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PROMs for Systemic Sclerosis (Scleroderma)

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Patient Reported Outcome Measures in Rheumatic Diseases

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Abstract

Scleroderma (SSc, systemic sclerosis) is a rare autoimmune connective tissue disease that often involves multiple organs and thus can affect many aspects from the view point of the patient. Patient-reported outcomes (PROs) are needed to capture these features. PROs that are commonly used in SSc include patient global assessment, function as measured by the Health Assessment Questionnaire Disability Index (HAQ, HAQ-DI), pain, fatigue, Raynaud’s Condition Score (RCS), gastrointestinal tract (UCLA SCTC GIT 2.0), the Symptom Burden Index (SBI), and the Scleroderma HAQ (SHAQ),which is the HAQ with added scales. Some measures are specific to SSc and others are general. General measures include health-related quality of life measures, such as the SF-36, that allow comparison between diseases. Specific measures have been developed for SSc and are usually validated using clinic patients or trial patients with SSc. The latter includes UCLA SCTC GIT 2.0, Symptom Burden Index, Mouth Handicap Scale in Systemic Sclerosis, and the UK SSc Functional Score. The RCS can be applied to any patient with either primary or secondary Raynaud’s Phenomenon (RP) including RP related to SSc. The majority of SSc patients have hand involvement. There are multiple measures for hand function that are not specific for SSc, including the HAQ; Cochin Hand Function Scale; Michigan Hand Questionnaire; and the Disabilities of the Arm, Shoulder and Hand (DASH). It is difficult to encompass all problems that occur in SSc with only one measurement tool. However, PROs in SSc are important to aid in understanding the impact of SSc on patients.

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References

  1. Pattanaik D, Brown M, Postlethwaite BC, Postlethwaite AE. Pathogenesis of systemic sclerosis. Front Immunol. 2015;6:272. doi:10.3389/fimmu.2015.00272.

    Article  PubMed  PubMed Central  CAS  Google Scholar 

  2. Allanore Y, Avouac J, Kahan A. Systemic sclerosis: an update in 2008. Joint Bone Spine. 2008;75(6):650–5. doi:10.1016/j.jbspin.2008.07.003.

    Article  PubMed  Google Scholar 

  3. Johnson SR, Glaman DD, Schentag CT, Lee P. Quality of life and functional status in systemic sclerosis compared to other rheumatic diseases. J Rheumatol. 2006;33(6):1117–22.

    PubMed  Google Scholar 

  4. Mayes MD, Lacey Jr JV, Beebe-Dimmer J, et al. Prevalence, incidence, survival, and disease characteristics of systemic sclerosis in a large US population. Arthritis Rheum. 2003;48:2246–55.

    Article  PubMed  Google Scholar 

  5. Paquette DL, Falanga V. Cutaneous concerns of scleroderma patients. J Dermatol. 2003;30:438–43.

    Article  PubMed  Google Scholar 

  6. Smyth AE, MacGregor AJ, Mukerjee D, Brough GM, Black CM, Denton CP. A cross-sectional comparison of three self-reported functional indices in scleroderma. Rheumatology (Oxford). 2003;42(6):732–8.

    Article  CAS  Google Scholar 

  7. Ouimet JM, Pope JE, Gutmanis I, Koval J. Work disability in scleroderma is greater than in rheumatoid arthritis and is predicted by high HAQ scores. Open Rheumatol J. 2008;2:44–52. doi:10.2174/1874312900802010044.

    Article  PubMed  PubMed Central  Google Scholar 

  8. Bassel M, Hudson M, Taillefer SS, et al. Frequency and impact of symptoms experienced by patients with systemic sclerosis: results from a Canadian National Survey. Rheumatology (Oxford). 2010;50:762–7.

    Article  Google Scholar 

  9. Schnitzer M, Hudson M, Baron M, Steele R, Canadian Scleroderma Research Group. Disability in systemic sclerosis—a longitudinal observational study. J Rheumatol. 2011;38(4):685–92. doi:10.3899/jrheum.100635.

    Article  PubMed  Google Scholar 

  10. Leroy EC, Black C, Fleischmajer R, et al. Scleroderma (systemic sclerosis): classification, subsets and pathogenesis. J Rheumatol. 1988;15:202–5.

