Abstract
While tethering of the skin is the clinical hallmark of SSc, many patients may develop musculoskeletal symptoms as an early sign of the disease or during the course of their illness. Manifestations may include varying degrees of rheumatic complaints ranging from symptoms of carpal tunnel syndrome, arthralgias to frank arthritis, or uncomfortable bony lesions. Musculoskeletal involvement strongly contributes to disability and impaired quality of life in SSc, reducing the performance of everyday occupation (Baron M, Lee P, Keystone EC. The articular manifestations of progressive systemic sclerosis (scleroderma). Ann Rheum Dis. 1982;41(2):147–52; Mau W, Listing J, Huscher D, Zeidler H, Zink A. Employment across chronic inflammatory rheumatic diseases and comparison with the general population. J Rheumatol. 2005;32(4):721–8; 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; 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; 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)1–5. Indeed, several cross-sectional studies highlight the major impact of musculoskeletal involvement on the quality of life in SSc. One report investigated the relationship between patient beliefs, symptom report, and clinical/demographic variables.
Similar content being viewed by others
References
Baron M, Lee P, Keystone EC. The articular manifestations of progressive systemic sclerosis (scleroderma). Ann Rheum Dis. 1982;41(2):147–52.
Mau W, Listing J, Huscher D, Zeidler H, Zink A. Employment across chronic inflammatory rheumatic diseases and comparison with the general population. J Rheumatol. 2005;32(4):721–8.
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.
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.
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.
Richards HL, Herrick AL, Griffin K, Gwilliam PD, Loukes J, Fortune DG. Systemic sclerosis: patients’ perceptions of their condition. Arthritis Rheum. 2003;49(5):689–96.
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.
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.
Tagliafico A, Panico N, Resmini E, Derchi LE, Ghio M, Martinoli C. The role of ultrasound imaging in the evaluation of peripheral nerve in systemic sclerosis (scleroderma). Eur J Radiol. 2009;77:377–82, e-pub ahead of print.
Machet L, Vaillant L, Machet MC, Esteve E, Muller C, Khallouf R, et al. Carpal tunnel syndrome and systemic sclerosis. Dermatology. 1992;185(2):101–3.
Rodnan GP, Medsger TA. The rheumatic manifestations of progressive systemic sclerosis (scleroderma). Clin Orthop Relat Res. 1968;57:81–93.
Rosenbaum LH, Swartz WM, Rodnan GP, Medsger Jr TA. Wrist drop in progressive systemic sclerosis (scleroderma): complete rupture of the extensor tendon mechanism. Arthritis Rheum. 1985;28(5):586–9.
La Montagna G, Baruffo A, Tirri R, Buono G, Valentini G. Foot involvement in systemic sclerosis: a longitudinal study of 100 patients. Semin Arthritis Rheum. 2002;31(4):248–55.
Steen VD, Medsger Jr TA. The palpable tendon friction rub: an important physical examination finding in patients with systemic sclerosis. Arthritis Rheum. 1997;40(6):1146–51.
Low AH, Lax M, Johnson SR, Lee P. Magnetic resonance imaging of the hand in systemic sclerosis. J Rheumatol. 2009;36(5):961–4.
Avouac J, Walker U, Tyndall A, Kahan A, Matucci-Cerinic M, Allanore Y, et al. Characteristics of joint involvement and relationships with systemic inflammation in systemic sclerosis: results from the EULAR Scleroderma Trial and Research Group (EUSTAR) database. J Rheumatol. 2010;37(7):1488–501.
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.
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.
Cohen MJ, Persellin RH. Coexistence of rheumatoid arthritis and systemic sclerosis in four patients. Scand J Rheumatol. 1982;11(4):241–5.
Armstrong RD, Gibson T. Scleroderma and erosive polyarthritis: a disease entity? Ann Rheum Dis. 1982;41(2):141–6.
Baron M, Srolovitz H, Lander P, Kapusta M. The coexistence of rheumatoid arthritis and scleroderma: a case report and review of the literature. J Rheumatol. 1982;9(6):947–50.
Avouac J, Airò P, Dieude P, Caramaschi P, Tiev K, Diot E, et al. Associated autoimmune diseases in systemic sclerosis define a subset of patients with milder disease: results from two large cohorts of European Caucasian patients. J Rheumatol. 2010;37(3):608–14.
