Fireside conference 19. Wegener's granulomatosis and lethal midline granuloma.
In summary, Wegener's granulomatosis is a systemic illness that is characterized pathologically by necrotizing granulomatosis and vasculitis. Lethal midline granuloma is not a pathological term. Lethal midline granuloma is a clinical term, and in reality, lethal midline granuloma has been proven to be polymorphic reticulosis and not Wegener's granulomatosis. Polymorphic reticulosis is now understood to be a T-cell lymphoma. Further study of these malignant lymphomas has shown that these T-cell lymphoproliferative disorders may be causally associated with Epstein-Barr virus. Although there is strong association between autoantibodies against cytoplasmic constituents of neurophils and monocytes in patients with active Wegener's granulomatosis, the exact pathogenic mechanism in Wegener's granulomatosis and the etiology is still unknown. IgG-C-ANAC (C-ANAC stands for Classical/Cytoplasmic Anti-Neutrophil Cytoplasm Antibodies) increases with increasing disease activity from undetectable levels to up to 95% of active Wegener's granulomatosis patients. Rhinologic symptoms in Wegener's granulomatosis include progressive nasal obstruction, bloody rhinorrhea with crusting, and vague pain and tenderness of the nasal dorsum. Usually these patients have mucosal ulcerations of the nose with or without a septal perforation. Other areas of the head and neck can be involved, and they include orbital, otologic, oral, and subglottic involvement. Hallmarks of malignant lymphoma (polymorphic reticulosis) when it involves the upper airway include rapid localized destruction of the nose, orbit, paranasal sinuses, and hard palate. Treatment for Wegener's granulomatosis includes antimicrobial agents in addition to a regimen of cyclophosphamide and glucocorticoids. The treatment for malignant lymphoma (polymorphic reticulosis) is primarily radiation, especially when confined to one site.
D019268:Antibodies, Antineutrophil Cytoplasmic; D001323:Autoantibodies / Q000276:immunology; D006103:Granuloma, Lethal Midline; D014890:Granulomatosis with Polyangiitis; D006801:Humans; D007074:Immunoglobulin G / Q000276:immunology; D053146:Myeloblastin; D012697:Serine Endopeptidases / Q000276:immunology
D019268:Antibodies, Antineutrophil Cytoplasmic; D001323:Autoantibodies; D007074:Immunoglobulin G; D012697:Serine Endopeptidases; D053146:Myeloblastin