Systemic sclerosis (scleroderma, SSc) is an autoimmune disease that causes significant dysfunction to multiple organ systems, including the musculoskeletal system. It poses significant challenges to the hand surgeon, including calcinosis, ischemic changes, Raynaud phenomenon, tendinopathies, synovitis, and joint contractures. Patients with SSc also suffer from multiorgan dysfunction, which makes them high-risk surgical patients. The hand surgeon must understand the pathophysiology, treatment strategies, and special operative considerations required in this population to avoid complications and help maintain or improve hand function.
HoogenFvan den KhannaDFransenJ, et al. 2013 classification criteria for systemic sclerosis: an American College of Rheumatology/European League against rheumatism collaborative initiative. Arthritis Rheum. 2013;65(11):2737-2747. doi:10.1002/art.38098.
2.
AssassiSMayesMDArnettFC, et al. Systemic sclerosis and lupus. Arthritis Rheum. 2010;62(2):589-598. doi:10.1002/art.27224.
3.
GabrielliAAvvedimentoEVKriegT.Scleroderma. N Engl J Med. 2009;360(19):1989-2003. doi:10.1056/NEJMra0806188.
4.
ChanderSGordonP.Soft tissue and subcutaneous calcification in connective tissue diseases. Curr Opin Rheumatol. 2012;24(2):158-164. doi:10.1097/BOR.0b013e32834ff5cd.
5.
BalinSJWetterDAAndersenLK, et al. Calcinosis cutis occurring in association with autoimmune connective tissue disease: the Mayo Clinic experience with 78 patients, 1996-2009. Arch Dermatol. 2012;148(4):455-462. doi:10.1001/archdermatol.2011.2052.
6.
MeloneCPJrMcLoughlinJCBeldnerS.Surgical management of the hand in scleroderma. Curr Opin Rheumatol. 1999;11(6):514-520.
7.
WilliamsAACarlHMLifchezSD.The scleroderma hand: manifestations of disease and approach to management. J Hand Surg Am. 2018;43(6):550-557. doi:10.1016/j.jhsa.2018.03.021.
8.
LapnerMAGoetzTJ.High-speed burr debulking of digital calcinosis cutis in scleroderma patients. J Hand Surg Am. 2014;39(3):503-510. doi:10.1016/j.jhsa.2013.12.003.
9.
ChamberlainAJWalkerNPJ. Successful palliation and significant remission of cutaneous calcinosis in CREST syndrome with carbon dioxide laser. Dermatol Surg. 2003;29(9):968-970. doi:10.1046/j.1524-4725.2003.29261.x.
10.
CappelliLWigleyFM.Management of Raynaud phenomenon and digital ulcers in scleroderma. Rheum Dis Clin North Am. 2015;41(3):419-438. doi:10.1016/j.rdc.2015.04.005.
11.
HachullaEClersonPLaunayD, et al. Natural history of ischemic digital ulcers in systemic sclerosis: single-center retrospective longitudinal study. J Rheumatol. 2007;34(12):2423-2430.
12.
HughesMPaulingJD.Exploring the patient experience of digital ulcers in systemic sclerosis. Semin Arthritis Rheum. 2019;48(5):888-894. doi:10.1016/j.semarthrit.2018.08.001.
13.
GiuggioliDManfrediAColaciM, et al. Osteomyelitis complicating scleroderma digital ulcers. Clin Rheumatol. 2013;32(5):623-627. doi:10.1007/s10067-012-2161-7.
14.
WigleyFM.Clinical practice. Raynaud’s phenomenon. N Engl J Med. 2002;347(13):1001-1008. doi:10.1056/NEJMcp013013.
15.
StringerTFemiaA.Raynaud’s phenomenon: current concepts. Clin Dermatol. 2018;36(4):498-507.
16.
ThompsonAESheaBWelchV, et al. Calcium-channel blockers for Raynaud’s phenomenon in systemic sclerosis. Arthritis Rheum. 2001;44(8):1841-1847. doi:10.1002/1529-0131(200108)44:8<1841::AID-ART322>3.0.CO;2-8.
17.
PaceCSMerrittWH.Extended periarterial sympathectomy: evaluation of long-term outcomes. Hand. 2018;13(4):395-402. doi:10.1177/1558944717715119.
18.
BelloRJCooneyCMMelamedE, et al. The therapeutic efficacy of Botulinum toxin in treating scleroderma-associated Raynaud’s phenomenon: a randomized, double-blind, placebo-controlled clinical trial. Arthritis Rheumatol. 2017;69(8):1661-1669. doi:10.1002/art.40123.
19.
WigleyFMFlavahanNA.Raynaud’s phenomenon. N Engl J Med2016;375:556-565. doi:10.1056/NEJMra1507638.
