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Sjogren Syndrome

Discussion in 'Immunology and Rheumatology' started by Dr.Scorpiowoman, Nov 23, 2016.

  1. Dr.Scorpiowoman

    Dr.Scorpiowoman Golden Member

    May 23, 2016
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    Sjögren syndrome is a systemic chronic inflammatory disorder characterized by lymphocytic infiltrates in exocrine organs. Most individuals with Sjögren syndrome present with sicca symptoms, such as xerophthalmia (dry eyes), xerostomia (dry mouth), and parotid gland enlargement, which is seen in the image below. [1]



    Marked bilateral parotid gland enlargement in a patient with primary Sjögren syndrome. Sicca syndrome is a common clinical finding.

    In addition, numerous extraglandular features may develop, such as the following:

    • Arthralgia
    • Arthritis
    • Raynaud phenomenon
    • Myalgia
    • Pulmonary disease
    • Gastrointestinal disease
    • Leukopenia
    • Anemia
    • Lymphadenopathy
    • Neuropathy
    • Vasculitis
    • Renal tubular acidosis
    • Lymphoma
    About 50% of patients with Sjögren syndrome have cutaneous findings, such as dry skin (xeroderma), palpable and nonpalpable purpura, and/or urticaria. [2]

    Primary Sjögren syndrome occurs in the absence of another underlying rheumatic disorder, whereas secondary Sjögren syndrome is associated with another underlying rheumatic disease, such as systemic lupus erythematosus (SLE), rheumatoid arthritis (RA), or scleroderma. Given the overlap of Sjögren syndrome with many other rheumatic disorders, it is sometimes difficult to determine whether a clinical manifestation is solely a consequence of Sjögren syndrome or is due to one of its overlapping disorders.

    Importantly, classic clinical features of Sjögren syndrome may also be seen in infections with certain viruses. These include hepatitis C virus, human immunodeficiency virus (HIV), and human T-cell lymphotrophic virus (HTLV).

    Treatment for Sjögren syndrome is largely based on symptoms (eg, lotion for dry skin, artificial tears for dry eyes). Rituximab has shown promise in the treatment of severe extraglandular manifestations (eg, vasculitis, cryoglobulinemia, peripheral neuropathy). Patients must be monitored carefully for the potential development of lymphoma, as the risk for this disease is significantly higher than in the general population.

    Classification criteria

    A number of classification criteria for Sjögren syndrome were designed primarily for clinical research studies but are also often used to help guide clinical diagnoses. The American-European Consensus Group’s criteria for the classification of Sjögren syndrome were proposed in 2002 and are the most commonly used criteria for the diagnosis of Sjögren syndrome. A new set of classification criteria has been developed by the Sjögren’s International Collaborative Clinical Alliance (SICCA) investigators and accepted as a provisional criteria set by the American College of Rheumatology (ACR) in 2012.

    American-European Consensus Group classification

    The American-European Consensus Group (AECG) criteria for the classification of Sjögren syndrome are outlined below. [3, 4] These criteria allow a diagnosis of Sjögren syndrome in patients without sicca symptoms or who have not undergone a biopsy.

    According to the American-European classification system (as modified by Tzioufas and Voulgarelis [5] ), diagnosis of primary Sjögren syndrome requires at least four of the criteria listed below; in addition, either criterion number 5 or criterion number 6 must be included. Sjögren syndrome can be diagnosed in patients who have no sicca symptoms if three of the four objective criteria are fulfilled. The criteria are as follows:

    1. Ocular symptoms - Dry eyes for more than 3 months, foreign-body sensation, use of tear substitutes more than 3 times daily
    2. Oral symptoms - Feeling of dry mouth, recurrently swollen salivary glands, frequent use of liquids to aid swallowing
    3. Ocular signs - Schirmer test performed without anesthesia (< 5 mm in 5 min), positive vital dye staining results
    4. Oral signs - Abnormal salivary scintigraphy findings, abnormal parotid sialography findings, abnormal sialometry findings (unstimulated salivary flow < 1.5 mL in 15 min)
    5. Positive minor salivary gland biopsy findings
    6. Positive anti–SSA or anti–SSB antibody results

    Secondary Sjögren syndrome is diagnosed when, in the presence of a connective-tissue disease, symptoms of oral or ocular dryness exist in addition to criterion 3, 4, or 5, above.

