Retroperitoneal Vasculitis With Perivascular Fibrosis

SUMMARY A florid case of retroperitoneal fibrosis with severe vascular as well as urinary complications is described. The ætiology is discussed and from a study of the available literature it is concluded that the basic disorder is a vasculitis with similarities to polyarteritis nodosa or the necrotising arteritis of Wegener. Widespread general effects, notably obscure pain, anorexia, and weight loss may precede, by months or years, the more overt ureteric obstructive manifestations: joint symptoms, pericarditis, and respiratory infection may also occur. Obstruction of the inferior vena cava and arterial insufficiency may develop. A raised sedimentation rate is an index of activity. Serum protein changes are non‐specific. Anaemia may be an early manifestation. There is evidence that the connective tissue changes may be widespread and even involve the mediastinum. There is a tendency towards eventual self‐limitation of the disease. Ureterolysis is an efficient method for treatment of the functional obstruction of the ureters and steroid therapy may be of help.