Hand Function in Scleroderma

  • Cosimo Bruni
  • Angela Del Rosso
  • Marco Matucci Cerinic
  • Susanna Maddali Bongi


Systemic sclerosis or scleroderma (SSc) is characterized by immunologic abnormalities, microvascular alterations, and excessive collagen production, leading to fibrosis of skin and internal organs (lungs, heart, gastrointestinal tract).

Hands are prominent targets of SSc and may be involved at different levels. Frequent signs and symptoms of the disease, such as skin and microcirculatory impairment as well as musculoskeletal involvement (of joints, tendons, and muscles), usually begin from the hands. Hand involvement evolves throughout all the course of the disease and leads to important disability in SSc.

This chapter will analyze the involvement of the hands in SSc, focusing on the causes and the kind of functional disturbances, the relation between hand impairment and other clinical parameters, hand function in different stages of the disease, and the tools to assess damage and function of the hand.


Systemic sclerosis Disability Handicap Quality of life Hand Fingers Scleroderma Claw type deformities Raynaud phenomenon Digital ulcers Arthritis Duruoz Hand Index (DHI) UK scleroderma functional score (UKFS) Hand Mobility in Scleroderma (HAMIS) test 


  1. 1.
    Varga J, Trojanowska M, Kuwana M. Pathogenesis of systemic sclerosis: recent insights of molecular and cellular mechanisms and therapeutic opportunities. J Scleroderma Relat Disord. 2017;2(3):137–52.CrossRefGoogle Scholar
  2. 2.
    Khanna D, Furst DE, Clements PJ, et al. Standardization of the modified Rodnan skin score for use in clinical trials of systemic sclerosis. J Scleroderma Relat Disord. 2017;2(1):11–8. Scholar
  3. 3.
    Leclair V, Hudson M, Proudman SM, Stevens WM, Fritzler MJ, Wang M, Nikpour M, Baron M. Subsets in systemic sclerosis: one size does not fit all. J Scleroderma Relat Disord. 2016;1(3):298–306. Scholar
  4. 4.
    Poole JL. Grasp pattern variations seen in the scleroderma hand. Am J Occup Ther. 1994;48(1):46–54. PubMed PMID: 8116783. Epub 1994 Jan 01.CrossRefGoogle Scholar
  5. 5.
    Maddali-Bongi S, Del Rosso A, Passalacqua M, Miccio S, Cerinic MM. Manual lymph drainage improving upper extremity edema and hand function in patients with systemic sclerosis in edematous phase. Arthritis Care Res (Hoboken). 2011;63(8):1134–41. PubMed PMID: 21523925. Epub 2011 Apr 28.CrossRefGoogle Scholar
  6. 6.
    Khanna PP, Furst DE, Clements PJ, Maranian P, Indulkar L, Khanna D, et al. Tendon friction rubs in early diffuse systemic sclerosis: prevalence, characteristics and longitudinal changes in a randomized controlled trial. Rheumatology (Oxford). 2010;49(5):955–9. PubMed PMID: 20144926; PubMed Central PMCID: PMCPMC2909791. Epub 2010 Feb 11.CrossRefGoogle Scholar
  7. 7.
    Entin MA, Wilkinson RD. Scleroderma hand: a reappraisal. Orthop Clin North Am. 1973;4(4):1031–8. PubMed PMID: 4744644. Epub 1973 Oct 01.Google Scholar
  8. 8.
    Palmer DG, Hale GM, Grennan DM, Pollock M. Bowed fingers. A helpful sign in the early diagnosis of systemic sclerosis. J Rheumatol. 1981;8(2):266–72. PubMed PMID: 7230157. Epub 1981 Mar 01.PubMedGoogle Scholar
  9. 9.
    Bandinelli F, Kaloudi O, Candelieri A, Conforti ML, Casale R, Cammarata S, et al. Early detection of median nerve syndrome at the carpal tunnel with high-resolution 18 MHz ultrasonography in systemic sclerosis patients. Clin Exp Rheumatol. 2010;28(5 Suppl 62):S15–8. PubMed PMID: 21050540. Epub 2010 Nov 26.PubMedGoogle Scholar
  10. 10.
    Czirjak L, Foeldvari I, Muller-Ladner U. Skin involvement in systemic sclerosis. Rheumatology (Oxford). 2008;47(Suppl 5):v44–5. PubMed PMID: 18784142. Epub 2008 Sept 17.CrossRefGoogle Scholar
  11. 11.