    Application of these criteria has yielded a sensitivity of 97.2% and a specificity of 48.6% for the diagnosis of primary Sjögren syndrome. For secondary Sjögren syndrome, the specificity is 97.2% and the sensitivity, 64.7%. [6]

    Exclusion criteria include any of the following:

    • Past head-and-neck irradiation
    • Hepatitis C virus infection
    • Acquired immunodeficiency syndrome (AIDS)
    • Prior lymphoma
    • Sarcoidosis
    • Graft versus host disease
    • Use of anticholinergic drugs

    ACR classification criteria for Sjogren syndrome

    These classification criteria were developed by SICCA investigators in an effort to improve specificity of criteria used for entry into clinical trials, especially in light of the emergence of biologic agents as potential treatments for Sjögren syndrome and their associated comorbidities. This high specificity makes the ACR criteria more suitable for application in situations in which misclassification may present a health risk. They were accepted by the ACR as a provisional criteria set in 2012. [7]

    According to the ACR criteria, the diagnosis of Sjögren syndrome requires at least two of the following three findings:

    • Positive serum anti-SSA and/or anti-SSB antibodies or positive rheumatoid factor and antinuclear antibody titer of at least 1:320
    • Ocular staining score of at least 3
    • Presence of focal lymphocytic sialadenitis with a focus score of at least 1 focus/4 mm 2 in labial salivary gland biopsy samples

    In comparison with commonly used AECG criteria, the ACR criteria are based entirely on a combination of objective tests that assess the three main components of Sjögren syndrome (serologic, ocular, and salivary) and do not include criteria based on subjective symptoms of ocular and oral dryness.

    Application of these criteria has yielded a sensitivity of 93% and a specificity of 95% for the diagnosis of Sjögren syndrome. These criteria do not distinguish between primary and secondary forms of Sjögren syndrome.


    Complications related to Sjögren syndrome include the following:

    • Emergence of disorders associated with Sjögren syndrome, such as SLE and RA
    • Infection of the parotid gland, typically staphylococcal, streptococcal, or pneumococcal - clues include unilateral worsening of symptoms, along with tenderness, warmth, and erythema
    • Emergence of parotid tumors - watch for unusually hard or unilateral parotid enlargement
    • Pregnant patients with antiRo/SS-A antidodies are at risk for fetal loss, complete heart block in the fetus ,and neonatal lupus syndrome in the newborn
    • Emergence of pseudolymphomas (pleomorphic cells that do not meet the criteria for malignancy) and non-Hodgkin B-cell lymphomas (see the image below) [5]

    • fd0ee9c6ab03b7124a50058dbe725133.jpg

    Clinical photograph and photomicrograph of a 48-year-old man with Sjögren syndrome with a large left parotid mass. On biopsy, B-cell lymphoma of mucosa-associated lymphoid tissue (MALT) type was identified. Microscopic section of parotid biopsy, stained with immunoperoxidase for kappa light chains (brown-stained cells), showed monoclonal population of B cells, confirming the diagnosis.

    Sjögren syndrome can occur as a primary disease of exocrine gland dysfunction or in association with several other autoimmune diseases (eg, systemic lupus erythematosus [SLE], rheumatoid arthritis, scleroderma, systemic sclerosis, cryoglobulinemia, polyarteritis nodosa). These primary and secondary types occur with similar frequency, but the sicca complex seems to cause more severe symptoms in the primary form.

    Virtually all organs may be involved. The disease commonly affects the eyes, mouth, parotid gland, lungs, kidneys, skin, and nervous system.

