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Cardiology367 papers

Scleroderma

Last edited: 4/23/2026

Overview

Scleroderma, also known as systemic sclerosis, is a chronic autoimmune disorder characterized by fibrosis and vascular abnormalities affecting the skin and internal organs. 1 25

Diagnosis

Key Diagnostic Criteria: Skin thickening, Raynaud's phenomenon, nailfold capillary abnormalities (detected via nailfold capillaroscopy), and presence of autoantibodies (e.g., anti-Scl-70, anti-centromere antibodies). 2 22 25

Recommended Tests:

- Nailfold capillaroscopy for capillary morphology assessment. 2 - Skin biopsy for histopathological evaluation. - Pulmonary function tests to assess lung involvement. - Echocardiography for cardiac evaluation. - Esophageal manometry and barium swallow for gastrointestinal involvement. 55 62

Grading: Limited vs. diffuse cutaneous involvement based on extent of skin changes. 25 88

Management

First-Line Treatments:

- Immunosuppressive Agents: Cyclophosphamide for severe internal organ involvement. 73 - Vasodilators: Calcium channel blockers for Raynaud's phenomenon. - Pulmonary Hypertension: Bosentan, epoprostenol, or treprostinil for pulmonary arterial hypertension. 19 80

Adjunctive Treatments:

- Vitamin D: To reduce oxidative DNA damage in patients with organ involvement. 4 - Botulinum Toxin: For managing Raynaud's phenomenon-related symptoms. 18 - IV Iloprost: For digital vasculopathy, showing potential for long-term stabilization. 13 - Prostaglandins: Transdermal prostaglandin E1 ethyl ester for trophic skin lesions. 52

Special Populations

Pregnancy: Limited data; careful monitoring of maternal and fetal outcomes required. 69

Pediatrics: Early diagnosis and multidisciplinary management crucial due to varied clinical presentations. 44 84

Elderly: Increased risk of comorbidities; tailored management focusing on organ involvement.

Comorbidities: Obstructive sleep apnea may exacerbate pulmonary involvement; consider polysomnography in affected patients. 8

Key Recommendations

Utilize nailfold capillaroscopy for early detection of microvascular abnormalities in suspected scleroderma patients. (Evidence: Moderate) 2

Screen for pulmonary hypertension in all patients with scleroderma, particularly those with limited cutaneous involvement, using echocardiography. (Evidence: Moderate) 80

Consider cyclophosphamide for severe internal organ involvement, especially in patients with significant lung or renal disease. (Evidence: Strong) 73

Monitor and manage Raynaud's phenomenon with calcium channel blockers and consider botulinum toxin for refractory symptoms. (Evidence: Moderate) 18

Evaluate gastrointestinal involvement using esophageal manometry and barium swallow to guide management strategies. (Evidence: Moderate) 62

References

Showing 100 most recent of 214 indexed papers.

