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Year : 2014  |  Volume : 5  |  Issue : 1  |  Page : 31-35

Sjogren's syndrome: A review

Department of Oral Medicine and Radiology, Institute of Dental Sciences, Bareilly, Uttar Pradesh, India

Date of Web Publication19-Mar-2014

Correspondence Address:
Mallika Kishore
Department of Oral Medicine and Radiology, Institute of Dental Sciences, Bareilly, Uttar Pradesh
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0976-433X.129070

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Sjogren's syndrome (SS) is a chronic autoimmune disease characterized by the lymphocytic infiltration of salivary and lacrimal glands leading to xerostomia and keratoconjunctivitis sicca. Prevalence of primary SS in the general population has been estimated to be around 1-3%. SS is an under-recognized disease in which most of the significant progress has been made in the past 25 years. The herald of newer diagnostic tools could help clinicians and thereby provide significant relief to patients through earlier treatments. The treatment of SS is limited to symptomatic management, and involves the use of solutions to replace salivary secretion and afford a measure of hydration. The purpose of present paper is to highlight the difficulties and complexities that are inherent in the diagnosis of SS and the important role that dental practitioners can play in the management of its oral manifestations.

Keywords: Diagnosis, oral, Sjogren′s syndrome

How to cite this article:
Kishore M, Panat SR, Aggarwal A, Agarwal N, Upadhyay N, Garg R. Sjogren's syndrome: A review. SRM J Res Dent Sci 2014;5:31-5

How to cite this URL:
Kishore M, Panat SR, Aggarwal A, Agarwal N, Upadhyay N, Garg R. Sjogren's syndrome: A review. SRM J Res Dent Sci [serial online] 2014 [cited 2023 Jan 28];5:31-5. Available from:

  Introduction Top

Sjogren's syndrome (SS) is a chronic autoimmune disease characterized by lymphocytic infiltration and subsequent destruction of the exocrine glands, including those found in the nose, ears, skin, vagina, respiratory and gastrointestinal systems. [1] It is among the group of diseases overseen by rheumatologists; however, its diagnosis and management require 3 areas of specialty practice: rheumatology, ophthalmology, and oral medicine. [2]

Therefore, this paper aims to review and critically address the recent advances on the aetiopathogenesis of the SS, taking into account the attained clinical features, with particular relevance given to the oral involvement.

  Epidemology Top

SS is estimated to affect 1-3% of the general population. [3] A cautious but realistic estimate from the studies presented so far will be that primary SS(pSS) is a disease with a prevalence not exceeding 0.6% of the general population (6/1000). [4] In an epidemiologic study, the calculated prevalence of SS in 705 randomly selected women aged ranges from 52-72 years was 2.7%. [5] SS, although a common disorder in Western countries with an estimated prevalence of 3 in 100 to 1 in 1000, has rarely been reported from India. [6] This report highlights the rarity of this disease in our geographic region.

  Etiology and Pathogenesis Top

Even if tremendous progress in the field of research has been made to unveil the different mechanistic processes underlying the development of SS, the initial triggering events of the disease have yet to be unearthed. Central to the pathophysiology of SS is chronic perpetual stimulation of the autoimmune system. Both B and T cells are implicated in the pathogenesis of the SS, even though the mechanisms underlying humoral and cellular abnormalities are not yet known. [7],[8]

It is probably the result of an environmental stimulus that promotes an autoimmune reaction in genetically susceptible persons. Infectious agents most commonly sialotropic viruses have been postulated to trigger the syndrome; however, associations with most of the potential viral candidates, including cytomegalovirus and Epstein-Barr virus, are weak. [9] The putative role of different viruses in SS can be viewed in the light that salivary glands are a site of latent viral infections. [10]

A genetic predisposition to SS has been suggested because of multiple reports of two or more members of the same family developing the syndrome. Affected individuals of different ethnic origins carry different human leucocyte antigen susceptibilities. [4]

The high percentage of females with SS compared to males suggests that the immune regulatory properties of sex hormones are involved in its development. [11] Androgen deficiency, both locally and systemically, has also been pointed out to be a key factor prompting SS. Reduced plasmatic levels (up to 40 - 50%) of dehydroepiandrosterone sulfate, the precursor sex steroid hormone produced in the adrenal cortex, has been identified in SS-affected individuals, comparing to age and sex matched controls. [12]