Avouac J, Gossec L, Dougados M. Diagnostic and predictive value of anti-cyclic citrullinated protein antibodies in rheumatoid arthritis: a systematic literature review. Ann Rheum Dis. 2006;65(7):845–51.
Morita Y, Muro Y, Sugiura K, Tomita Y. Anti-cyclic citrullinated peptide antibody in systemic sclerosis. Clin Exp Rheumatol. 2008;26(4):542–7.
Marrone M, Chiala A, Tampoia M, Iannone F, Raho L, Covelli M, et al. Prevalence of anti-CCP antibodies in systemic sclerosis. Reumatismo. 2007;59(1):20–4.
Horiki T, Moriuchi J, Takaya M, Uchiyama M, Hoshina Y, Inada K, et al. The coexistence of systemic sclerosis and rheumatoid arthritis in five patients. Clinical and immunogenetic features suggest a distinct entity. Arthritis Rheum. 1996;39(1):152–6.
Blocka KL, Bassett LW, Furst DE, Clements PJ, Paulus HE. The arthropathy of advanced progressive systemic sclerosis. A radiographic survey. Arthritis Rheum. 1981;24(7):874–84.
Misra R, Darton K, Jewkes RF, Black CM, Maini RN. Arthritis in scleroderma. Br J Rheumatol. 1995;34(9):831–7.
Avouac J, Sordet C, Depinay C, Ardizonne M, Vacher-Lavenu MC, Sibilia J, et al. Systemic sclerosis-associated Sjogren’s syndrome and relationship to the limited cutaneous subtype: results of a prospective study of sicca syndrome in 133 consecutive patients. Arthritis Rheum. 2006;54(7):2243–9.
Santiago M, Baron M, Miyachi K, Fritzler MJ, Abu-Hakima M, Leclercq S, et al. A comparison of the frequency of antibodies to cyclic citrullinated peptides using a third generation anti-CCP assay (CCP3) in systemic sclerosis, primary biliary cirrhosis and rheumatoid arthritis. Clin Rheumatol. 2008;27(1):77–83.
Ingegnoli F, Galbiati V, Zeni S, Meani L, Zahalkova L, Lubatti C, et al. Use of antibodies recognizing cyclic citrullinated peptide in the differential diagnosis of joint involvement in systemic sclerosis. Clin Rheumatol. 2007;26(4):510–4.
Generini S, Steiner G, Miniati I, Conforti ML, Guiducci S, Skriner K, et al. Anti-hnRNP and other autoantibodies in systemic sclerosis with joint involvement. Rheumatology (Oxford). 2009;48(8):920–5.
Ueda-Hayakawa I, Hasegawa M, Kumada S, Tanaka C, Komura K, Hamaguchi Y, et al. Usefulness of anti-cyclic citrullinated peptide antibody and rheumatoid factor to detect rheumatoid arthritis in patients with systemic sclerosis. Rheumatology (Oxford). 2010;49(11):2135–9.
La Montagna G, D’Angelo S, Valentini G. Cross-sectional evaluation of YKL-40 serum concentrations in patients with systemic sclerosis. Relationship with clinical and serological aspects of disease. J Rheumatol. 2003;30(10):2147–51.
Rozman B, Cucnik S, Sodin-Semrl S, Czirjak L, Varju C, Distler O, et al. Prevalence and clinical associations of anti-Ku antibodies in patients with systemic sclerosis: a European EUSTAR-initiated multi-centre case-control study. Ann Rheum Dis. 2008;67(9):1282–6.
Schumacher Jr HR. Joint involvement in progressive systemic sclerosis (scleroderma): a light and electron microscopic study of synovial membrane and fluid. Am J Clin Pathol. 1973;60(5):593–600.
Hollander JL, McCarty Jr DJ, Astorga G, Castro-Murillo E. Studies on the Pathogenesis of Rheumatoid Joint Inflammation. I. The “R.A. Cell” and a Working Hypothesis. Ann Intern Med. 1965;62:271–80.
Tuffanelli DL, Winkelmann RK. Systemic scleroderma, a clinical study of 727 cases. Arch Dermatol. 1961;84:359–71.
