Combined CNS and pituitary involvement as a primary manifestation of Wegener granulomatosis.
Wegener granulomatosis (WG) is a systemic vasculitis of small and medium vessels. It predominantly affects the upper and/or lower respiratory airway and kidneys. Its pathogenesis is not fully understood. WG relatively frequently affects the nervous system (in 30-50% according to the different studies). Most frequently, it manifests as necrotizing vasculitis that leads to the peripheral neuropathies or to the cranial nerves palsy. Impairment of the central nervous system (CNS) is less frequent and occurs in 2-8% of patients. Three major pathogenetic mechanisms were described: CNS vasculitis, spreading of granulomas from the adjacent anatomical areas (paranasal cavities, orbit etc.), and new formation of granulomas in brain tissue. This case report describes patients in whom WG manifested in the form of localized skin involvement and combined CNS involvement that included pituitary gland. Atypical presentation of WG impedes and slows down the process of diagnosis and emphasizes the need for collaboration between medical specialists.
Spísek
Radek
R
Department of Immunology, 2nd Faculty of Medicine,, Charles University, V Uvalu 84, 150 06, Prague 5, Czech Republic. radek.spisek@lfmotol.cuni.cz
Kolouchová
Elena
E
Jensovský
Jirí
J
Rusina
Robert
R
Fendrych
Pavel
P
Plas
Jaroslav
J
Bartůnková
Jirina
J
D000038:Abscess / Q000209:etiology / Q000473:pathology; D000328:Adult; D001921:Brain / Q000098:blood supply / Q000473:pathology*; D002493:Central Nervous System Diseases / Q000175:diagnosis* / Q000209:etiology; D003520:Cyclophosphamide / Q000627:therapeutic use; D014890:Granulomatosis with Polyangiitis / Q000150:complications / Q000175:diagnosis* / Q000188:drug therapy; D006801:Humans; D007018:Hypopituitarism / Q000175:diagnosis* / Q000209:etiology; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008279:Magnetic Resonance Imaging; D008297:Male; D010903:Pituitary Gland, Anterior / Q000098:blood supply / Q000473:pathology*; D012867:Skin / Q000473:pathology; D012871:Skin Diseases / Q000209:etiology / Q000473:pathology
D007166:Immunosuppressive Agents; D003520:Cyclophosphamide