  Clinical Features Top

Glandular manifestations


The dry mouth is often manifest as the 'cream cracker' sign an inability to swallow dry dental caries is a common feature that may prompt a referral from a dentist. About half of the patients complain of recurrent parotid swelling, particularly relatively young patients in whom the inflammatory phase predominates. [13]

In advanced disease, the oral mucosa appears dry and glazed and tends to form fine wrinkles. Extreme dryness of the mouth, causing the tongue to stick to the palate, may lead to a "clicking" quality in the speech of patients with SS. In general, the surface of the tongue becomes red and lobulated, with partial or complete depapillation. [14]

Ocular manifestations

Diminished tear production due to lacrimal gland involvement leads to the destruction of both corneal and bulbar conjunctival epithelium and a constellation of clinical findings termed keratoconjunctivitis sicca (KCS). [15]

Symptoms of dry eye may include sensations of itching, grittiness, or soreness, even though the eyes' appearance is normal. Ocular complaints may include photosensitivity, erythema, eye fatigue, decreased visual acuity, a discharge in the eyes, and the sensation of a film across the visual field. [14]

In more severe disease, functional disability with visual impairment occurs. Complications of KCS include corneal ulcerations that can lead to perforations and iridocyclitis. [16]

Extraglandular manifestations

Musculoskeletal involvement

Joint disease associated with SS is commonly a polyarticular arthropathy which intermittently affects small joints. Evidence of joint deformity and erosion may be encountered in primary SS-affected individuals, as well as nonerosive arthritis. Arthralgias, myalgias and fibromyalgia-like features are also commonly found. [17]

Pulmonary involvement

Although common, pulmonary involvement is seldom clinically significant in patients with SS. [18] Interstitial lung disease is a classic feature of SS. The clinical manifestations include cough, dyspnea on exertion, bilateral pulmonary infiltrates on plain chest radiographs and other abnormalities on computer tomography scanner such as wall thickening at the segmental bronchi. With disease progression, fibrosis and neutrophilic alveolitis are present. [19]

Renal involvement

Kidney involvement is a frequent extraglandular manifestation of pSS. The renal involvement is rarely overt, and more often follows a subclinical course. In some cases, it may precede the onset of subjective sicca syndrome. [20] In SS patients who show evidence of glomerular lesions, hematuria, proteinuria, and renal insufficiency may be exhibited. Some patients may progress to nephrotic syndrome. [14]

Gastroenterologic involvement

The manifestations of gastrointestinal tract are not very specific and include esophageal dysmotility and gastro-intestinal reflux. Patients often complain of dysphagia, nausea and epigastric pain. Subclinical pancreatic involvement is present in approximately 25% of cases. There are no specific liver abnormalities, which can be attributed to SS, but autoimmune hepatitis and primary biliary cirrhosis can be associated diseases. [16],[21]

Neurologic involvement

Neurologic disease is one of the most common manifestations of SS, affecting cranial and peripheral nerves, and more rarely the central nervous system. The eclectic permutation of peripheral nervous system syndromes which occur in SS patients are among the most common and severe extraglandular complications. [22]

Hematologic/Oncologic involvement

Patients with SS frequently have leucopenia, thrombocytopenia, a high erythrocyte sedimentation rate and anti-nuclear antibodies. [13] Anemia of chronic disease and hypergammaglobulinemia are the most prevalent hematologic manifestations encountered at diagnosis and during the course of pSS. [23]

Thyroid Involvement

Hypothyroidism seems to be common in SS. [24] A study has shown that 45% of patients with SS also had associated thyroid dysfunction. This is further supported by a study suggesting that the prevalence of SS is 10 times higher in patients with autoimmune thyroiditis. [25]

Obstetrical and gynecological involvement

Dyspareunia secondary to impaired lubrication is verified in many premenopausal women with pSS. Other gynecological problems include vaginal dryness, endometriosis, episodes of amenorrhea and menorrhagia/metrorrhagia. [26]

Cardiac Involvement

Pericarditis and pulmonary hypertension can occur in SS. Cardiovascular tests suggestive of autonomic neuropathy, such as response of blood pressure to sustained hand grip, valsalva maneuver, heart-rate response to deep breathing, heart-rate and blood-pressure response to standing are abnormal in some patients with SS. [27]


The diagnosis of SS is not straightforward as many of the symptoms are subjective and vague and can be dismissed initially as other conditions or effect of medications. [28] Although minor salivary gland biopsy has been traditionally considered "the gold standard" for the diagnosis of SS, newer criteria have emerged to assist on this disease identification.

