Info

Sjögren’s syndrome

Definition & epidemiology

  • Chronic dysfxn of exocrine glands (eg, salivary/lacrimal) due to lymphoplasmacytic infiltration, extraglandular manifestations common in primary form
  • Can be primary or secondary (a/w RA, scleroderma, SLE, PM, hypothyroidism, HIV)
  • ~1/1000 prevelance with 9:1 ♀:♂ ratio; typically presents between age 40 & 60
  • Clinical manifestations 󰒖
  • Diagnostic studies 󰒖

Classification criteria (≥4 points 96% Se & 95% Sp; Arthritis Rheumatol 2017;69:35)

  • 3 points: ⊕ anti-Ro; labial saliv. gland bx w/ lymphocytic sialadenitis & score ≥1 foci/4 mm2
  • 1 point: abnormal ocular staining score ≥5; Schirmer’s test ≤5 mm/5 min; unstimulated salivary flow rate of ≤0.1 mL/min

Treatment (Ann Rheum Dis 2020;79:3)

  • Ocular: artificial tears, cyclosporine eyedrops, autologous tears
  • Oral: sugar-free gum, lemon drops, saliva substitute, hydration, pilocarpine, cevimeline
  • Systemic: depends on extraglandular manifest.; NSAIDs, steroids, DMARDs, rituximab