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CREST Syndrome Workup

  • Author: Jeanie C Yoon, MD; Chief Editor: Dirk M Elston, MD  more...
 
Updated: Oct 15, 2015
 

Laboratory Studies

Laboratory studies are as follows:

  • ANAs: Limited scleroderma is associated with an early rise in ANA levels, particularly of the immunoglobulin G3 subclass. The overall sensitivity of ANA in systemic sclerosis is 85%, while the specificity is approximately 54%. Serial testing of ANAs to monitor the progress of disease is not currently recommended.[50]
  • Anticentromere antibodies: These are found in approximately 50-90% of patients with limited forms of scleroderma; Anticentromere antibodies are present in 82-96% of patients with the CREST variant. The specificity of this test is 95%.[51]  Anti-Scl-70 (anti-topoisomerase I) antibody is associated with diffuse scleroderma, early internal organ involvement, and a worse prognosis. Perform this laboratory test early in the course of the patient's presentation to determine if the patient is at risk for this course of scleroderma. Nonspecific indicators of inflammation (eg, mild leukocytosis, normocytic-normochromic anemia, thrombocytosis, elevated erythrocyte sedimentation rate, elevated C-reactive protein) are rare but may be present in persons with limited scleroderma.
  • Calcinosis: Evaluate serum calcium and phosphorus levels to exclude a metabolic disturbance; however, calcinosis resulting from limited scleroderma is not associated with calcium or phosphorus abnormalities.
  • Raynaud phenomenon: The presence of ANA predicts the development of connective-tissue disease. The positive and negative predictive values of ANA values by immunofluorescence are 65% and 93%, respectively.
  • Esophageal dysmotility: Patients who are positive for ANAs and anticentromere antibodies while also being negative for anti-Scl70 antibody appear to have more esophageal involvement.[52]
  • Sclerodactyly: A thyrotropin level may help exclude the presence of thyroid disease as another potential cause of edematous or thickened skin.
  • Telangiectasia: No laboratory data are necessary.
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Imaging Studies

Imaging studies are as follows:

  • Calcinosis: The diagnosis of calcinosis (suspected based on physical examination finding of palpable, hard, subcutaneous nodules) is confirmed with plain radiographs demonstrating dermal or subcutaneous radiodense deposits. Computed tomography and bone scanning (skeletal scintigraphy with diphosphate compounds) are more sensitive for identifying calcinosis when plain radiography findings are normal.[53]  Videodermatoscopy may also be a useful tool for diagnosing dystrophic calcifications such as those resulting from CREST syndrome; however, it is not yet widely used.[54]
  • Raynaud phenomenon: Digital ischemia and/or infarction secondary to Raynaud disease may prompt Doppler ultrasonography or angiography to identify anatomic occlusive disease, which may be amenable to angioplasty or bypass surgery.
  • Esophageal dysmotility: Multiple modalities are available to evaluate esophageal dysmotility. The least invasive evaluation involves radiologic barium studies with attention to the esophagus (cine-esophagram). Findings include dilatation with decreased or absent peristalsis in the distal esophagus, indicating advanced disease. Esophageal stricture and ulcerations indicate late findings of erosive esophagitis. The best use of a barium swallow is to exclude a stricture when the patient reports dysphagia. Esophageal transit time can be demonstrated by fluoroscopy, but this is not clinically useful. Radionuclide scanning is specific for gastroesophageal reflux and pulmonary aspiration, but it is expensive and is not sensitive.
  • Sclerodactyly: No imaging studies are necessary.
  • Telangiectasia: No imaging studies are necessary for cutaneous telangiectasia.
  • Cough or fatigue: If patients have cough or fatigue, perform transthoracic echocardiography to look for pulmonary hypertension. More advanced pulmonary hypertension may demonstrate signs of right-sided heart enlargement on chest radiographs.
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Other Tests

Other tests that may be needed are as follows:

  • Raynaud phenomenon: Research tools, such as strain plethysmography, laser Doppler flowmetry, thermography, or finger systolic blood pressure, are not useful clinically for diagnosis, monitoring disease progression, or monitoring response to treatment.
  • Esophageal dysmotility: Esophageal manometry is highly sensitive for abnormal motility. Findings include diminished or absent distal peristalsis. LES pressure often is diminished. Manometry is fairly simple and inexpensive; abnormal results predict the development of erosive esophagitis, and findings are more likely to be positive in CREST syndrome patients.[55]  Twenty-four–hour pH monitoring is greater than 90% sensitive for the presence of gastroesophageal reflux; however, this test is more cumbersome and expensive. Esophagogastroduodenoscopy can identify the gross or histologic presence of esophagitis and the complications of esophagitis (eg, ulcers, stricture, Barrett esophagitis, adenocarcinoma). A biopsy is required to make the diagnosis of Barrett esophagitis or adenocarcinoma.
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Contributor Information and Disclosures
Author

Jeanie C Yoon, MD Clinical Instructor, Department of General Internal Medicine, University of Washington School of Medicine

Jeanie C Yoon, MD is a member of the following medical societies: American College of Physicians, Society of Hospital Medicine

Disclosure: Nothing to disclose.

