Sjogren's Syndrome

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Thalassemia

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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