Sudden death and Wegener's granulomatosis of the pituitary.
Involvement of brain parenchyma or meninges in ANCA-associated small-vessel vasculitis such as Wegener's granulomatosis (WG) is not uncommon. In contrast, involvement of the pituitary is exceedingly rare with only a few cases reported so far. The diagnosis is usually made on the basis of imaging techniques and abnormal pituitary function tests in the setting of active systemic vasculitis. However, histology-proven involvement of the pituitary by WG has not been reported so far. We report a case of WG with histology-proven granulomatous necrotizing inflammation of the pituitary and hypothalamo-pituitary stalk, disclosed at autopsy after the patient had died suddenly and unexpectedly in his sleep. In a setting of histology-proven WG, these findings were regarded as a pituitary manifestation of the disorder. A distinct cause of death could not be found, hence we speculate that hypothalamo-pituitary inflammation due to WG may have caused the sudden death in this patient.
Woywodt
A
A
Department of Nephrology, Franz-Volhard-Clinic, Humboldt-University, Berlin, Germany. woywodt@aol.com
Knoblauch
H
H
Kettritz
R
R
Schneider
W
W
Göbel
U
U
D000328:Adult; D003645:Death, Sudden; D014890:Granulomatosis with Polyangiitis / Q000175:diagnosis* / Q000188:drug therapy / Q000473:pathology; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use; D008279:Magnetic Resonance Imaging; D008297:Male; D010900:Pituitary Diseases / Q000175:diagnosis* / Q000473:pathology; D010903:Pituitary Gland, Anterior / Q000473:pathology*; D014667:Vasopressins / Q000627:therapeutic use
D016756:Immunoglobulins, Intravenous; D014667:Vasopressins