Ioannidis et al evaluated patients with primary Sjogren's syndrome (pSS) for prognostic factors related to lymphoproliferative disorders and death. These can help to identify a patient who may benefit from more aggressive management. The authors are from the University of Athens
Predictors for a lymphoproliferative disorder from univariate analysis:
(1) parotid enlargement
(2) palpable purpura at first study visit
(3) low serum C4 concentration (< 20 mg/dL)
(4) lymphadenopathy
(5) anti-Ro and/or anti-La antibodies
Predictors for a lymphoproliferative disorder from multivariate analysis:
(1) parotid enlargement
(2) palpable purpura at first study visit
(3) low serum C4 concentration (< 20 mg/dL)
The risk of developing a lymphoproliferative disorder was 9-fold greater if >= 1 of these 3 risk factors was present.
Predictors of mortality from univariate analysis:
(1) parotid enlargement
(2) splenomegaly
(3) lymphadenopathy
(4) palpable purpura
(5) rheumatoid factor
(6) low serum C4 concentration at first study visit (< 20 mg/dL)
Predictor of mortality from multivariate analysis:
(1) low serum C4 concentration at first study visit
Risk factors for malignant lymphoma that resulted in death:
(1) low serum C4 concentration at first study visit (< 20 mg/dL)
(2) palpable purpura at first study visit
|
Number of Risk Factors
|
Type pSS
|
Risk
|
|
>= 1
|
Type I
|
high
|
|
0
|
Type II
|
low
|
Cryoglobulins were not considered to be a routine test but were common in high-risk patients. They may be related to the low complement levels and the palpable purpura.