    CAS  PubMed  Google Scholar 

  11. Seibold J. Connective tissue diseases characterized by fibrosis. In: Ruddy S, Harris Jr ED, Sledge CB, editors. Textbook of rheumatology. Philadelphia: WB Saunders; 2001. p. 1133–59.

    Google Scholar 

  12. Wigley F, Systemic SB. Clinical features. In: Klippel JH, editor. Primer on the rheumatic diseases. Atlanta: The Arthritis Foundation; 2001. p. 357–64.

    Google Scholar 

  13. Medsger Jr TA. Natural history of systemic sclerosis and the assessment of disease activity, severity, functional status, and psychologic well-being. Rheum Dis Clin North Am. 2003;29:255–73.

    Article  PubMed  Google Scholar 

  14. Hudson M, Thombs BD, Steele R, Panopalis P, Newton E, Baron M, Canadian Scleroderma Research Group. Quality of life in patients with systemic sclerosis compared to the general population and patients with other chronic conditions. J Rheumatol. 2009;36:768–72. doi:10.3899/jrheum.080281.

    Article  PubMed  Google Scholar 

  15. Sekhon S, Pope J, Canadian Scleroderma Research Group (CSRG), Baron M. The Minimally Important Difference (MID) for patient centered outcomes including Health Assessment Questionnaire (HAQ), Fatigue, Pain, Sleep, Global VAS and SF-36 in Scleroderma (SSc). J Rheumatol. 2010;37(3):591–8. doi:10.3899/jrheum.090375.

    Article  PubMed  Google Scholar 

  16. Varga J, Denton CP, Wigley FM, editors. Scleroderma: from pathogenesis to comprehensive management. New York: Springer; 2012. doi:10.1007/978-1-4419-5774-0_7.

    Google Scholar 

  17. Schieir O, Thombs BD, Hudson M, Boivin JF, Steele R, Bernatsky S, Hanley J, Baron M, Canadian Scleroderma Research Group. Prevalence, severity, and clinical correlates of pain in patients with systemic sclerosis. Arthritis Care Res (Hoboken). 2010;62(3):409–17. doi:10.1002/acr.20108.

    Article  Google Scholar 

  18. El-Baalbaki G, Razykov I, Hudson M, Bassel M, Baron M, Thombs BD, Canadian Scleroderma Research Group. Association of pruritus with quality of life and disability in systemic sclerosis. Arthritis Care Res (Hoboken). 2010;62(10):1489–95. doi:10.1002/acr.20257.

    Article  Google Scholar 

  19. Zucker I, Yosipovitch G, David M, Gafter U, Boner G. Prevalence and characterization of uremic pruritus in patients undergoing hemodialysis: uremic pruritus is still a major problem for patients with end-stage renal disease. J Am Acad Dermatol. 2003;49:842–6.

    Article  PubMed  Google Scholar 

  20. Thombs BD, Hudson M, Taillefer SS, Baron M, Canadian Scleroderma Research Group. Prevalence and clinical correlates of symptoms of depression in patients with systemic sclerosis. Arthritis Rheum. 2008;59(4):504–9. doi:10.1002/art.23524.

    Article  PubMed  Google Scholar 

  21. Jewett LR, Hudson M, Malcarne VL, Baron M, Thombs BD, Canadian Scleroderma Research Group. Sociodemographic and disease correlates of body image distress among patients with systemic sclerosis. PLoS One. 2012;7(3), e33281. doi:10.1371/journal.pone.0033281.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  22. Knafo R, Thombs BD, Jewett L, Hudson M, Wigley F, Haythornthwaite JA. (Not) talking about sex: a systematic comparison of sexual impairment in women with systemic sclerosis and other chronic disease samples. Rheumatology (Oxford). 2009;48(10):1300–3. doi:10.1093/rheumatology/kep240.

    Article  Google Scholar 

  23. Hudson M, Steele R, Lu Y, Thombs BD, Canadian Scleroderma Research Group, Baron M. Work disability in systemic sclerosis. J Rheumatol. 2009;36(11):2481–6. doi:10.3899/jrheum.081237.

