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https://www.readbyqxmd.com/read/28088339/evidence-based-management-of-systemic-sclerosis-navigating-recommendations-and-guidelines
#1
REVIEW
Russell Edward Pellar, Janet Elizabeth Pope
OBJECTIVES: Systemic sclerosis (SSc) is a rare heterogeneous connective tissue disease. Recommendations addressing the major issues in the management of SSc including screening and treatment of organ complications are needed. METHODS: The updated European League Against Rheumatism/European Scleroderma Trial and Research (EULAR/EUSTAR) and the British Society of Rheumatology (BSR) and British Health Professionals in Rheumatology (BHPR) guidelines were compared and contrasted...
December 9, 2016: Seminars in Arthritis and Rheumatism
https://www.readbyqxmd.com/read/28058540/juvenile-dermatomyositis-a-tertiary-center-experience
#2
Kenan Barut, Pinar Ozge Avar Aydin, Amra Adrovic, Sezgin Sahin, Ozgur Kasapcopur
Juvenile dermatomyositis (JDM) is a rare chronic inflammatory disease of unknown etiology and primarily involves muscle and skin. It is the most common idiopathic inflammatory myopathy of childhood. This study aimed to evaluate demographic and clinical features, laboratory data, treatment modalities, and outcome of patients with JDM at a referral pediatric rheumatology center in Turkey. We retrospectively reviewed medical records of patients diagnosed with JDM between the years 2003-2016 at the Pediatric Rheumatology Department Cerrahpasa Medical Faculty...
January 5, 2017: Clinical Rheumatology
https://www.readbyqxmd.com/read/28056476/-advanced-systemic-sclerosis-endovascular-reconstruction-of-the-aortic-bifurcation-for-limb-salvage
#3
Florian Stein, Lars Kamper, Konstantinos Meletiadis, Ulrich Kusenack, Patrick Haage
History and admission findings A 69-year-old patient was initially hospitalized because of a 1.5 cm ulceration at the back of the right foot which had existed for the last year and become increasingly swollen and painful. Medical history revealed PmScl-positive systemic sclerosis presenting with a massive calcinosis cutis, advanced pulmonary fibrosis and peripheral artery disease. Examinations Inflammatory markers were normal. MR-Angiography of the lower extremities revealed a multi-segmental high-grade stenosis of the aortic bifurcation due to extensively calcified plaques...
January 2017: Deutsche Medizinische Wochenschrift
https://www.readbyqxmd.com/read/28018840/hand-impairment-in-systemic-sclerosis-various-manifestations-and-currently-available-treatment
#4
Amber Young, Rajaie Namas, Carole Dodge, Dinesh Khanna
Systemic sclerosis (SSc) is an autoimmune disease initially recognized by hand involvement due to characteristic Raynaud's phenomenon (RP), puffy hands, skin thickening, and contractures resembling claw deformities. SSc contributes to hand impairment through inflammatory arthritis, joint contractures, tendon friction rubs (TFRs), RP, digital ulcers (DU), puffy hands, skin sclerosis, acro-osteolysis, and calcinosis. These manifestations, which often co-exist, can contribute to difficulty with occupational activities and activities of daily living (ADL), which can result in impaired quality of life...
September 2016: Current Treatment Options in Rheumatology
https://www.readbyqxmd.com/read/28017514/acro-osteolysis-and-calcinosis-in-patient-with-scleroderma-a-case-report
#5
Wan-Sun Choi, So-Yeon Park, Yoon-Seong Ban, Joo-Hak Kim
Acro-osteolysis is a rare disease characterized by bone resorption involving the distal phalanges of the hand. 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. The medical treatment was arranged under the supervision of a rheumatologist.
December 22, 2016: Acta Orthopaedica et Traumatologica Turcica
https://www.readbyqxmd.com/read/27968927/autoimmune-collagen-vascular-diseases-kids-are-not-just-little-people
#6
Sean Timpane, Heather Brandling-Bennett, Arni K Kristjansson
Morphea, dermatomyositis (DM), and discoid lupus erythematosus (DLE) are autoimmune collagen vascular diseases that can present at any age. In all three of these diseases, the tenants of diagnosis and treatment are largely the same in both children and adults, with a few notable differences. Children with morphea are more likely to present with the linear subtype and have a higher incidence of extracutaneous manifestations. Children often need early aggressive systemic treatment to try to prevent long-term sequelae of morphea...
