Sjögren syndrome: a review for the maxillofacial surgeon

Sj?gren syndrome is a multisystemic condition that predominantly involves the salivary and lacrimal glands. Also known as sicca complex, the disease often is underdiagnosed and poorly understood. This article provides a comprehensive review on Sj?gren syndrome, with an emphasis on diagnosis and treatment modalities.     

Dental implants in patients with Sjögren’s syndrome: a case series and a systematic review

The purpose of this study was to assess the clinical outcomes of dental implants in patients with Sjögren’s syndrome (SS). The study consisted of two parts: report of a case series and a systematic review of the literature. The results of the clinical series revealed that 19 patients received 107 implants and were followed for a mean of 125 months. Two patients lost three implants (failure rate 2.8%, 3/107). At the last follow-up, there was a mean marginal bone loss (MBL) of ?2.190 ± 1.384 mm; estimated MBL after 30 years was 4.39 mm. The review identified 18 studies, resulting in 19 studies for analysis including the present clinical series. A total of 712 implants were placed in 186 patients; 705 implants were followed up for a mean of 72.5 months (failure rate 4.1%, 29/705; failed at a mean time of 12.9 ± 31.7 months). The probability of failure was 2.8% (95% confidence interval 1.6–4.1%). Primary SS patients had a lower implant failure rate (2.5%, 3/118) than secondary SS patients (6.5%, 12/184). In conclusion, dental implants should be considered by dentists as a viable treatment option for patients with SS, as the failure rate is fairly low. SS patients may, however, present a higher MBL around implants than patients from the general population.     

Eagle’s syndrome – A case report and review of the literature

Eagle’s syndrome (ES) occurs when an elongated styloid process or calcified stylohyoid ligament causes recurrent throat pain or foreign body sensation, dysphagia, or facial pain. Additional symptoms may include neck or throat pain with radiation to the ipsilateral ear. The symptoms related to this condition can be confused with those attributed to a wide variety of facial neuralgias. ES can be diagnosed radiologically and by physical examination. The treatment of ES is primarily surgical. The styloid process can be shortened through an intraoral or external approach. In this paper a case of ES exhibiting unilateral symptoms with bilateral elongation of styloid process is reported and the literature is reviewed.     

Sjögren's syndrome: clinical and therapeutic features. A review of the literature

Sj?gren' Syndrome (SS), also named Sicca Syndrome, is a complex disease, characterized by a series of clinical symptoms and signs chiefly represented by xerostomia, xerophthalmia and connectival diseases. The pathogenetic mechanisms consist of an autoimmune process leading to salivary and lacrimal glands progressive destruction. There is a primary form with salivary and lacrimal glands compromission only and a second form in which xerostomia and/or xerophthalmia are associated with connectival diseases like rheumatoid arthritis, systemic lupus erythematosus and scleroderma. The diagnosis of SS is rather difficult and it is based on various world-wide established and accepted criteria: the labial minor salivary glands biopsy and the research of specific seric autoantibodies are the basic elements. From the therapeutic point of view, various types of immunomodulant treatments based on cyclosporine, corticosteroids, methotrexate or alpha-interferon have been proposed with different RESULTS: Cholinergic drugs, like pilocarpine and cevimeline, are also used in order to stimulate the gland functionality.     

Primary and secondary Sjögren’s syndrome in children – a comparative study

Sjögren’s syndrome is a chronic inflammatory systemic autoimmune disease mainly affecting the exocrine and, particularly, the salivary and lacrimal glands. The condition usually occurs in adults. In 1994, the criteria for this syndrome were redefined in a multicenter European study. In children, Sjögren’s syndrome is a rare and probably underdiagnosed disease. To date, Sjögren’s syndrome in children has only been described in case reports and in the comparative presentation of various study results. So far, no study of a comparative classification into primary and secondary Sjögren’s syndrome has been carried out in a patient population of any size. Sjögren’s syndrome should be considered in the differential diagnosis of children with recurrent parotitis, keratoconjunctivitis sicca, or pronounced and early tooth decay associated with xerostomia. In this study of 23 children and adolescents under the age of 16 with the clinical symptoms and laboratory findings of Sjögren’s syndrome, we differentiate between primary and secondary Sjögren’s syndrome. The value of the individual methods of assessing the oral and the ophthalmological components and the manifestation of the underlying rheumatic condition are discussed on the basis of the EULAR criteria. The EULAR diagnostic criteria are of limited applicability in children because reliable anamnestic data are frequently lacking. Another problem in diagnosing Sjögren’s syndrome is the short-term detection of serological alterations and clinical symptoms. Even if young patients do not completely fulfill the required criteria, Sjögren’s syndrome can be assumed or confirmed in the presence of positive testing for oral and ocular manifestations and recurrent salivary gland enlargement.     

