Predicting adverse outcomes in primary Sjogren's syndrome: identification of prognostic factors

Rheumatology (Oxford). 2007 Aug;46(8):1359-62. doi: 10.1093/rheumatology/kem079. Epub 2007 Jun 14.


Objective: To identify features present at diagnosis that were prospectively associated with adverse outcomes in a large cohort of patients with primary Sjögren's syndrome (SS).

Methods: Two hundred and sixty-six patients diagnosed with primary SS in our department between 1984 and 2002 were consecutively included and followed up. Outcomes measured were vasculitis, B-cell lymphoma and death. Cox regression analysis was used to evaluate the effect of variables at diagnosis on outcomes.

Results: Twenty-five (9%) patients developed vasculitis. Multivariate analysis identified parotid scintigraphy grades III or IV (HR 3.55, P = 0.05) and C4 levels <0.11 g/l (HR 8.26, P < 0.001) as variables predicting the development of vasculitis. Nine (3%) patients developed B-cell lymphoma. Multivariate analysis identified C3 levels <0.82 g/l (HR 7.54, P = 0.016) as a predictive factor of lymphoma development. Twenty-five (9%) patients died during follow-up. Systemic involvement (HR 4.51, P = 0.022), vasculitis (HR 4.58, P = 0.042), C4 levels <0.11 g/l (HR 5.47, P = 0.027) and cryoglobulins (HR 4.58, P = 0.013) were independently associated with death. The presence of at least two of the above-mentioned predictive factors (parotid scintigraphy, vasculitis, hypocomplementaemia and cryoglobulinaemia) was associated with a lower survival in comparison with patients with no factor (log rank and Breslow tests <0.001).

Conclusion: The main prognostic factors for an adverse outcome identified in our cohort of patients with primary SS were vasculitis, severe involvement in parotid scintigraphy, hypocomplementaemia and/or cryoglobulins at diagnosis. Patients with at least two of these factors need a closer follow-up.

MeSH terms

  • Aged
  • Complement C3 / deficiency
  • Complement C4 / deficiency
  • Cryoglobulinemia / etiology
  • Epidemiologic Methods
  • Female
  • Humans
  • Lymphoma, B-Cell / etiology
  • Male
  • Middle Aged
  • Parotid Gland / diagnostic imaging
  • Prognosis
  • Radionuclide Imaging
  • Severity of Illness Index
  • Sjogren's Syndrome / complications
  • Sjogren's Syndrome / diagnosis*
  • Sjogren's Syndrome / diagnostic imaging
  • Vasculitis / etiology


  • Complement C3
  • Complement C4