    Clements PJ, Lachenbruch PA, Seibold JR, Zee B, Steen VD, Brennan P, et al. Skin thickness score in systemic sclerosis: an assessment of interobserver variability in 3 independent studies. J Rheumatol. 1993;20(11):1892–6. PubMed PMID: 8308774. Epub 1993 Nov 01.PubMedGoogle Scholar
  12. 12.
    Peytrignet S, Denton CP, Lunt M, Hesselstrand R, Mouthon L, Silman A, et al. Disability, fatigue, pain and their associates in early diffuse cutaneous systemic sclerosis: the European Scleroderma Observational Study. Rheumatology (Oxford). 2018;57(2):370–81. PubMed PMID: 29207002; PubMed Central PMCID: PMCPMC5850714. Epub 2017 Dec 06.CrossRefGoogle Scholar
  13. 13.
    Herrick AL, Gallas A. Systemic sclerosis-related calcinosis. J Scleroderma Relat Disord. 2016;1(2):194–203.CrossRefGoogle Scholar
  14. 14.
    Avouac J, Guerini H, Wipff J, Assous N, Chevrot A, Kahan A, et al. Radiological hand involvement in systemic sclerosis. Ann Rheum Dis. 2006;65(8):1088–92. PubMed PMID: 16414976; PubMed Central PMCID: PMCPMC1798258. Epub 2006 Jan 18.CrossRefGoogle Scholar
  15. 15.
    Bartoli F, Fiori G, Braschi F, Amanzi L, Bruni C, Blagojevic J, et al. Calcinosis in systemic sclerosis: subsets, distribution and complications. Rheumatology (Oxford). 2016;55(9):1610–4. PubMed PMID: 27241706. Epub 2016 June 01CrossRefGoogle Scholar
  16. 16.
    Morardet L, Avouac J, Sammour M, Baron M, Kahan A, Feydy A, et al. Late nailfold videocapillaroscopy pattern associated with hand calcinosis and acro-osteolysis in systemic sclerosis. Arthritis Care Res (Hoboken). 2016;68(3):366–73. PubMed PMID: 26223810. Epub 2015 Aug 01.CrossRefGoogle Scholar
  17. 17.
    Tuffanelli DL, Winkelmann RK. Systemic scleroderma, a clinical study of 727 cases. Arch Dermatol. 1961;84:359–71. PubMed PMID: 13778561. Epub 1961 Sept 01.CrossRefGoogle Scholar
  18. 18.
    Baron M, Lee P, Keystone EC. The articular manifestations of progressive systemic sclerosis (scleroderma). Ann Rheum Dis. 1982;41(2):147–52. PubMed PMID: 7073343; PubMed Central PMCID: PMCPMC1000899. Epub 1982 Apr 01.CrossRefGoogle Scholar
  19. 19.
    Erre GL, Marongiu A, Fenu P, Faedda R, Masala A, Sanna M, et al. The “sclerodermic hand”: a radiological and clinical study. Joint Bone Spine. 2008;75(4):426–31. PubMed PMID: 18455947. Epub 2008 May 06.CrossRefPubMedGoogle Scholar
  20. 20.
    Varjú C, Péntek M, Lóránd V, Nagy G, Minier T, Czirják L. Musculoskeletal involvement in systemic sclerosis: an unexplored aspect of the disease. J Scleroderma Relat Disord. 2017;2(1):19–32. Scholar
  21. 21.
    Ashida R, Ihn H, Mimura Y, Jinnin M, Asano Y, Kubo M, et al. Clinical features of scleroderma patients with contracture of phalanges. Clin Rheumatol. 2007;26(8):1275–7. PubMed PMID: 17171315. Epub 2006 Dec 16.CrossRefPubMedGoogle Scholar
  22. 22.
    Brower LM, Poole JL. Reliability and validity of the Duruoz hand index in persons with systemic sclerosis (scleroderma). Arthritis Rheum. 2004;51(5):805–9. PubMed PMID: 15478150. Epub 2004 Oct 13.CrossRefPubMedGoogle Scholar
  23. 23.
