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Sjogren syndrome

Mechanism
  • Autoimmune (anti-SSA/Ro & anti-SSB/la antibodies) damage to secretory glands
    • Lacrimal
    • Salivary
  • Theory: viral infection of salivary glands (EBV, HTLV-1, HCV) may stimulate immune system
 
Classification
  • Primary
  • Secondary → associated with other autoimmune diseases (rheumatoid arthritis, systemic lupus erythematosus, systemic sclerosis)
 
Epidemiolgy
  • 4th or 5th decade
  • Female to male ratio is 9:1
  • 0,4-0,6 of the total population (one of the most common autoimmune disorders)
 
Clinical presentation
  • Xerophtalmia or Keratoconjuntivitis sicca
    • Ocular dryness, foreign body sensation, pain, photophobia
    • Decreased tear production → Shirmer test (≤5mm in 5min)
    • Assess integrity of cornea → lissamine green stain, fluorescein stain, rose bengal dye
    • Risk: keratitis, corneal thinning, ulceration, recurrent infections
  • Xerostomia
    • Dryness, soreness, burning of the mouth and lips
    • Difficulty swallowing
    • Require frequent ingestion of fluids
    • Perlèche or thrush (candidal overgrowth)
    • Dental caries
    • GERD (saliva has high pH that neutralizes acid), may mimic upper respiratory infections
  • Arthritis
    • Polyarticular, non-erosive, chronic, progressive, asymmetric
  • Xerosis of vagina
    • Dryness, burning, dyspareunia
  • Other skin manifestations
    • Annular erythema (similar to subacute cutaneous LE - overlap?)
    • Vasculitis
      • Palpable or non-palpable purpura
      • Urticarial vasculitis
      • Presence of purpura should prompt assessment for cryoglobulinemia
    • Erythema nodosum
    • Nodular amyloidosis
    • Sweet syndrome
  • Extraglandular and extracutaneous
    • Lungs: interstitial pneumonitis (often subclinical)
    • Kidneys (intertitial nephritis, tubular dysfunction
    • Bone marrow
    • Peripheral and central nervous systems
 
Complications/Associations
  • Subacute cutaneous lupus drug reactions
  • Cutaneous small vessel vasculitis
  • (!) Chronic B cell activation → marginal zone B cell lymphoma in lacrimal glands (20x higher risk) 💀
 
Exams
  • Hypergammaglobulinemia
  • Anti-SSA (Ro) & Anti-SSB (La)
  • Positive Rheumatoid Factor
  • Salivary gland sialometry or parotid sialography (not commonly used)
  • Salivary gland biopsy
    • Presence of one or more foci of inflammatory cells (≥50 lymphocytes) in 4mm2 of salivary gland tissue
 
Diagnosis
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DDx
  • Xerostomia
    • anticholinergic drugs
    • Age related atrophy of glands
    • Previous irradiation
 
Treatment
  • Oral xerostomia
    • Tratamento de xerostomia: hydral gum para hidratar a boca
    • Pilocarpina Salagen x3
    • Oculotect
    • Siccafluid
    • Fórmula magistral com lidocaina, triancinolona e nistatina (boca seca secundária a radioterapia)
      • Lidocaína 2%, triancinolona 0,1%/diprofos, nistatina 1000.000 UI QBP M100mL solução aquosa, carboximetilcelulose ou base para colutório
      • Bochechar com 5–10 mL durante 1–2 minutos, 4x/dia, antes das refeições e ao deitar. Não engolir (ou engolir, dependendo da indicação).
  • Preservative free artificial tears and lubricant ointments
  • Punctal plugs that occlude nasolacrimal ducts
  • Home humidifiers
  • Cyclosporine eye drops
  • Autologous serum tears
  • Vaginal
    • Lubricants
    • Prophylatic antifungal
    • Intravaginal or systemic estrogen
  • Systemic therapy if vasculitis or complications
    • Hydroxychloroquine
    • MTX
    • Azathioprine
    • MMF
    • TNF inhibitors
    • Belimumab
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