Orthognathic surgery in Melnick–Needles syndrome: a review of the literature and report of two siblings

Melnick–Needles syndrome (MNS) is a rare congenital X-linked dominant skeletal dysplasia, characterized by exophthalmos, a prominent forehead, and mandibular hypoplasia and retrognathism. Dental features may include anodontia, hypodontia, or oligodontia. Increased collagen content, unpredictable collagen synthesis, and abnormal bony architecture have raised concerns regarding bone splitting intraoperatively and bone healing postoperatively. This report describes the cases of two sisters with MNS, who successfully underwent orthognathic surgery consisting of bilateral mandibular ramus osteotomies combined with advancement genioplasty and iliac crest bone grafting, to correct the classical MNS facial deformity of mandibular retrognathia.     

Primary Sjögren syndrome in a 2-year-old patient: role of the dentist in diagnosis and dental management with a 6-year follow-up

International Journal of Paediatric Dentistry 2011; 21: 471–475 Background. Primary Sjögren syndrome is a rare autoimmune disease, especially in children, mainly affecting girls (77%), and usually diagnosed around 10 years of age. Diagnosis during childhood is difficult, especially because of the diversity of the clinical presentation and difficulty obtaining reliable history data, accounting for a higher frequency of underdiagnosed cases. Differential conditions should be considered, especially the ones that promote xerostomia, such as diabetes, ectodermal dysplasia, rheumatoid arthritis, scleroderma, systemic lupus erythematosus, sarcoidosis, lymphoma, HIV and HTLV infection. Conditions associated with parotid enlargement should also be excluded, including juvenile recurrent parotitis (JRP), sialadenosis, sarcoidosis, lymphoma, infectious parotitis caused by streptococcal and staphylococcal infections, viral infections (paramyxovirus, Epstein–Barr virus, cytomegalovirus, and parvovirus), and diffuse infiltrative lymphocytosis syndrome (associated with HIV infection), and rare congenital conditions, such as polycystic parotid disease. Case report. A paediatric female patient was referred to our clinic for dental treatment complaining about dry mouth, oral discomfort, and dysphagia. The patient presented five of the required criteria to establish the diagnosis of pSS, including ocular symptoms, oral symptoms, evidence of keratoconjunctivitis sicca, focal sialadenitis confirmed by minor salivary gland biopsy, and evidence of major salivary gland involvement. Our patient did not have positive SS‐A and SS‐B autoantibodies. According to the literature, about 29% of individuals with pSS can present seronegativity for SS‐A (anti‐Ro) antibodies and about 33% can present seronegativity for SS‐B (anti‐La) antibodies. Conclusion. To the best of our knowledge, this is the youngest patient reported in the scientific English literature with pSS. Primary Sjögren syndrome has a wide clinical and immunologic spectrum and may progress with increased morbidity. Clinicians must be aware of the development of pSS in such an early age and exclude all possible differential findings to provide early diagnosis and treatment.     

Primary sialoangiectasia--a diagnostic pitfall in Sj?gren's syndrome: case report.

A case of primary sialoangiectasia, which in this case was initially misdiagnosed as Sj?gren's syndrome, is described. Other diseases, including HIV infection, psoriatic arthritis, and acute parotitis, may cause glandular changes similar to the changes found in the syndrome. Therefore, sialography must be combined with other methods of assessment of the oral cavity when suspicion is high for Sj?gren's syndrome. Properly applied, sialography provides essential information regarding the severity of glandular damage and the progression of the disease.     

Salivary dysfunction and quality of life in Sjögren syndrome: a critical oral-systemic connection

BACKGROUND: The importance of oral health to systemic health and quality of life (QOL) is gaining attention. Although several studies have examined generic (general) QOL in Sj?gren syndrome (SS), little information exists on the effect of oral health on QOL and relationships among self-reported oral health, systemic health and objective clinical measures of health. The authors conducted this study to characterize these relationships in a sample of patients with SS. METHODS: Thirty-nine patients with a diagnosis of SS ascertained by means of the 2002 American-European Consensus criteria completed both the Oral Health Impact Profile (OHIP-14) and the Medical Outcomes Study 36-Item Short-Form Health Survey (SF-36) QOL questionnaires. OHIP-14 measures pain; functional limitation; and psychological, emotional and social disability associated with the mouth. SF-36 measures physical and emotional health and the ability to perform usual activities. Additional measures included the number of self-reported autoimmune symptoms and an index of disease damage. Statistical analysis was performed by using hierarchical regression analysis. RESULTS: Both generic and oral health-related QOL were poor in these patients. Specifically, the findings indicated that salivary flow rate was correlated significantly with both Disease Damage Index and OHIP-14 ratings, the number of autoimmune symptoms was correlated significantly with both oral and generic QOL, and oral health accounted for a significant percentage of variance in SF-36 domains of general health and social function. CONCLUSIONS: Oral health appears to have an independent influence on general QOL in patients with SS. These findings underscore the importance of proactive dental management of the oral manifestations of SS. CLINICAL IMPLICATIONS: Dentists and physicians must work collaboratively to maintain oral health and quality of life for patients with Sj?gren syndrome. The dentist should address patients' concerns of xerostomia and hyposalivation in an aggressive manner.     

