Send to

Choose Destination
Medicine (Baltimore). 2015 Oct;94(42):e1598. doi: 10.1097/MD.0000000000001598.

Risk Factors for Autoimmune Diseases Development After Thrombotic Thrombocytopenic Purpura.

Author information

From the Centre hospitalier universitaire Bretonneau, Service de néphrologie, Tours (MR, CB, J-MH, FTM); Centre hospitalier universitaire Hôtel-Dieu, Service de médecine interne A, Nantes (ML, MH); Centre hospitalier de Valenciennes, Service de néphrologie, Valenciennes (JD, YD); Centre de Référence des Microangiopathies Thrombotiques, Hôpital Saint-Antoine, APHP (EA, LG, AW, J-LB, FP, FP, J-PM, CP, PP, TK, AS, DB, PP, DC, AV, J-MH, MH, PC, FTM, MH, PC); Université Paris Diderot, Sorbonne Paris Cité (EA, LG); Service de Réanimation Médicale, Hôpital Saint-Louis, AP-HP (EA); Service d'Immunologie Clinique, Hôpital Saint-Louis, AP-HP, Paris (LG); Service de Néphrologie, Hôpital Maison Blanche, Reims (AW); Sorbonne Université, UPMC Univ Paris 06 (J-LB); Service de Réanimation Médicale, CHU Saint-Antoine, AP-HP, Paris (J-LB); Service de Néphrologie, Hôpital Albert Calmette, Lille (FP); Service de Réanimation Polyvalente, Hôpital Cochin, AP-HP (FP, J-PM); Université Paris 5, Paris (FP, J-PM); Service de Néphrologie - Médecine Interne, Hôpital Sud, Amiens (CP); Service d'Hémaphérèse, Hôpital de Marseille Conception, Marseille (PP); Service de néphrologie-transplantation-dialyse, Hôpital Pellegrin, Bordeaux (YD); Unité d'Hémaphérèse thérapeutique, CHU de Montpellier (TK); Service de Réanimation Médicale, Centre Hospitalier Universitaire de Caen (AS); Service de Néphrologie, Dijon (CM); Service de Néphrologie, Hôpital Necker-Enfants Malades, AP-HP, Paris (AS); Service d'Hématologie Clinique et de Thérapie Cellulaire, CHU Dupuytren, Limoges (DB); Service de Réanimation, CHU Brabois, Nancy (PP); Service de néphrologie-immunologie clinique, hôpital Rangueil, Toulouse (DC); Service d'Hématologie Biologique, Hôpital Lariboisière, AP-HP, Paris (AV); Université Paris-Sud 11, Le Kremlin-Bicêtre (AV); Inserm U1009, Institut Gustave Roussy, Villejuif (PC); and Service d'Hématologie, Hôpital Saint-Antoine, AP-HP, Paris, France (PC)


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.

[Indexed for MEDLINE]
Free PMC Article

Supplemental Content

Full text links

Icon for Wolters Kluwer Icon for PubMed Central
Loading ...
Support Center