Key Points
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Improves dental practitioners' understanding of systemic sclerosis.
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Improves recognition of key orofacial manifestations of systemic sclerosis.
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Highlights difficulties faced by patients with regards to oral hygiene measures and access to routine treatment.
Abstract
Systemic sclerosis (SSc) is a multisystem disease of unknown aetiology characterised by microangiopathy, dysregulated immune function and tissue remodelling, which commonly involves the oral cavity. Orofacial manifestations of SSc contribute greatly to overall disease burden and yet are regularly overlooked and under-treated. This may reflect a pre-occupation amongst rheumatology clinicians on potentially life-threatening internal organ involvement, but is also a consequence of insufficient engagement between rheumatologists and dental professionals. A high proportion of SSc patients report difficulty accessing a dentist with knowledge of the disease and there is recognition amongst dentists that this could impact negatively on patient care. This review shall describe the clinical features and burden of orofacial manifestations of SSc and the management of such problems. The case is made for greater collaborative working between rheumatologists and dental professionals with an interest in SSc in both the research and clinical setting.
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References
Leader D, Papas A, Finkelman M . A survey of dentists' knowledge and attitudes with respect to the treatment of scleroderma patients. J Clin Rheumatol. 2014; 20: 189–194.
Mayes M D, Lacey JV Jr, Beebe-Dimmer J et al. Prevalence, incidence, survival, and disease characteristics of systemic sclerosis in a large US population. Arthritis Rheum. 2003; 48: 2246–2255.
Nikpour M, Stevens W M, Herrick A L, Proudman S M . Epidemiology of systemic sclerosis. Best Pract Res Clin Rheumatol. 2010; 24: 857–869.
D'Angelo W A, Fries J F, Masi A T, Shulman L E . Pathologic observations in systemic sclerosis (scleroderma). A study of fifty-eight autopsy cases and fifty-eight matched controls. Am J Med. 1969; 46: 428–440.
LeRoy E C, Black C, Fleischmajer R et al. Scleroderma (systemic sclerosis): classification, subsets and pathogenesis. J Rheumatol. 1988; 15: 202–205.
LeRoy EC . Systemic sclerosis. A vascular perspective. Rheum Dis Clin North Am. 1996; 22: 675–694.
van den Hoogen F, Khanna D, Fransen J et al. 2013 classification criteria for systemic sclerosis: an American College of Rheumatology/European League against Rheumatism collaborative initiative. Arthritis Rheum. 2013; 65: 2737–2747.
Thoua N M, Bunce C, Brough G, Forbes A, Emmanuel A V, Denton C P . Assessment of gastrointestinal symptoms in patients with systemic sclerosis in a UK tertiary referral centre. Rheumatology (Oxford). 2010; 49: 1770–1775.
Duchini A, Sessoms S L . Gastrointestinal hemorrhage in patients with systemic sclerosis and CREST syndrome. Am J Gastroenterol. 1998; 93: 1453–1456.
Rosenthal I H . Generalized scleroderma; hidebound disease, its relation to the oral cavity, with case history and dental restoration. Oral Surg Oral Med Oral Pathol. 1948; 1: 1019–1028.
Albilia J B, Lam D K, Blanas N, Clokie C M, Sandor G K . Small mouths. Big problems? A review of scleroderma and its oral health implications. J Can Dent Assoc. 2007; 73: 831–836.
Alantar A, Cabane J, Hachulla E et al. Recommendations for the care of oral involvement in patients with systemic sclerosis. Arthritis Care Res (Hoboken). 2011; 63: 1126–1133.
Baron M, Hudson M, Tatibouet S et al. The Canadian systemic sclerosis oral health study: orofacial manifestations and oral health-related quality of life in systemic sclerosis compared with the general population. Rheumatology (Oxford). 2014; 53: 1386–1394.
Vitali C, Baldanzi C, Polini F, Montesano A, Ammenti P, Cattaneo D . Instrumented Assessment of Oral Motor Function in Healthy Subjects and People with Systemic Sclerosis. Dysphagia 2015; 30: 286–295.
Frech T M, Pauling J D, Murtaugh M A, Kendall K, Domsic R T . Sublingual Abnormalities in Systemic Sclerosis. J Clin Rheumatol. 2016; 22: 19–21.
Paquette D L, Falanga V . Cutaneous concerns of scleroderma patients. J Dermatol 2003; 30: 438–443.
Apfelberg D B, Varga J, Greenbaum S S . Carbon dioxide laser resurfacing of peri-oral rhytids in scleroderma patients. Dermatol Surg. 1998; 24: 517–519.
Yuen H K, Marlow N M, Reed S G et al. Effect of orofacial exercises on oral aperture in adults with systemic sclerosis. Disabil Rehabil. 2012; 34: 84–89.
Pizzo G, Scardina G A, Messina P . Effects of a nonsurgical exercise program on the decreased mouth opening in patients with systemic scleroderma. Clin Oral Investig 2003; 7: 175–178.