    Maddali-Bongi S, Del Rosso A, Galluccio F, Sigismondi F, Miniati I, Conforti ML, et al. Efficacy of connective tissue massage and Mc Mennell joint manipulation in the rehabilitative treatment of the hands in systemic sclerosis. Clin Rheumatol. 2009;28(10):1167–73. PubMed PMID: 19554274. Epub 2009 June 26.CrossRefGoogle Scholar
  24. 24.
    Sandqvist G, Hesselstrand R, Eberhardt K. A longitudinal follow-up of hand involvement and activities of daily living in early systemic sclerosis. Scand J Rheumatol. 2009;38(4):304–10. PubMed PMID: 19296402. Epub 2009 Mar 20.CrossRefPubMedGoogle Scholar
  25. 25.
    Sandqvist G, Scheja A, Hesselstrand R. Pain, fatigue and hand function closely correlated to work ability and employment status in systemic sclerosis. Rheumatology (Oxford). 2010;49(9):1739–46. PubMed PMID: 20511345. Epub 2010 June 01.CrossRefGoogle Scholar
  26. 26.
    Misra R, Darton K, Jewkes RF, Black CM, Maini RN. Arthritis in scleroderma. Br J Rheumatol. 1995;34(9):831–7. PubMed PMID: 7582722. Epub 1995 Sept 01.CrossRefGoogle Scholar
  27. 27.
    Catoggio LJ, Evison G, Harkness JA, Maddison PJ. The arthropathy of systemic sclerosis (scleroderma); comparison with mixed connective tissue disease. Clin Exp Rheumatol. 1983;1(2):101–12. PubMed PMID: 6335854. Epub 1983 Apr 01.PubMedGoogle Scholar
  28. 28.
    Bruni C, Guiducci S, Bellando-Randone S, Matucci-Cerinic M. Avascular bone necrosis: an underestimated complication of systemic sclerosis. Semin Arthritis Rheum. 2017;47(1):e3–5. PubMed PMID: 28438381. Epub 2017 Apr 26.CrossRefPubMedGoogle Scholar
  29. 29.
    Steen VD, Medsger TA, Jr. The palpable tendon friction rub: an important physical examination finding in patients with systemic sclerosis. Arthritis Rheum. 1997;40(6):1146–51.<1146::AID-ART19>3.0.CO;2-9. PubMed PMID: 9182926. Epub 1997 June 01.CrossRefGoogle Scholar
  30. 30.
    Rodnan GP, Medsger TA. The rheumatic manifestaions of progressive systemic sclerosis (scleroderma). Clin Orthop Relat Res. 1968;57:81–93. PubMed PMID: 4876122. Epub 1968 Mar 01.PubMedGoogle Scholar
  31. 31.
    Clements PJ, Furst DE, Campion DS, Bohan A, Harris R, Levy J, et al. Muscle disease in progressive systemic sclerosis: diagnostic and therapeutic considerations. Arthritis Rheum. 1978;21(1):62–71. PubMed PMID: 623695. Epub 1978 Jan 01.CrossRefGoogle Scholar
  32. 32.
    Bruni C, Cuomo G, Rossi FW, Praino E, Bellando-Randone S. Kidney involvement in systemic sclerosis: from pathogenesis to treatment. J Scleroderma Relat Disord. 2018;3(1):43–52.CrossRefGoogle Scholar
  33. 33.
    Sunderkotter C, Riemekasten G. Pathophysiology and clinical consequences of Raynaud’s phenomenon related to systemic sclerosis. Rheumatology (Oxford). 2006;45(Suppl 3):iii33–5. PubMed PMID: 16987831. Epub 2006 Sept 22.PubMedGoogle Scholar
  34. 34.
    Suliman YA, Bruni C, Johnson SR, Praino E, Aleman H, Borazan N, Cometi L, Myers B, Khanna D, Allanore Y, Baron M, Krieg T, Herrick A, Afonso A, Distler O, Kafaja S, Denton CP, Matucci-Cerinic M, Furst DE. Defining skin ulcers in systemic sclerosis: systematic literature review and proposed World Scleroderma Foundation (WSF) definition. J Scleroderma Relat Disord. 2017;2(2):115–20.CrossRefGoogle Scholar
  35. 35.
    Guillevin L, Hunsche E, Denton CP, Krieg T, Schwierin B, Rosenberg D, et al. Functional impairment of systemic scleroderma patients with digital ulcerations: results from the DUO registry. Clin Exp Rheumatol. 2013;31(2 Suppl 76):71–80. PubMed PMID: 23910613. Epub 2013 Aug 16.PubMedGoogle Scholar
  36. 36.
