Xerostomia-keratoconjunctivitis sicca syndrome

Summary about Disease


Xerostomia-keratoconjunctivitis sicca syndrome, more commonly known as Sjögren's syndrome, is a chronic autoimmune disorder where the body's immune system mistakenly attacks its own moisture-producing glands. This primarily affects the lacrimal (tear) glands and salivary glands, leading to dry eyes (keratoconjunctivitis sicca) and dry mouth (xerostomia). Sjögren's syndrome can occur alone (primary Sjögren's) or in association with other autoimmune disorders (secondary Sjögren's), such as rheumatoid arthritis or lupus.

Symptoms


Dry Eyes: Gritty sensation, burning, itching, redness, blurred vision, light sensitivity.

Dry Mouth: Difficulty swallowing, difficulty speaking for extended periods, altered taste, increased dental cavities, mouth sores, dry or cracked lips.

Other Possible Symptoms: Fatigue, joint pain, muscle pain, dry skin, vaginal dryness, cough, difficulty concentrating, enlarged salivary glands, Raynaud's phenomenon (fingers and toes turning white or blue in response to cold or stress).

Causes


Sjögren's syndrome is an autoimmune disorder, meaning the body's immune system attacks its own tissues. The exact cause is unknown, but it is believed to be a combination of genetic predisposition and environmental factors, such as viral infections.

Medicine Used


There is no cure for Sjögren's syndrome, so treatment focuses on managing symptoms and preventing complications. Common medications include:

Artificial Tears: For dry eyes.

Saliva Substitutes: For dry mouth.

Prescription Eye Drops: Such as cyclosporine (Restasis) or lifitegrast (Xiidra) to increase tear production.

Cholinergic Agonists: Such as pilocarpine (Salagen) or cevimeline (Evoxac) to stimulate saliva production.

Nonsteroidal Anti-Inflammatory Drugs (NSAIDs): For joint pain and muscle pain.

Disease-Modifying Antirheumatic Drugs (DMARDs): Such as hydroxychloroquine (Plaquenil) for more systemic symptoms.

Immunosuppressants: In severe cases, medications like methotrexate, azathioprine, or biologics may be used to suppress the immune system.

Corticosteroids: For short term relief of inflammation.

Is Communicable


No, Sjögren's syndrome is not communicable. It is an autoimmune disorder, not an infectious disease.

Precautions


Eye Care: Use artificial tears frequently, avoid dry environments, use humidifiers, and consider punctal plugs (small devices inserted into tear ducts to block drainage).

Oral Care: Drink plenty of water, chew sugar-free gum or candies to stimulate saliva, practice good oral hygiene, and see a dentist regularly.

Skin Care: Use moisturizers to prevent dry skin.

Avoid Irritants: Avoid smoking, alcohol, and caffeine, as these can worsen dry mouth.

Manage Stress: Stress can exacerbate symptoms, so practice relaxation techniques.

How long does an outbreak last?


Sjögren's syndrome is a chronic condition, not an "outbreak." Symptoms can fluctuate in severity, with periods of increased symptoms (flares) followed by periods of relative remission. The duration of these flares varies from person to person and can last from days to weeks or longer.

How is it diagnosed?


Diagnosis of Sjögren's syndrome typically involves a combination of:

Medical History and Physical Exam: Review of symptoms and overall health.

Eye Exams: Schirmer's test (measures tear production), slit-lamp examination (examines the surface of the eye).

Salivary Gland Tests: Salivary flow rate measurement, salivary gland biopsy.

Blood Tests: To detect antibodies associated with Sjögren's syndrome (e.g., anti-Ro/SSA, anti-La/SSB), and to rule out other conditions.

Imaging: Sialography or salivary gland scintigraphy may be used to visualize the salivary glands.

Timeline of Symptoms


The onset of Sjögren's symptoms can be gradual and insidious. Some people may experience symptoms for years before being diagnosed.

Early Stages: Mild dry eyes and dry mouth, often attributed to other causes (e.g., allergies, medications).

Progression: Symptoms become more persistent and bothersome, leading to significant discomfort and affecting daily activities. Other symptoms, such as fatigue and joint pain, may develop.

Advanced Stages: Complications such as corneal damage, dental problems, and systemic involvement (e.g., kidney problems, lung problems) may occur. The timeline varies greatly from person to person. Some may have mild symptoms that remain stable for years, while others experience a more rapid progression.

Important Considerations


Sjögren's syndrome can affect individuals differently.

Early diagnosis and treatment are important to prevent complications.

Regular follow-up with a rheumatologist and other specialists (e.g., ophthalmologist, dentist) is essential.

People with Sjögren's syndrome have a slightly increased risk of developing lymphoma.

Pregnancy requires careful monitoring due to the risk of neonatal lupus in the baby.

Support groups and educational resources can provide valuable information and emotional support.