Overview

Sjögren’s syndrome is a chronic autoimmune disease characterized by the body’s immune system mistakenly attacking its own moisture-producing glands. This leads to two hallmark symptoms: dry eyes (keratoconjunctivitis sicca) and dry mouth (xerostomia). Beyond these, Sjögren’s syndrome can also affect other organs, including the joints, skin, kidneys, lungs, liver, and nerves, making it a systemic condition. It predominantly affects middle-aged women, although it can occur in both genders and all age groups. Sjögren’s syndrome is classified as either primary (occurring alone) or secondary (occurring alongside another autoimmune disease, such as rheumatoid arthritis or lupus).

Causes

The exact cause of Sjögren’s syndrome remains unknown, but it is believed to result from a combination of genetic, environmental, and hormonal factors:

• Genetic predisposition: People with certain genes (such as HLA-DR and HLA-DQ types) are at higher risk of developing Sjögren’s syndrome.
• Autoimmune response: The immune system becomes overactive and targets the body’s own glands and tissues, causing inflammation and damage.
• Viral triggers: Infections such as Epstein-Barr virus (EBV), cytomegalovirus (CMV), or hepatitis C may trigger the autoimmune response in genetically susceptible individuals.
• Hormonal factors: The condition is more common in women, suggesting a hormonal link, particularly related to estrogen levels.
• Secondary association: Sjögren’s syndrome frequently occurs in conjunction with other autoimmune diseases like rheumatoid arthritis, systemic lupus erythematosus (SLE), and scleroderma.

Symptoms

The symptoms of Sjögren’s syndrome vary in severity and can affect multiple body systems. The most common and classic symptoms include:

• Dry eyes: Gritty, burning, or itchy eyes, with a sensation of sand in the eyes and sensitivity to light.
• Dry mouth: Difficulty swallowing, speaking, or tasting, along with an increased risk of dental cavities, oral infections, and mouth sores.

Other systemic symptoms may include:

• Joint pain and stiffness: Similar to arthritis, causing discomfort and reduced mobility.
• Swollen salivary glands: Particularly the parotid glands, located in front of the ears.
• Skin dryness and rashes: Including increased sensitivity to sunlight.
• Vaginal dryness: Leading to discomfort or pain during intercourse.
• Fatigue: Persistent, profound tiredness affecting daily functioning.
• Neurological symptoms: Numbness, tingling, or peripheral neuropathy in some cases.
• Organ involvement: Rarely, Sjögren’s can cause complications in the kidneys, lungs, or liver.

Diagnosis

Diagnosing Sjögren’s syndrome can be challenging due to the wide range of symptoms and its overlap with other conditions. A combination of clinical evaluation, laboratory tests, and specialized exams are used:

• Medical history and physical examination: A thorough evaluation of dryness symptoms, joint pain, and other systemic complaints.
• Blood tests: Detecting autoantibodies such as anti-SSA (Ro), anti-SSB (La), rheumatoid factor (RF), and antinuclear antibodies (ANA).
• Schirmer’s test: Measures tear production by placing small strips of paper under the lower eyelids.
• Salivary flow test: Quantifies saliva production over a specific period.
• Salivary gland biopsy: A minor lip biopsy to check for lymphocytic infiltration, a hallmark of Sjögren’s syndrome.
• Imaging studies: Ultrasound or sialography of salivary glands to assess gland structure and function.

Treatment

There is no cure for Sjögren’s syndrome, but treatments aim to relieve symptoms, manage complications, and improve quality of life. Treatment options include:

• Artificial tears and eye drops: To relieve dry eyes and prevent corneal damage.
• Saliva substitutes: Mouth sprays, lozenges, or chewing sugar-free gum to alleviate dry mouth.
• Good oral hygiene: Regular dental care and fluoride treatments to prevent dental complications.
• Medications to stimulate saliva and tear production: Drugs like pilocarpine or cevimeline may be prescribed.
• Anti-inflammatory medications: NSAIDs to relieve joint pain and swelling.
• Immunosuppressive therapy: In cases with systemic involvement, drugs like hydroxychloroquine, methotrexate, or biologics may be used.
• Moisturizing products: For skin and vaginal dryness, including lotions and lubricants.
• Lifestyle adjustments: Avoiding smoking, alcohol, and caffeine, all of which can worsen dryness symptoms.

Prognosis

The prognosis for Sjögren’s syndrome varies depending on the severity of symptoms and the degree of organ involvement. Many people experience mild symptoms that are manageable with treatment, and life expectancy is generally normal. However, some individuals may develop more serious complications, such as severe joint pain, chronic fatigue, or organ damage. There is also a slightly increased risk of developing non-Hodgkin’s lymphoma in people with Sjögren’s syndrome. With early diagnosis, regular monitoring, and a personalized treatment plan, most patients can lead active and fulfilling lives, although ongoing care is often required to manage symptoms and prevent complications.