Development and validation of a predictive model for death in acquired severe ADAMTS13 deficiency-associated idiopathic thrombotic thrombocytopenic purpura: the French TMA Reference Center experience.

Background: Acquired thrombotic thrombocytopenic purpura is still associated with a 10-20% death rate. It has still not been possible to clearly identify early prognostic factors of death. This study involved thrombotic thrombocytopenic purpura patients with acquired severe (<10% of normal activity) ADAMTS13 deficiency and aimed to identify prognostic factors associated with 30-day death.

[Giant cell arteritis-related upper/lower limb vasculitis].

Upper/lower limb vasculitis has been considered an uncommon manifestation of giant cell arteritis, occurring in 3 to 16% of patients. Upper/lower limb vasculitis is still associated with significant morbidity, leading to limb/toe amputation in 5.6 to 15.

Long-term follow-up of upper and lower extremity vasculitis related to giant cell arteritis: a series of 36 patients.

We conducted this retrospective study to determine the prevalence of giant cell arteritis (GCA) in patients exhibiting nonatherosclerotic upper and/or lower extremity arterial involvement and to evaluate the clinical features and long-term outcome of those patients.From January 1997 to March 2008, 36 consecutive patients in the Department of Internal Medicine at the University of Rouen medical center received a diagnosis of symptomatic upper/lower extremity vasculitis related to GCA. In the 36 patients, upper/lower extremity vasculitis preceded the initial GCA diagnosis in 7 patients (19.