Published: 18 Jun 2025

ICD9: 710.2      ICD10: M35.00      ICD11: 4A43.2

Sjogren's Syndrome (often referred to as Sicca Syndrome when focusing on its hallmark dryness symptoms) is a chronic autoimmune disorder that primarily affects the moisture-producing glands of the body, specifically the lacrimal glands (tear glands) and salivary glands.
This leads to the hallmark symptoms of dry eyes and dry mouth.

Here's a breakdown of key aspects:

Key Features:

Autoimmune Disorder: The immune system mistakenly attacks the body's own tissues, specifically the moisture-producing glands.
Chronic Inflammation: The inflammation in these glands causes damage and reduces their ability to produce tears and saliva.
Primary Symptoms (Sicca Symptoms):
Dry Eyes (Keratoconjunctivitis Sicca): Feeling of grittiness, burning, itching, sensitivity to light, blurred vision, and excessive tearing (a paradoxical response to dryness).
Dry Mouth (Xerostomia): Difficulty swallowing, speaking, taste changes, increased risk of dental caries, and a feeling of cottonmouth.
Systemic Involvement: While dry eyes and dry mouth are the defining features, Sjogren's can affect other parts of the body, leading to a wide range of symptoms, including:
Fatigue: A profound and persistent feeling of tiredness.
Joint Pain (Arthralgia) and Arthritis: Pain, stiffness, and swelling in the joints.
Skin Rashes: Dry, itchy skin.
Vaginal Dryness: In women.
Neuropathy: Nerve damage, causing numbness, tingling, or pain.
Kidney Problems: Less common, but can affect kidney function.
Lung Problems: Cough, shortness of breath.
Vasculitis: Inflammation of blood vessels.

Types of Sjogren's Syndrome:

Primary Sjogren's Syndrome: Occurs on its own, without the presence of another autoimmune disorder.
Secondary Sjogren's Syndrome: Occurs in conjunction with another autoimmune disease, such as rheumatoid arthritis, lupus, or scleroderma.

Causes:

The exact cause of Sjogren's Syndrome is not fully understood, but it is believed to involve a combination of genetic predisposition and environmental factors, such as viral infections.

Diagnosis:

Diagnosis can be challenging because symptoms can mimic other conditions. It typically involves:

Medical History and Physical Exam: The doctor will ask about symptoms and conduct a physical examination.
Eye Exams: Schirmer's test (measures tear production) and slit-lamp examination.
Salivary Gland Function Tests: Salivary flow rate measurement, salivary gland biopsy.
Blood Tests: To check for specific antibodies (e.g., anti-Ro/SSA, anti-La/SSB), inflammatory markers, and other abnormalities.
Lip Biopsy: A biopsy of the minor salivary glands in the lip can help confirm the diagnosis.

Treatment:

There is no cure for Sjogren's Syndrome, so treatment focuses on managing symptoms and preventing complications. Treatment options include:

Dry Eyes: Artificial tears, lubricating eye ointments, prescription eye drops (e.g., cyclosporine, lifitegrast), punctal plugs (to block tear drainage).
Dry Mouth: Frequent sips of water, sugar-free gum or candy to stimulate saliva production, saliva substitutes, prescription medications to increase saliva production (e.g., pilocarpine, cevimeline), good oral hygiene.
Joint Pain: Pain relievers, anti-inflammatory medications, physical therapy.
Systemic Symptoms: Immunosuppressant medications (e.g., hydroxychloroquine, methotrexate) may be used to manage systemic inflammation and symptoms.
Lifestyle Modifications: Avoiding dry environments, staying hydrated, quitting smoking, and practicing good oral hygiene.

Complications:

Dental Problems: Increased risk of cavities, gum disease, and tooth loss due to dry mouth.
Eye Damage: Corneal ulcers, infections, and vision problems due to chronic dry eyes.
Lymphoma: Slightly increased risk of developing lymphoma (a type of cancer of the lymphatic system).
Other Autoimmune Diseases: Individuals with Sjogren's may be at increased risk of developing other autoimmune disorders.

In summary, Sjogren's Syndrome is a chronic autoimmune disorder characterized by dry eyes and dry mouth, but it can also affect other parts of the body. While there is no cure, treatment can help manage symptoms and improve quality of life.