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Observational Study
. 2015 Oct;94(42):e1598.
doi: 10.1097/MD.0000000000001598.

Risk Factors for Autoimmune Diseases Development After Thrombotic Thrombocytopenic Purpura

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Free PMC article
Observational Study

Risk Factors for Autoimmune Diseases Development After Thrombotic Thrombocytopenic Purpura

Mélanie Roriz et al. Medicine (Baltimore). .
Free PMC article

Abstract

Autoimmune thrombotic thrombocytopenic purpura (TTP) can be associated with other autoimmune disorders, but their prevalence following autoimmune TTP remains unknown. To assess the prevalence of autoimmune disorders associated with TTP and to determine risk factors for and the time course of the development of an autoimmune disorder after a TTP episode, we performed a cross sectional study. Two-hundred sixty-one cases of autoimmune TTP were included in the French Reference Center registry between October, 2000 and May, 2009. Clinical and laboratory data available at time of TTP diagnosis were recovered. Each center was contacted to collect the more recent data and diagnosis criteria for autoimmunity. Fifty-six patients presented an autoimmune disorder in association with TTP, 9 years before TTP (median; min: 2 yr, max: 32 yr) (26 cases), at the time of TTP diagnosis (17 cases) or during follow-up (17 cases), up to 12 years after TTP diagnosis (mean, 22 mo). The most frequent autoimmune disorder reported was systemic lupus erythematosus (SLE) (26 cases) and Sjögren syndrome (8 cases). The presence of additional autoimmune disorders had no impact on outcomes of an acute TTP or the occurrence of relapse. Two factors evaluated at TTP diagnosis were significantly associated with the development of an autoimmune disorder during follow-up: the presence of antidouble stranded (ds)DNA antibodies (hazard ratio (HR): 4.98; 95% confidence interval (CI) [1.64-15.14]) and anti-SSA antibodies (HR: 9.98; 95% CI [3.59-27.76]). A follow-up across many years is necessary after an acute TTP, especially when anti-SSA or anti-dsDNA antibodies are present on TTP diagnosis, to detect autoimmune disorders early before immunologic events spread to prevent disabling complications.

Conflict of interest statement

The authors have no funding and conflicts of interest to disclose.

Figures

FIGURE 1
FIGURE 1
Study flow chart. HIV indicates human immunodeficiency virus; HUS = hemolytic and uremic syndrome; USS = Upshaw–Shulman syndrome.
FIGURE 2
FIGURE 2
Number of autoimmune diseases cases according to date of TTP diagnosis.
FIGURE 3
FIGURE 3
Kaplan–Meier survival estimates of autoimmune disease-free survival after autoimmune TTP. Autoimmune disease-free survival as a function of (A) anti-SSA and (B) anti-dsDNA antibody detection at TTP diagnosis.

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