    The etiology of Sjögren syndrome is not well understood. The presence of activated salivary gland epithelial cells expressing major histocompatibility complex (MHC) class II molecules and the identification of inherited susceptibility markers suggest that environmental or endogenous antigens trigger a self-perpetuating inflammatory response in susceptible individuals. In addition, the continuing presence of active interferon pathways in Sjögren syndrome suggests ongoing activation of the innate immune system. [8, 9] Together, these findings suggest an ongoing interaction between the innate and acquired immune systems in Sjögren syndrome.

    Association with the human leukocyte antigen

    The frequency of HLA-DR52 in patients with primary Sjögren syndrome is estimated to be 87%, but it is also significantly increased in secondary Sjögren syndrome that occurs with rheumatoid arthritis or systemic lupus erythematosus.

    The genetic associations in Sjögren syndrome vary among ethnic groups. In white persons, for instance, the condition is linked to human leukocyte antigen (HLA)–DR3, HLA-DQ2, and HLA-B8, [10] whereas the linkage is to HLA-DRB1*15 in Spanish persons [11] and to HLA-DR5 in Greek and Israeli persons. [12]

    Some evidence indicates that the true association of Sjögren syndrome may be with HLA-DQA1, which is in linkage disequilibrium with HLA-DR3 and HLA-DR5. [13] These HLA associations appear restricted to individuals with Sjögren syndrome who have antibodies to the antigens SSA and SSB rather than to the disease manifestations themselves. [14]

    Possible disease triggers

    Viruses are viable candidates as environmental triggers, although proof of causation has remained elusive, and certainly no single virus has been implicated. Epstein-Barr virus (EBV), HTLV-1, human herpesvirus 6 (HHV-6), HIV, hepatitis C virus (HCV), and cytomegalovirus (CMV) may have a role. Sjögrenlike syndromes are seen in patients infected with HIV, HTLV-1, and hepatitis C. [15, 16, 17]

    Damage and/or cell death due to viral infection or other causes may provide triggering antigens to Toll-like receptors in or on dendritic or epithelial cells, which, by recognizing pathogen-associated patterns, are activated and begin producing cytokines, chemokines, and adhesion molecules. As T and B lymphocytes migrate into the gland, they themselves become activated by dendritic and epithelial cells, thereafter acting as antigen-presenting cells. [18]

    Expressed antigens include SSA/Ro, SSB/La, alpha-fodrin and beta-fodrin, and cholinergic muscarinic receptors. [14] Dendritic cell triggering by immune complexes formed from SSA ̶ anti-SSA (or other immune complexes) may propagate the ongoing innate and acquired immune activation.

    Glandular pathology

    The pathology of a typical involved salivary or lacrimal gland in Sjögren syndrome reveals aggregations of lymphocytes—periductal at first, then panlobular. These cells are primarily CD4 T cells (75%) and memory cells, with 10% B cells and immunoglobulin-secreting plasma cells. Although individual lobules can be destroyed, salivary gland biopsy samples from patients with Sjögren syndrome typically retain 40%-50% of their viable glandular structure. Therefore, inflammatory destruction of salivary and lacrimal glands may not fully account for the symptoms of Sjögren syndrome. [19]

    Studies suggest that the disease process of Sjögren syndrome has a neuroendocrine component. Proinflammatory cytokines released by epithelial cells and lymphocytes may impair neural release of acetylcholine. In addition, antibodies to acetylcholine (muscarinic) receptors may interfere with the neural stimulation of local glandular secretion, [20] perhaps by interfering with aquaporin. [21] Moreover, a study reports that M3 muscarinic receptor antibodies may cause autonomic dysfunction in patients with Sjögren syndrome. [22, 23]

    Current studies have also focused on the role of apoptotic mechanisms in the pathogenesis of primary Sjögren syndrome. A defect in Fas-mediated apoptosis, which is necessary for down-regulation of the immune response, can result in a chronic inflammatory destruction of the salivary gland, resembling Sjögren syndrome. [24]

    Owing to these structural and functional changes in the lacrimal and salivary glands, their aqueous output is diminished. In the eye, tear hyperosmolarity results and is itself a proinflammatory stimulus, resulting in an inflammatory cascade on the ocular surface, [25] with evidence of immune activation of the conjunctival epithelium and local cytokine and metalloproteinase production. Damage to the corneal epithelium, already vulnerable due to inadequate tear film protection, ensues, with resultant epithelial erosions and surface irregularity.

