further studies should correlate radiological studies with treatment of Calcinosis and underlying ACTD.
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Calcinosis cutis occurring in association with autoimmune connective tissue disease the mayo clinic experience with 78 patients 1996 2009
Archives of Dermatology, 2012Co-Authors: Samuel J Balin, David A Wetter, Louise K Andersen, Mark D P DavisAbstract:Objective To describe characteristics and treatment of patients with Calcinosis cutis in the clinical setting of autoimmune connective tissue disease (ACTD). Design Retrospective study. Setting Tertiary referral center. Patients Seventy-eight patients with Calcinosis cutis and ACTD between 1996 and 2009. Main Outcome Measures Clinical features, treatments, and outcomes of patients with Calcinosis cutis in the clinical setting of ACTD. Results Of 78 patients (mean age at onset of Calcinosis cutis, 40.1 years), 64 (82%) were female. The following diseases were associated with Calcinosis cutis: dermatomyositis (n = 30) with classic (n = 15), juvenile (n = 14), and amyopathic (n = 1) subtypes; systemic sclerosis with limited cutaneous scleroderma (n = 24); lupus panniculitis (n = 4); systemic lupus erythematosus (n = 2); mixed connective tissue disease (n = 4); overlap connective tissue disease (n = 6); undifferentiated connective tissue disease (n = 6); polymyositis (n = 1); and rheumatoid arthritis (n = 1). Therapy for Calcinosis cutis consisted of medical treatment alone (n = 19), surgical therapy alone (n = 11), combined medical and surgical treatment (n = 17), no treatment (n = 30), and unknown (n = 1). Diltiazem hydrochloride was the most commonly used medical therapy, with 9 of 17 patients having a partial response. Twenty-eight patients had surgical excision of 1 or more lesions of Calcinosis cutis: 22 had a complete response, 5 had a partial response, and 1 had no response. Conclusions Dermatomyositis and systemic sclerosis were the most common ACTDs associated with Calcinosis cutis. Although no treatment was uniformly effective, surgical excision of symptomatic lesions and medical treatment with diltiazem provided benefit for some patients.
