Wegener’s granulomatosis with thrombotic thrombocytopenic purpura – neurologic manifestations

Authors: J. Vítová
Authors‘ workplace: Interní oddělení Nemocnice Č. Budějovice, a. s.
Published in: Čes. Revmatol., 16, 2008, No. 2, p. 92-96.
Category: Case Report


Wegener’s granulomatosis (WG) is a systemic vasculitis of medium-sized and small-sized vessels. Most of all, the kidneys and respiratory tract, peripheral nerves, less frequently CNS are affected with either vasculitis or granulomas. Other organs may be affected, as well. Thrombotic thrombocytopenic purpura (TTP) is a disease caused by deficiency of von Willebrand factor’s protease resulting in hyperadhesiveness of thrombocytes with various clinical consequences. The case report of a patient with coincidental course of WG and TTP is described. The disease was recurrent, diffusing and potentiating the clinical manifestations, which took on dramatic picture. The case draws attention to the possibility of combination of two rare conditions and the need of thorough examination of clinical manifestations and laboratory findings, which do not fit in the image of one disease.

Key words:
Wegener’s granulomatosis, thrombotic thrombocytopenic purpura, neurologic manifestations


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