Coauthor(s)

Gregory J Raugi, MD, PhD Professor, Department of Internal Medicine, Division of Dermatology, University of Washington at Seattle School of Medicine; Chief, Dermatology Section, Primary and Specialty Care Service, Veterans Administration Medical Center of Seattle

Gregory J Raugi, MD, PhD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Nothing to disclose.

Specialty Editor Board

Richard P Vinson, MD Assistant Clinical Professor, Department of Dermatology, Texas Tech University Health Sciences Center, Paul L Foster School of Medicine; Consulting Staff, Mountain View Dermatology, PA

Richard P Vinson, MD is a member of the following medical societies: American Academy of Dermatology, Texas Medical Association, Association of Military Dermatologists, Texas Dermatological Society

Disclosure: Nothing to disclose.

Jeffrey P Callen, MD Professor of Medicine (Dermatology), Chief, Division of Dermatology, University of Louisville School of Medicine

Jeffrey P Callen, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American College of Physicians, American College of Rheumatology

Disclosure: Received income in an amount equal to or greater than $250 from: XOMA; Biogen/IDEC; Novartis; Janssen Biotech, Abbvie, CSL pharma<br/>Received honoraria from UpToDate for author/editor; Received honoraria from JAMA Dermatology for associate editor and intermittent author; Received royalty from Elsevier for book author/editor; Received dividends from trust accounts, but I do not control these accounts, and have directed our managers to divest pharmaceutical stocks as is fiscally prudent from Stock holdings in various trust accounts include some pharmaceutical companies and device makers for i inherited these trust accounts; for: Celgene; Pfizer; 3M; Johnson and Johnson; Merck; Abbott Laboratories; AbbVie; Procter and Gamble; Amgen.

Chief Editor

Dirk M Elston, MD Professor and Chairman, Department of Dermatology and Dermatologic Surgery, Medical University of South Carolina College of Medicine

Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Nothing to disclose.

Acknowledgements

The authors and editors of Medscape Reference wish to thank Dr. Bruce Gilliland for assistance in reviewing the manuscript and Dr. Jan V. Hirschmann and Dr. Netayna Sandler for their images.

The authors and editors of Medscape Reference also gratefully acknowledge the contributions of previous authors, Mary A. Wemple, MD, and Kyle L. Horner, MD, MS, to the development and writing of this article.

References
  1. Meyer O. [From Thibierge-Weissenbach syndrome (1910) to anti-centromere antibodies (1980). Clinical and biological features of scleroderma]. Ann Med Interne (Paris). 1999 Jan. 150(1):47-52. [Medline].

  2. Winterbauer RH. Multiple telangiectasia, Raynaud's phenomenon, sclerodactyly, and subcutaneous calcinosis: A syndrome mimicking hereditary hemorrhagic telangiectasia. Bull Johns Hopkins Hosp. 1964 Jun. 114:361-83. [Medline].

  3. Frayha RA, Scarola JA, Shulman LE. Calcinosis in scleroderma: A reevaluation of the CRST syndrome, abstracted. Arthritis Rheum. 1973. 16:542.

  4. Velayos EE, Masi AT, Stevens MB, Shulman LE. The 'CREST' syndrome. Comparison with systemic sclerosis (scleroderma). Arch Intern Med. 1979 Nov. 139(11):1240-4. [Medline].

  5. Rodnan GP, Jablonska S, Medsger TA. Classification and nomenclature of progressive systemic sclerosis (scleroderma). Clin Rheum Dis. 1979. 5:5-13.

  6. Barnett AJ, Miller MH, Littlejohn GO. A survival study of patients with scleroderma diagnosed over 30 years (1953-1983): the value of a simple cutaneous classification in the early stages of the disease. J Rheumatol. 1988 Feb. 15(2):276-83. [Medline].

  7. Tuffanelli DL, Winkelmann RK. Diffuse systemic scleroderma. A comparison with acrosclerosis. Ann Intern Med. 1962 Aug. 57:198-203. [Medline].