    Bruni C, Guiducci S, Bellando-Randone S, Lepri G, Braschi F, Fiori G, et al. Digital ulcers as a sentinel sign for early internal organ involvement in very early systemic sclerosis. Rheumatology (Oxford). 2015;54(1):72–6. PubMed PMID: 25065009. Epub 2014 July 30.CrossRefGoogle Scholar
  37. 37.
    Denton CP, Krieg T, Guillevin L, Schwierin B, Rosenberg D, Silkey M, et al. Demographic, clinical and antibody characteristics of patients with digital ulcers in systemic sclerosis: data from the DUO Registry. Ann Rheum Dis. 2012;71(5):718–21. PubMed PMID: 22247218; PubMed Central PMCID: PMCPMC3329234. Epub 2012 Jan 17.CrossRefGoogle Scholar
  38. 38.
    Amanzi L, Braschi F, Fiori G, Galluccio F, Miniati I, Guiducci S, et al. Digital ulcers in scleroderma: staging, characteristics and sub-setting through observation of 1614 digital lesions. Rheumatology (Oxford). 2010;49(7):1374–82. PubMed PMID: 20400463. Epub 2010 Apr 20.CrossRefGoogle Scholar
  39. 39.
    Matucci-Cerinic M, Krieg T, Guillevin L, Schwierin B, Rosenberg D, Cornelisse P, et al. Elucidating the burden of recurrent and chronic digital ulcers in systemic sclerosis: long-term results from the DUO Registry. Ann Rheum Dis. 2016;75(10):1770–6. PubMed PMID: 26612339; PubMed Central PMCID: PMCPMC5036212. Epub 2015 Nov 28.CrossRefGoogle Scholar
  40. 40.
    Mouthon L, Mestre-Stanislas C, Berezne A, Rannou F, Guilpain P, Revel M, et al. Impact of digital ulcers on disability and health-related quality of life in systemic sclerosis. Ann Rheum Dis. 2010;69(1):214–7. PubMed PMID: 19221115. Epub 2009 Feb 18.CrossRefPubMedGoogle Scholar
  41. 41.
    Jaeger VK, Distler O, Maurer B, Czirjak L, Lorand V, Valentini G, et al. Functional disability and its predictors in systemic sclerosis: a study from the DeSScipher project within the EUSTAR group. Rheumatology (Oxford). 2018;57(3):441–50. PubMed PMID: 28499034. Epub 2017 May 13.CrossRefGoogle Scholar
  42. 42.
    Lori S, Matucci-Cerinic M, Casale R, Generini S, Lombardi A, Pignone A, et al. Peripheral nervous system involvement in systemic sclerosis: the median nerve as target structure. Clin Exp Rheumatol. 1996;14(6):601–5. PubMed PMID: 8978953. Epub 1996 Nov 01.PubMedGoogle Scholar
  43. 43.
    Casale R, Buonocore M, Matucci-Cerinic M. Systemic sclerosis (scleroderma): an integrated challenge in rehabilitation. Arch Phys Med Rehabil. 1997;78(7):767–73. PubMed PMID: 9228882. Epub 1997 July 01.CrossRefGoogle Scholar
  44. 44.
    Cerinic MM, Generini S, Pignone A, Casale R. The nervous system in systemic sclerosis (scleroderma). Clinical features and pathogenetic mechanisms. Rheum Dis Clin N Am. 1996;22(4):879–92. PubMed PMID: 8923601. Epub 1996 Nov 01.CrossRefGoogle Scholar
  45. 45.
    Bassel M, Hudson M, Taillefer SS, Schieir O, Baron M, Thombs BD. Frequency and impact of symptoms experienced by patients with systemic sclerosis: results from a Canadian National Survey. Rheumatology (Oxford). 2011;50(4):762–7. PubMed PMID: 21149249. Epub 2010 Dec 15.CrossRefGoogle Scholar
  46. 46.
    van Lankveld WG, Vonk MC, Teunissen H, van den Hoogen FH. Appearance self-esteem in systemic sclerosis—subjective experience of skin deformity and its relationship with physician-assessed skin involvement, disease status and psychological variables. Rheumatology (Oxford). 2007;46(5):872–6. PubMed PMID: 17308314. Epub 2007 Feb 20.CrossRefGoogle Scholar
  47. 47.