    Extraglandular involvement

    Extraglandular involvement in Sjögren syndrome manifests in part as hypergammaglobulinemia and the production of multiple autoantibodies, especially ANA and RF. This may be due to polyclonal B-cell activation, but the cause of this expanded activation is not known.

    Involvement of other organs and tissues may result from effects of these antibodies, immune complexes, or lymphocytic infiltration and occurs in one third of patients with Sjögren syndrome. Prolonged hyperstimulation of B cells may lead to disturbances in their differentiation and maturation and may account for the greatly increased incidence of lymphoma in these patients. [26]

    Sex hormones

    Sex hormones may influence the immunologic manifestations of primary Sjögren syndrome, because the disease is much more common in women than in men. The prevalence of serologic markers tends to be lower in male patients than in female patients. Although the role of sex hormones (eg, estrogens, androgens) in the pathogenesis of primary Sjögren syndrome remains unknown, adrenal and gonadal steroid hormone deficiency probably affects immune function.


    Sjögren syndrome carries a generally good prognosis. In patients who develop a disorder associated with Sjögren syndrome, the prognosis is more closely related to the associated disorder (eg, SLE, lymphoma).

    Although salivary and lacrimal function generally stabilize, the presence of SSA and/or hypocomplementemia may predict a decline in function.[28]

    Morbidity and mortality

    Morbidity associated with Sjögren syndrome is mainly associated with the gradually decreased function of exocrine organs, which become infiltrated with lymphocytes. The increased mortality rate associated with the condition is primarily related to disorders commonly associated with Sjögren syndrome, such as SLE, RA, and primary biliary cirrhosis. Patients with primary Sjögren syndrome who do not develop a lymphoproliferative disorder have a normal life expectancy. [29]


    Among patients with Sjögren syndrome, the incidence of non-Hodgkin lymphoma is 4.3% (18.9 times higher than in the general population), with a median age at diagnosis of 58 years. The mean time to the development of non-Hodgkin lymphoma after the onset of Sjögren syndrome is 7.5 years.

    The most common histologic subtype of non-Hodgkin lymphoma in Sjögren syndrome is mucosa-associated lymphoid tissue (MALT) lymphoma, which can develop in any nonlymphoid tissue infiltrated by periepithelial lymphoid tissue—most commonly the salivary glands, but also the stomach, nasopharynx, skin, liver, kidneys, and lungs. The progression of these infiltrates to lymphoma occurs slowly and in a stepwise fashion. Lymphoma is present at more than 1 site in 20% of patients at initial diagnosis.

    The results of one study suggest that diagnostic labial salivary gland tissue biopsy can be used to detect germinal center ̶ like lesions, which can be a highly predictive and easily obtained marker for non-Hodgkin lymphoma in primary Sjögren syndrome patients. [30]

    Patients at an increased risk of lymphoma include those with regional or generalized lymphadenopathy, hepatosplenomegaly, palpable purpura, leukopenia, renal insufficiency, loss of a previously positive polyclonal gammopathy or RF, development of a monoclonal gammopathy, or the development of a monoclonal cryoglobulinemia.

    Neonatal lupus and congenital heart block

    Children born to mothers with antibodies against SSA/Ro are at an increased risk of neonatal lupus and congenital heart block. If one such child develops congenital heart block, the risk for congenital heart block during a subsequent pregnancy is 15%.

    Antiphospholipid syndrome

    Patients with Sjögren syndrome who have antiphospholipid antibodies can develop the clinical features of antiphospholipid syndrome, which include increased fetal wastage and vascular thromboses.

