Sjögren’s syndrome(S.S) is a systemic, chronic, autoimmune, inflammatory disorder. Characterized by lymphocytic infiltrates in exocrine organs. Most individuals with Sjögren syndrome present with sicca symptoms.
In addition, numerous extraglandular features may develop.
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 (SLE), rheumatoid arthritis (RA), or scleroderma. primary and secondary types occur with similar frequency.
- The etiology of Sjögren syndrome is not well understood.
- Autoimmune disease
- ongoing interaction between the innate and acquired immune systems.
- There is presence of activated salivary gland epithelial cells expressing major histocompatibility complex (MHC) class II molecules.
- Association with the human leukocyte antigen(HLA)
- Some evidence indicates that the true association of Sjögren syndrome may be with HLA-DQA1.
- According to Israeli research ,HLA-DR5 is associated with S.S in Israeli persons.
- Possible disease triggers
- Viruses are viable candidates as environmental triggers, uncertain.
- Epstein-Barr virus (EBV), HTLV-1, human herpesvirus 6 (HHV-6), HIV, hepatitis C virus (HCV), and cytomegalovirus (CMV) may have a role.
New studies (Jan-2003) : 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 .
- IN USA:
- Second most common rheumatologic disorder, behind SLE.
- Sjögren syndrome affects 0.1-4% of the population.
- Between different ethnic groups , the disease occurs worldwide with similar prevalence.
- The female-to-male ratio of Sjögren’s syndrome is 9:1.
-The Journal of Clinical Endocrinology & Metabolism Vol. 94, No. 6 2044-2051(2009)- Dehydroepiandrosterone (DHEA) Role.
-2011 -Charles E. Schmidt College of Medicine, Florida- Atlantic University.
- Sjögren syndrome can affect individuals of any age but is most common in elderly people. Onset typically occurs in the fourth to fifth decade of life.
- Sjögren syndrome carries a generally good prognosis.
- Morbidity associated with Sjögren syndrome is mainly associated with the gradually decreased function of exocrine organs.
- Patients with primary Sjögren syndrome have a normal life expectancy.
- Children born to mothers with antibodies against SSA/Ro are at an increased risk of neonatal lupus and congenital heart block.
The first symptoms in primary Sjögren syndrome can be easily overlooked and overlapped.
1- Xerophthalmia (dry eyes) and xerostomia (dry mouth) are the main clinical presentations in adults. Bilateral parotid swelling is the most common sign of onset in children.
Extraglandular involvement in Sjögren syndrome falls into 2 general categories:
- Periepithelial infiltrative processes include interstitial nephritis, liver involvement, and bronchiolitis and generally follow a benign course.
- Extraepithelial extraglandular involvement in Sjögren syndrome is related to B-cell hyperreactivity, hypergammaglobulinemia, and immune complex formation and includes palpable purpura, glomerulonephritis, and peripheral neuropathy .
- Sicca symptoms (dry eyes and dry mouth and dry vagina).
- Cutaneous symptoms
- Pulmonary symptoms
- (xerotrachea),which can manifest as a dry cough.
- Gastrointestinal symptoms.
- Cardiac symptoms.
- Renal disorders.
- A history of recurrent miscarriages or stillbirths
- Neurologic symptoms.(see neurology corner).
- Diagnosis of primary Sjögren syndrome requires 4 out of 6 of the below criteria:
- Ocular symptoms – Dry eyes for more than 3 months, foreign-body sensation, use of tear substitutes more than 3 times daily.
- Oral symptoms – Feeling of dry mouth, recurrently swollen salivary glands, frequent use of liquids to aid swallowing.
- Ocular signs – Schirmer’s test performed (< 5 mm in 5 min).
- Oral signs – Abnormal salivary scintigraphy findings, abnormal parotid sialography findings, abnormal sialometry findings (unstimulated salivary flow < 1.5mL in 15min) .
- Positive minor salivary gland biopsy findings
- Positive anti–SSA or anti–SSB antibody results
- No curative agents for Sjögren syndrome exist.
- The treatment of the disorder is essentially symptomatic.
- In secondary Sjögren syndrome, treatment is based on the accompanying disease and its clinical features.
- Skin and vaginal dryness
- Patients should use skin creams, such as Eucerin, or skin lotions, such as Lubriderm.
- Arthralgias and arthritis
- Acetaminophen or nonsteroidal anti-inflammatory drugs (NSAIDs).
- Dry Eyes:
- Artificial tears should be applied liberally.
- Topical steroids.
- Nutritional supplements.
- Dry mouth:
- Patients with dry mouth can liberally drink sips of water and take bottled water with them on trips.
- 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.
- Surgical Therapy
- Occlusion of the lacrimal puncta can be corrected surgically. Electrocautery and other techniques can be used for permanent punctal occlusion.