November 2016: Clinics in Dermatology
https://www.readbyqxmd.com/read/27955821/intravenous-sodium-thiosulfate-for-treating-tumoral-calcinosis-associated-with-systemic-disorders-report-of-four-cases
#7
Arthur Mageau, Vincent Guigonis, Voa Ratzimbasafy, Thomas Bardin, Pascal Richette, Pablo Urena, Hang-Korng Ea
Intravenous sodium thiosulfate (ivSTS) is a promising new therapeutic option for calciphylaxis related to end-stage renal disease. However, its effect on tumoral calcinosis (TC) complicating autoimmune connective-tissue diseases has been scarcely described. We report here 4 cases (3 adults and 1 child) of TC treated with ivSTS. TC was secondary to CREST syndrome, dermatomyositis (1 adult and 1 child) and systemic erythematous lupus and involved multiple sites in all cases. In all 4 patients, TC was responsible for joint pain, reduced mobility, inflammatory flares and skin fistulations...
December 7, 2016: Joint, Bone, Spine: Revue du Rhumatisme
https://www.readbyqxmd.com/read/27908312/calcinosis-in-poly-dermatomyositis-clinical-and-laboratory-predictors-and-treatment-options
#8
Micaela Fredi, Francesca Bartoli, Ilaria Cavazzana, Angela Cerebelli, Nice Carabellese, Angela Tincani, Minoru Satoh, Franco Franceschini
OBJECTIVES: We aimed to identify the possible clinical and laboratory predictors of calcinosis in a cohort of patients with a diagnosis of polymyositis (PM) and dermatomyositis (DM). METHODS: We carried out a retrospective analysis of a cohort of myositis patients attending our clinic between January 2013 and May 2014. RESULTS: 74 patients (58 females, 16 males) with PM (30 cases), DM (30 cases), overlap syndrome (13 cases) and inclusion body myositis (1 case) were enrolled...
November 14, 2016: Clinical and Experimental Rheumatology
https://www.readbyqxmd.com/read/27835954/characteristics-and-outcome-of-children-with-juvenile-dermatomyositis-in-cape-town-a-cross-sectional-study
#9
Lawrence Owino Okong'o, Monika Esser, Jo Wilmshurst, Christiaan Scott
BACKGROUND: Juvenile dermatomyositis (JDM) is a rare idiopathic inflammatory childhood myopathy of uncertain aetiology. The demographic and clinical presentation of JDM may differ by race and geographic regions. Few studies have described the characteristics of JDM patients from Africa. METHODS: We conducted a retrospective observational study to determine clinical characteristics and outcomes of patients satisfying the Bohan and Peter criteria for probable JDM seen between 2004 and 2013 in three hospitals in Cape Town, South Africa...
November 11, 2016: Pediatric Rheumatology Online Journal
https://www.readbyqxmd.com/read/27833984/a-case-of-a-generalized-symptomatic-calcinosis-in-systemic-sclerosis
#10
Cristian Radu Jecan, Ştefan Iulian Bedereag, Ruxandra Diana Sinescu, Valentin Titus Grigorean, Cristina Nicoleta Cozma, Anca Bordianu, Ioan Petre Florescu
Calcinosis consists of abnormal calcium deposition in soft tissues, which appears often in patients with limited systemic sclerosis, being one of the criteria of CREST (calcinosis cutis, Raynaud phenomenon, esophageal dysfunction, sclerodactyly, and telangiectasia) syndrome. 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. We describe the case of a 52-year-old woman with systemic sclerosis and CREST syndrome who was admitted in our Department with multiple painful and disabling tumoral masses, situated in nearly all joints...