  8. Ferri C, Valentini G, Cozzi F, Sebastiani M, Michelassi C, La Montagna G, et al. Systemic sclerosis: demographic, clinical, and serologic features and survival in 1,012 Italian patients. Medicine (Baltimore). 2002 Mar. 81(2):139-53. [Medline].

  9. Scussel-Lonzetti L, Joyal F, Raynauld JP, Roussin A, Rich E, Goulet JR, et al. Predicting mortality in systemic sclerosis: analysis of a cohort of 309 French Canadian patients with emphasis on features at diagnosis as predictive factors for survival. Medicine (Baltimore). 2002 Mar. 81(2):154-67. [Medline].

  10. LeRoy EC, Black C, Fleischmajer R, Jablonska S, Krieg T, Medsger TA Jr, et al. Scleroderma (systemic sclerosis): classification, subsets and pathogenesis. J Rheumatol. 1988 Feb. 15(2):202-5. [Medline].

  11. Mayes MD. Scleroderma epidemiology. Rheum Dis Clin North Am. 1996 Nov. 22(4):751-64. [Medline].

  12. Nadashkevich O, Davis P, Fritzler MJ. A proposal of criteria for the classification of systemic sclerosis. Med Sci Monit. 2004 Nov. 10(11):CR615-21. [Medline].

  13. Maricq HR, Valter I. A working classification of scleroderma spectrum disorders: a proposal and the results of testing on a sample of patients. Clin Exp Rheumatol. 2004 Jan-Feb. 22(3 Suppl 33):S5-13. [Medline].

  14. Wollheim FA. Classification of systemic sclerosis. Visions and reality. Rheumatology (Oxford). 2005 Oct. 44(10):1212-6. [Medline].

  15. Postiglione L, Montagnani S, Riccio A, Montuori N, Sciorio S, Ladogana P, et al. Enhanced expression of the receptor for granulocyte macrophage colony stimulating factor on dermal fibroblasts from scleroderma patients. J Rheumatol. 2002 Jan. 29(1):94-101. [Medline].

  16. Kawakami T, Soma Y, Mizoguchi M, Saito R. Immunohistochemical expression of transforming growth factor beta3 in calcinosis in a patient with systemic sclerosis and CREST syndrome. Br J Dermatol. 2000 Nov. 143(5):1098-100. [Medline].

  17. Postiglione L, Ladogana P, Montagnani S, di Spigna G, Castaldo C, Turano M, et al. Effect of granulocyte macrophage-colony stimulating factor on extracellular matrix deposition by dermal fibroblasts from patients with scleroderma. J Rheumatol. 2005 Apr. 32(4):656-64. [Medline].

  18. Kikuchi K, Kadono T, Furue M, Tamaki K. Tissue inhibitor of metalloproteinase 1 (TIMP-1) may be an autocrine growth factor in scleroderma fibroblasts. J Invest Dermatol. 1997 Mar. 108(3):281-4. [Medline].

  19. de Oliveira JG, Guedes AC, Lanna CC, Coelho LF, Prados RZ, Feghali C, et al. Protease nexin-1 messenger RNA levels are not affected by serum or interferon beta in cultured systemic sclerosis fibroblasts. Arch Dermatol Res. 2002 Jan. 293(11):584-9. [Medline].

  20. Helmbold P, Fiedler E, Fischer M, Marsch WCh. Hyperplasia of dermal microvascular pericytes in scleroderma. J Cutan Pathol. 2004 Jul. 31(6):431-40. [Medline].

  21. LeRoy EC. Systemic sclerosis. A vascular perspective. Rheum Dis Clin North Am. 1996 Nov. 22(4):675-94. [Medline].

  22. Klareskog L, Gustafsson R, Scheynius A, Hällgren R. Increased expression of platelet-derived growth factor type B receptors in the skin of patients with systemic sclerosis. Arthritis Rheum. 1990 Oct. 33(10):1534-41. [Medline].

  23. Nelson JL. Microchimerism and the pathogenesis of systemic sclerosis. Curr Opin Rheumatol. 1998 Nov. 10(6):564-71. [Medline].

  24. Mayes MD, Lacey JV Jr, Beebe-Dimmer J, Gillespie BW, Cooper B, Laing TJ, et al. Prevalence, incidence, survival, and disease characteristics of systemic sclerosis in a large US population. Arthritis Rheum. 2003 Aug. 48(8):2246-55. [Medline].