    Article  PubMed  Google Scholar 

  24. Malcarne VL, Fox RS, Mills SD, Gholizadeh S. Psychosocial aspects of systemic sclerosis. Curr Opin Rheumatol. 2013;25:707–13. doi:10.1097/01.bor.0000434666.47397.c2.

    Article  PubMed  Google Scholar 

  25. Thombs BD, van Lankveld W, Bassel M, et al. Psychological health and well-being in systemic sclerosis: state of the science and consensus research agenda. Arthritis Care Res (Hoboken). 2010;62:1181–9. doi:10.1002/acr.20187.

    Article  Google Scholar 

  26. Suarez-Almazor ME, Kallen MA, Roundtree AK, Mayes M. Disease and symptom burden in systemic sclerosis: a patient perspective. J Rheumatol. 2007;34(8):1718–26.

    PubMed  Google Scholar 

  27. Fries JF, Spitz P, Kraines RG, Holman HR. Measurement of patient outcome in arthritis. Arthritis Rheum. 1980;23:137–45.

    Article  CAS  PubMed  Google Scholar 

  28. Pope J. Measures of systemic sclerosis (scleroderma). Arthritis Care Res. 2011;63(S11):S98–111. doi:10.1002/acr.20598.

    Article  Google Scholar 

  29. Johnson SR, Hawker GA, Davis AM. The Health Assessment Questionnaire disability index and Scleroderma Health Assessment Questionnaire in Scleroderma Trials: an evaluation of their measurement properties. Arthritis Rheum. 2005;53:256–62.

    Article  PubMed  Google Scholar 

  30. Merkel PA, Herlyn K, Martin RW, Anderson JJ, Mayes MD, Bell P, et al. Measuring disease activity and functional status in patients with scleroderma and Raynaud’s phenomenon. Arthritis Rheum. 2002;46:2410–20.

    Article  PubMed  Google Scholar 

  31. Clements PJ, Wong WK, Hurwitz EL, Furst DE, Mayes M, White B, et al. The disability index of the health assessment questionnaire is a predictor and correlate of outcome in the high-dose versus low-dose penicillamine in systemic sclerosis trial. Arthritis Rheum. 2001;44(3):653–61.

    Article  CAS  PubMed  Google Scholar 

  32. Steen VD, Medsger Jr TA. The value of the Health Assessment Questionnaire and special patient-generated scales to demonstrate change in systemic sclerosis patients over time. Arthritis Rheum. 1997;40:1984–91. doi:10.1002/art.1780401110.

    Article  CAS  PubMed  Google Scholar 

  33. Sultan N, Pope JE, Clements PJ, Scleroderma Trials Study Group. The health assessment questionnaire (HAQ) is strongly predictive of good outcome in early diffuse scleroderma: results from an analysis of two randomized controlled trials in early diffuse scleroderma. Rheumatology (Oxford). 2004;43:472–8.

    Article  CAS  Google Scholar 

  34. Khanna D, Clements PJ, Postlethwaite AE, Furst DE. Does incorporation of aids and devices make a difference in the score of the health assessment questionnaire-disability index? Analysis from a scleroderma clinical trial. J Rheumatol. 2008;35:466–8.

    Article  PubMed  Google Scholar 

  35. The EuroQol Group. EuroQol-a new facility for the measurement of health-related quality of life. Health Policy. 1990;16:199–208.

    Article  Google Scholar 

  36. Rabin R, de Charro F. EQ-SD: a measure of health status from the EuroQol Group. Ann Med. 2001;33:337–43.

    Article  CAS  PubMed  Google Scholar 

  37. Gualtierotti R, Scalone L, Ingegnoli F, Cortesi P, Lubatti C, Zeni S, et al. Health related quality of life assessment in patients with systemic sclerosis. Reumatismo. 2010;62(3):210–4.

    CAS  PubMed  Google Scholar 

  38. Muller H, Rehberger P, Gunther C, Schmitt J. Determinants of disability, quality of life and depression in dermatological patients with systemic scleroderma. Br J Dermatol. 2011;166(2):343–53. doi:10.1111/j.1365-2133.2011.10624.x.

    Article  PubMed  Google Scholar 

  39. Strickland G, Pauling J, Cavill C, McHugh N. Predictors of health-related quality of life and fatigue in systemic sclerosis: evaluation of the EuroQol-5D and FACIT-F assessment tools. Clin Rheumatol. 2012;31:1215–22. doi:10.1007/s10067-012-1997-1.