1 Hossain AS, Clarin MTRDC, Kimura K, Biggin G, Taga Y, Uto K et al.. Fibrillin-1 G234D mutation in the hybrid1 domain causes tight skin associated with dysregulated elastogenesis and increased collagen cross-linking in mice. Matrix biology : journal of the International Society for Matrix Biology 2025. link 2 Roberts-Thomson PJ, Patterson KA, Walker JG. Clinical utility of nailfold capillaroscopy. Internal medicine journal 2023. link 3 Rodríguez-Reyna TS, Hant FN, Cutolo M, Smith V. Training the Next Generation of Rheumatologists: What Is the Best Way to Teach Fellows About Scleroderma?. Rheumatic diseases clinics of North America 2023. link 4 Dal-Bekar NE, İşlekel GH, Köken-Avşar A, Yarkan-Tuğsal H, Tuna G, Zengin B et al.. Vitamin D attenuates elevated oxidative DNA damage in scleroderma patients with organ involvement: A prospective study. The Journal of steroid biochemistry and molecular biology 2023. link 5 Hamaguchi Y. Drug-induced scleroderma-like lesion. Allergology international : official journal of the Japanese Society of Allergology 2022. link 6 Benz K, Baulig C, Knippschild S, Strietzel FP, Hunzelmann N, Jackowski J. Prevalence of Oral and Maxillofacial Disorders in Patients with Systemic Scleroderma-A Systematic Review. International journal of environmental research and public health 2021. link 7 Mengoni M, Braun AD, Gaffal E. Generalized Nodules and Sclerosis of the Skin: A Quiz. Acta dermato-venereologica 2021. link 8 Yakut T, Balcan B, Karakurt S, Direskeneli H, Yalcinkaya Y, Peker Y. Impact of concomitant obstructive sleep apnea on pulmonary involvement and main pulmonary artery diameter in adults with scleroderma. Sleep & breathing = Schlaf & Atmung 2021. link 9 Martínez-Milla J, Gaebelt HP, Sánchez-Pernaute O, Kallmeyer A, Romero J, Farré J. Scleroderma Renal Crisis: A Reversible Cause of Left Ventricular Dysfunction. Reumatologia clinica 2020. link 10 Frontin JB, Anthony BW. Quantifying Dermatology: Method and Device for User-Independent Ultrasound Measurement of Skin Thickness. Annual International Conference of the IEEE Engineering in Medicine and Biology Society. IEEE Engineering in Medicine and Biology Society. Annual International Conference 2019. link 11 Dellalana LE, Chen F, Vain A, Gandelman JS, Põldemaa M, Chen H et al.. Reproducibility of the durometer and myoton devices for skin stiffness measurement in healthy subjects. Skin research and technology : official journal of International Society for Bioengineering and the Skin (ISBS) [and] International Society for Digital Imaging of Skin (ISDIS) [and] International Society for Skin Imaging (ISSI) 2019. link 12 Akakpo AS, Teclessou JN, Mouhari-Touré A, Saka B, Matakloe H, Kakpovi K et al.. Scleroderma in hospital settings in Lomé: 50 cases. Medecine et sante tropicales 2017. link 13 Foti R, Visalli E, Amato G, Benenati A, Converso G, Farina A et al.. Long-term clinical stabilization of scleroderma patients treated with a chronic and intensive IV iloprost regimen. Rheumatology international 2017. link 14 Dixit S, Kalkur C, Sattur AP, Bornstein MM, Melton F. Scleroderma and dentistry: Two case reports. Journal of medical case reports 2016. link 15 Takahashi T, Asano Y, Oka T, Miyagaki T, Tamaki Z, Nonaka S et al.. Scleroderma en coup de sabre with recurrent episodes of brain hemorrhage. The Journal of dermatology 2016. link 16 Bose N, Chiesa-Vottero A, Chatterjee S. Scleroderma renal crisis. Seminars in arthritis and rheumatism 2015. link 17 Gerede DM, Turhan S, Hural R, Acıbuca A, Kucuksahin O, Ozcan OU et al.. Evaluation of global function of the heart in scleroderma patients. Echocardiography (Mount Kisco, N.Y.) 2015. link 18 Uppal L, Dhaliwal K, Butler PE. A prospective study of the use of botulinum toxin injections in the treatment of Raynaud's syndrome associated with scleroderma. The Journal of hand surgery, European volume 2014. link 19 Marini C, Formichi B, Bauleo C, Michelassi C, Pancani R, Prediletto R et al.. Improved survival in limited scleroderma-related pulmonary artery hypertension. Internal and emergency medicine 2014. link 20 Fox BD, Shimony A, Langleben D, Hirsch A, Rudski L, Schlesinger R et al.. High prevalence of occult left heart disease in scleroderma-pulmonary hypertension. The European respiratory journal 2013. link 21 Shah AA, Chung SE, Wigley FM, Wise RA, Hummers LK. Changes in estimated right ventricular systolic pressure predict mortality and pulmonary hypertension in a cohort