Clinical characteristics of p-ANCA (anti-neutrophil cytoplasmic antibody)-related nephritis: a single center experience

Kang, Hyeock Joo; Park, Sun Hee; Park, Ja Yong; Shin, Yong Bong; Kim, Chan Duck; Tak, Woo Taek; Lee, Jeong Ho; Kim, Yong Lim

Abstract

BACKGROUND/AIMS: We evaluated the clinical characteristics and prognostic value of the clinical, laboratory, pathologic features, at time of diagnosis, and the renal survival of patients with ANCA (anti-neutrophil cytoplasmic antibody)-related nephritis. METHOD: We retrospectively analyzed 17 patients who were diagnosed with ANCA-related nephritis at a single center. The risks of progression to ESRD or death according to the clinical parameters, the ANCA pattern and the renal pathologic findings were evaluated.
RESULTS
The major symptoms were hematuria (100%), proteinuria (100%), uremic symptoms (41.2%), edema (35.5%), upper respiratory symptoms (29.4%) and oliguria (23.5%), which were not correlated with renal survival. All the patients showed a p-ANCA pattern. The BUN level (p=0.032) and GFR (p=0.023) at the time of diagnosis were different between the improved and the progressed patients in terms of renal function. The pathology indices were not predictive factors of both renal and patient survival. Eight patients (47.1%) were treated with steroid IV pulse, 4 (23.5%) with steroid IV pulse and cyclophosphamide IV pulse, 2 (11.8%) with steroid IV pulse, cyclophosphamide IV pulse and plasma exchange, and 2 (11.8%) with steroid IV pulse and plasma exchange. Fourteen patients (82.4%) needed hemodialysis. There were 3 (17.6%) disease-related deaths, 13 patients (76.5%) reached ESRD and 4 (23.5%) showed recovery of renal function. The mean percent of patients who survived was 80.2% and the mean percent of renal survival was 33.3% at the 1st and 3rd year, respectively.
CONCLUSIONS
Poor renal function at presentation was associated with a high risk for disease progression, but age, gender, the clinical patterns of presentation and the pathologic findings were not associated with the prognosis. Early diagnosis and treatment seems to be essential to improve the renal outcomes.