  25. Arnett FC, Howard RF, Tan F, Moulds JM, Bias WB, Durban E, et al. Increased prevalence of systemic sclerosis in a Native American tribe in Oklahoma. Association with an Amerindian HLA haplotype. Arthritis Rheum. 1996 Aug. 39(8):1362-70. [Medline].

  26. Steen VD, Powell DL, Medsger TA Jr. Clinical correlations and prognosis based on serum autoantibodies in patients with systemic sclerosis. Arthritis Rheum. 1988 Feb. 31(2):196-203. [Medline].

  27. Pakunpanya K, Verasertniyom O, Vanichapuntu M, Pisitkun P, Totemchokchyakarn K, Nantiruj K, et al. Incidence and clinical correlation of anticentromere antibody in Thai patients. Clin Rheumatol. 2006 May. 25(3):325-8. [Medline].

  28. Silman AJ, Newman J. Epidemiology of systemic sclerosis. Curr Opin Rheumatol. 1996 Nov. 8(6):585-9. [Medline].

  29. Alamanos Y, Tsifetaki N, Voulgari PV, Siozos C, Tsamandouraki K, Alexiou GA, et al. Epidemiology of systemic sclerosis in northwest Greece 1981 to 2002. Semin Arthritis Rheum. 2005 Apr. 34(5):714-20. [Medline].

  30. Allcock RJ, Forrest I, Corris PA, Crook PR, Griffiths ID. A study of the prevalence of systemic sclerosis in northeast England. Rheumatology (Oxford). 2004 May. 43(5):596-602. [Medline].

  31. Le Guern V, Mahr A, Mouthon L, Jeanneret D, Carzon M, Guillevin L. Prevalence of systemic sclerosis in a French multi-ethnic county. Rheumatology (Oxford). 2004 Sep. 43(9):1129-37. [Medline].

  32. Englert H, Small-McMahon J, Davis K, O'Connor H, Chambers P, Brooks P. Systemic sclerosis prevalence and mortality in Sydney 1974-88. Aust N Z J Med. 1999 Feb. 29(1):42-50. [Medline].

  33. Akesson A, Wollheim FA. Organ manifestations in 100 patients with progressive systemic sclerosis: a comparison between the CREST syndrome and diffuse scleroderma. Br J Rheumatol. 1989 Aug. 28(4):281-6. [Medline].

  34. Zamost BJ, Hirschberg J, Ippoliti AF, Furst DE, Clements PJ, Weinstein WM. Esophagitis in scleroderma. Prevalence and risk factors. Gastroenterology. 1987 Feb. 92(2):421-8. [Medline].

  35. Johnson DA, Drane WE, Curran J, Cattau EL Jr, Ciarleglio C, Khan A, et al. Pulmonary disease in progressive systemic sclerosis. A complication of gastroesophageal reflux and occult aspiration?. Arch Intern Med. 1989 Mar. 149(3):589-93. [Medline].

  36. Lachter J, Suissa A, Schiff E, Rosner I. Anemia in CREST syndrome. Isr Med Assoc J. 2003 Jun. 5(6):449. [Medline].

  37. Meyer O. [Crest Syndrome]. Ann med Interne (Paris). 2002. 153(3):183-188. [Medline].

  38. Mavrogeni S, Bratis C, Manoussakis M. Coronary artery abnormalities in CREST syndrome revealed by cardiovascular magnetic resonance imaging. Can J Cardiol. 2011 May-Jun. 27(3):390.e5-7. [Medline].

  39. Kouraklis G, Glinavou A, Karatzas G. Primary biliary cirrhosis accompanied by CREST syndrome. South Med J. 2002 Sep. 95(9):1058-9. [Medline].

  40. Tojo J, Ohira H, Suzuki T, Takeda I, Shoji I, Kojima T, et al. Clinicolaboratory characteristics of patients with primary biliary cirrhosis associated with CREST symptoms. Hepatol Res. 2002 Mar. 22(3):187-195. [Medline].

  41. Hill CL, Nguyen AM, Roder D, Roberts-Thomson P. Risk of cancer in patients with scleroderma: a population based cohort study. Ann Rheum Dis. 2003 Aug. 62(8):728-31. [Medline].

  42. Desai K, Mian NZ. Osteonecrosis of multiple joints in a patient with limited scleroderma/CREST syndrome. J Clin Rheumatol. 2015 Apr. 21 (3):169-70. [Medline].

  43. Sjogren RW. Gastrointestinal motility disorders in scleroderma. Arthritis Rheum. 1994 Sep. 37(9):1265-82. [Medline].

  44. Gladman DD, Kung TN, Siannis F, Pellett F, Farewell VT, Lee P. HLA markers for susceptibility and expression in scleroderma. J Rheumatol. 2005 Aug. 32(8):1481-7. [Medline].