2016: Romanian Journal of Morphology and Embryology, Revue Roumaine de Morphologie et Embryologie
https://www.readbyqxmd.com/read/27693175/esophageal-abnormalities-in-juvenile-localized-scleroderma-is-it-associated-with-other-extracutaneous-manifestations
#11
Clarissa C M Valões, Glaucia V Novak, Juliana B Brunelli, Katia T Kozu, Ricardo K Toma, Clovis A Silva
OBJECTIVE: To assess esophageal involvement (EI) in juvenile localized scleroderma (JLS) population and the possible association between this gastrointestinal manifestation and demographic data, clinical features, laboratory exams, treatments and outcomes. METHODS: For a period of 31 years, 5,881 patients with rheumatic diseases were followed in our Pediatric Rheumatology Division. EI was defined by the presence of symptoms (solid/liquid dysphagia, heartburn, esophageal regurgitation, nausea/vomiting and epigastralgia) and confirmed by at least one EI exam abnormality: barium contrast radiography, upper gastrointestinal endoscopy and 24-hour esophageal pH-monitoring...
September 17, 2016: Revista Brasileira de Reumatologia
https://www.readbyqxmd.com/read/27660938/a-novel-approach-in-the-treatment-of-calcinosis-cutis
#12
Robert Hawkins, Ricky Mehta
No abstract text is available yet for this article.
October 2016: Journal of Clinical Rheumatology: Practical Reports on Rheumatic & Musculoskeletal Diseases
https://www.readbyqxmd.com/read/27622522/calciphylaxis-and-martorell-hypertensive-ischemic-leg-ulcer-same-pattern-one-pathophysiology
#13
Jürg Hafner
This review presents a closer look at four diseases which are probably closely related to one another pathophysiologically: (a) calciphylaxis (distal pattern); (b) calciphylaxis (proximal pattern); (c) Martorell hypertensive ischemic leg ulcer; (d) calciphylaxis with normal renal and parathyroid function (synonym: eutrophication). The four diseases have largely the same risk factors: (1) arterial hypertension, (2) diabetes mellitus (types 1 and 2), (3) secondary or tertiary hyperparathyroidism (in end-stage kidney disease) and (4) oral anticoagulation with vitamin K antagonists...
2016: Dermatology: International Journal for Clinical and Investigative Dermatology
https://www.readbyqxmd.com/read/27608765/bilateral-strio-pallido-dentate-calcinosis-fahr-s-disease-report-of-seven-cases-and-revision-of-literature
#14
Elisabetta Savino, Cecilia Soavi, Eleonora Capatti, Massimo Borrelli, Giovanni B Vigna, Angelina Passaro, Giovanni Zuliani
BACKGROUND: Fahr's disease is rare a neurodegenerative idiopathic condition characterized by symmetric and bilateral calcifications of basal ganglia, usually associated with progressive neuropsychiatric dysfunctions and movement disorders. The term "Fahr's syndrome" is used in presence of calcifications secondary to a specific cause, but the variability of etiology, pathogenesis, and clinical picture underlying this condition have raised the question of the real existence of a syndrome...
September 8, 2016: BMC Neurology
https://www.readbyqxmd.com/read/27606883/the-erbium-yag-laser-a-new-technique-for-the-treatment-of-scrotal-calcinosis
#15
Markus Meissner, Johannes Kleemann, Roland Kaufmann, Falk Ochsendorf
Scrotal calcinosis is a rare local entity characterized by multiple, calcified and painless nodules of the scrotal skin and was first described by Lewinski in 1883 (Figure 1a).(1) It normally appears in men aged 20-40 years.(2) For a long time, the pathological origin of this disease was unclear. In the last years it became more and more obvious, that dystrophic calcification of epithelial cysts involving inflammation, rupture, calcification and finally obliteration of the cyst wall is the main pathological correlate for scrotal calcinosis...
September 8, 2016: Journal of the European Academy of Dermatology and Venereology: JEADV
https://www.readbyqxmd.com/read/27494629/safety-and-efficacy-of-extracorporeal-shock-wave-therapy-eswt-in-calcinosis-cutis-associated-with-systemic-sclerosis
#16
Sandra Blumhardt, Diana P Frey, Martin Toniolo, Hatem Alkadhi, Ulrike Held, Oliver Distler
OBJECTIVES: Calcinosis cutis is a frequent, difficult to treat manifestation of systemic sclerosis (SSc) associated with high morbidity. The aim of this prospective, controlled, monocentric study was to assess safety and efficacy of extracorporeal shock wave therapy (ESWT) for calcinosis cutis of the finger in SSc patients. METHODS: A 12-week proof of concept study in which 4 SSc patients with calcinosis cutis were treated at one painful finger with high-energy, focused ESWT, in 3 sessions with one-week interval between each session...
