RT Journal Article
SR Electronic
T1 Wegener's granulomatosis in two sisters.
JF Annals of the Rheumatic Diseases
JO Ann Rheum Dis
FD BMJ Publishing Group Ltd and European League Against Rheumatism
SP 417
OP 421
DO 10.1136/ard.45.5.417
VO 45
IS 5
A1 Muniain, M A
A1 Moreno, J C
A1 Gonzalez Cámpora, R
YR 1986
UL http://ard.bmj.com/content/45/5/417.abstract
AB We describe two sisters with necrotising granulomas. The intervals between the onset of symptoms and diagnosis were eight and nine years. The pathological findings showed necrotising granulomas in both patients, this being consistent with a diagnosis of pathergic (Wegener's) granulomatosis. The one patient that accepted treatment responded well to cyclophosphamide, though the facial lesions responded only to radiation therapy. Since the second was almost asymptomatic we conclude that relatives of patients with Wegener's disease should be investigated.