What are people with Sjogren's syndrome at risk of developing?

Risks Associated with Sjögren's Syndrome

Patients with Sjögren's syndrome are at significantly increased risk of developing non-Hodgkin lymphoma (10-44 times higher risk than the general population), particularly marginal zone B-cell lymphomas affecting the salivary glands. 1

Major Complications and Risks

Lymphoproliferative Disorders

• Non-Hodgkin Lymphoma (NHL)
  • Most serious complication with 10-44 fold increased risk compared to healthy individuals 1
  • Predominantly low-grade B-cell lymphomas of marginal zone histological type 1
  • Primary extranodal involvement of major salivary glands, especially parotid 1
  • Diffuse large B-cell lymphoma is the most common NHL subtype 2
  • Risk factors include presence of cryoglobulinemia and higher EULAR Sjögren's syndrome disease activity index 1

Organ-Specific Complications

• Oral/Dental Complications

  • Dental decay and oral infections 3
  • Gum inflammation and mouth sores 3
  • Cracked or chipped teeth 3

• Ocular Complications

  • Eye damage and vision problems 3
  • Corneal ulceration and scarring (from severe dry eye) 3

• Pulmonary Manifestations

  • Interstitial lung disease 3
  • Chronic dry cough 3

• Renal Complications

  • Tubulointerstitial nephritis 3
  • Renal tubular acidosis 3

• Neurological Complications

  • Peripheral neuropathy (numbness or tingling in extremities) 3
  • Autonomic neuropathy 3

Other Associated Conditions

• Thyroid Cancer

  • Increased risk compared to general population 4

• Multiple Myeloma

  • Higher incidence in Sjögren's patients 4

• Skin Cancers

  • Elevated risk reported in some studies 4

• Other Autoimmune Conditions

  • Increased risk of developing other autoimmune disorders 5
  • Particularly systemic lupus erythematosus and rheumatoid arthritis 5

Risk Stratification and Monitoring

High-Risk Factors for Lymphoma Development

• Presence of cryoglobulinemia 1
• Elevated disease activity (high EULAR Sjögren's syndrome disease activity index) 1
• Salivary gland enlargement 3
• Persistent parotid enlargement 3

Protective Factors

• Salivary gland biopsies appear to be associated with lower lymphoma risk 2
• Immunomodulator therapy (hydroxychloroquine, azathioprine, cyclosporine, methotrexate, rituximab) may reduce lymphoma risk 2

Recommended Monitoring

• Regular monitoring for lymphoproliferative disorders 3
• Pulmonary function tests for respiratory complications 3
• Vigilance for vasculitic manifestations 3
• Thyroid function monitoring 3
• In pregnant women with anti-Ro antibodies, fetal echocardiography between 16-26 weeks to monitor for congenital heart block 3

Clinical Pearls

• The risk of lymphoma in Sjögren's syndrome exceeds that seen in other autoimmune diseases like systemic lupus erythematosus and rheumatoid arthritis 1
• Some lymphomas in Sjögren's syndrome may evolve slowly or even regress spontaneously 6
• Multiple risk factors in an individual correlate with higher lymphoma risk 1
• Co-management with a rheumatologist is essential due to potential systemic complications 3