Clinical analysis of 100 patients with Wegener's granulomatosis / 中华风湿病学杂志

ABSTRACT

Objective To investigate the clinical features of 100 cases with Wegener's granulomatosis (WG). Methods One hundred patients with WG admitted to our center in recent 11 years were retrospectively analyzed. Results The ratio of male to female was 1.041. The average age was (39±17) years (ranging from 4 to 72 years). The upper respiratory tract (86%), lung (82%), kidney (70%) and ocular (53%) were the major affected organs, followed by neurological system ( 12% ) and cardiovascular system ( 11% ). cANCA was positive in 77% of patients, while ANCA was negative in 8%. Images mostly showed multiple nodules/mass with/without cavity in lung (59%) and sinusitis (57%). The pathologic features were necrotic granulomatosis and/or microvasculitis,which was 78% in nasal mucosa/mass biopsy and 75% in lung.Focal segmental necrotic glomerulonephritis ( 59% ) was an important feature for confirming the diagnosis. Of the WG groups, 49% of patients were in generalized subgroup followed by localized (22%), early systemic ( 15% ), severe renal (9%) and refractory group (5%). The patients were treated with corticosteroid and cyclophosphamide. The remission rate in the induction phase was 78%, while the mortality rate was 4%. The follow-up duration ranged from 1 to 145 months. Complications included infection (22%), chronic renal failure( 12% ), deep venous thrombosis( 11% ). Five patients died(8% ), in which 2 patients died of infection.Conclusion The clinical manifestations of Wegener's granulomatosis are complicate. ANCA testing, images of sinus and lung and histological biopsy have played important roles in early diagnosis, which is significant to initiate appropriate and prompt treatment in order to reduce complications and improve prognosis.