  45. Bovenzi M, Barbone F, Pisa FE, Betta A, Romeo L, Tonello A, et al. A case-control study of occupational exposures and systemic sclerosis. Int Arch Occup Environ Health. 2004 Jan. 77(1):10-6. [Medline].

  46. Maître A, Hours M, Bonneterre V, Arnaud J, Arslan MT, Carpentier P, et al. Systemic sclerosis and occupational risk factors: role of solvents and cleaning products. J Rheumatol. 2004 Dec. 31(12):2395-401. [Medline].

  47. Mayes MD. Endothelin and endothelin receptor antagonists in systemic rheumatic disease. Arthritis Rheum. 2003 May. 48(5):1190-9. [Medline].

  48. Anastasopoulos G, Marinis A, Konstantinidis C, Theodosopoulos T, Fragulidis G, Vassiliou I. Adenocarcinoma of the third portion of the duodenum in a man with CREST syndrome. World J Surg Oncol. 2008 Oct 1. 6:106. [Medline]. [Full Text].

  49. Lauritano D, Bussolati A, Baldoni M, Leonida A. Scleroderma and CREST syndrome: a case report in dentistry. Minerva Stomatol. 2011 Sep. 60(9):443-66. [Medline].

  50. van Paassen P, Damoiseaux J, Tervaert JW. Laboratory assessment in musculoskeletal disorders. Best Pract Res Clin Rheumatol. 2003 Jun. 17(3):475-94. [Medline].

  51. Aeschlimann A, Meyer O, Bourgeois P, Haim T, Belmatoug N, Palazzo E, et al. Anti-Scl-70 antibodies detected by immunoblotting in progressive systemic sclerosis: specificity and clinical correlations. Ann Rheum Dis. 1989 Dec. 48(12):992-7. [Medline].

  52. Gonzalez R, Storr M, Bloching H, Seige M, Ott R, Allescher HD. Autoantibody profile in progressive systemic sclerosis as markers for esophageal involvement. J Clin Gastroenterol. 2001 Feb. 32(2):123-7. [Medline].

  53. Bar-Sever Z, Mukamel M, Harel L, Hardoff R. Scintigraphic evaluation of calcinosis in juvenile dermatomyositis with Tc-99m MDP. Clin Nucl Med. 2000 Dec. 25(12):1013-6. [Medline].

  54. Strumia R. Videodermatoscopy: a useful tool for diagnosing cutaneous dystrophic calcifications. Dermatol Online J. 2005. 11(1):28. [Medline].

  55. Ling TC, Johnston BT. Esophageal investigations in connective tissue disease: which tests are most appropriate?. J Clin Gastroenterol. 2001 Jan. 32(1):33-6. [Medline].

  56. Joslin N. Early identification key to scleroderma treatment. Nurse Pract. 2004 Jul. 29(7):24-39; quiz 40-1. [Medline].

  57. Rus V, White B. Systemic sclerosis: multiple therapies control morbidity. J Musculoskel Med. 2002. 19:110-121.

  58. Samuelson UK, Ahlmen EM. Development and evaluation of a patient education program for persons with systemic sclerosis (scleroderma). Arthritis Care Res. 2000 Jun. 13(3):141-8. [Medline].

  59. Legendre C, Allanore Y, Ferrand I, Kahan A. Evaluation of depression and anxiety in patients with systemic sclerosis. Joint Bone Spine. 2005 Oct. 72(5):408-11. [Medline].

  60. Matsuura E, Ohta A, Kanegae F, Haruda Y, Ushiyama O, Koarada S, et al. Frequency and analysis of factors closely associated with the development of depressive symptoms in patients with scleroderma. J Rheumatol. 2003 Aug. 30(8):1782-7. [Medline].

  61. Farge D, Passweg J, van Laar JM, Marjanovic Z, Besenthal C, Finke J, et al. Autologous stem cell transplantation in the treatment of systemic sclerosis: report from the EBMT/EULAR Registry. Ann Rheum Dis. 2004 Aug. 63(8):974-81. [Medline].

  62. Thomas-Golbanov CK, Wilke WS, Fessler BJ, Hoffman GS. Open label trial of tamoxifen in scleroderma. Clin Exp Rheumatol. 2003 Jan-Feb. 21(1):99-102. [Medline].

  63. Fink CW, Cook JD. Spontaneous resolution of calcinosis in childhood dermatomyositis. Arthritis Rheum. 1986. 29(suppl):S91.