of scleroderma patients. Annals of the rheumatic diseases 2013. link 22 Tang DM, Pathikonda M, Harrison M, Fisher RS, Friedenberg FK, Parkman HP. Symptoms and esophageal motility based on phenotypic findings of scleroderma. Diseases of the esophagus : official journal of the International Society for Diseases of the Esophagus 2013. link 23 Borowiec A, Dabrowski R, Wozniak J, Jasek S, Chwyczko T, Kowalik I et al.. Cardiovascular assessment of asymptomatic patients with juvenile-onset localized and systemic scleroderma: 10 years prospective observation. Scandinavian journal of rheumatology 2012. link 24 Mishra N, Shrestha D, Poudyal RB, Shiva Raj KC. Atypical presentation of scleroderma in infancy. Rheumatology international 2012. link 25 Hassoun PM. Lung involvement in systemic sclerosis. Presse medicale (Paris, France : 1983) 2011. link 26 Hissaria P, Lester S, Hakendorf P, Woodman R, Patterson K, Hill C et al.. Survival in scleroderma: results from the population-based South Australian Register. Internal medicine journal 2011. link 27 Patanè S, Marte F, Sturiale M, Dattilo G, Luzza F. Atrial flutter, ventricular tachycardia and changing axis deviation associated with scleroderma. International journal of cardiology 2011. link 28 Watanabe R, Nakaya I, Yahata M, Takahashi T, Sasajima T, Takeuchi K et al.. Pulmonary hypertension confirmed histologically five months prior to scleroderma renal crisis onset. Modern rheumatology 2011. link 29 Panda S. Scleroderma in children: emerging management issues. Indian journal of dermatology, venereology and leprology 2010. link 30 Shah AA, Wigley FM, Hummers LK. Telangiectases in scleroderma: a potential clinical marker of pulmonary arterial hypertension. The Journal of rheumatology 2010. link 31 Lo CY, Shyur SD, Chu SH, Huang LH, Kao YH, Lei WT et al.. Juvenile scleroderma: experience in one institution. Asian Pacific journal of allergy and immunology 2010. link 32 Batal I, Domsic RT, Shafer A, Medsger TA, Kiss LP, Randhawa P et al.. Renal biopsy findings predicting outcome in scleroderma renal crisis. Human pathology 2009. link 33 Ng K, Gow P. Management of scleroderma in a New Zealand tertiary rheumatology centre: emphasis on pulmonary complications. The New Zealand medical journal 2007. link 34 Gazi H, Pope JE, Clements P, Medsger TA, Martin RW, Merkel PA et al.. Outcome measurements in scleroderma: results from a delphi exercise. The Journal of rheumatology 2007. link 35 Attaran RR, Guarraia D. Ascending aortic aneurysm in a man with scleroderma. Clinical rheumatology 2007. link 36 Rho YH, Choi SJ, Lee YH, Ji JD, Song GG. Scleroderma associated with ANCA-associated vasculitis. Rheumatology international 2006. link 37 Rho YH, Choi SJ, Lee YH, Ji JD, Song GG. Scleroderma associated with ANCA-associated vasculitis. Rheumatology international 2006. link 38 Dziadzio M, Anastassiades CP, Hawkins PN, Potter M, Gabrielli A, Brough GM et al.. From scleredema to AL amyloidosis: disease progression or coincidence? Review of the literature. Clinical rheumatology 2006. link 39 Massad MG, Powell CR, Kpodonu J, Tshibaka C, Hanhan Z, Snow NJ et al.. Outcomes of lung transplantation in patients with scleroderma. World journal of surgery 2005. link 40 Marasini B, Massarotti M, Cossutta R. Scleroderma heart disease. International journal of immunopathology and pharmacology 2005. link 41 Sparsa A, Lesaux N, Kessler E, Bonnetblanc JM, Blaise S, Lebrun-Ly V et al.. Treatment of cutaneous calcinosis in CREST syndrome by extracorporeal shock wave lithotripsy. Journal of the American Academy of Dermatology 2005. link 42 Lucivero V, Mezzapesa DM, Petruzzellis M, Carella A, Lamberti P, Federico F. Ischaemic stroke in progressive systemic sclerosis. Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology 2004. link 43 Zoumalan RA, Bendok BR, Parkinson RJ, Sorin J, Burke AM, Batjer HH. Association of an irregularly shaped anterior choroidal aneurysm with CREST syndrome. Case report. Journal of neurosurgery 2004. link 44 Wise RA, Wigley FM, White B, Leatherman G, Zhong J, Krasa H et al.. Efficacy and tolerability of a selective alpha(2C)-adrenergic receptor blocker in recovery from cold-induced vasospasm in scleroderma patients: a single-center, double-blind, placebo-controlled, randomized crossover study. Arthritis and rheumatism 2004. link 45 Clements PJ, Becvar R, Drosos AA, Ghattas L, Gabrielli A. Assessment of gastrointestinal involvement. Clinical and experimental