    Malcarne VL, Greenbergs HL. Psychological adjustment to systemic sclerosis. Arthritis Care Res. 1996;9(1):51–9. PubMed PMID: 8945113. Epub 1996 Feb 01.CrossRefGoogle Scholar
  48. 48.
    Sandqvist G, Eklund M, Akesson A, Nordenskiold U. Daily activities and hand function in women with scleroderma. Scand J Rheumatol. 2004;33(2):102–7. PubMed PMID: 15163111. Epub 2004 May 28.CrossRefGoogle Scholar
  49. 49.
    Sandqvist G, Scheja A, Eklund M. Working ability in relation to disease severity, everyday occupations and well-being in women with limited systemic sclerosis. Rheumatology (Oxford). 2008;47(11):1708–11. PubMed PMID: 18815157. Epub 2008 Sept 26.CrossRefGoogle Scholar
  50. 50.
    Berezne A, Seror R, Morell-Dubois S, de Menthon M, Fois E, Dzeing-Ella A, et al. Impact of systemic sclerosis on occupational and professional activity with attention to patients with digital ulcers. Arthritis Care Res (Hoboken). 2011;63(2):277–85. PubMed PMID: 20824802. Epub 2010 Sept 09.CrossRefGoogle Scholar
  51. 51.
    Boutry N, Hachulla E, Zanetti-Musielak C, Morel M, Demondion X, Cotten A. Imaging features of musculoskeletal involvement in systemic sclerosis. Eur Radiol. 2007;17(5):1172–80. PubMed PMID: 17021702. Epub 2006 Oct 06.CrossRefPubMedGoogle Scholar
  52. 52.
    Avouac J, Mogavero G, Guerini H, Drape JL, Mathieu A, Kahan A, et al. Predictive factors of hand radiographic lesions in systemic sclerosis: a prospective study. Ann Rheum Dis. 2011;70(4):630–3. PubMed PMID: 21131648. Epub 2010 Dec 07.CrossRefPubMedGoogle Scholar
  53. 53.
    Steen VD, Medsger TA Jr. 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(11):1984–91.<1984::AID-ART10>3.0.CO;2-R. PubMed PMID: 9365087. Epub 1997 Nov 19.CrossRefPubMedGoogle Scholar
  54. 54.
    Follansbee WP, Zerbe TR, Medsger TA Jr. Cardiac and skeletal muscle disease in systemic sclerosis (scleroderma): a high risk association. Am Heart J. 1993;125(1):194–203. PubMed PMID: 8417518. Epub 1993 Jan 01.CrossRefGoogle Scholar
  55. 55.
    West SG, Killian PJ, Lawless OJ. Association of myositis and myocarditis in progressive systemic sclerosis. Arthritis Rheum. 1981;24(5):662–8. PubMed PMID: 7236323. Epub 1981 May 01.CrossRefGoogle Scholar
  56. 56.
    Sunderkotter C, Herrgott I, Bruckner C, Moinzadeh P, Pfeiffer C, Gerss J, et al. Comparison of patients with and without digital ulcers in systemic sclerosis: detection of possible risk factors. Br J Dermatol. 2009;160(4):835–43. PubMed PMID: 19183180. Epub 2009 Feb 03.CrossRefPubMedGoogle Scholar
  57. 57.
    Tiev KP, Diot E, Clerson P, Dupuis-Simeon F, Hachulla E, Hatron PY, et al. Clinical features of scleroderma patients with or without prior or current ischemic digital ulcers: post-hoc analysis of a nationwide multicenter cohort (ItinerAIR-Sclerodermie). J Rheumatol. 2009;36(7):1470–6. PubMed PMID: 19487271. Epub 2009 June 03.CrossRefPubMedGoogle Scholar
  58. 58.
    Steen V, Denton CP, Pope JE, Matucci-Cerinic M. Digital ulcers: overt vascular disease in systemic sclerosis. Rheumatology (Oxford). 2009;48 Suppl 3:iii19–24. PubMed PMID: 19487218. Epub 2009 June 12.CrossRefGoogle Scholar
  59. 59.
    Poole JL, Steen VD. The use of the Health Assessment Questionnaire (HAQ) to determine physical disability in systemic sclerosis. Arthritis Care Res. 1991;4(1):27–31. PubMed PMID: 11188583. Epub 1991 Mar 01.CrossRefGoogle Scholar
  60. 60.