  64. Merlino G, Germano S, Carlucci S. Surgical management of digital calcinosis in CREST syndrome. Aesthetic Plast Surg. 2013 Dec. 37 (6):1214-9. [Medline].

  65. Hazen PG, Walker AE, Carney JF, Stewart JJ. Cutaneous calcinosis of scleroderma. Successful treatment with intralesional adrenal steroids. Arch Dermatol. 1982 May. 118(5):366-7. [Medline].

  66. Vayssairat M, Hidouche D, Abdoucheli-Baudot N, Gaitz JP. Clinical significance of subcutaneous calcinosis in patients with systemic sclerosis. Does diltiazem induce its regression?. Ann Rheum Dis. 1998 Apr. 57(4):252-4. [Medline].

  67. Berger RG, Featherstone GL, Raasch RH, McCartney WH, Hadler NM. Treatment of calcinosis universalis with low-dose warfarin. Am J Med. 1987 Jul. 83(1):72-6. [Medline].

  68. Cukierman T, Elinav E, Korem M, Chajek-Shaul T. Low dose warfarin treatment for calcinosis in patients with systemic sclerosis. Ann Rheum Dis. 2004 Oct. 63(10):1341-3. [Medline].

  69. Lassoued K, Saiag P, Anglade MC, Roujeau JC, Touraine RL. Failure of warfarin in treatment of calcinosis universalis. Am J Med. 1988 Apr. 84(4):795-6. [Medline].

  70. Boulman N, Slobodin G, Rozenbaum M, Rosner I. Calcinosis in rheumatic diseases. Semin Arthritis Rheum. 2005 Jun. 34(6):805-12. [Medline].

  71. Rabens SF, Bethune JE. Disodium etidronate therapy for dystrophic cutaneous calcification. Arch Dermatol. 1975 Mar. 111(3):357-61. [Medline].

  72. Metzger AL, Singer FR, Bluestone R, Pearson CM. Failure of disodium etidronate in calcinosis due to dermatomyositis and scleroderma. N Engl J Med. 1974 Dec 12. 291(24):1294-6. [Medline].

  73. Ambler GR, Chaitow J, Rogers M, McDonald DW, Ouvrier RA. Rapid improvement of calcinosis in juvenile dermatomyositis with alendronate therapy. J Rheumatol. 2005 Sep. 32(9):1837-9. [Medline].

  74. Robertson LP, Marshall RW, Hickling P. Treatment of cutaneous calcinosis in limited systemic sclerosis with minocycline. Ann Rheum Dis. 2003 Mar. 62(3):267-9. [Medline].

  75. Taborn J, Bole GG, Thompson GR. Colchicine suppression of local and systemic inflammation due to calcinosis universalis in chronic dermatomyositis. Ann Intern Med. 1978 Nov. 89(5 Pt 1):648-9. [Medline].

  76. Kalajian AH, Perryman JH, Callen JP. Intravenous immunoglobulin therapy for dystrophic calcinosis cutis: unreliable in our hands. Arch Dermatol. 2009 Mar. 145(3):334; author reply 335. [Medline].

  77. Goodfield MJ, Hume A, Rowell NR. The effect of simple warming procedures on finger blood flow in systemic sclerosis. Br J Dermatol. 1988 May. 118(5):661-8. [Medline].

  78. Freedman RR. Quantitative measurements of finger blood flow during behavioral treatments for Raynaud''s disease. Psychophysiology. 1989 Jul. 26(4):437-41. [Medline].

  79. Karavidas MK, Tsai PS, Yucha C, McGrady A, Lehrer PM. Thermal biofeedback for primary Raynaud's phenomenon: a review of the literature. Appl Psychophysiol Biofeedback. 2006 Sep. 31(3):203-16. [Medline].

  80. Meyrick Thomas RH, Rademaker M, Grimes SM, MacKay A, Kovacs IB, Cook ED, et al. Nifedipine in the treatment of Raynaud's phenomenon in patients with systemic sclerosis. Br J Dermatol. 1987 Aug. 117(2):237-41. [Medline].

  81. Thompson AE, Pope JE. Calcium channel blockers for primary Raynaud's phenomenon: a meta-analysis. Rheumatology (Oxford). 2005 Feb. 44(2):145-50. [Medline].

  82. Fischer M, Reinhold B, Falck H, Torok M, Alexander K. Topical nitroglycerin ointment in Raynaud's phenomenon. Z Kardiol. 1985 May. 74(5):298-302. [Medline].