September 2016: Clinical and Experimental Rheumatology
https://www.readbyqxmd.com/read/27437158/assessment-and-surgical-treatment-of-calcinosis-of-the-shoulder-associated-with-crest-syndrome
#17
R Manohara, S J Breusch
We report an unusual case of a 65-year-old lady with CREST syndrome with multiple upper and lower limb calcinosis, who presented with severe shoulder pain and stiffness, with widespread intra- and extra-articular calcinosis, which was refractory to conservative measures. We were able to identify the main cause of her symptoms through serial diagnostic injections as calcific biceps tendinosis. We will discuss her assessment and surgical management and the pathophysiology and various treatment modalities for managing the soft tissue calcinosis in rheumatological diseases...
2016: Case Reports in Rheumatology
https://www.readbyqxmd.com/read/27247203/is-rituximab-an-effective-treatment-of-refractory-calcinosis
#18
Maria Dubos, Kim Ly, Clothilde Martel, Anne Laure Fauchais
Calcinosis, the deposition of calcified material in soft tissues, is frequently seen in systemic sclerosis and dermatomyositis. Treatment options are limited, with disappointing results. Some recent case reports suggest that rituximab may be an attractive therapeutic option. In case 1, a 54-year-old woman who presented with rheumatoid arthritis in association with scleromyositis was treated with rituximab for rheumatoid arthritis. Despite this, she developed multiple progressive calcinosis, necessitating extracorporeal shock wave lithotripsy to limit calcinosis extension and pain...
May 31, 2016: BMJ Case Reports
https://www.readbyqxmd.com/read/27241706/calcinosis-in-systemic-sclerosis-subsets-distribution-and-complications
#19
Francesca Bartoli, Ginevra Fiori, Francesca Braschi, Laura Amanzi, Cosimo Bruni, Jelena Blagojevic, Silvia Bellando-Randone, Laura Cometi, Carolina de Souza Mueller, Serena Guiducci, Laura Rasero, Francesco Epifani, Daniel E Furst, Marco Matucci-Cerinic
OBJECTIVE: To retrospectively analyse the features of calcinosis in a cohort of SSc patients. METHODS: Charts of SSc patients attending the Ulcer Unit of the Rheumatology Department, University of Florence and presenting a clinical suspicion of calcinosis were considered in the study. Data on clinical history, including recent skin changes, and clinical examination of all areas with suspected calcinosis, radiological imaging of the calcinotic area, demographics and SSc-related organ involvement and pain measured by a visual analogue scale were recorded...
September 2016: Rheumatology
https://www.readbyqxmd.com/read/27164190/phenotypic-and-genotypic-characterization-and-treatment-of-a-cohort-with-familial-tumoral-calcinosis-hyperostosis-hyperphosphatemia-syndrome
#20
Mary Scott Ramnitz, Pravitt Gourh, Raphaela Goldbach-Mansky, Felasfa Wodajo, Shoji Ichikawa, Michael J Econs, Kenneth E White, Alfredo Molinolo, Marcus Y Chen, Theo Heller, Jaydira Del Rivero, Patricia Seo-Mayer, Bita Arabshahi, Malaka B Jackson, Sarah Hatab, Edward McCarthy, Lori C Guthrie, Beth A Brillante, Rachel I Gafni, Michael T Collins
Familial tumoral calcinosis (FTC)/hyperostosis-hyperphosphatemia syndrome (HHS) is a rare disorder caused by mutations in the genes encoding fibroblast growth factor-23 (FGF23), N-acetylgalactosaminyltransferase 3 (GALNT3), or KLOTHO. The result is functional deficiency of, or resistance to, intact FGF23 (iFGF23), causing hyperphosphatemia, increased renal tubular reabsorption of phosphorus (TRP), elevated or inappropriately normal 1,25-dihydroxyvitamin D3 (1,25D), ectopic calcifications, and/or diaphyseal hyperostosis...
October 2016: Journal of Bone and Mineral Research: the Official Journal of the American Society for Bone and Mineral Research
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