Sjogren Syndrome

Question 1

Classification for Primary Sjogrens

Answer 1

Need 4

1. CLINICAL
   - Oral (dry mouth, recurrent parotitis or enlargement of parotid glands)
   - Ocular (recurrent conjunctivitis without obvious allergy or infection etiology, keratoconjunctivits sicca)
   - Other mucosal involvement (recurrent vaginitis)
   - Systemic (FUP, non inflammatory arthralgias, hypokalemic paralysis, abdominal pain)
2. Immunological abnormalities
   - Ro, LA, high titer ANA, RF
3. Other lab abnormalities
   - Biochemical: Elevated amylase
   - Hematological: Leukopenia, high ESR
   - Immunological: Hypergamma
   - Nephrological: Renal tubular acidosis
   - Histological proof of lymphocytic infiltrate of salivary glands or other organs
   - Objective documentation of parotid gland involvement
   - Objective documentation of parotid gland involvement

Exclusion of all other autoimmune diseases

Question 2

What suggests poor prognosis?

Answer 2

Purpura, pernio, cyroglobulins

Question 3

Lab features indicating a predisposition to lymphoma

Answer 3

Mixed monoclonal cryoglobulins, low level of C4, monoclonal bands in the serum or urine

Question 4

Clinical features predictive of lymphoma

Answer 4

Persistent enlargement of parotid gland, LAD, splenomegaly, palpable purpora