  83. Anderson ME, Moore TL, Hollis S, Jayson MI, King TA, Herrick AL. Digital vascular response to topical glyceryl trinitrate, as measured by laser Doppler imaging, in primary Raynaud's phenomenon and systemic sclerosis. Rheumatology (Oxford). 2002 Mar. 41(3):324-8. [Medline].

  84. Teh LS, Manning J, Moore T, Tully MP, O'Reilly D, Jayson MI. Sustained-release transdermal glyceryl trinitrate patches as a treatment for primary and secondary Raynaud's phenomenon. Br J Rheumatol. 1995 Jul. 34(7):636-41. [Medline].

  85. Torley HI, Madhok R, Capell HA, Brouwer RM, Maddison PJ, Black CM, et al. A double blind, randomised, multicentre comparison of two doses of intravenous iloprost in the treatment of Raynaud's phenomenon secondary to connective tissue diseases. Ann Rheum Dis. 1991 Nov. 50(11):800-4. [Medline].

  86. Milio G, Corrado E, Genova C, Amato C, Raimondi F, Almasio PL, et al. Iloprost treatment in patients with Raynaud's phenomenon secondary to systemic sclerosis and the quality of life: a new therapeutic protocol. Rheumatology (Oxford). 2006 Aug. 45(8):999-1004. [Medline].

  87. Kaminaga T, Takada K, Kutomi K, Imai E, Furui S. Improvement in cold-induced Raynaud's syndrome with administration of ticlopidine hydrochloride detected by Tc-99m DTPA human serum albumin scintigraphy. Clin Nucl Med. 2002 Feb. 27(2):131-2. [Medline].

  88. Rajagopalan S, Pfenninger D, Somers E, Kehrer C, Chakrabarti A, Mukherjee D, et al. Effects of cilostazol in patients with Raynaud's syndrome. Am J Cardiol. 2003 Dec 1. 92(11):1310-5. [Medline].

  89. Dean SM, Satiani B. Three cases of digital ischemia successfully treated with cilostazol. Vasc Med. 2001 Nov. 6(4):245-8. [Medline].

  90. Bridges AJ, Spadone DP. Tissue plasminogen activator treatment of digital thrombosis in severe Raynaud's phenomenon--a case report. Angiology. 1993 Jul. 44(7):566-9. [Medline].

  91. Denton CP, Howell K, Stratton RJ, Black CM. Long-term low molecular weight heparin therapy for severe Raynaud's phenomenon: a pilot study. Clin Exp Rheumatol. 2000 Jul-Aug. 18(4):499-502. [Medline].

  92. Jacobs MJ, Jörning PJ, Van Rhede van der Kloot EJ, Kitslaar PJ, Lemmens HA, Slaaf DW, et al. Plasmapheresis in Raynaud's phenomenon in systemic sclerosis: a microcirculatory study. Int J Microcirc Clin Exp. 1991 Feb. 10(1):1-11. [Medline].

  93. O'Reilly MJ, Talpos G, Roberts VC, White JM, Cotton LT. Controlled trial of plasma exchange in treatment of Raynaud's syndrome. Br Med J. 1979 Apr 28. 1(6171):1113-5. [Medline].

  94. Koo AP. Therapeutic apheresis in autoimmune and rheumatic diseases. J Clin Apher. 2000. 15(1-2):18-27. [Medline].

  95. Dziadzio M, Denton CP, Smith R, Howell K, Blann A, Bowers E, et al. Losartan therapy for Raynaud''s phenomenon and scleroderma: clinical and biochemical findings in a fifteen-week, randomized, parallel-group, controlled trial. Arthritis Rheum. 1999 Dec. 42(12):2646-55. [Medline].

  96. Pancera P, Sansone S, Secchi S, Covi G, Lechi A. The effects of thromboxane A2 inhibition (picotamide) and angiotensin II receptor blockade (losartan) in primary Raynaud's phenomenon. J Intern Med. 1997 Nov. 242(5):373-6. [Medline].

  97. Wood HM, Ernst ME. Renin-angiotensin system mediators and Raynaud's phenomenon. Ann Pharmacother. 2006 Nov. 40(11):1998-2002. [Medline].

  98. Baumhaekel M, Scheffler P, Boehm M. Use of tadalafil in a patient with a secondary Raynaud's phenomenon not responding to sildenafil. Microvasc Res. 2005 May. 69(3):178-9. [Medline].

  99. Gore J, Silver R. Oral sildenafil for the treatment of Raynaud's phenomenon and digital ulcers secondary to systemic sclerosis. Ann Rheum Dis. 2005 Sep. 64(9):1387. [Medline].

