Sjogren’s syndrome

Vignette says a 38 years old female presents to her primary care physician with chief complaints of dry eyes and dry mouths for the past 6 months; She reports of burning, itching and gritty sensation in both eyes and uses artificial tear drops frequently; She also reports of difficulty swallowing dry foods and needs to drink water frequently while eating foods; Examination shows non tender, enlarged bilateral parotid glands; She was diagnosed with rheumatoid arthritis 2 years back and takes 15 mg of methotrexate once weekly with folinic acid; Vital signs show blood pressure of 120/80 mm of Hg, pulse rate of 78 bpm, respiratory rate of 16 breaths/min, oxygen saturation of 98% in RA and temperature of 96.9 F; Serology shows positive ANA, rheumatoid factor, Anti SS-A (Ro) and Anti SS-B (La) antibodies; Schirmer test shows reduced tear production; Salivary gland biopsy shows lymphocytic infiltration; Diagnosis?

Diagnosis is Sjogren’s syndrome.

Sjogren’s syndrome is an autoimmune disease that leads to destruction of lacrimal and salivary glands due to lymphocytic infiltration; It commonly affects women; It can be divided into primary and secondary (associated with other autoimmune disorders like RA, SLE).

Clinical features:-
1. Dry eyes (keratoconjunctivitis sicca)
2. Dry mouth (xerostomia), dental caries
3. Symmetric painless enlargement of parotid glands
4. Vaginal dryness and dyspareunia
5. Dry skin
6. Extra glandular features include arthritis, vasculitis, interstitial nephritis, interstitial pneumonitis, PNS or CNS disease, PBC, Raynaud phenomenon, cryoglobulinemia.

Diagnosis:-
1. ANA (+) in 90% of cases, RF (+) in 75 % of patients with secondary disease.
2. Anti ribonucleoprotein antibodies (Anti SS-A (Ro) and Anti SS-B (La) antibodies) are positive.
3. Schirmer test to access tear production.
4. Rose Bengal stain shows damaged epithelium of cornea and conjunctiva.
5. Lip (minor salivary gland) biopsy shows lymphocytic infiltration.

Management:-
1. Pilocarpine or Cevimeline (enhance oral and ocular secretions via acetylcholine).
2. Artificial tears for dry eyes.
3. Good oral hygiene.
4. NSAIDs, Steroids, DMARDs for extra glandular manifestations.