    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(2):256–62. PubMed PMID: 15818719. Epub 2005 Apr 09.CrossRefPubMedGoogle Scholar
  61. 61.
    Ruof J, Bruhlmann P, Michel BA, Stucki G. Development and validation of a self-administered systemic sclerosis questionnaire (SySQ). Rheumatology (Oxford). 1999;38(6):535–42. PubMed PMID: 10402074. Epub 1999 July 13.CrossRefGoogle Scholar
  62. 62.
    Ostojic P, Damjanov N. The Scleroderma Assessment Questionnaire (SAQ). A new self-assessment questionnaire for evaluation of disease status in patients with systemic sclerosis. Z Rheumatol. 2006;65(2):168–75. PubMed PMID: 16501926. Epub 2006 Feb 28.CrossRefPubMedGoogle Scholar
  63. 63.
    Ostojic P, Damjanov N. Indices of the Scleroderma Assessment Questionnaire (SAQ) can be used to demonstrate change in patients with systemic sclerosis over time. Joint Bone Spine. 2008;75(3):286–90. PubMed PMID: 18378177. Epub 2008 Apr 02.CrossRefPubMedGoogle Scholar
  64. 64.
    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(8):1692–8. PubMed PMID: 20516027; PubMed Central PMCID: PMCPMC3887547. Epub 2010 June 03.CrossRefGoogle Scholar
  65. 65.
    Duruoz MT, Poiraudeau S, Fermanian J, Menkes CJ, Amor B, Dougados M, et al. Development and validation of a rheumatoid hand functional disability scale that assesses functional handicap. J Rheumatol. 1996;23(7):1167–72. PubMed PMID: 8823687. Epub 1996 July 01.Google Scholar
  66. 66.
    Poiraudeau S, Chevalier X, Conrozier T, Flippo RM, Liote F, Noel E, et al. Reliability, validity, and sensitivity to change of the Cochin hand functional disability scale in hand osteoarthritis. Osteoarthr Cartil. 2001;9(6):570–7. PubMed PMID: 11520171. Epub 2001 Aug 25.CrossRefPubMedGoogle Scholar
  67. 67.
    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(1):94–102. PubMed PMID: 17266096. Epub 2007 Feb 03.CrossRefPubMedGoogle Scholar
  68. 68.
    Ingegnoli F, Galbiati V, Boracchi P, Comi D, Gualtierotti R, Zeni S, et al. Reliability and validity of the Italian version of the hand functional disability scale in patients with systemic sclerosis. Clin Rheumatol. 2008;27(6):743–9. PubMed PMID: 18040599. Epub 2007 Nov 28.CrossRefPubMedGoogle Scholar
  69. 69.
    Poole JL, Gallegos M, O’Linc S. Reliability and validity of the arthritis hand function test in adults with systemic sclerosis (scleroderma). Arthritis Care Res. 2000;13(2):69–73. PubMed PMID: 14635280. Epub 2003 Nov 26.CrossRefGoogle Scholar
  70. 70.
    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. PubMed PMID: 19032808. Epub 2008 Nov 27.PubMedGoogle Scholar
  71. 71.
    Penta M, Thonnard JL, Tesio L. ABILHAND: a Rasch-built measure of manual ability. Arch Phys Med Rehabil. 1998;79(9):1038–42. PubMed PMID: 9749680. Epub 1998 Sept 28.CrossRefGoogle Scholar
  72. 72.
    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. PubMed PMID: 19405012. Epub 2009 May 01.CrossRefPubMedGoogle Scholar
  73. 73.
    Silman A, Akesson A, Newman J, Henriksson H, Sandquist G, Nihill M, et al. Assessment of functional ability in patients with scleroderma: a proposed new disability assessment instrument. J Rheumatol. 1998;25(1):79–83. PubMed PMID: 9458207. Epub 1998 Feb 11.PubMedGoogle Scholar
  74. 74.
    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. PubMed PMID: 12730528. Epub 2003 May 06.CrossRefGoogle Scholar
  75. 75.
    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. PubMed PMID: 17426141. Epub 2007 Apr 12.CrossRefGoogle Scholar
  76. 76.
    Sandqvist G, Eklund M. Hand Mobility in Scleroderma (HAMIS) test: the reliability of a novel hand function test. Arthritis Care Res. 2000;13(6):369–74. PubMed PMID: 14635312. Epub 2003 Nov 26.CrossRefGoogle Scholar
  77. 77.