    Article  PubMed  Google Scholar 

  40. Kwakkenbos L, Fransen J, Vonk MC, Becker ES, Jeurissen M, van den Hoogen FH, van den Ende CH. A comparison of the measurement properties and estimation of minimal important differences of the EQ-5D and SF-6D utility measures in patients with systemic sclerosis. Clin Exp Rheumatol. 2013;31(2 Suppl 76):50–6.

    PubMed  Google Scholar 

  41. Hudson M, Impens A, Baron M, Seibold JR, Thombs BD, Walker JG, et al. Discordance between patient and physician assessments of disease severity in systemic sclerosis. J Rheumatol. 2010;37:2307–12. doi:10.3899/jrheum.100354.

    Article  PubMed  Google Scholar 

  42. Clements PJ, Hurwitz EL, Wong WK, Seibold JR, Mayes M, White B, et al. Skin thickness score as a predictor and correlate of outcome in systemic sclerosis: high-dose versus low-dose penicillamine trial. Arthritis Rheum. 2000;43:2445–54.

    Article  CAS  PubMed  Google Scholar 

  43. Khanna D, Furst DE, Clements PJ, Park GS, Hays RD, Yoon J, et al. Responsiveness of the SF-36 and the Health Assessment Questionnaire disability index in a systemic sclerosis clinical trial. J Rheumatol. 2005;32:832–40.

    PubMed  Google Scholar 

  44. Ware Jr JE, Sherbourne CD. The MOS 36-Item Short Health Survey (SF-36) I. Conceptual framework and item selection. Med Care. 1992;30:473–83.

    Article  PubMed  Google Scholar 

  45. Ware Jr JE, Kosinki M, Keller S. SF-36 physical and mental health summary scales: a user’s manual. Boston: New England Medical Center Health Institute; 1994.

    Google Scholar 

  46. Danieli E, Airo P, Bettoni L, Cinquini M, Antonioli CM, Cavazzana I, et al. Health-related quality of life measured by the short form in systemic sclerosis: correlations with indexes of disease severity, disability, and depressive symptoms. Clin Rheumatol. 2005;24:48–54. doi:10.1007/s10067-004-0970-z.

    Article  PubMed  Google Scholar 

  47. Cosutta R, Zeni S, Soldi A, Colombelli P, Belotti Masserini A, Fantini F. Evaluation of quality of life in patients with systemic sclerosis by the SR-36 questionnaire (In Italian). Rheum. 2002;54:122–7.

    Google Scholar 

  48. McHorney CA, Ware JE, Raczek AE. The MOS 36-item short-form health survey (SF-36): II. Psychometric and clinical validity in measuring physical and mental health constructs. Med Care. 1993;31(3):247–63.

    Article  CAS  PubMed  Google Scholar 

  49. Khanna D. Measuring disease activity and outcomes in clinical trials. In: Varga J, Denton CP, Wigley FM, editors. Scleroderma: from pathogenesis to comprehensive management. New York: Springer; 2012. p. 661–72.

    Chapter  Google Scholar 

  50. Brazier L, Roberts J, Deverill M. The estimation of a preference-based measure of health from the SF-36. J Health Econ. 2002;21(2):271–92.

    Article  PubMed  Google Scholar 

  51. Khanna D, Furst DE, Wong WK, Tsevat J, Clements PJ, Park GS, Postlethwaite AE, Ahmed M, Ginsburg S, Hays RD, Scleroderma Collagen Type 1 Study Group. Reliability, validity and minimally important differences of the SF-6D in systemic sclerosis. Qual Life Res. 2007;16:1083–92. doi:10.1007/s11136-007-9207-3.

    Article  PubMed  Google Scholar 

  52. Kallen MA, Mayes MD, Kriseman YL, de Achaval SB, Cox VL, Suarez-Almazor ME. The symptom burden index: development and initial findings from use with patients with systemic sclerosis. J Rheumatol. 2010;37:1692–8. doi:10.3899/jrheum.090504.

    Article  PubMed  Google Scholar 

  53. Silman A, Akesson A, Newman J, Henriksson H, Sandquist G, Nihill M, Palfrey S, et al. Assessment of functional ability in patients with scleroderma: a proposed disability assessment instrument. J Rheumatol. 1998;25(1):79–83.