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  2. Dr.Scorpiowoman

    Dr.Scorpiowoman Golden Member

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    Approach Considerations

    No curative agents for Sjögren syndrome exist. The treatment of the disorder is essentially symptomatic. [71]

    In secondary Sjögren syndrome, treatment is based on the accompanying disease and its clinical features. Sjögren syndrome and associated SLE improve more than primary Sjögren syndrome. In Sjögren syndrome associated with polymyositis, monthly cyclophosphamide pulse therapy has been used successfully.

    In annular erythema associated with Sjögren syndrome in Japanese patients, prednisolone (10-20 mg/d) is effective. No evidence-based strategies are available for the management of anular erythema in Western populations because it is rare. However, case reports have demonstrated that hydroxycloroquine can be effective in whites. [72]

    The inhibition of protease activity in EBV-mediated apoptotic cells may be a potential therapeutic approach in the treatment of Sjögren syndrome.

    Skin and vaginal dryness

    Patients should use skin creams, such as Eucerin, or skin lotions, such as Lubriderm, to help with dry skin. Vaginal lubricants, such as Replens, can be used for vaginal dryness. Vaginal estrogen creams can be considered in postmenopausal women. Watch for and treat vaginal yeast infections.

    Arthralgias and arthritis

    Acetaminophen or nonsteroidal anti-inflammatory drugs (NSAIDs) can be taken for arthralgias. Consider hydroxychloroquine if NSAIDs are not sufficient for the synovitis occasionally associated with primary Sjögren syndrome. However, hydroxychloroquine does not relieve sicca symptoms. Patients with RA associated with Sjögren syndrome likely require other disease-modifying agents.

    Additional treatment considerations

    In patients with major organ involvement, such as lymphocytic interstitial lung disease, consider therapy with steroids and immunosuppressive agents, such as cyclophosphamide.

    While cyclophosphamide and similar agents may be helpful for treating serious manifestations of Sjögren syndrome or disorders associated with Sjögren syndrome, clinicians should understand that these agents are also associated with the development of lymphomas.

    Long-term anticoagulation may be needed in patients with vascular thrombosis related to antiphospholipid antibody syndrome.

    In a small group of patients with primary Sjögren syndrome, mycophenolate sodium reduced subjective, but not objective, ocular dryness and significantly reduced hypergammaglobulinemia and RF. [73]

    Among the biologic therapies, the greatest experience in primary Sjögren syndrome is with rituximab, an anti-CD20 (which is expressed on B-cell precursors) monoclonal antibody. Anti-B–cell strategies, particularly rituximab, have a promising effect in the treatment of patients with severe extraglandular manifestations of Sjögren syndrome.

    Reports on the use of rituximab in patients with primary Sjögren syndrome have emerged in the literature. [74, 75, 76] In a double-blind, randomized, placebo-controlled trial, Meijer et al found that rituximab significantly improved saliva flow rate, lacrimal gland function, and other variables in patients with primary Sjögren syndrome. [77]

    In an open-label clinical trial, modest improvements were noted in patient-reported symptoms of fatigue and oral dryness. However, no significant improvement in the objective measures of lacrimal and salivary gland function was noted, despite effective depletion of blood B cells. [78] In a randomized, placebo-controlled, parallel-group study of 120 patients with primary Sjögren syndrome, treatment with rituximab did not alleviate disease activity or symptoms at week 24, although it did alleviate some symptoms at weeks 6 and 16. [79]

    Rituximab appears promising in the treatment of vasculitis and intravenous immunoglobulin (IVIG)–dependent ataxic neuropathy. [80, 81]Results from the AIR registry (French) indicated that rituximab appears to be effective in cryoglobulinemia or vasculitis-related peripheral nervous system involvement in primary Sjögren syndrome. [82]

    In a prospective study of 78 patients with primary Sjögren syndrome treated with rituximab, significant improvement in extraglandular manifestations was reported, as measured by EULAR [European League Against Rheumatism] Sjögren Syndrome Disease Activity Index (ESSDAI) (disease activity score) and overall good tolerance reported. [83] Several smaller studies of rituximab revealed improvement of arthralgias, regression of parotid gland swelling, [84] and improvement of immune-related thrombocytopenia. [85]

    Of the TNF inhibitors, both etanercept and infliximab have failed to demonstrate significant benefit in Sjögren syndrome.