The revised diagnostic criteria established in 2002 by the American-European Consensus Group are listed in [Table 1]. [4] Early recognition of SS may prevent complications such as dental caries, corneal ulceration, chronic oral infection, and sialadenitis, and it allows for clinical surveillance for the development of serious extraglandular systemic manifestations. [14]
Table 1: Diagnostic criteria of Sjögren's syndrome

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Diagnostic Tests

Oral diagnostic tests


Patients with clinically overt SS have reduced flow rate.Measuring submandibular/sublingual flow rates may contribute to an early diagnosis of SS. In contrast, parotid flow rates are decreased in SS and Non-SS sicca patients. [29] The test should therefore be standardized; the unstimulated whole saliva collection test is performed for 15 min, and the test is considered positive when <1.5 ml whole saliva is collected. [4]


The sialography typically shows sialectasias in contrast to the fine arborization seen in normal parotid ductules. Diagnosis is generally based on the classification given in [Table 2]. [30]
Table 2: Sialographic staging

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In the scintigraphic test, 99mTc-pertechnate is given intravenously, and in SS patients the typical finding is decreased uptake in response to stimulation of the parotid and submandibular salivary glands. This test is a sensitive and valid method to measure abnormalities in salivary gland function in the hands of skilled personal. [31]


SS is a mixture of increased inflammatory proteins and decreased acinar proteins when compared with healthy controls. [32] Furthermore ionic changes were observed in SS-affected individuals, namely regarding the levels of chloride, potassium, calcium, sodium and magnesium. [29]

Magnetic Resonance (MR) and ultrasonography (US)

MR imaging (MRI), MR sialography and US are noninvasive methodologies that allow the imaging of salivary glands in their physiological state without artefacts induced by intraductal contrast media or biopsy procedures. MRI was shown to provide a reliable imaging procedure to evaluate glandular alterations. It allows multiplanar evaluation and processes a high contrast tissue resolution. Characteristically, in SS, MRI reveals an inhomogeneous internal pattern on both T1 and T2 sequences, with multiple hypo- and hyper-intense nodules of different sizes. [33]

Serologic tests

Serology is used to establish the presence of anti-SS-A/Ro and anti-SS-B/La auto-antibodies,based on (enzyme-linked immunosorbent assay). Anti-SS-A/Ro antibodies can also be detected in other autoimmune processes such as rheumatoid arthritis and systemic lupus erythematosus; for this reason, anti-SS-B/La antibodies are considered to be more specific of SS. Anti- SS-A/Ro can be isolated in 25-65% of cases, and anti- SS-B/La in 13-48%. [34]

Ocular Diagnostic tests

  1. Schirmer test
  2. Rose Bengal Staining
  3. Tear break up time (BUT)

  Histopathology Top

Salivary gland Biopsy

A positive biopsy is defined as at least one focus of dense, inflammatory infiltrate containing at least 50 lymphocytes/4 mm 2 . The lip biopsy may be useful in ambiguous cases or when therapy beyond symptom management is being considered. [35]

Differential Diagnosis

The differential diagnosis of SS includes conditions and medications that can produce KCS, xerostomia, and parotid gland enlargement [Table 3]. [14]
Table 3: Differential Diagnosis

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At present, treatment for most patients is essentially symptomatic.The patient should regularly visit a rheumatologist as well as an ophthalmologist and dentist in order to prevent and treat the consequences of mucosal dryness, in addition to extraglandular manifestations and other associated complications [Table 4]. [4],[36],[37],[38],[39]
Table 4: Management of SS

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  Conclusion Top

People with SS may be more susceptible to drug allergies and care is needed to monitor their condition if medication is required. Like all chronic diseases it is important to have regular contact with your doctor and eye specialist to monitor your condition. Regular dental checkups are essential. Although SS is not life threatening, careful attention to the problems it causes can help minimize the "nuisance" aspect of this condition and assist in a more relaxed way.

  References Top

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37.Tsifetaki N, Kitsos G, Paschides CA, Alamanos Y, Eftaxias V, Voulgari PV, et al. Oral pilocarpine for the treatment of ocular symptoms in patients with Sjögren′s syndrome: A randomised 12 week controlled study. Ann Rheum Dis 2003;62:1204-7.  Back to cited text no. 37
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  [Table 1], [Table 2], [Table 3], [Table 4]


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