Sjögren’s syndrome is widely recognized for causing dry eyes and dry mouth, resulting from damage to the body's moisture-producing glands. However, this chronic autoimmune disease can go much further. In many cases, it extends beyond the salivary and lacrimal glands, impacting a variety of internal organs and systems. Understanding how Sjögren’s syndrome affects the body beyond dryness is essential for comprehensive care, early detection of complications, and better long-term health outcomes.

Overview of Sjögren’s Syndrome

Sjögren’s syndrome occurs when the immune system mistakenly attacks the body’s exocrine glands, especially those responsible for producing saliva and tears. There are two types:

  • Primary Sjögren’s syndrome: Occurs independently of other autoimmune conditions.

  • Secondary Sjögren’s syndrome: Occurs in conjunction with other autoimmune diseases, such as lupus or rheumatoid arthritis.

While dryness is the hallmark symptom, up to 60% of patients experience extraglandular manifestations, meaning the disease affects other tissues and organs.

The Immune System and Systemic Inflammation

In Sjögren’s, the immune system becomes chronically overactive. This results in systemic inflammation that can damage organs and interfere with normal body functions. The presence of autoantibodies (e.g., anti-SSA/Ro and anti-SSB/La) signals widespread immune activity, even in organs not directly involved in fluid production.

Organs Commonly Affected Beyond the Glands

1. Joints and Muscles

Joint pain and muscle aches are common in Sjögren’s. In some cases, inflammation causes symptoms similar to arthritis, including:

  • Joint stiffness and swelling

  • Limited range of motion

  • Morning stiffness

Although not usually associated with joint erosion like rheumatoid arthritis, the discomfort can significantly impact mobility and quality of life.

2. Lungs

Pulmonary involvement occurs in a significant number of patients. Sjögren’s can cause:

  • Interstitial lung disease (ILD): Inflammation and scarring of lung tissue, leading to shortness of breath and dry cough.

  • Bronchitis or bronchiolitis: Inflammation of the airways, sometimes causing wheezing.

  • Pleuritis: Inflammation of the lining around the lungs.

If untreated, lung complications can lead to long-term respiratory issues.

3. Kidneys

Renal involvement is often silent but potentially serious. Inflammation can affect kidney function and structure, resulting in:

  • Interstitial nephritis: Inflammation in the spaces between kidney tubules.

  • Renal tubular acidosis: Impaired acid balance regulation, leading to fatigue, muscle weakness, and bone problems.

Routine blood and urine tests help detect kidney problems early in people with Sjögren’s.

4. Nervous System

Neurological symptoms can be diverse and complex. Sjögren’s may affect both the central and peripheral nervous systems:

  • Peripheral neuropathy: Causes numbness, tingling, burning pain, or weakness in the limbs.

  • Cranial nerve involvement: May lead to facial numbness or hearing problems.

  • Cognitive dysfunction ("brain fog"): Includes memory issues and difficulty concentrating.

  • Myelitis: Rarely, inflammation of the spinal cord may occur.

These symptoms require careful evaluation by a neurologist to determine the best course of treatment.

5. Liver and Bile Ducts

Liver involvement is less common but possible. Patients may develop:

  • Autoimmune hepatitis

  • Primary biliary cholangitis (PBC): A chronic disease affecting the bile ducts.

Liver enzyme tests may be recommended as part of regular monitoring in Sjögren’s patients.

6. Gastrointestinal Tract

Sjögren’s can lead to gastrointestinal discomfort due to reduced digestive secretions and immune-related inflammation. Common issues include:

  • Acid reflux (GERD)

  • Difficulty swallowing

  • Bloating and abdominal discomfort

  • Pancreatic insufficiency (in rare cases)

Nutrient absorption may be impaired in severe cases, necessitating dietary adjustments or supplements.

7. Skin and Vasculature

Skin involvement can include:

  • Dry skin (xerosis)

  • Purpura: Purple spots caused by bleeding under the skin.

  • Vasculitis: Inflammation of small or medium-sized blood vessels, which can cause skin ulcers or more serious internal complications.

Vasculitis in Sjögren’s may also affect nerves, kidneys, or internal organs and must be managed urgently.

8. Blood and Lymphatic System

Blood abnormalities are frequently observed, including:

  • Anemia

  • Leukopenia (low white blood cells)

  • Thrombocytopenia (low platelets)

Sjögren’s also slightly increases the risk of developing non-Hodgkin lymphoma, particularly in those with severe gland involvement, persistent swelling, or systemic complications.

Risk Factors for Systemic Involvement

Certain factors may raise the likelihood of extraglandular involvement:

  • Presence of anti-SSA/Ro and anti-SSB/La antibodies

  • Early disease onset

  • Male gender (though rare, often more severe)

  • High levels of inflammation markers (e.g., ESR, CRP)

  • Longstanding or untreated disease

Patients with these risk factors require closer monitoring for systemic complications.

Diagnosis and Monitoring

Systemic manifestations of Sjögren’s may require evaluation by multiple specialists, including rheumatologists, nephrologists, pulmonologists, and neurologists. Tests may include:

  • Blood tests (autoantibodies, inflammatory markers)

  • Imaging (X-rays, CT scans, MRIs)

  • Organ function panels (liver, kidney, lung capacity tests)

  • Biopsies (for skin, nerves, or glands if needed)

Routine check-ups and symptom tracking are vital to detect changes early.

Treatment Approaches

While there is no cure, systemic symptoms can often be managed effectively. Treatment may include:

  • Corticosteroids: To control inflammation during flares.

  • Immunosuppressants: Such as methotrexate, azathioprine, or mycophenolate mofetil.

  • Biologic therapies: Like rituximab, used in more severe or refractory cases.

  • Organ-specific care: Involving inhalers for lung issues, pain relievers for neuropathy, or diet modification for GI symptoms.

Tailoring treatment based on organ involvement is key to improving function and preventing damage.

Conclusion

Sjögren’s syndrome is far more than a disease of dryness. Its potential to affect organs throughout the body underscores the importance of comprehensive care and proactive management. While not everyone with Sjögren’s will experience systemic symptoms, awareness and early intervention can make a significant difference. By working closely with healthcare providers, patients can manage symptoms effectively, reduce complications, and maintain a good quality of life even in the presence of this complex autoimmune condition.