Sjögren’s Syndrome Complete Guide
Understanding Sjögren’s Syndrome
Introduction to Sjögren’s Syndrome
Sjögren’s syndrome is a chronic autoimmune disorder characterized by lymphocytic infiltration of exocrine glands, leading to the classic symptoms of dry eyes (keratoconjunctivitis sicca) and dry mouth (xerostomia). The condition can occur as primary Sjögren’s syndrome or in association with other autoimmune diseases, most commonly rheumatoid arthritis and systemic lupus erythematosus.
The syndrome is named after Henrik Sjögren, a Swedish ophthalmologist who first described the condition in 1933. Since then, our understanding of Sjögren’s has expanded significantly, revealing it to be a systemic disease with diverse manifestations beyond glandular involvement.
Sjögren’s syndrome primarily affects women, with a female-to-male ratio of approximately 9:1. The condition typically presents in middle age, though it can occur at any age, including in children. The prevalence is estimated at 0.1-4% of the population, making it one of the more common autoimmune conditions.
In Dubai and the UAE, Sjögren’s syndrome is increasingly recognized and managed by rheumatologists. Awareness of the condition’s varied presentations helps ensure timely diagnosis and appropriate treatment.
The Immunology of Sjögren’s
Sjögren’s syndrome involves autoimmune attack on lacrimal and salivary glands, leading to decreased tear and saliva production.
Lymphocytic infiltration is the histological hallmark, with T cells and B cells forming focal aggregates in glandular tissue. Over time, this infiltration leads to glandular destruction and fibrosis.
Autoantibodies are characteristic of Sjögren’s syndrome. Anti-SSA (Ro) and anti-SSB (La) antibodies are present in the majority of patients and are included in diagnostic criteria. Rheumatoid factor is also commonly present.
Interferon signature, with elevated type I interferons, is characteristic of Sjögren’s syndrome and may contribute to disease pathogenesis.
B cell hyperactivity is a feature of Sjögren’s, with increased risk of B cell lymphoma, particularly in patients with severe disease and certain prognostic factors.
The systemic nature of Sjögren’s reflects immune-mediated inflammation in multiple organs. Fatigue, musculoskeletal pain, and neuropathy are common systemic manifestations.
Clinical Manifestations
Glandular Symptoms
The hallmark symptoms of Sjögren’s result from exocrine gland dysfunction.
Dry eyes (keratoconjunctivitis sicca) causes a sandy or gritty sensation, redness, burning, and light sensitivity. Untreated, severe dryness can damage the cornea.
Dry mouth (xerostomia) causes difficulty swallowing dry foods, altered taste, increased dental caries, oral ulcers, and oral infections including candidiasis.
Other exocrine involvement may include dry skin, vaginal dryness, and reduced sweat production.
Systemic Manifestations
Sjögren’s is a systemic disease with diverse manifestations.
Fatigue is extremely common and often severe, frequently reported as one of the most disabling symptoms.
Musculoskeletal pain, including arthralgia and myalgia, is common and may be mistaken for rheumatoid arthritis.
Peripheral neuropathy occurs in some patients, causing numbness, tingling, or pain in the hands and feet.
Lung involvement includes interstitial lung disease and bronchial dryness causing cough.
Kidney involvement is less common but includes interstitial nephritis and renal tubular acidosis.
Cutaneous manifestations include vasculitis, which may present as purpura or skin ulcers.
Complications
Complications of Sjögren’s include dental decay and oral infections from reduced saliva.
Corneal damage and vision problems from severe dry eyes.
Increased risk of B cell lymphoma, particularly in patients with severe disease, persistent salivary gland enlargement, and low complement levels.
Systemic vasculitis affecting multiple organs.
Diagnosis and Treatment
Diagnostic Evaluation
Diagnosis of Sjögren’s involves a combination of clinical assessment, laboratory testing, and objective measures of glandular function.
Schirmer’s test measures tear production using filter paper placed under the lower eyelid.
Salivary flow testing measures saliva production over a timed period.
Salivary gland imaging, including ultrasound, MRI, and sialography, can demonstrate characteristic changes.
Minor salivary gland biopsy, typically from the lip, shows focal lymphocytic sialadenitis and is highly specific for diagnosis.
Blood tests for anti-SSA (Ro) and anti-SSB (La) antibodies are essential for diagnosis. ANA and rheumatoid factor are also commonly positive.
Treatment
Symptomatic treatment of dryness is the cornerstone of management.
Artificial tears and lubricating eye drops manage dry eyes. Punctal plugs may be inserted to reduce tear drainage.
Saliva substitutes, sugar-free candies, and good oral hygiene manage dry mouth. Pilocarpine and cevimeline stimulate saliva production.
Hydroxychloroquine may help systemic symptoms including fatigue, musculoskeletal pain, and rash.
Biologics targeting B cells (rituximab) or BLyS (belimumab) may be used for severe systemic disease.
Immunosuppressive medications may be needed for organ-threatening disease.
Medical Disclaimer
This guide is provided for educational purposes only and is not intended as a substitute for professional medical advice, diagnosis, or treatment. Always seek the advice of your physician or other qualified health provider with any questions you may have regarding a medical condition.
Never disregard professional medical advice or delay in seeking it because of something you have read in this guide.
The services and programs mentioned in this guide are provided by Healers Clinic in Dubai and the UAE.
Services at Healers Clinic
Healers Clinic in Dubai offers integrative approaches that complement conventional Sjögren’s management:
- Nutritional Consultation for dietary guidance
- IV Nutrition Therapy for addressing nutritional needs
- Immune System Reboot Program for comprehensive support
Schedule a consultation: Book Your Appointment
Frequently Asked Questions
1. What is Sjögren’s syndrome? An autoimmune condition causing dry eyes and dry mouth from immune attack on exocrine glands.
2. What causes Sjögren’s? Genetic predisposition combined with environmental triggers leading to autoimmune response.
3. How is Sjögren’s diagnosed? Combination of symptoms, blood tests for autoantibodies, and objective testing of tear/saliva production.
4. What are the symptoms? Dry eyes, dry mouth, fatigue, joint pain, and possible systemic organ involvement.
5. Is Sjögren’s curable? No, but symptoms can be managed effectively.
6. What treatments are available? Symptomatic treatments for dryness, hydroxychloroquine, and biologics for severe disease.
7. Does Sjögren’s affect life expectancy? Generally not, but increased lymphoma risk requires monitoring.
8. Can Sjögren’s affect other organs? Yes, lungs, kidneys, nerves, and skin can be involved.
9. Is Sjögren’s hereditary? Family history increases risk, but most cases are sporadic.
10. Can diet help Sjögren’s? Staying hydrated and avoiding drying foods may help symptoms.
11. What is the connection to lymphoma? Sjögren’s increases B cell lymphoma risk, particularly with severe disease.
12. Can men get Sjögren’s? Yes, though it is 9 times more common in women.
13. Does weather affect symptoms? Dry environments may worsen dry eyes and mouth.
14. Can Sjögren’s affect pregnancy? Women with anti-SSA antibodies have increased risk of neonatal lupus.
15. What is the outlook? Good with appropriate management and monitoring.
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