rheumatology 2003. link 46 Schachna L, Wigley FM, Chang B, White B, Wise RA, Gelber AC. Age and risk of pulmonary arterial hypertension in scleroderma. Chest 2003. link 47 Chang B, Wigley FM, White B, Wise RA. Scleroderma patients with combined pulmonary hypertension and interstitial lung disease. The Journal of rheumatology 2003. link 48 Shor R, Halabe A. New trends in the treatment of scleroderma renal crisis. Nephron 2002. link 49 Schachna L, Wigley FM. Targeting mediators of vascular injury in scleroderma. Current opinion in rheumatology 2002. link 50 Tung A, Sweitzer B, Cutter T. Cardiac arrest after labetalol and metoclopramide administration in a patient with scleroderma. Anesthesia and analgesia 2002. link 51 Morse J, Barst R, Horn E, Cuervo N, Deng Z, Knowles J. Pulmonary hypertension in scleroderma spectrum of disease: lack of bone morphogenetic protein receptor 2 mutations. The Journal of rheumatology 2002. link 52 Schlez A, Kittel M, Scheurle B, Diehm C, Jünger M. Transdermal application of prostaglandin E1 ethyl ester for the treatment of trophic acral skin lesions in a patient with systemic scleroderma. Journal of the European Academy of Dermatology and Venereology : JEADV 2002. link 53 Das CP, Prabhakar S, Lal V, Kharbanda PS. Scleroderma, stroke, optic neuropathy: a rare association. Neurology India 2002. link 54 Gokula RM, Gupta AK, Shirley SE, Coard K, Ramphal PS. Scleroderma with cardiac tamponade, hyperthyroidism and incidental papillary thyroid carcinoma. The West Indian medical journal 2002. link 55 Knight LR, Smeathers JE, Isdale AH, Helliwell PS. Evaluating the cutaneous involvement in scleroderma: torsional stiffness revisited. Rheumatology (Oxford, England) 2001. link 56 Furspan PB, Mayes MD, Freedman RR. Effect of temperature and modulators of protein tyrosine kinase activity on the reactivity of isolated venules in secondary Raynaud's phenomenon. The Journal of rheumatology 2001. link 57 Coghlan JG, Mukerjee D. The heart and pulmonary vasculature in scleroderma: clinical features and pathobiology. Current opinion in rheumatology 2001. link 58 Tomaino MM. Digital arterial occlusion in scleroderma: is there a role for digital arterial reconstruction?. Journal of hand surgery (Edinburgh, Scotland) 2000. link 59 Mould TL, Roberts-Thomson PJ. Pathogenesis of telangiectasia in scleroderma. Asian Pacific journal of allergy and immunology 2000. link 60 French LE, Braun R, Masouyé I, Ramelet AA, Humbert PG, Saurat JH. Post-stripping sclerodermiform dermatitis. Archives of dermatology 1999. link 61 Freedman RR, Girgis R, Mayes MD. Endothelial and adrenergic dysfunction in Raynaud's phenomenon and scleroderma. The Journal of rheumatology 1999. link 62 Melone CP, McLoughlin JC, Beldner S. Surgical management of the hand in scleroderma. Current opinion in rheumatology 1999. link 63 Freedman RR, Girgis R, Mayes MD. Acute effect of nitric oxide on Raynaud's phenomenon in scleroderma. Lancet (London, England) 1999. link03557-6) 64 Tarrago D, Aguilera I, Melero J, Wichmann I, Nuñez-Roldan A, Sanchez B. Identification of cation-independent mannose 6-phosphate receptor/insulin-like growth factor type-2 receptor as a novel target of autoantibodies. Immunology 1999. link 65 Matsumoto AK, Moore R, Alli P, Wigley FM. Three cases of osteonecrosis of the lunate bone of the wrist in scleroderma. Clinical and experimental rheumatology 1999. link 66 Phan TG, Cass A, Gillin A, Trew P, Fertig N, Sturgess A. Anti-RNA polymerase III antibodies in the diagnosis of scleroderma renal crisis sine scleroderma. The Journal of rheumatology 1999. link 67 Stafford L, Englert H, Gover J, Bertouch J. Distribution of macrovascular disease in scleroderma. Annals of the rheumatic diseases 1998. link 68 Minai OA, Dweik RA, Arroliga AC. Manifestations of scleroderma pulmonary disease. Clinics in chest medicine 1998. link70112-x) 69 Doss BJ, Jacques SM, Mayes MD, Qureshi F. Maternal scleroderma: placental findings and perinatal outcome. Human pathology 1998. link90025-9) 70 Chaffee NR. CREST syndrome: clinical manifestations and dental management. Journal of prosthodontics : official journal of the American College of Prosthodontists 1998. link 71 Unsal E, Kilinç O, Kargi A, Necla T. Early assessment of pulmonary involvement in limited scleroderma. A case report. The Turkish journal of pediatrics 1998. link 72 Thompson AE, Pope JE. A study of the frequency of pericardial and pleural effusions in