Maddali Bongi S, Del Rosso A, Galluccio F et al. Efficacy of a tailored rehabilitation program for systemic sclerosis. Clin Exp Rheumatol. 2009; 27(Suppl 54): 44–50.
Naylor W P, Douglass C W, Mix E . The nonsurgical treatment of microstomia in scleroderma: a pilot study. Oral Surg Oral Med Oral Pathol. 1984; 57: 508–511.
Buchbinder D, Currivan R B, Kaplan A J, Urken M L . Mobilization regimens for the prevention of jaw hypomobility in the radiated patient: a comparison of three techniques. J Oral Maxillofac Surg 1993; 51: 863–867.
Conine T A, Carlow D L, Stevenson-Moore P . Dynamic orthoses for the management of microstomia. J Rehabil Res Dev. 1987; 24: 43–48.
Conine T A, Carlow D L, Stevenson-Moore P . The Vancouver microstomia orthosis. J Prosthet Dent. 1989; 61: 476–483.
Davis S, Thompson J G, Clark J, Kowal-Vern A, Latenser BA . A prototype for an economical vertical microstomia orthosis. J Burn Care Res 2006; 27: 352–356.
Dougherty M E, Warden GD . A thirty-year review of oral appliances used to manage microstomia, 1972 to 2002. J Burn Care Rehabil. 2003; 24: 418–431.
Ridgway C L, Warden G D . Evaluation of a vertical mouth stretching orthosis: two case reports. J Burn Care Rehabil. 1995; 16: 74–78.
Naylor W P, Manor R C . Fabrication of a flexible prosthesis for the edentulous scleroderma patient with microstomia. J Prosthet Dent 1983; 50: 536–538.
Comstedt L R, Svensson A, Troilius A . Improvement of microstomia in scleroderma after intense pulsed light: A case series of four patients. J Cosmet Laser Ther 2012; 14: 102–106.
Koymen R, Gulses A, Karacayli U, Aydintug Y S . Treatment of microstomia with commissuroplasties and semidynamic acrylic splints. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2009; 107: 503–507.
Del Papa N, Caviggioli F, Sambataro D et al. Autologous fat grafting in the treatment of fibrotic perioral changes in patients with systemic sclerosis. Cell Transplant. 2015; 24: 63–72.
Mould T L, Roberts-Thomson P J . Pathogenesis of telangiectasia in scleroderma. Asian Pac J Allergy Immunol. 2000; 18: 195–200.
Dinsdale G, Murray A, Moore T et al. A comparison of intense pulsed light and laser treatment of telangiectases in patients with systemic sclerosis: a within-subject randomized trial. Rheumatology (Oxford). 2014; 53: 1422–1430.
Porter S R, Leao J C . Review article: oral ulcers and its relevance to systemic disorders. Aliment Pharmacol Ther. 2005; 21: 295–306.
Bassel M, Hudson M, Taillefer S S, Schieir O, Baron M, Thombs B D . Frequency and impact of symptoms experienced by patients with systemic sclerosis: results from a Canadian National Survey. Rheumatology (Oxford). 2011; 50: 762–767.
Kobak S, Oksel F, Aksu K, Kabasakal Y . The frequency of sicca symptoms and Sjogren's syndrome in patients with systemic sclerosis. Int J Rheum Dis. 2013; 16: 88–92.
Swaminathan S, Goldblatt F, Dugar M, Gordon T P, Roberts-Thomson P J . Prevalence of sicca symptoms in a South Australian cohort with systemic sclerosis. Intern Med J. 2008; 38: 897–903.
Baron M, Hudson M, Tatibouet S et al. Relationship between disease characteristics and orofacial manifestations in systemic sclerosis: Canadian Systemic Sclerosis Oral Health Study III. Arthritis Care Res (Hoboken). 2015; 67: 681–690.
Avouac J, Sordet C, Depinay C 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: 2243–2249.
Janin A, Gosselin B, Gosset D, Hatron P Y, Sauvezie B . Histological criteria of Sjogren's syndrome in scleroderma. Clin Exp Rheumatol 1989; 7: 167–169.
Montgomery-Cranny J, Hodgson T, Hegarty A M . Aetiology and management of xerostomia and salivary gland hypofunction. Br J Hosp Med (Lond) 2014; 75: 509–510: 11–4.
Marmary Y, Glaiss R, Pisanty S . Scleroderma: oral manifestations. Oral Surg Oral Med Oral Pathol 1981; 52: 32–37.
White S C, Frey N W, Blaschke D D et al. Oral radiographic changes in patients with progressive systemic sclerosis (scleroderma). J Am Dent Assoc 1977; 94: 1178–1182.
Alexandridis C, White S C . Periodontal ligament changes in patients with progressive systemic sclerosis. Oral Surg Oral Med Oral Pathol 1984; 58: 113–118.
Dagenais M, MacDonald D, Baron M et al. The Canadian Systemic Sclerosis Oral Health Study IV: oral radiographic manifestations in systemic sclerosis compared with the general population. Oral Surg Oral Med Oral Pathol Oral Radiol 2015; 120: 104–111.