    Sandqvist G, Eklund M. Validity of HAMIS: a test of hand mobility in scleroderma. Arthritis Care Res. 2000;13(6):382–7. PubMed PMID: 14635314. Epub 2003 Nov 26.CrossRefGoogle Scholar
  78. 78.
    Del Rosso A, Maddali-Bongi S, Sigismondi F, Miniati I, Bandinelli F, Matucci-Cerinic M. The Italian version of the Hand Mobility in Scleroderma (HAMIS) test: evidence for its validity and reliability. Clin Exp Rheumatol. 2010;28(5 Suppl 62):S42–7. PubMed PMID: 21050544. Epub 2010 Nov 26.PubMedGoogle Scholar
  79. 79.
    Ingegnoli F, Boracchi P, Ambrogi F, Gualtierotti R, Galbiati V, Meroni PL. Hand impairment in systemic sclerosis: association of different hand indices with organ involvement. Scand J Rheumatol. 2010;39(5):393–7. PubMed PMID: 20476855. Epub 2010 May 19.CrossRefPubMedGoogle Scholar
  80. 80.
    Maddali Bongi S, Del Rosso A, Galluccio F, Tai G, Sigismondi F, Passalacqua M, et al. Efficacy of a tailored rehabilitation program for systemic sclerosis. Clin Exp Rheumatol. 2009;27(3 Suppl 54):44–50. PubMed PMID: 19796561. Epub 2009 Dec 04.PubMedGoogle Scholar
  81. 81.
    Furst DE, Clements PJ, Harris R, Ross M, Levy J, Paulus HE. Measurement of clinical change in progressive systemic sclerosis: a 1 year double-blind placebo-controlled trial of N-acetylcysteine. Ann Rheum Dis. 1979;38(4):356–61. PubMed PMID: 386962; PubMed Central PMCID: PMCPMC1000371. Epub 1979 Aug 01.CrossRefGoogle Scholar
  82. 82.
    Merkel PA, Clements PJ, Reveille JD, Suarez-Almazor ME, Valentini G, Furst DE, et al. Current status of outcome measure development for clinical trials in systemic sclerosis. Report from OMERACT 6. J Rheumatol. 2003;30(7):1630–47. PubMed PMID: 12858472. Epub 2003 July 15.PubMedGoogle Scholar
  83. 83.
    Torok KS, Baker NA, Lucas M, Domsic RT, Boudreau R, Medsger TA, Jr. Reliability and validity of the delta finger-to-palm (FTP), a new measure of finger range of motion in systemic sclerosis. Clin Exp Rheumatol. 2010;28(2 Suppl 58):S28–36. PubMed PMID: 20576211; PubMed Central PMCID: PMCPMC2935276. Epub 2010 Sept 02.Google Scholar
  84. 84.
    Roberts-Thomson AJ, Massy-Westropp N, Smith MD, Ahern MJ, Highton J, Roberts-Thomson PJ. The use of the hand anatomic index to assess deformity and impaired function in systemic sclerosis. Rheumatol Int. 2006;26(5):439–44. PubMed PMID: 16237530. Epub 2005 Oct 21.CrossRefPubMedGoogle Scholar
  85. 85.
    Phumethum V, Jamal S, Johnson SR. Biologic therapy for systemic sclerosis: a systematic review. J Rheumatol. 2011;38(2):289–96. PubMed PMID: 21041277. Epub 2010 Nov 03.CrossRefPubMedGoogle Scholar
  86. 86.
    Mugii N, Hamaguchi Y, Maddali-Bongi S. Clinical significance and usefulness of rehabilitation for systemic sclerosis. J Scleroderma Relat Disord. 2018;3(1):71–80. Scholar

Copyright information

© Springer Nature Switzerland AG 2019

Authors and Affiliations

  • Cosimo Bruni
    • 1
    • 2
  • Angela Del Rosso
    • 1
    • 2
  • Marco Matucci Cerinic
    • 1
    • 2
  • Susanna Maddali Bongi
    • 1
    • 2
  1. 1.Department of Experimental and Clinical MedicineUniversity of FlorenceFlorenceItaly
  2. 2.Division of RheumatologyAzienda Ospedaliera Universitaria CareggiFlorenceItaly

Personalised recommendations