scleroderma. British journal of rheumatology 1998. link 73 Akesson A. Cyclophosphamide therapy for scleroderma. Current opinion in rheumatology 1998. link 74 Stratton RJ, Coghlan JG, Pearson JD, Burns A, Sweny P, Abraham DJ et al.. Different patterns of endothelial cell activation in renal and pulmonary vascular disease in scleroderma. QJM : monthly journal of the Association of Physicians 1998. link 75 Dyck PJ, Hunder GG, Dyck PJ. A case-control and nerve biopsy study of CREST multiple mononeuropathy. Neurology 1997. link 76 Roddy J, Holtby S, Seigel S. Scleroderma concurrent with culture proven tuberculosis in a Japanese Canadian patient. The Journal of rheumatology 1996. link 77 Nishioka K, Katayama I, Kondo H, Shinkai H, Ueki H, Tamaki K et al.. Epidemiological analysis of prognosis of 496 Japanese patients with progressive systemic sclerosis (SSc). Scleroderma Research Committee Japan. The Journal of dermatology 1996. link 78 Battle RW, Davitt MA, Cooper SM, Buckley LM, Leib ES, Beglin PA et al.. Prevalence of pulmonary hypertension in limited and diffuse scleroderma. Chest 1996. link 79 Wigley FM. Raynaud's phenomenon and other features of scleroderma, including pulmonary hypertension. Current opinion in rheumatology 1996. link 80 Armstrong GP, Whalley GA, Doughty RN, Gamble GD, Flett SM, Tan PL et al.. Left ventricular function in scleroderma. British journal of rheumatology 1996. link 81 Wigley FM, Flavahan NA. Raynaud's phenomenon. Rheumatic diseases clinics of North America 1996. link70300-8) 82 Richard MD, Park CH. Tc-99m MIBI uptake secondary to pulmonary interstitial fibrosis in progressive systemic sclerosis. Clinical nuclear medicine 1995. link 83 Kahaleh MB. Raynaud's phenomenon and the vascular disease in scleroderma. Current opinion in rheumatology 1995. link 84 Uziel Y, Miller ML, Laxer RM. Scleroderma in children. Pediatric clinics of North America 1995. link40058-1) 85 Poirier NC, Taillefer R, Topart P, Duranceau A. Antireflux operations in patients with scleroderma. The Annals of thoracic surgery 1994. link91073-1) 86 Kahaleh MB. Raynaud's phenomenon and vascular disease in scleroderma. Current opinion in rheumatology 1994. link 87 Hermosillo AG, Ortiz R, Dábague J, Casanova JM, Martínez-Lavín M. Autonomic dysfunction in diffuse scleroderma vs CREST: an assessment by computerized heart rate variability. The Journal of rheumatology 1994. link 88 Zuber M, Gotzen R, Filler I. Clinical correlation of anticentromere antibodies. Clinical rheumatology 1994. link 89 Shibata S, Harpel P, Bona C, Fillit H. Monoclonal antibodies to heparan sulfate inhibit the formation of thrombin-antithrombin III complexes. Clinical immunology and immunopathology 1993. link 90 Boschi A, Snyers B, Lambert M. Bilateral optic neuropathy associated with the crest variant of scleroderma. European journal of ophthalmology 1993. link 91 Gates C, Morand EF, Davis M, Maddison PJ. Sclerotherapy as treatment of recurrent bleeding from upper gastrointestinal telangiectasia in CREST syndrome. British journal of rheumatology 1993. link 92 Kerr LD, Spiera H. Myocarditis as a complication in scleroderma patients with myositis. Clinical cardiology 1993. link 93 Martin L, Pauls JD, Ryan JP, Fritzler MJ. Identification of a subset of patients with scleroderma with severe pulmonary and vascular disease by the presence of autoantibodies to centromere and histone. Annals of the rheumatic diseases 1993. link 94 Paley M, McLoughlin P. Oral problems associated with CREST syndrome: a case report. British dental journal 1993. link 95 Miller M. Scleroderma. Australian family physician 1993. link 96 Mazagri R, Shuaib A. Flunarizine is effective in prophylaxis of headache associated with scleroderma. Headache 1992. link 97 Stefanato C, Chlebus E, Janniger CK, Jablonska S. Subcutaneous symmetrical morphea with unusual onset as nodular vasculitis. Cutis 1992. link 98 Saitoh H, Tomkiel J, Cooke CA, Ratrie H, Maurer M, Rothfield NF et al.. CENP-C, an autoantigen in scleroderma, is a component of the human inner kinetochore plate. Cell 1992. link90538-n) 99 Englert H, Brennan P, McNeil D, Black C, Silman AJ. Reproductive function prior to disease onset in women with scleroderma. The Journal of rheumatology 1992. link 100 O'Brien BM, Kumar PA, Mellow CG, Oliver TV. Radical microarteriolysis in the treatment of vasospastic disorders of the hand, especially scleroderma. Journal of hand surgery (Edinburgh, Scotland) 1992. link80272-4)