    CAS  PubMed  Google Scholar 

  54. Poole JL, Brower L. Validity of the scleroderma functional assessment questionnaire. J Rheumatol. 2004;31:402–3.

    PubMed  Google Scholar 

  55. Serednicka K, Smyth AE, Black CM, Denton CP. Using a self-reported functional score to assess disease progression in systemic sclerosis. Rheumatology (Oxford). 2007;46(7):1107–10.

    Article  CAS  Google Scholar 

  56. Sjogren RW. Gastrointestinal motility disorders in scleroderma [review]. Arthritis Rheum. 1994;37:1265–82.

    Article  CAS  PubMed  Google Scholar 

  57. Lock G, Holstege A, Lang B, Scholmerich J. Gastrointestinal manifestations of progressive systemic sclerosis. Am J Gastroenterol. 1997;92:763–71.

    CAS  PubMed  Google Scholar 

  58. Nietert PJ, Mitchell HC, Bolster MB, Curran MY, Tilley BC, Silver RM. Correlates of depression, including overall and gastrointestinal functional status, among patients with systemic sclerosis. J Rheumatol. 2005;32:51–7.

    PubMed  Google Scholar 

  59. Gliddon AE, Dore CJ, Maddison PJ, QUINS Trial Study Group. Influence of clinical features on the health status of patients with limited cutaneous systemic sclerosis. Arthritis Rheum. 2006;55:473–9.

    Article  PubMed  Google Scholar 

  60. Khanna D, Hays RD, Park GS, Braun-Moscovici Y, Mayes MD, McNearney TA, et al. Development of a preliminary scleroderma gastrointestinal tract 1.0 quality of life instrument. Arthritis Rheum. 2007;57:1280–6.

    Article  PubMed  Google Scholar 

  61. Khanna D, Hays RD, Maranian P, Seibold JR, Impens A, Mayes MD, et al. Reliability and validity of the University of California, Los Angeles Scleroderma Clinical Trial Consortium Gastrointestinal Tract Instrument. Arthritis Rheum. 2009;61:1257–63. doi:10.1002/art.24730.

    Article  PubMed  PubMed Central  Google Scholar 

  62. Baron M, Hudson M, Steele R, Lo E. Canadian Scleroderma Research G. Validation of the UCLA Scleroderma Clinical Trial Gastrointestinal Tract Instrument version 2.0 for systemic sclerosis. J Rheumatol. 2011;38(9):1925–30. doi:10.3899/jrheum.110060.

    Article  PubMed  Google Scholar 

  63. Frech TM, Khanna D, Maranian P, Frech EJ, Sawitzke AD, Murtaugh MA. Probiotics for the treatment of systemic sclerosis-associated gastrointestinal bloating/distention. Clin Exp Rheumatol. 2011;29(2 Suppl 65):S22–5.

    PubMed  Google Scholar 

  64. Eypasch E, Williams JI, Wood-Dauphinee S, Ure BM, Schmulling C, Neugebauer E, et al. Gastrointestinal quality of life index: development, validation and application of a new instrument. Br J Surg. 1995;82:216–22.

    Article  CAS  PubMed  Google Scholar 

  65. Poole JL. Measures of hand function. Arthritis Care Res (Hoboken). 2011;63:S189–99.

    Article  Google Scholar 

  66. Duruoz MT, Poiraudeau S, Fermanian J, Menkes C, Amor B, Dougados M, et al. Development and validation of a rheumatoid hand functional disability scale that assesses functional handicap. J Rheumatol. 1996;23:1167–72.

    CAS  PubMed  Google Scholar 

  67. Brower LM, Poole JL. Reliability and validity of the Duruoz Hand Index in persons with systemic sclerosis (scleroderma). Arthritis Rheum. 2004;51:805–9.

    Article  PubMed  Google Scholar 

  68. Rannou F, Poiraudeau S, Berezne A, Baubet T, Le-Guern V, Cabane J, et al. Assessing disability and quality of life in systemic sclerosis: construct validities of the Cochin Hand Function Scale, Health Assessment Questionnaire (HAQ), Systemic Sclerosis HAQ, and Medical Outcomes Study 36-Item Short Form Health Survey. Arthritis Rheum. 2007;57:94–102.