20.
ElhaiMGueriniHBazeliR, et al. Ultrasonographic hand features in systemic sclerosis and correlates with clinical, biologic, and radiographic findings. Arthritis Care Res. 2012;64(8):1244-1249. doi:10.1002/acr.21668.
21.
AvouacJWalkerUTyndallA, 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):1488-1501. doi:10.3899/jrheum.091165.
22.
KhannaPPFurstDEClementsPJ, et al. Tendon friction rubs in early diffuse systemic sclerosis: prevalence, characteristics and longitudinal changes in a randomized controlled trial. Rheumatology. 2010;49(5):955-959. doi:10.1093/rheumatology/kep464.
StoenoiuMSHoussiauFALecouvetFE.Tendon friction rubs in systemic sclerosis: a possible explanation—an ultrasound and magnetic resonance imaging study. Rheumatology. 2013;52(3):529-533. doi:10.1093/rheumatology/kes307.
25.
DoréALucasMIvancoD, et al. The significance of palpable tendon friction rubs in early diffuse cutaneous systemic sclerosis. Arthritis Care Res. 2013;65(8):1385-1389. doi:10.1002/acr.21964.
26.
BaronMLeePKeystoneEC.The articular manifestations of progressive systemic sclerosis (scleroderma). Ann Rheum Dis. 1982;41(2):147-152. doi:10.1136/ard.41.2.147.
27.
AvouacJGueriniHWipffJ, et al. Radiological hand involvement in systemic sclerosis. Ann Rheum Dis. 2006;65(8):1088-1092. doi:10.1136/ard.2005.044602.
28.
LowAHLLaxMJohnsonSR, et al. Magnetic resonance imaging of the hand in systemic sclerosis. J Rheumatol. 2009;36(5):961-964. doi:10.3899/jrheum.080795.
29.
La MontagnaGSodanoACapurroV, et al. The arthropathy of systemic sclerosis: a 12 month prospective clinical and imaging study. Skeletal Radiol. 2005;34(1):35-41. doi:10.1007/s00256-004-0830-6.
30.
JonesNFImbrigliaJESteenVD, et al. Surgery for scleroderma of the hand. J Hand Surg Am. 1987;12(3):391-400. doi:10.1016/s0363-5023(87)80012-6.
31.
LipscombPRSimonsGWWinkelmannRK.Surgery for sclerodactylia of the hand: experience with six cases. J Bone Joint Surg Am. 1969;51(6):1112-1117.
32.
MeloneCPJrDayanE.Articular surgery of the ischemic hand in systemic scleroderma: a vascular basis for arthrodesis and arthroplasty. J Hand Surg Am. 2018;43(6):574.e1-574.e9. doi:10.1016/j.jhsa.2018.03.008.
33.
GilbartMKJollesBMLeeP, et al. Surgery of the hand in severe systemic sclerosis. J Hand Surg Br. 2004;29(6):599-603. doi:10.1016/j.jhsb.2004.03.013.
34.
BogochERGrossDK.Surgery of the hand in patients with systemic sclerosis: outcomes and considerations. J Rheumatol. 2005;32(4):642-648.
35.
PolitikouOGiesenTReissnerL, et al. Hand and wrist joint procedures in patients with scleroderma: a systematic review. J Hand Surg Eur Vol. 2019;44(4):402-407. doi:10.1177/1753193418795632.
36.
TagliaficoAPanicoNResminiE, et al. The role of ultrasound imaging in the evaluation of peripheral nerve in systemic sclerosis (scleroderma). Eur J Radiol. 2011;77(3):377-382. doi:10.1016/j.ejrad.2009.08.010.
37.
NimitbanchaTSirasapornPWattanapanP, et al. The prevalence of median neuropathy at wrist in systemic sclerosis patients at Srinagarind Hospital. J Sci Soc. 2015;42(3):180. doi:10.4103/0974-5009.165566.
38.
AmaralTNPeresFALapaAT, et al. Neurologic involvement in scleroderma: a systematic review. Semin Arthritis Rheum. 2013;43(3):335-347. doi:10.1016/j.semarthrit.2013.05.002.
39.
BignottiBGhioMPanicoN, et al. High-resolution ultrasound of peripheral nerves in systemic sclerosis: a pilot study of computer-aided quantitative assessment of nerve density. Skeletal Radiol. 2015;44(12):1761-1767. doi:10.1007/s00256-015-2230-5.
40.
DempseyZSRowellSMcRobertR.The role of regional and neuroaxial anesthesia in patients with systemic sclerosis. Local Reg Anesth. 2011;4:47-56. doi:10.2147/LRA.S23808.
