What is Sjögren's syndrome?

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From the Guidelines

The management of Sjögren's syndrome should prioritize a multidisciplinary approach, with the first therapeutic approach for dryness being symptomatic relief using topical therapies, as recommended by the EULAR guidelines 1.

Overview of Sjögren's Syndrome

Sjögren's syndrome is an autoimmune disorder characterized by dry eyes and dry mouth due to lymphocytic infiltration of exocrine glands. The primary goal of treatment is to manage symptoms and prevent complications.

Treatment Approach

  • For dry eyes, artificial tears (preservative-free) should be used frequently during the day, with lubricating ointments at night 1.
  • Prescription options include cyclosporine eye drops (Restasis) 0.05% twice daily or lifitegrast (Xiidra) 5% twice daily.
  • For dry mouth, patients should sip water frequently, use sugar-free lozenges, and consider saliva substitutes like Biotene 1.
  • Pilocarpine (5-10mg three times daily) or cevimeline (30mg three times daily) can stimulate saliva production.

Systemic Manifestations

  • For systemic manifestations, hydroxychloroquine (200-400mg daily) may help with fatigue and joint pain 1.
  • More severe cases might require immunosuppressants like methotrexate (starting at 7.5mg weekly) or rituximab for refractory disease.
  • Patients should maintain good oral hygiene, have regular dental and ophthalmological check-ups, and avoid medications that worsen dryness like antihistamines 1.

Disease Activity Assessment

  • The EULAR Sjögren's Syndrome Disease Activity Index (ESSDAI) should be used to assess disease activity 1.
  • The ESSDAI score can help guide treatment decisions, with higher scores indicating more severe disease activity.

Key Recommendations

  • A multidisciplinary approach involving various health professionals is essential 1.
  • The first therapeutic approach to dryness should be symptomatic relief using topical therapies 1.
  • Systemic therapies may be considered for the treatment of active systemic disease 1.

From the Research

Definition and Symptoms of Sjögren Syndrome

  • Sjögren syndrome is a systemic autoimmune disease characterized by dry eyes and dry mouth 2.
  • It is classified as primary or secondary, with primary disease being a solitary process and secondary disease accompanying another autoimmune disease, often rheumatoid arthritis 2.
  • Symptoms include dry eyes, dry mouth, and other symptoms related to decreased salivary and lacrimal gland function 3.

Treatment and Management of Sjögren Syndrome

  • The treatment of primary Sjögren's syndrome is based principally on the management of sicca features and systemic manifestations 4.
  • Topical therapies, such as saliva substitutes and artificial tears, are used to treat sicca manifestations 4.
  • Stimulation of salivary flow with a sialogogue, such as pilocarpine, is the therapy of choice for patients with residual salivary gland function 4.
  • Pilocarpine has been shown to be more effective than artificial saliva for enhancing salivary and lacrimal secretion in patients with Sjögren syndrome 5.

Diagnosis and Epidemiology of Sjögren Syndrome

  • Sjögren syndrome is a challenging diagnosis, requiring coordination with a team of specialists, including dentists, otolaryngologists, rheumatologists, and ophthalmologists 2.
  • Approximately 4 million Americans are diagnosed with Sjögren's Syndrome 6.
  • The disease is characterized by lymphocytic infiltration of salivary and lacrimal glands, leading to the systemic production of autoantibodies 3.

Management of Symptoms

  • Interventions to manage symptoms such as fatigue, dry eyes, mouth, and skin are important for patients with Sjögren syndrome 6.
  • Chronic pain is a common symptom of Sjögren syndrome, and management of this symptom is crucial 6.

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Professional Medical Disclaimer

This information is intended for healthcare professionals. Any medical decision-making should rely on clinical judgment and independently verified information. The content provided herein does not replace professional discretion and should be considered supplementary to established clinical guidelines. Healthcare providers should verify all information against primary literature and current practice standards before application in patient care. Dr.Oracle assumes no liability for clinical decisions based on this content.

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