    Article  PubMed  Google Scholar 

  69. Poiraudeau S, Chevalier X, Conrozier T, Flippo RM, Liote F, Lefevre-Colau MM, et al. Reliability, validity, and sensitivity to change of the Cochin Hand Functional Disability Scale in hand osteoarthritis. Osteoarthritis Cartilage. 2001;9:570–7.

    Article  CAS  PubMed  Google Scholar 

  70. Lefevre-Colau MM, Poiraudeau S, Fermanian J, Etchepare F, Alnot JY, Le Viet D, et al. Responsiveness of the Cochin rheumatoid disability scale after surgery. Rheumatology (Oxford). 2001;40:843–50.

    Article  CAS  Google Scholar 

  71. Poiraudeau S, Lefevre-Colau MM, Fermanian J, Revel M. The ability of the Cochin Rheumatoid Arthritis Hand Functional Scale to detect change during the course of disease. Arthritis Care Res. 2000;13:296–303.

    Article  CAS  PubMed  Google Scholar 

  72. Hudak PL, Amadio PC, Bombardier C. Development of an upper extremity outcome measure: the DASH (Disabilities of the Arm, Shoulder and Hand). Am J Ind Med. 1996;29:602–8.

    Article  CAS  PubMed  Google Scholar 

  73. Kennedy CA, Beaton DE, Solway S, McConnell S, Bombardier C. The DASH outcome measure user’s manual. 3rd ed. Toronto: Institute for Work & Health; 2011.

    Google Scholar 

  74. Gabel CP, Yelland M, Melloh M, Burkett B. A modified QuickDASH-9 provides a valid outcome instrument for upper limb function. BMC Musculoskelet Disord. 2009;10:161. doi:10.1186/1471-2474-10-161.

    Article  PubMed  PubMed Central  Google Scholar 

  75. Varju C, Balint Z, Solyom AI, Farkas H, Karpati E, Berta B, et al. Cross-cultural adaptation of the disabilities of the arm, shoulder, and hand (DASH) questionnaire into Hungarian and investigation of its validity in patients with systemic sclerosis. Clin Exp Rheumatol. 2008;26(5):776–83.

    CAS  PubMed  Google Scholar 

  76. Penta M, Thonnard JL, Tesio L. ABILHAND: a Rasch-built measure of manual ability. Arch Phys Med Rehabil. 1998;79:1038–42.

    Article  CAS  PubMed  Google Scholar 

  77. Vanthuyne M, Smith V, Arat S, Westhovens R, De Keyser F, Houssiau FA, et al. Validation of a manual ability questionnaire in patients with systemic sclerosis. Arthritis & Rheum. 2009;61(5):695–703. doi:10.1002/art.24426.

    Article  Google Scholar 

  78. Chung KC, Pillsbury MS, Walters MR, Hayward RA. Reliability and validity testing of the Michigan Hand Outcomes Questionnaire. J Hand Surg Am. 1998;23:575–87.

    Article  CAS  PubMed  Google Scholar 

  79. Mouthon L, Rannou F, Berezne A, Pagnoux C, Arene JP, Fois E, et al. Development and validation of a scale for mouth handicap in systemic sclerosis: the Mouth Handicap in Systemic Sclerosis scale. Ann Rheum Dis. 2007;66:1651–5.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  80. Stevens A, Pope JE. Retest reliabilities and variability among scleroderma patients for 4 tests of disability: support for a better measure. Presented at the Canadian Rheumatology Association Meeting, 1995. J Rheumatol. 1995;22:1603.

    Google Scholar 

  81. Thompson AE, Shea B, Welch V, Fenlon D, Pope JE. Calcium channel blockers for Raynaud’s phenomenon in progressive systemic sclerosis. Arthritis Rheum. 2001;44:1841–7.

    Article  CAS  PubMed  Google Scholar 

  82. Hewlett S, Dures E, Almeida C. Measures of fatigue. Arthritis Care Res (Hoboken). 2011;63:S263–86.

    Article  Google Scholar 

  83. Harel D, Thombs BD, Hudson M, Baron M, Steele R, Canadian Scleroderma Research Group. Measuring fatigue in SSc: A comparison of the Short Form-36 vitality subscale and Functional Assessment of Chronic Illness Therapy-Fatigue scale. Rheumatology. 2012;51:2177–85. doi:10.1093/rheumatology/kes206.

