Midline granuloma and Wegener's granulomatosis: clinical & therapeutic considerations

• Published in: Journal of rheumatology Vol. 3; no. 3; p. 241
• Main Authors: Schechter, S L, Bole, G G, Walker, S E
• Format: Journal Article
• Language: English
• Published: Canada 01-09-1976
• Subjects: Adrenal Cortex Hormones - therapeutic use; Adult; Antineoplastic Agents - therapeutic use; Azathioprine - therapeutic use; Cyclophosphamide - therapeutic use; Drug Therapy, Combination; Female; Granuloma, Lethal Midline - drug therapy; Granuloma, Lethal Midline - radiotherapy; Granuloma, Lethal Midline - therapy; Granulomatosis with Polyangiitis - drug therapy; Granulomatosis with Polyangiitis - radiotherapy; Granulomatosis with Polyangiitis - therapy; Humans; Male; Middle Aged; Nitrogen Mustard Compounds - therapeutic use; Prednisone - therapeutic use; Prognosis
• ISSN: 0315-162X

Midline granuloma (MG), limited Wegener's granulomatosis (LWG), and generalized Wegener's granulomatosis (WG) have been viewed by some investigators as representing an interrelated disease spectrum. Others believe that MG and WG are two distinct clinicopathologic entities. A series of cases is presented suggesting that therapy of MG should be individualized. Treatment may include corticosteroids, high-dose irradiation, and/or immunosuppressive drugs. LWG may be treated initially with corticosteroids alone, but lack of response requires the addition of an immunosuppressive agent. WG should be treated with an immunosuppressive drug and, at times, corticosteroids as well. None of the cases of MG described in this report progressed to WG. This may be interpreted as supporting the contention that MG and WG are separate diseases. Alternatively, aggressive treatment of MG with irradiation or immunosuppressives may prevent its transition to more generalized disease.