Original source

[1]
Fibrillin-1 G234D mutation in the hybrid1 domain causes tight skin associated with dysregulated elastogenesis and increased collagen cross-linking in mice. — Hossain AS, Clarin MTRDC, Kimura K, Biggin G, Taga Y, Uto K et al. Matrix biology : journal of the International Society for Matrix Biology (2025)
[2]
Clinical utility of nailfold capillaroscopy. — Roberts-Thomson PJ, Patterson KA, Walker JG Internal medicine journal (2023)
[3]
Training the Next Generation of Rheumatologists: What Is the Best Way to Teach Fellows About Scleroderma? — Rodríguez-Reyna TS, Hant FN, Cutolo M, Smith V Rheumatic diseases clinics of North America (2023)
[4]
Vitamin D attenuates elevated oxidative DNA damage in scleroderma patients with organ involvement: A prospective study. — Dal-Bekar NE, İşlekel GH, Köken-Avşar A, Yarkan-Tuğsal H, Tuna G, Zengin B et al. The Journal of steroid biochemistry and molecular biology (2023)
[5]
Drug-induced scleroderma-like lesion. — Hamaguchi Y Allergology international : official journal of the Japanese Society of Allergology (2022)
[6]
Prevalence of Oral and Maxillofacial Disorders in Patients with Systemic Scleroderma-A Systematic Review. — Benz K, Baulig C, Knippschild S, Strietzel FP, Hunzelmann N, Jackowski J International journal of environmental research and public health (2021)
[7]
Generalized Nodules and Sclerosis of the Skin: A Quiz. — Mengoni M, Braun AD, Gaffal E Acta dermato-venereologica (2021)
[8]
Impact of concomitant obstructive sleep apnea on pulmonary involvement and main pulmonary artery diameter in adults with scleroderma. — Yakut T, Balcan B, Karakurt S, Direskeneli H, Yalcinkaya Y, Peker Y Sleep & breathing = Schlaf & Atmung (2021)
[9]
Scleroderma Renal Crisis: A Reversible Cause of Left Ventricular Dysfunction. — Martínez-Milla J, Gaebelt HP, Sánchez-Pernaute O, Kallmeyer A, Romero J, Farré J Reumatologia clinica (2020)
[10]
Quantifying Dermatology: Method and Device for User-Independent Ultrasound Measurement of Skin Thickness. — Frontin JB, Anthony BW Annual International Conference of the IEEE Engineering in Medicine and Biology Society. IEEE Engineering in Medicine and Biology Society. Annual International Conference (2019)
[11]
Reproducibility of the durometer and myoton devices for skin stiffness measurement in healthy subjects. — Dellalana LE, Chen F, Vain A, Gandelman JS, Põldemaa M, Chen H et al. Skin research and technology : official journal of International Society for Bioengineering and the Skin (ISBS) [and] International Society for Digital Imaging of Skin (ISDIS) [and] International Society for Skin Imaging (ISSI) (2019)
[12]
Scleroderma in hospital settings in Lomé: 50 cases. — Akakpo AS, Teclessou JN, Mouhari-Touré A, Saka B, Matakloe H, Kakpovi K et al. Medecine et sante tropicales (2017)
[13]
Long-term clinical stabilization of scleroderma patients treated with a chronic and intensive IV iloprost regimen. — Foti R, Visalli E, Amato G, Benenati A, Converso G, Farina A et al. Rheumatology international (2017)
[14]
Scleroderma and dentistry: Two case reports. — Dixit S, Kalkur C, Sattur AP, Bornstein MM, Melton F Journal of medical case reports (2016)
[15]
Scleroderma en coup de sabre with recurrent episodes of brain hemorrhage. — Takahashi T, Asano Y, Oka T, Miyagaki T, Tamaki Z, Nonaka S et al. The Journal of dermatology (2016)
[16]
Scleroderma renal crisis. — Bose N, Chiesa-Vottero A, Chatterjee S Seminars in arthritis and rheumatism (2015)
[17]
Evaluation of global function of the heart in scleroderma patients. — Gerede DM, Turhan S, Hural R, Acıbuca A, Kucuksahin O, Ozcan OU et al. Echocardiography (Mount Kisco, N.Y.) (2015)
[18]
A prospective study of the use of botulinum toxin injections in the treatment of Raynaud's syndrome associated with scleroderma. — Uppal L, Dhaliwal K, Butler PE The Journal of hand surgery, European volume (2014)
[19]
Improved survival in limited scleroderma-related pulmonary artery hypertension. — Marini C, Formichi B, Bauleo C, Michelassi C, Pancani R, Prediletto R et al. Internal and emergency medicine (2014)
[20]
High prevalence of occult left heart disease in scleroderma-pulmonary hypertension. — Fox BD, Shimony A, Langleben D, Hirsch A, Rudski L, Schlesinger R et al. The European respiratory journal (2013)
[21]
Changes in estimated right ventricular systolic pressure predict mortality and pulmonary hypertension in a cohort of scleroderma patients. — Shah AA, Chung SE, Wigley FM, Wise RA, Hummers LK Annals of the rheumatic diseases (2013)
[22]
Symptoms and esophageal motility based on phenotypic findings of scleroderma. — Tang DM, Pathikonda M, Harrison M, Fisher RS, Friedenberg FK, Parkman HP Diseases of the esophagus : official journal of the International Society for Diseases of the Esophagus (2013)
[23]
Cardiovascular assessment of asymptomatic patients with juvenile-onset localized and systemic scleroderma: 10 years prospective observation. — Borowiec A, Dabrowski R, Wozniak J, Jasek S, Chwyczko T, Kowalik I et al. Scandinavian journal of rheumatology (2012)
[24]
Atypical presentation of scleroderma in infancy. — Mishra N, Shrestha D, Poudyal RB, Shiva Raj KC Rheumatology international (2012)
[25]
Lung involvement in systemic sclerosis. — Hassoun PM Presse medicale (Paris, France : 1983) (2011)
[26]
Survival in scleroderma: results from the population-based South Australian Register. — Hissaria P, Lester S, Hakendorf P, Woodman R, Patterson K, Hill C et al. Internal medicine journal (2011)
[27]
Atrial flutter, ventricular tachycardia and changing axis deviation associated with scleroderma. — Patanè S, Marte F, Sturiale M, Dattilo G, Luzza F International journal of cardiology (2011)
[28]
Pulmonary hypertension confirmed histologically five months prior to scleroderma renal crisis onset. — Watanabe R, Nakaya I, Yahata M, Takahashi T, Sasajima T, Takeuchi K et al. Modern rheumatology (2011)
[29]
Scleroderma in children: emerging management issues. — Panda S Indian journal of dermatology, venereology and leprology (2010)
[30]
Telangiectases in scleroderma: a potential clinical marker of pulmonary arterial hypertension. — Shah AA, Wigley FM, Hummers LK The Journal of rheumatology (2010)
[31]
Juvenile scleroderma: experience in one institution. — Lo CY, Shyur SD, Chu SH, Huang LH, Kao YH, Lei WT et al. Asian Pacific journal of allergy and immunology (2010)
[32]
Renal biopsy findings predicting outcome in scleroderma renal crisis. — Batal I, Domsic RT, Shafer A, Medsger TA, Kiss LP, Randhawa P et al. Human pathology (2009)
[33]
Management of scleroderma in a New Zealand tertiary rheumatology centre: emphasis on pulmonary complications. — Ng K, Gow P The New Zealand medical journal (2007)
[34]