    Fewer data are available with regard to the role of anti-CD22, anti-BAFF, anti-IL-1, type 1 interferon, and anti-T–cell agents in treatment of primary Sjögren syndrome, with further investigations ongoing. The overall paucity of evidence in therapeutic studies in primary Sjögren syndrome suggests that much larger trials of the most promising therapies are necessary.

    Emergency department care

    The diagnosis of Sjögren syndrome can be made from the ED if the index of suspicion is high. Patients may present with mild symptoms (eg, eye grittiness, eye dryness or discomfort, dry mouth, recurrent caries). Bilateral parotid gland swelling is also a common presentation.

    Patients with known Sjögren syndrome should not be taken lightly for their complaint of dry eyes or dry mouth, as these chronic problems can be very distressing and obtrusive.

    Inpatient care

    Give attention to artificial lubricants and humidified oxygen for intubated and/or sedated patients with Sjögren syndrome.

    Outpatient care

    Encourage patients with Sjögren syndrome to be active. In addition, patients should be encouraged to avoid exacerbation of dryness symptoms (eg, through smoking or exposure to low-humidity environments). All patients with Sjögren syndrome should be monitored by an ophthalmologist and dentist, in addition to their rheumatologist. Certain patients may be candidates for punctal occlusion, which is usually performed by an ophthalmologist.


    Most patients with Sjögren syndrome can be monitored at follow-up visits every 3 months and, if the patient is stable, up to every 6 months. Patients with active problems or in whom an emerging associated illness is a concern can be seen as often as monthly.

    Surgical Therapy

    Occlusion of the lacrimal puncta can be corrected surgically. Electrocautery and other techniques can be used for permanent punctal occlusion.

    During surgery, the anesthesiologist should administer as little anticholinergic medication as possible and use humidified oxygen to help avoid inspissation of pulmonary secretions. Good postoperative respiratory therapy should also be provided. Patients are at higher risk for corneal abrasions, so ocular lubricants should be considered.

    Biopsies that may be performed in association with Sjögren syndrome include the following:

    • Minor salivary gland biopsy - For diagnostic purposes
    • Parotid gland biopsy - If malignancy is suggested
    • Biopsy on an enlarged lymph node - To help rule out pseudolymphoma or lymphoma


    Sjögren syndrome and its associated disorders necessitate a total patient perspective that is often best provided by an internist. A rheumatologist with specific training and experience in Sjögren syndrome and its associated disorders is also essential to the management of the condition. In addition, good oral prophylaxis and therapy are necessary.

    Involve ophthalmologists early in the care of patients, for rose bengal and fluorescein staining to help to establish the diagnosis and for assessment of the degree of eye damage.

    Consultation with an otolaryngologist may be needed early to perform a minor or major salivary gland biopsy if this is deemed necessary to establish a diagnosis. The specialist may also need to perform a parotid biopsy if malignant transformation is suggested.

    Depending on the problems, patients with Sjögren syndrome may need to be seen by other specialists, including the following:

    • Nephrologist - To help manage renal tubular acidosis
    • Pulmonologist - To help manage interstitial lung disease
    • Hematologist/oncologist - If pseudolymphoma or lymphoma develops

    Dry Eyes

    The treatment of dry eyes depends on the severity of the dryness, which is best determined by an ophthalmologist and is graded according to the degree of symptoms, conjunctival injection and staining, corneal damage, tear quality, and lid involvement. [86, 87, 88, 89, 90, 91]

    New therapeutic strategies designed to facilitate AQP5 trafficking to the apical plasma membrane may prove useful in the management of dry eyes in Sjögren syndrome. In addition, data on novel secretagogues and androgen therapies for dry eyes are promising. [80]

    A consensus clinical guideline for the management of dry eyes associated with Sjögren syndrome recommends the following [92] :

