Sjogren's Syndrome

Primary: occurs alone as a solitary process

Secondary: associated w/ other autoimmune disorders such as rheumatoid arthritis, Hashimotos, SLE

Most common in females (90%), 40-60 yo

one of the three most common systemic autoimmune diseases

Associated with HLA-SR52 gene

Inflammatory destruction of the exocrine glands characterized by focal lymphocyte infiltration of exocrine glands

Aggregation of lymphocytes, primarily CD4+ T-cells and memory cells

Minor salivary gland biopsy or parotid gland biops

Findings of gland fibrosis & lymphocytic infiltration confirm diagnosis

Ocular tests:

Rose Bengal stain: abnormal corneal epithelium

Positive Schirmer test (decreased tear production - wetting <5mm of filter paper)

Screening labs:

Could have + rheumatoid factor, elevated ESR

ANA: Anti SSA/Ro, Anti SSB/La

Oral findings: Decreased salivation, oral ulcers, dental caries, periodontal disease, bilateral parotid gland enlargement & tenderness

Ocular findings: Xerophthalmia, conjunctivitis, corneal ulcers

Pericarditis

Dry mucous membranes

Fever

Nonerosive arthritis

Raynauds

Peripheral neuropathy

Sensorineural hearing loss

ENT symptoms: nasal dryness, chronic cough

Gi symptoms

Dry mucous membranes

Xerostomia

Keratoconjunctivitis sicca 2/2 decreased tear production

Dyspareunia 2/2 decr vaginal secretions

Constitutional symptoms:

fatigue, weakness, sleep disturbances, anxiety, depression

generalized pain, arthralgias

Artificial tears

Artificial saliva, fluoride

Pilocarpine or Cevimeline (cholinergics aka muscarinic antagonists)

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

MOA: increases lacrimation and salivation

Increased fluid intake

Chew sugar-free gum

Vitamin D supplementation to decrease risk of neuropathy & lymphoma