Cholinoreceptor autoantibodies in Sjögren syndrome

Previous studies have demonstrated that antibodies against cholinoreceptors of exocrine glands correlate with dry mouth in persons with primary Sj?gren syndrome (pSS). The aim of the present investigation was to establish if serum IgG antibodies (pSS IgG) were able to interact with cholinoreceptors in rat submandibular gland-dependent stimulation of cyclooxygenase 2 (COX-2) mRNA expression and PGE(2) production. Our findings indicated that pSS IgG-stimulating M(3), M(4), and M(1) cholinoreceptors exerted an increase in COX-2 mRNA without affecting COX-1 mRNA expression and increased PGE(2) production. Inhibitors of phospholipase A(2), COX- s, L-type calcium channel currents, and Ca(2+)-ATPase from sarcoplasmic reticulum prevented the pSS IgG effect on PGE(2) production. An ionophore of calcium mimicked pSS IgG action, suggesting a crucial role of calcium homeostasis in the cholinoreceptor-stimulated increase in PGE(2) production. Moreover, the amounts of PGE(2) in saliva and in sera from persons with pSS were significantly higher than in pre- or post-menopausal women. These findings illustrate the importance of autoantibodies to cholinoreceptors in the generation of chronic inflammation of target tissues in SS.     

Marathon of eponyms: 19 Sjögren syndrome

Oral Diseases (2011) 17 , 538–540 The use of eponyms has long been contentious, but many remain in common use, as discussed elsewhere (Editorial: Oral Diseases. 2009: 15; 185). The use of eponyms in diseases of the head and neck is found mainly in specialties dealing with medically compromised individuals (paediatric dentistry, special care dentistry, oral and maxillofacial medicine, oral and maxillofacial pathology, oral and maxillofacial radiology and oral and maxillofacial surgery) and particularly by hospital‐centred practitioners. This series has selected some of the more recognized relevant eponymous conditions and presents them alphabetically. The information is based largely on data available from MEDLINE and a number of internet websites as noted below: the authors would welcome any corrections. This document summarizes data about Sjögren syndrome.     

Oral facial digital syndrome—Case report and review of the literature

A patient is described with developmental deformities of the mouth, face and limbs. Patients with similar deformities have previously been described and referred to as the oral-facial-digital syndrome, Type I or Type II. This patient, however, had characteristics of both types in addition to malformation of the limbs and temporal lobe epilepsy features not previously described.     

B cell dysregulation in primary Sjögren’s syndrome: A review

Primary Sjögren’s syndrome is a chronic autoimmune disorder of unknown etiology and is characterized by progressive focal lymphocytic infiltration of the lacrimal and salivary glands. Comparison of B cell subsets from the peripheral blood and salivary glands of patients with primary Sjögren’s syndrome and those from healthy individuals shows dysregulation and derangement of B cell subsets in both peripheral circulation and in inflamed glandular tissues. This dysregulation is expressed as a decrease in the percentage of CD27+ memory B cells in peripheral blood and an increase in the CD27+ memory B cells in the affected glands. Further, the overall percentage of long-lived autoantibodies-producing plasma cells within the affected glands is increased. In the last two decades, several studies have shown growing evidences that B cells play multiple roles in primary Sjögren’s syndrome pathophysiology, and that dysregulation of these cells may actually play a central role in the disease development.     

Oral involvement in primary Sjögren syndrome

BackgroundIn small studies, investigators have described oral features and their sequelae in primary Sjögren syndrome (PSS), but they have not provided a full picture of the aspects and implications of oral involvement. The authors describe what is, to their knowledge, the first large-scale evaluation to do so. In addition, they report data regarding utilization and cost of dental care among patients with PSS.MethodsThe authors surveyed patients with primary Sjögren syndrome as identified by their physicians (PhysR-PSS), patient-members of the Sjögren's Syndrome Foundation (SSF-PSS) and control subjects who did not have PSS. They made comparisons between the three groups.ResultsSubjects were 277 patients with PhysR-PSS, 1,225 patients with SSF-PSS and 606 control subjects. More than 96 percent of those in the patient groups experienced oral problems. An oral complaint was the initial symptom in more than one-half of the patients. Xerostomia-associated signs and symptoms were common and severe, as evidenced by scores on an inventory of sicca symptoms. These patients' rate of dental care utilization was high, and the care was costly.ConclusionsOral and dental disease in PSS is extensive and persistent and represents a significant burden of illness.Clinical ImplicationsOral symptoms and signs are common in patients with PSS. Early recognition of the significance of these findings by oral specialists could accelerate diagnosis and minimize oral morbidities.     

Gorham–Stout disease of the mandible: case report and review of literature of a rare type of osteolysis

Oral Radiology - Gorham–Stout disease (GSD) is a rare form of osteolysis, the aetiology and pathogenesis of which remains controversial to this date. Although more than 200 cases of GSD have...