    • Patient evaluation should include symptoms of both discomfort and visual disturbance along with evaluation of the relative contribution of aqueous production deficiency and evaporative loss of tear volume
    • Objective parameters of tear film stability, tear osmolarity, degree of lid margin disease, and ocular surface damage should be used to stage disease severity and assist in guiding treatment
    • Successful management requires patient education on the nature of the problem, aggravating factors, and treatment goals

    Treatment options are used according to the severity and character of dry eye disease and include the following [92] :

    • Tear supplementation and stabilization
    • Control of inflammation of the lacrimal glands and ocular surface
    • Possible stimulation of tear production

    Level 1 - Mild symptoms, no corneal signs

    Artificial tears should be applied liberally. Patients may need to apply artificial tears more often if they enter a low-humidity environment (ie, air conditioning, airplanes). Artificial tears with hydroxymethylcellulose or dextran are more viscous and can last longer before reapplication is needed. Encourage patients to try various preparations to determine what works best for them.

    If artificial tears burn when they are instilled, the preservative in the artificial tears is likely irritating the eye. If artificial tears are used more often than every 4 hours, patients should use a preservative-free preparation to avoid eye irritation from the preservatives.

    The use of humidifiers may help. If the patient is living in an area with hard water, he or she should use distilled water. Patients should avoid medications with anticholinergic and antihistamine effects.

    Level 2 - Moderate or severe symptoms with tear film signs or visual signs, or mild corneal/conjunctival staining

    Patients should use unpreserved tears or gels or nighttime ointments. Patients who wake up in the morning with severe matting in the eyes should use a more viscous preparation (eg, Lacri-Lube) at night. While the more viscous preparations can be applied less often, they can make patients' vision filmy. Therefore, they are best used at night. The more viscous preparations occasionally lead to blepharitis, which can exacerbate sicca symptoms.

    The following agents may also be indicated:

    • Topical steroids
    • Secretagogues
    • Cyclosporine A [93]
    • Nutritional supplements

    Level 3 - Severe symptoms with marked corneal changes or filamentary keratitis

    The following treatments may be indicated:

    • Tetracyclines
    • Autologous serum tears
    • Temporary plugging of the lacrimal puncta to increase the amount of tears that remain in the eyes

    Level 4 - Extremely severe symptoms with altered lifestyle, or severe corneal staining, erosions, or conjunctival scarring

    The following therapies may be indicated:

    • Systemic anti-inflammatory therapy, including acetylcysteine
    • Topical vitamin A
    • Electrocautery and other techniques for permanent punctal occlusion
    • Glasses fitted with moisture shields to decrease evaporation

    Dry Mouth

    Patients with dry mouth can liberally drink sips of water and take bottled water with them on trips. They can also place a glass of water at their bedside for nighttime use, as needed. [94] Sugar-free lemon drops can also be used as needed to stimulate salivary secretion. Artificial saliva can be used as needed, although patient tolerance varies. Preparations include Salivart, Saliment, Saliva Substitute, MouthKote, and Xero-Lube. Patients should avoid medications with anticholinergic and antihistamine effects.

    The use of humidifiers may help. Distilled water is best in patients living in an area with hard water.

    Patients should be seen regularly by a dentist, who may advise fluoride treatments. Toothpaste without detergents can reduce mouth irritation in patients with Sjögren syndrome. Brands include Biotene toothpaste, Biotene mouth rinse, Dental Care toothpaste, and Oral Balance gel.

    Watch for oral candidiasis and angular cheilitis and treat them with topical antifungal agents, such as nystatin troches. Oral fluconazole may occasionally be needed. Patients also need to be sure to disinfect their dentures.

    Sinusitis and sinus blockade should be treated because these problems may contribute to mouth breathing. Emphasize the use of isotonic sodium chloride solution nasal sprays to avoid antihistamine use.

    Pilocarpine or cevimeline tablets are options. Some small studies suggest that interferon alfa may be a useful therapy in the future.


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