La Montagna G, Sodano A, Capurro V, Malesci D, Valentini G. The arthropathy of systemic sclerosis: a 12 month prospective clinical and imaging study. Skeletal Radiol. 2005;34(1):35–41.
Cuomo G, Zappia M, Abignano G, Iudici M, Rotondo A, Valentini G. Ultrasonographic features of the hand and wrist in systemic sclerosis. Rheumatology (Oxford). 2009;48(11):1414–7.
Chitale S, Ciapetti A, Hodgson R, Grainger A, O’Connor P, Goodson NJ, et al. Magnetic resonance imaging and musculoskeletal ultrasonography detect and characterize covert inflammatory arthropathy in systemic sclerosis patients with arthralgia. Rheumatology (Oxford). 2008;49(12):2357–61.
Wang J, Yarnykh VL, Molitor JA, Nash RA, Chu B, Wilson GJ, et al. Micro magnetic resonance angiography of the finger in systemic sclerosis. Rheumatology (Oxford). 2008;47(8):1239–43.
Sheehan JJ, Fan Z, Davarpanah AH, Hodnett PA, Varga J, Carr JC, et al. Nonenhanced MR Angiography of the hand with flow-sensitive dephasing-prepared balanced SSFP sequence: initial experience with systemic sclerosis. Radiology. 2011;259(1):248–56.
Allanore Y, Seror R, Chevrot A, Kahan A, Drape JL. Hand vascular involvement assessed by magnetic resonance angiography in systemic sclerosis. Arthritis Rheum. 2007;56(8):2747–54.
Resnick D, Greenway G, Vint VC, Robinson CA, Piper S. Selective involvement of the first carpometacarpal joint in scleroderma. AJR Am J Roentgenol. 1978;131(2):283–6.
Koutaissoff S, Vanthuyne M, Smith V, De Langhe E, Depresseux G, Westhovens R, et al. Hand radiological damage in systemic sclerosis: comparison with a control group and clinical and functional correlations. Semin Arthritis Rheum. 2010;40(5):455–60.
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.
Mugino H, Ikemura K. Progressive systemic sclerosis with spontaneous fracture due to resorption of the mandible: a case report. J Oral Maxillofac Surg. 2006;64(7):1137–9.
Braun-Moscovici Y, Furst DE, Markovits D, Rozin A, Clements PJ, Nahir AM, et al. Vitamin D, parathyroid hormone, and acroosteolysis in systemic sclerosis. J Rheumatol. 2008;35(11):2201–5.
Vacca A, Cormier C, Piras M, Mathieu A, Kahan A, Allanore Y. Vitamin D deficiency and insufficiency in 2 independent cohorts of patients with systemic sclerosis. J Rheumatol. 2009;36(9):1924–9.
Scharer L, Smith DW. Resorption of the terminal phalanges in scleroderma. Arthritis Rheum. 1969;12(1):51–63.
Au K, Mayes MD, Maranian P, Clements PJ, Khanna D, Steen VD, et al. Course of dermal ulcers and musculoskeletal involvement in systemic sclerosis patients in the scleroderma lung study. Arthritis Care Res (Hoboken). 2010;62(12):1772–8.
Nacci F, Righi A, Conforti ML, Miniati I, Fiori G, Martinovic D, et al. Intravenous immunoglobulins improve the function and ameliorate joint involvement in systemic sclerosis: a pilot study. Ann Rheum Dis. 2007;66(7):977–9.
Lam GK, Hummers LK, Woods A, Wigley FM. Efficacy and safety of etanercept in the treatment of scleroderma-associated joint disease. J Rheumatol. 2007;34(7):1636–7.
Allanore Y, Devos-Francois G, Caramella C, Boumier P, Jounieaux V, Kahan A. Fatal exacerbation of fibrosing alveolitis associated with systemic sclerosis in a patient treated with adalimumab. Ann Rheum Dis. 2006;65(6):834–5.
Ostor AJ, Crisp AJ, Somerville MF, Scott DG. Fatal exacerbation of rheumatoid arthritis associated fibrosing alveolitis in patients given infliximab. BMJ. 2004;329(7477):1266.
Smith V, Van Praet JT, Vandooren B, Van der Cruyssen B, Naeyaert JM, Decuman S, et al. Rituximab in diffuse cutaneous systemic sclerosis: an open-label clinical and histopathological study. Ann Rheum Dis. 2010;69(1):193–7.