  100. Caglayan E, Huntgeburth M, Karasch T, Weihrauch J, Hunzelmann N, Krieg T, et al. Phosphodiesterase type 5 inhibition is a novel therapeutic option in Raynaud disease. Arch Intern Med. 2006 Jan 23. 166(2):231-3. [Medline].

  101. 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 Rheum. 2004 Dec. 50(12):3994-4001. [Medline].

  102. Johnson S, Iazzetta J, Dewar C. Severe Raynaud's phenomenon with yohimbine therapy for erectile dysfunction. J Rheumatol. 2003 Nov. 30(11):2503-5. [Medline].

  103. Rubin LJ, Badesch DB, Barst RJ, Galie N, Black CM, Keogh A, et al. Bosentan therapy for pulmonary arterial hypertension. N Engl J Med. 2002 Mar 21. 346(12):896-903. [Medline].

  104. Coleiro B, Marshall SE, Denton CP, Howell K, Blann A, Welsh KI, et al. Treatment of Raynaud's phenomenon with the selective serotonin reuptake inhibitor fluoxetine. Rheumatology (Oxford). 2001 Sep. 40(9):1038-43. [Medline].

  105. Garcia-Porrua C, Margarinos CC, Gonzalez-Gay MA. Raynaud's phenomenon and serotonin reuptake inhibitors. J Rheumatol. 2004 Oct. 31(10):2090; author reply 2090-1. [Medline].

  106. Herrick AL. Treatment of Raynaud's phenomenon: new insights and developments. Curr Rheumatol Rep. 2003 Apr. 5(2):168-74. [Medline].

  107. Wang SJ, La JL, Chen DY, Chen YH, Hsieh TY, Lin WY. Effects of cisapride on oesophageal transit of solids in patients with progressive systemic sclerosis. Clin Rheumatol. 2002 Feb. 21(1):43-5. [Medline].

  108. Hulshof MM, Bouwes Bavinck JN, Bergman W, Masclee AA, Heickendorff L, Breedveld FC, et al. Double-blind, placebo-controlled study of oral calcitriol for the treatment of localized and systemic scleroderma. J Am Acad Dermatol. 2000 Dec. 43(6):1017-23. [Medline].

  109. Clements PJ, Furst DE, Wong WK, Mayes M, White B, Wigley F, et al. High-dose versus low-dose D-penicillamine in early diffuse systemic sclerosis: analysis of a two-year, double-blind, randomized, controlled clinical trial. Arthritis Rheum. 1999 Jun. 42(6):1194-203. [Medline].

  110. Alam M, Dover JS, Arndt KA. Treatment of facial telangiectasia with variable-pulse high-fluence pulsed-dye laser: comparison of efficacy with fluences immediately above and below the purpura threshold. Dermatol Surg. 2003 Jul. 29(7):681-4; discussion 685. [Medline].

  111. Saddic N, Miller JJ, Miller OF 3rd, Clarke JT. Surgical debridement of painful fingertip calcinosis cutis in CREST syndrome. Arch Dermatol. 2009 Feb. 145(2):212-3. [Medline].

  112. Melone CP Jr, McLoughlin JC, Beldner S. Surgical management of the hand in scleroderma. Curr Opin Rheumatol. 1999 Nov. 11(6):514-20. [Medline].

  113. Chamberlain AJ, Walker NP. Successful palliation and significant remission of cutaneous calcinosis in CREST syndrome with carbon dioxide laser. Dermatol Surg. 2003 Sep. 29(9):968-70. [Medline].

  114. Bottomley WW, Goodfield MJ, Sheehan-Dare RA. Digital calcification in systemic sclerosis: effective treatment with good tissue preservation using the carbon dioxide laser. Br J Dermatol. 1996 Aug. 135(2):302-4. [Medline].

  115. Medsger TA. Systemic sclerosis (scleroderma): Clinical aspects. Koopman WJ, ed. Arthritis and Allied Conditions: A Textbook of Rheumatology. 13th ed. Williams and Wilkins; 1997. 1433-64.

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Calcinosis on dorsal forearm.
Close-up view of calcinosis.
Raynaud phenomenon showing pallor of most of the finger tips with a violaceous discoloration (hyperemia) of the thumb tip.
Sclerodactyly (also with Raynaud phenomenon).
Sclerodactyly.
Telangiectasia of the face.
Telangiectasia of the lip.
Telangiectasia of the finger.
Close-up view of telangiectasia.
 
 
 
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