About Sicca/Sjogren’s Disease

Sjogren’s is a chronic, inflammatory autoimmune disease involving exocrine gland inflammation.

  • Extra-glandular symptoms affect up to 40% of pts
  • F:M = 9:1
  • Peak age 50-60 yo

Generally presents with inflammation of exocrine glands +/- extraglandular features.

Sjogren’s can present as a primary  disease called Sjogren’s disease, in the absence of another underlying disease, or a patient can have secondary Sjogren’s. In secondary Sjogren’s, patients have the symptom of dry mouth (xerostomia) and/or dry eyes (xerophthalmia), associated with another underlying rheumatic disease (rheumatoid arthritis, lupus, systemic sclerosis, etc.)

  • Most common symption: sicca = dry eyes and/or dry mouth in >85% pts
  • Ocular:
    • Predominant dryness in the morning
    • Dry and itchy feeling
    • Foreign body/gritty sensation
    • Eye redness
  • Dry mouth → frequent cavities (view image)
    • Inability to eat dry food (bread, crackers, etc.)
    • Difficult speaking for long period due to dryness
    • Mouth burning
  • Parotid gland enlargement (view image)
  • Extraglandular features:
    • Arthritis in up to 45%, arthralgia more common
    • Neuropathy: PNS in up to 35%
    • Renal and GU: RTA IV (hypokalemia, acidosis), interstitial cystitis, vaginal dryness
    • Pulmonary: rarely lymphocytic interstitial pneumonitis (LIP)
    • Cardiac: pericarditis, pulmonary hypertension
    • GI
  • Rash
  • Raynaud’s phenomenon
  • Cytopenias

Lab workup

  • SSa/SSb+ in 60-80%
  • Other rheum labs that can be seen in Sjogren’s but nonspecific and does not help with diagnosis: ANA+, RF+
  • CBC with differential: anemia of chronic disease
  • RF+ (+/- low C4) independently associated with higher risk of non-Hodgkin’s lymphoma in primary Sjogren’s
  • Given features can overlap with SLE, consider checking other extractable nuclear antigens

Objective Testing

  • Ocular surface staining (view image)
  • Salivary gland scintigraphy
  • Whole sialometry
  • Salivary gland biopsy
  • Schirmer test (view image)
  • Extraglandular evaluation driven by organ(s) involved
    • Concern for interstitial lung involvement: CT ILD protocol
    • For peripheral small fiber neuropathy: skin biopsy may be needed (EMG/NCV will not
      pick up small fiber neuropathy)

Sicca (Dry Eyes and/or Dry Mouth)

Avoid contributing medications and desiccants

Oral Symptoms

  • Good hydration
  • Salivary stimulants
  • Artificial saliva
  • Muscarinic agonist: pilocarpine, cevimeline
  • 1.1% sodium fluoride
  • ENT referral if oral ulcers, refractory disease

Ocular Symptoms

  • Artificial tears/ointment
  • Ophtho referral:
    • Topical cyclosporine
    • Punctal plugs

Extra-Glandular Manifestations

  • Arthritis: DMARDs –  hydroxychloroquine, methotrexate, etc.
  • Neuropathy: IVIG, rituximab
  • Lymphocytic ILD: rituximab, cyclophosphamide
  • Heme abnormalities like ITP: rituximab

Poor Prognostic Factors

  • RF+ (+/- low C4) independently associated with higher risk of non-Hodgkin’s lymphoma in primary Sjogren’s
    • Disappearance of previously positive RF concerning for development of lymphoma
  • Low complements
  • Parotid gland enlargement
  • Lymphadenopathy

Complications

  • Increased risk of non-Hodgkin’s lymphoma
  • Cryoglobulinemic vasculitis
  • Important to rule out mimics: oral candidiasis, medications, smoking, contacts overuse
  • 25-60% of peripheral neuropathy precedes Sjogren’s diagnosis
  • If sicca but SSA/SSB neg: consider cancer or IgG4 disease, recommend biopsy
  • Sjogren’s can be associated with cryoglobulinemic vasculitis (consider this diagnosis if new rash; labs will show very low C4, normal C3, high RF)
  • RF+, parotid gland enlargement, +/- low C4 in adults independently associated with higher
    risk of non-Hodgkin’s lymphoma in primary Sjogren’s

Related Links

Sjogren’s Disease Case Studies

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