Lafyatis R, Kissin E, York M, Farina G, Viger K, Fritzler MJ, et al. B cell depletion with rituximab in patients with diffuse cutaneous systemic sclerosis. Arthritis Rheum. 2009;60(2):578–83.
Daoussis D, Liossis SN, Tsamandas AC, Kalogeropoulou C, Kazantzi A, Sirinian C, et al. Experience with rituximab in scleroderma: results from a 1-year, proof-of-principle study. Rheumatology (Oxford). 2010;49(2):271–80.
Poole JL. Musculoskeletal rehabilitation in the person with scleroderma. Curr Opin Rheumatol. 2010;22(2):205–12.
Bogoch ER, Gross DK. Surgery of the hand in patients with systemic sclerosis: outcomes and considerations. J Rheumatol. 2005;32(4):642–8.
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;57(4):252–4.
Farah MJ, Palmieri GM, Sebes JI, Cremer MA, Massie JD, Pinals RS. The effect of diltiazem on calcinosis in a patient with the CREST syndrome. Arthritis Rheum. 1990;33(8):1287–93.
Dolan AL, Kassimos D, Gibson T, Kingsley GH. Diltiazem induces remission of calcinosis in scleroderma. Br J Rheumatol. 1995;34(6):576–8.
Palmieri GM, Sebes JI, Aelion JA, Moinuddin M, Ray MW, Wood GC, et al. Treatment of calcinosis with diltiazem. Arthritis Rheum. 1995;38(11):1646–54.
Matsumoto I, Ito Y, Tomo H, Nakao Y, Takaoka K. Case reports: ossified mass of the rotator cuff tendon in the subacromial bursa. Clin Orthop Relat Res. 2005;437:247–50.
Metzger AL, Singer FR, Bluestone R, Pearson CM. Failure of disodium etidronate in calcinosis due to dermatomyositis and scleroderma. N Engl J Med. 1974;291(24):1294–6.
Cukierman T, Elinav E, Korem M, Chajek-Shaul T. Low dose warfarin treatment for calcinosis in patients with systemic sclerosis. Ann Rheum Dis. 2004;63(10):1341–3.
Robertson LP, Marshall RW, Hickling P. Treatment of cutaneous calcinosis in limited systemic sclerosis with minocycline. Ann Rheum Dis. 2003;62(3):267–9.
Loucks J, Pope JE. Osteoporosis in scleroderma. Semin Arthritis Rheum. 2005;34(4):678–82.
Souza RB, Borges CT, Takayama L, Aldrighi JM, Pereira RM. Systemic sclerosis and bone loss: the role of the disease and body composition. Scand J Rheumatol. 2006;35(5):384–7.
Sampaio-Barros PD, Costa-Paiva L, Filardi S, Sachetto Z, Samara AM, Marques-Neto JF. Prognostic factors of low bone mineral density in systemic sclerosis. Clin Exp Rheumatol. 2005;23(2):180–4.
Neumann K, Wallace DJ, Metzger AL. Osteoporosis–less than expected in patients with scleroderma? J Rheumatol. 2000;27(7):1822–3.
Brun B, Serup J, Hagdrup H. Radiological changes of the hands in systemic sclerosis. Acta Derm Venereol. 1983;63(4):349–52.
Bassett LW, Blocka KL, Furst DE, Clements PJ, Gold RH. Skeletal findings in progressive systemic sclerosis (scleroderma). AJR Am J Roentgenol. 1981;136(6):1121–6.
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.
Author information
Authors and Affiliations
Corresponding author
Editor information
Editors and Affiliations
Rights and permissions
Copyright information
© 2012 Springer Science+Business Media, LLC
About this chapter
Cite this chapter
Avouac, J., Allanore, Y. (2012). Tendons, Joints, and Bone. In: Varga, J., Denton, C., Wigley, F. (eds) Scleroderma. Springer, Boston, MA. https://doi.org/10.1007/978-1-4419-5774-0_44
Download citation
DOI: https://doi.org/10.1007/978-1-4419-5774-0_44
Published:
Publisher Name: Springer, Boston, MA
Print ISBN: 978-1-4419-5773-3
Online ISBN: 978-1-4419-5774-0
eBook Packages: MedicineMedicine (R0)