Mehra A . Periodontal space widening in patients with systemic sclerosis: a probable explanation. Dentomaxillofac Radiol 2008; 37: 183; author reply 184.
Haers P E, Sailer H F . Mandibular resorption due to systemic sclerosis. Case report of surgical correction of a secondary open bite deformity. Int J Oral Maxillofac Surg 1995; 24: 261–267.
Hopper F E, Giles A D . Orofacial changes in systemic sclerosis-report of a case of resorption of mandibular angles and zygomatic arches. Br J Oral Surg 1982; 20: 129–134.
Ramon Y, Samra H, Oberman M . Mandibular condylosis and apertognathia as presenting symptoms in progressive systemic sclerosis (scleroderma). Pattern of mandibular bony lesions and atrophy of masticatory muscles in PSS, presumably caused by affected muscular arteries. Oral Surg Oral Med Oral Pathol 1987; 63: 269–274.
Seifert M H, Steigerwald J C, Cliff M M . Bone resorption of the mandible in progressive systemic sclerosis. Arthritis Rheum 1975; 18: 507–512.
Auluck A, Pai K M, Shetty C, Shenoi S D . Mandibular resorption in progressive systemic sclerosis: a report of three cases. Dentomaxillofac Radiol 2005; 34: 384–386.
Farrell D A, Medsger T A, Jr. Trigeminal neuropathy in progressive systemic sclerosis. Am J Med 1982; 73: 57–62.
Fischoff D K, Sirois D . Painful trigeminal neuropathy caused by severe mandibular resorption and nerve compression in a patient with systemic sclerosis: case report and literature review. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2000; 90: 456–459.
MacIntosh R B, Shivapuja P K, Naqvi R . Scleroderma and the temporomandibular joint: reconstruction in 2 variants. J Oral Maxillofac Surg 2015; 73: 1199–1210.
Doucet J C, Morrison A D . Bilateral mandibular condylysis from systemic sclerosis: case report of surgical correction with bilateral total temporomandibular joint replacement. Craniomaxillofac Trauma Reconstr 2011; 4: 11–18.
Scardina G A, Pizzigatti M E, Messina P . Periodontal microcirculatory abnormalities in patients with systemic sclerosis. J Periodontol 2005; 76: 1991–1995.
Ozcelik O, Haytac M C, Ergin M, Antmen B, Seydaoglu G . The immunohistochemical analysis of vascular endothelial growth factors A and C and microvessel density in gingival tissues of systemic sclerosis patients: their possible effects on gingival inflammation. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2008; 105: 481–485.
Yuen H K, Weng Y, Reed S G, Summerlin L M, Silver R M . Factors associated with gingival inflammation among adults with systemic sclerosis. Int J Dent Hyg 2014; 12: 55–61.
Poole J L, Brewer C, Rossie K, Good C C, Conte C, Steen V . Factors related to oral hygiene in persons with scleroderma. Int J Dent Hyg 2005; 3: 13–17.
Poole J, Conte C, Brewer C et al. Oral hygiene in scleroderma: The effectiveness of a multi-disciplinary intervention program. Disabil Rehabil 2010; 32: 379–384.
Elhai M, Meune C, Avouac J, Kahan A, Allanore Y . Trends in mortality in patients with systemic sclerosis over 40 years: a systematic review and meta-analysis of cohort studies. Rheumatology (Oxford) 2012; 51: 1017–1026.
Moinzadeh P, Fonseca C, Hellmich M et al. Association of anti-RNA polymerase III autoantibodies and cancer in scleroderma. Arthritis Res Ther 2014; 16: R53.
Onishi A, Sugiyama D, Kumagai S, Morinobu A . Cancer incidence in systemic sclerosis: meta-analysis of population-based cohort studies. Arthritis Rheum 2013; 65: 1913–1921.
Derk C T, Rasheed M, Spiegel J R, Jimenez S A . Increased incidence of carcinoma of the tongue in patients with systemic sclerosis. J Rheumatol 2005; 32: 637–641.
Derk C T, Rasheed M, Artlett C M, Jimenez SA . A cohort study of cancer incidence in systemic sclerosis. J Rheumatol 2006; 33: 1113–1116.
Kuo C F, Luo S F, Yu K H et al. Cancer risk among patients with systemic sclerosis: a nationwide population study in Taiwan. Scand J Rheumatol 2012; 41: 44–49.
Baron M, Hudson M, Tatibouet S et al. The Canadian systemic sclerosis oral health study II: the relationship between oral and global health-related quality of life in systemic sclerosis. Rheumatology (Oxford) 2015; 54: 692–696.
Mouthon L, Rannou F, Berezne A 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: 1651–1655.
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Veale, B., Jablonski, R., Frech, T. et al. Orofacial manifestations of systemic sclerosis. Br Dent J 221, 305–310 (2016). https://doi.org/10.1038/sj.bdj.2016.678
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DOI: https://doi.org/10.1038/sj.bdj.2016.678
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