Systemic sclerosis (SSc) is a rare multisystem connective tissue disorder characterized by the triad fibrosis, vasculopathy and immune dysregulation. This chronic disease has a significant impact on the orofacial region that is involved in more than two-thirds of the cases. SSc patients can show a wide array of oral manifestations, which are usually associated with a severe impairment of the quality of life. They often present a decreased the salivary flow and a reduced mouth opening that contribute substantially to the worsening of the oral health status. Therefore, SSc patients require specific and multidisciplinary interventions that should be initiated as early as possible. The identification of specific radiological and clinical signs at the early stage will improve the management of such patients. This study reviews the wide spectrum of orofacial manifestations associated with SSc and suggests clues for the oral management that remains challenging.
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
Progressive systemic sclerosis presenting with Raynaud's phenomenon in the tongue and sensory trigeminal neuropathy
Mandibular resorption due to systemic sclerosis. Case report of surgical correction of a secondary open bite deformity
E-selectin expression in salivary endothelial cells and sera from patients with systemic sclerosis. Role of resident mast cell-derived tumor necrosis factor alpha
Xerostomia in systemic sclerosis: systematic evaluation by salivary scintigraphy and lip biopsy in thirty-four patients
Early expression of E-selectin, tumor necrosis factor alpha, and mast cell infiltration in the salivary glands of patients with systemic sclerosis
The value of the Health Assessment Questionnaire and special patient-generated scales to demonstrate change in systemic sclerosis patients over time
Bilateral oral commissurotomy using buccal mucosa flaps for management of microstomia: report of a case
The correlation between salivary endothelial expression of E-selectin and clinical and biological parameters in systemic scleroderma
Pilocarpine tablets for the treatment of dry mouth and dry eye symptoms in patients with Sjögren syndrome: a randomized, placebo-controlled, fixed-dose, multicenter trial. P92-01 Study Group
Mast cells, extracellular matrix components, TGFbeta isoforms and TGFbeta receptor expression in labial salivary glands in systemic sclerosis
Painful trigeminal neuropathy caused by severe mandibular resorption and nerve compression in a patient with systemic sclerosis: case report and literature review
The use of the Health Assessment Questionnaire (HAQ) to determine physical disability in systemic sclerosis
Osteolysis of the cervical spine and mandible in systemic sclerosis: a case report with computed tomography and magnetic resonance imaging findings
Analysis of systemic sclerosis in twins reveals low concordance for disease and high concordance for the presence of antinuclear antibodies
Prevalence, incidence, survival, and disease characteristics of systemic sclerosis in a large US population
Effects of a nonsurgical exercise program on the decreased mouth opening in patients with systemic scleroderma
Cytokine and chemokine levels in systemic sclerosis: relationship with cutaneous and internal organ involvement
Progressive systemic sclerosis with spontaneous fracture due to resorption of the mandible: a case report
Impact of systemic sclerosis oral manifestations on patients' health-related quality of life: A systematic review
Volumetric Cone Beam Computed Tomography for the Assessment of Oral Manifestations in Systemic Sclerosis: Data from an EUSTAR Cohort
Multiple External Root Resorption of Teeth as a New Manifestation of Systemic Sclerosis-A Cross-Sectional Study in Japan
Dental management of scleroderma patients using pentoxifylline plus vitamin E with and without TheraBite® to reduce trismus: Two case reports and brief review of literature
Reduced salivary flow and caries status are correlated with disease activity and severity in patients with diffuse cutaneous systemic sclerosis.
Measuring the impact of systemic sclerosis on oral health-related quality of life in a UK population.
Prevalence of Oral and Maxillofacial Disorders in Patients with Systemic Scleroderma-A Systematic Review.
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