Outcome measurements in scleroderma: results from a delphi exercise. — Gazi H, Pope JE, Clements P, Medsger TA, Martin RW, Merkel PA et al. The Journal of rheumatology (2007)
[35]
Ascending aortic aneurysm in a man with scleroderma. — Attaran RR, Guarraia D Clinical rheumatology (2007)
[36]
Scleroderma associated with ANCA-associated vasculitis. — Rho YH, Choi SJ, Lee YH, Ji JD, Song GG Rheumatology international (2006)
[37]
Scleroderma associated with ANCA-associated vasculitis. — Rho YH, Choi SJ, Lee YH, Ji JD, Song GG Rheumatology international (2006)
[38]
From scleredema to AL amyloidosis: disease progression or coincidence? Review of the literature. — Dziadzio M, Anastassiades CP, Hawkins PN, Potter M, Gabrielli A, Brough GM et al. Clinical rheumatology (2006)
[39]
Outcomes of lung transplantation in patients with scleroderma. — Massad MG, Powell CR, Kpodonu J, Tshibaka C, Hanhan Z, Snow NJ et al. World journal of surgery (2005)
[40]
Scleroderma heart disease. — Marasini B, Massarotti M, Cossutta R International journal of immunopathology and pharmacology (2005)
[41]
Treatment of cutaneous calcinosis in CREST syndrome by extracorporeal shock wave lithotripsy. — Sparsa A, Lesaux N, Kessler E, Bonnetblanc JM, Blaise S, Lebrun-Ly V et al. Journal of the American Academy of Dermatology (2005)
[42]
Ischaemic stroke in progressive systemic sclerosis. — Lucivero V, Mezzapesa DM, Petruzzellis M, Carella A, Lamberti P, Federico F Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology (2004)
[43]
Association of an irregularly shaped anterior choroidal aneurysm with CREST syndrome. Case report. — Zoumalan RA, Bendok BR, Parkinson RJ, Sorin J, Burke AM, Batjer HH Journal of neurosurgery (2004)
[44]
Efficacy and tolerability of a selective alpha(2C)-adrenergic receptor blocker in recovery from cold-induced vasospasm in scleroderma patients: a single-center, double-blind, placebo-controlled, randomized crossover study. — Wise RA, Wigley FM, White B, Leatherman G, Zhong J, Krasa H et al. Arthritis and rheumatism (2004)
[45]
Assessment of gastrointestinal involvement. — Clements PJ, Becvar R, Drosos AA, Ghattas L, Gabrielli A Clinical and experimental rheumatology (2003)
[46]
Age and risk of pulmonary arterial hypertension in scleroderma. — Schachna L, Wigley FM, Chang B, White B, Wise RA, Gelber AC Chest (2003)
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Scleroderma patients with combined pulmonary hypertension and interstitial lung disease. — Chang B, Wigley FM, White B, Wise RA The Journal of rheumatology (2003)
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New trends in the treatment of scleroderma renal crisis. — Shor R, Halabe A Nephron (2002)
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Targeting mediators of vascular injury in scleroderma. — Schachna L, Wigley FM Current opinion in rheumatology (2002)
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Cardiac arrest after labetalol and metoclopramide administration in a patient with scleroderma. — Tung A, Sweitzer B, Cutter T Anesthesia and analgesia (2002)
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Pulmonary hypertension in scleroderma spectrum of disease: lack of bone morphogenetic protein receptor 2 mutations. — Morse J, Barst R, Horn E, Cuervo N, Deng Z, Knowles J The Journal of rheumatology (2002)
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Transdermal application of prostaglandin E1 ethyl ester for the treatment of trophic acral skin lesions in a patient with systemic scleroderma. — Schlez A, Kittel M, Scheurle B, Diehm C, Jünger M Journal of the European Academy of Dermatology and Venereology : JEADV (2002)
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Scleroderma, stroke, optic neuropathy: a rare association. — Das CP, Prabhakar S, Lal V, Kharbanda PS Neurology India (2002)
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Scleroderma with cardiac tamponade, hyperthyroidism and incidental papillary thyroid carcinoma. — Gokula RM, Gupta AK, Shirley SE, Coard K, Ramphal PS The West Indian medical journal (2002)
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Evaluating the cutaneous involvement in scleroderma: torsional stiffness revisited. — Knight LR, Smeathers JE, Isdale AH, Helliwell PS Rheumatology (Oxford, England) (2001)
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Effect of temperature and modulators of protein tyrosine kinase activity on the reactivity of isolated venules in secondary Raynaud's phenomenon. — Furspan PB, Mayes MD, Freedman RR The Journal of rheumatology (2001)
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The heart and pulmonary vasculature in scleroderma: clinical features and pathobiology. — Coghlan JG, Mukerjee D Current opinion in rheumatology (2001)
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Digital arterial occlusion in scleroderma: is there a role for digital arterial reconstruction? — Tomaino MM Journal of hand surgery (Edinburgh, Scotland) (2000)
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Pathogenesis of telangiectasia in scleroderma. — Mould TL, Roberts-Thomson PJ Asian Pacific journal of allergy and immunology (2000)
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Post-stripping sclerodermiform dermatitis. — French LE, Braun R, Masouyé I, Ramelet AA, Humbert PG, Saurat JH Archives of dermatology (1999)
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Endothelial and adrenergic dysfunction in Raynaud's phenomenon and scleroderma. — Freedman RR, Girgis R, Mayes MD The Journal of rheumatology (1999)
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Surgical management of the hand in scleroderma. — Melone CP, McLoughlin JC, Beldner S Current opinion in rheumatology (1999)
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Acute effect of nitric oxide on Raynaud's phenomenon in scleroderma. — Freedman RR, Girgis R, Mayes MD Lancet (London, England) (1999)
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Identification of cation-independent mannose 6-phosphate receptor/insulin-like growth factor type-2 receptor as a novel target of autoantibodies. — Tarrago D, Aguilera I, Melero J, Wichmann I, Nuñez-Roldan A, Sanchez B Immunology (1999)
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Three cases of osteonecrosis of the lunate bone of the wrist in scleroderma. — Matsumoto AK, Moore R, Alli P, Wigley FM Clinical and experimental rheumatology (1999)
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Anti-RNA polymerase III antibodies in the diagnosis of scleroderma renal crisis sine scleroderma. — Phan TG, Cass A, Gillin A, Trew P, Fertig N, Sturgess A The Journal of rheumatology (1999)
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Distribution of macrovascular disease in scleroderma. — Stafford L, Englert H, Gover J, Bertouch J Annals of the rheumatic diseases (1998)