    Article  PubMed  Google Scholar 

  84. Mahler DA, Weinberg DH, Wells CK, Feinstein AR. The measurement of dyspnea: contents, interobserver agreement and physiologic correlates of two new clinical indexes. Chest. 1984;85:751–8.

    Article  CAS  PubMed  Google Scholar 

  85. Tashkin DP, Elashoff R, Clements PJ, Goldin J, Roth MD, Furst DE, et al. Cyclophosphamide versus placebo in scleroderma lung disease. N Engl J Med. 2006;354:2655–66. doi:10.1056/NEJMoa055120.

    Article  CAS  PubMed  Google Scholar 

  86. Borg G. Psychophysical basis of perceived exertion. Med Sci Sports Exerc. 1982;14:377–81.

    CAS  PubMed  Google Scholar 

  87. Khanna D, Clements PJ, Furst DE, Chon Y, Elashoff R, Roth MD, et al. Correlation of the degree of dyspnea with health-related quality of life, functional abilities, and diffusing capacity for carbon monoxide in patients with systemic sclerosis and active alveolitis: results from the Scleroderma Lung Study. Arthritis Rheum. 2005;52:592–600.

    Article  PubMed  Google Scholar 

  88. Khanna D, Yan X, Tashkin DP, Furst DE, Elashoff R, Roth MD, et al. Impact of oral cyclophosphamide on health-related quality of life in patients with active scleroderma lung disease: results from the scleroderma lung study. Arthritis Rheum. 2007;56:1676–84.

    Article  CAS  PubMed  Google Scholar 

  89. Khanna D, Tseng CH, Furst DE, Clements PJ, Elashoff R, Roth M, for Scleroderma Lung Study Investigators, et al. Minimally important differences in the Mahler’s Transition Dyspnoea Index in a large randomized controlled trial: results from the Scleroderma Lung Study. Rheumatology (Oxford). 2009;48:1537–40.

    Article  Google Scholar 

  90. Goldin J, Elashoff R, Kim HJ, et al. Treatment of scleroderma-interstitial lung disease with cyclophosphamide is associated with less progressive fibrosis on serial thoracic high-resolution CT scan than placebo: findings from the scleroderma lung study. Chest. 2009;136:1333–40.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  91. McKenna SP, Doughty N, Meads DM, Doward LC, Pepke-Zaba J. The Cambridge Pulmonary Hypertension Outcome Review (CAMPHOR): a measure of health-related quality of life and quality of life for patients with pulmonary hypertension. Quality Life Res. 2006;15:103–15.

    Article  CAS  Google Scholar 

  92. Meads DM, McKenna SP, Doughty N, Das C, Gin-Sing W, Langley J, et al. The responsiveness and validity of the CAMPHOR Utility Index. Eur Respir J. 2008;32:1513–9.

    Article  CAS  PubMed  Google Scholar 

  93. Gomberg-Maitland M, Thenappan T, Rizvi K, Chandra S, Meads DM, McKenna SP. United States validation of the Cambridge Pulmonary Hypertension Outcome Review (CAMPHOR). J Heart Lung Transplant. 2008;27:124–30.

    Article  PubMed  Google Scholar 

  94. Shenoy PD, Kumar S, Jha LK, Choudhary SK, Singh U, Misra R, et al. Efficacy of tadalafil in secondary Raynaud’s phenomenon resistant to vasodilator therapy: a double-blind randomized cross-over trial. Rheumatology (Oxford). 2010;49:2420–8. doi:10.1093/rheumatology/keq291.

    Article  CAS  Google Scholar 

  95. Khanna PP, Maranian P, Gregory J, Khanna D. The minimally important difference and patient acceptable symptom state for the Raynaud’s condition score in patients with Raynaud’s phenomenon in a large randomised controlled clinical trial. Ann Rheum Dis. 2010;69:588–91. doi:10.1136/ard.2009.107706.

