Sjogren's Syndrome
Health Promotions
Vitamin D supplementation to decrease risk of neuropathy & lymphoma
Chew sugar-free gum
Increased fluid intake
Treatment
Pilocarpine or Cevimeline (cholinergics aka muscarinic antagonists)
MOA: increases lacrimation and salivation
SE: diaphoresis, flushing, bradycardia, diarrhea, N/V, incontinence, blurred vision (2/2 pupil constriction), bronchoconstriction
Cevimeline has less of an effect on cardiac & lung tissue
Artificial saliva, fluoride
Artificial tears
Symptoms
Constitutional symptoms:
generalized pain, arthralgias
fatigue, weakness, sleep disturbances, anxiety, depression
Dyspareunia 2/2 decr vaginal secretions
Keratoconjunctivitis sicca 2/2 decreased tear production
Xerostomia
Gi symptoms
ENT symptoms: nasal dryness, chronic cough
Signs
Sensorineural hearing loss
Peripheral neuropathy
Raynauds
Nonerosive arthritis
Fever
Dry mucous membranes
Pericarditis
Ocular findings: Xerophthalmia, conjunctivitis, corneal ulcers
Oral findings: Decreased salivation, oral ulcers, dental caries, periodontal disease, bilateral parotid gland enlargement & tenderness
Diagnostics
Screening labs:
ANA: Anti SSA/Ro, Anti SSB/La
Could have + rheumatoid factor, elevated ESR
Ocular tests:
Positive Schirmer test (decreased tear production - wetting <5mm of filter paper)
Rose Bengal stain: abnormal corneal epithelium
Minor salivary gland biopsy or parotid gland biops
Findings of gland fibrosis & lymphocytic infiltration confirm diagnosis
Pathophysiology
Inflammatory destruction of the exocrine glands characterized by focal lymphocyte infiltration of exocrine glands
Aggregation of lymphocytes, primarily CD4+ T-cells and memory cells
Genetics
Associated with HLA-SR52 gene
Epidemiology
one of the three most common systemic autoimmune diseases
Most common in females (90%), 40-60 yo
Etiology
Secondary: associated w/ other autoimmune disorders such as rheumatoid arthritis, Hashimotos, SLE
Primary: occurs alone as a solitary process