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Manifestations of scleroderma pulmonary disease. — Minai OA, Dweik RA, Arroliga AC Clinics in chest medicine (1998)
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Maternal scleroderma: placental findings and perinatal outcome. — Doss BJ, Jacques SM, Mayes MD, Qureshi F Human pathology (1998)
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CREST syndrome: clinical manifestations and dental management. — Chaffee NR Journal of prosthodontics : official journal of the American College of Prosthodontists (1998)
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Early assessment of pulmonary involvement in limited scleroderma. A case report. — Unsal E, Kilinç O, Kargi A, Necla T The Turkish journal of pediatrics (1998)
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A study of the frequency of pericardial and pleural effusions in scleroderma. — Thompson AE, Pope JE British journal of rheumatology (1998)
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Cyclophosphamide therapy for scleroderma. — Akesson A Current opinion in rheumatology (1998)
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Different patterns of endothelial cell activation in renal and pulmonary vascular disease in scleroderma. — Stratton RJ, Coghlan JG, Pearson JD, Burns A, Sweny P, Abraham DJ et al. QJM : monthly journal of the Association of Physicians (1998)
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A case-control and nerve biopsy study of CREST multiple mononeuropathy. — Dyck PJ, Hunder GG, Dyck PJ Neurology (1997)
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Scleroderma concurrent with culture proven tuberculosis in a Japanese Canadian patient. — Roddy J, Holtby S, Seigel S The Journal of rheumatology (1996)
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Epidemiological analysis of prognosis of 496 Japanese patients with progressive systemic sclerosis (SSc). Scleroderma Research Committee Japan. — Nishioka K, Katayama I, Kondo H, Shinkai H, Ueki H, Tamaki K et al. The Journal of dermatology (1996)
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Prevalence of pulmonary hypertension in limited and diffuse scleroderma. — Battle RW, Davitt MA, Cooper SM, Buckley LM, Leib ES, Beglin PA et al. Chest (1996)
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Raynaud's phenomenon and other features of scleroderma, including pulmonary hypertension. — Wigley FM Current opinion in rheumatology (1996)
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Left ventricular function in scleroderma. — Armstrong GP, Whalley GA, Doughty RN, Gamble GD, Flett SM, Tan PL et al. British journal of rheumatology (1996)
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Raynaud's phenomenon. — Wigley FM, Flavahan NA Rheumatic diseases clinics of North America (1996)
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Tc-99m MIBI uptake secondary to pulmonary interstitial fibrosis in progressive systemic sclerosis. — Richard MD, Park CH Clinical nuclear medicine (1995)
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Raynaud's phenomenon and the vascular disease in scleroderma. — Kahaleh MB Current opinion in rheumatology (1995)
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Scleroderma in children. — Uziel Y, Miller ML, Laxer RM Pediatric clinics of North America (1995)
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Antireflux operations in patients with scleroderma. — Poirier NC, Taillefer R, Topart P, Duranceau A The Annals of thoracic surgery (1994)
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Raynaud's phenomenon and vascular disease in scleroderma. — Kahaleh MB Current opinion in rheumatology (1994)
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Autonomic dysfunction in diffuse scleroderma vs CREST: an assessment by computerized heart rate variability. — Hermosillo AG, Ortiz R, Dábague J, Casanova JM, Martínez-Lavín M The Journal of rheumatology (1994)
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Clinical correlation of anticentromere antibodies. — Zuber M, Gotzen R, Filler I Clinical rheumatology (1994)
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Monoclonal antibodies to heparan sulfate inhibit the formation of thrombin-antithrombin III complexes. — Shibata S, Harpel P, Bona C, Fillit H Clinical immunology and immunopathology (1993)
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Bilateral optic neuropathy associated with the crest variant of scleroderma. — Boschi A, Snyers B, Lambert M European journal of ophthalmology (1993)
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Sclerotherapy as treatment of recurrent bleeding from upper gastrointestinal telangiectasia in CREST syndrome. — Gates C, Morand EF, Davis M, Maddison PJ British journal of rheumatology (1993)
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Myocarditis as a complication in scleroderma patients with myositis. — Kerr LD, Spiera H Clinical cardiology (1993)
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Identification of a subset of patients with scleroderma with severe pulmonary and vascular disease by the presence of autoantibodies to centromere and histone. — Martin L, Pauls JD, Ryan JP, Fritzler MJ Annals of the rheumatic diseases (1993)
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Oral problems associated with CREST syndrome: a case report. — Paley M, McLoughlin P British dental journal (1993)
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Scleroderma. — Miller M Australian family physician (1993)
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Flunarizine is effective in prophylaxis of headache associated with scleroderma. — Mazagri R, Shuaib A Headache (1992)
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Subcutaneous symmetrical morphea with unusual onset as nodular vasculitis. — Stefanato C, Chlebus E, Janniger CK, Jablonska S Cutis (1992)
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CENP-C, an autoantigen in scleroderma, is a component of the human inner kinetochore plate. — Saitoh H, Tomkiel J, Cooke CA, Ratrie H, Maurer M, Rothfield NF et al. Cell (1992)
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Reproductive function prior to disease onset in women with scleroderma. — Englert H, Brennan P, McNeil D, Black C, Silman AJ The Journal of rheumatology (1992)
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Radical microarteriolysis in the treatment of vasospastic disorders of the hand, especially scleroderma. — O'Brien BM, Kumar PA, Mellow CG, Oliver TV Journal of hand surgery (Edinburgh, Scotland) (1992)

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