    Article  PubMed  Google Scholar 

  96. Hinchcliff M, Cella D. Patient-reported outcomes. In: Varga J, Denton CP, Wigley FM, editors. Scleroderma: from pathogenesis to comprehensive management. New York: Springer; 2012. p. 673–8.

    Chapter  Google Scholar 

  97. Hinchcliff M, Beaumont JL, Thavarajah K, Varga J, Chung A, Podlusky S, et al. Validity of two new patient-reported outcome measures in systemic sclerosis: Patient-Reported Outcomes Measurement Information System 29-item Health Profile and Functional Assessment of Chronic Illness Therapy-Dyspnea short form. Arthritis Care Res (Hoboken). 2011;63(11):1620–8. doi:10.1002/acr.20591.

    Article  Google Scholar 

  98. Cella D, Yount S, Rothrock N, Gershon R, Cook K, Reeve B, Ader D, et al. The Patient-Reported Outcomes Measurement Information System (PROMIS): Progress of an NIH Roadmap Cooperative Group during its first two years. Med Care. 2007;45(5):S3–11.

    Article  PubMed  PubMed Central  Google Scholar 

  99. Webster K, Cella D, Yost K. The Functional Assessment of Chronic Illness Therapy (FACIT) Measurement System: properties, applications, and interpretation. Health Qual Life Outcomes. 2003;1:79. doi:10.1186/1477-7525-1-79.

    Article  PubMed  PubMed Central  Google Scholar 

  100. Fries JF, Krishnan E, Bruce B. Items, instruments, crosswalks, and PROMIS. J Rheumatol. 2009;36:1093–5.

    Article  PubMed  Google Scholar 

  101. Khanna D, Krishnan E, Dewitt EM, Khanna PP, Spiegel B, Hays RD. The future of measuring patient-reported outcomes in rheumatology: Patient-Reported Outcomes Measurement Information System (PROMIS). Arthritis Care Res. 2011;63:S486–90. doi:10.1002/acr.20581.

    Article  Google Scholar 

  102. Cella D, Riley W, Stone A, et al. The Patient-Reported Outcomes Measurement Information System (PROMIS) developed and tested its first wave of adult self-reported health outcome item banks: 2005–2008. J Clin Epidemiol. 2010;63(11):1179–94. doi:10.1016/j.jclinepi.2010.04.011.

    Article  PubMed  PubMed Central  Google Scholar 

  103. Khanna D, Maranian P, Rothrock N, Cella D, Gershon R, Khanna P, et al. Feasibility and construct validity of PROMIS and “legacy” instruments in an academic scleroderma clinic. Value Health. 2012;15:128–34.

    Article  PubMed  Google Scholar 

  104. Hinchcliff M, Beaumont JL, Carns MA, Podlusky S, Thavarajah K, Varga J, et al. Longitudinal evaluation of PROMIS-29 and FACIT-dyspnea short forms in systemic sclerosis. J Rheumatol. 2015;42(1):64–72. doi:10.3899/jrheum.140143.

    Article  CAS  PubMed  Google Scholar 

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Authors and Affiliations

  1. Schulich School of Medicine and Dentistry, University of Western Ontario, London, ON, Canada

    Russell E. Pellar B.E.Sc. & Theresa M. Tingey B.Arts.Sc.

  2. Division of Rheumatology, Schulich School of Medicine and Dentistry, University of Western Ontario, London, ON, Canada

    Janet E. Pope M.D., M.P.H., F.R.C.P.C.

  3. Division of Rheumatology, Department of Medicine, St. Joseph’s Health Care London, London, ON, Canada

    Janet E. Pope M.D., M.P.H., F.R.C.P.C.

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  1. Russell E. Pellar B.E.Sc.
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  2. Theresa M. Tingey B.Arts.Sc.
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  3. Janet E. Pope M.D., M.P.H., F.R.C.P.C.
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Editors and Affiliations

  1. Honorary Senior Clinical Lecturer, King’s College London, London, United Kingdom

    Yasser El Miedany

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Pellar, R.E., Tingey, T.M., Pope, J.E. (2016). PROMs for Systemic Sclerosis (Scleroderma). In: El Miedany, Y. (eds) Patient Reported Outcome Measures in Rheumatic Diseases. Springer, Cham. https://doi.org/10.1007/978-3-319-32851-5_11

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