Prognostic value of histopathological scoring and grading in patients with renal AA amyloidosis.

OBJECTIVE: The amount and distribution pattern of amyloid deposits may contribute to renal function and outcome, given the great diversity of renal involvement in amyloidosis. The aim of this study was to analyze the impact of histological characteristics of patients with biopsy-proven renal AA amyloidosis (AAA) on renal outcome. METHODS: Renal biopsies of 37 patients with AAA were re-evaluated. The distribution pattern of glomerular amyloid (GA) deposits was classified, the extent of amyloid deposits in glomeruli, vessel, and interstitium and other histopathologic lesions were scored, and renal amyloid prognostic score (RAPS) was determined by summing all scores. Their potential prognostic relevance on renal outcome was investigated. RESULTS: GA and vascular amyloid (VA) deposits were noted in all cases, interstitial amyloid (IA) was detected in 70.2%. GA deposits were predominantly seen in diffuse mesengiocapillary fashions (class IV) (51.4%). GA class, the extent of GA, VA, IA deposit, and RAPS, as well as interstitial fibrosis (IF) and interstitial inflammation were correlated to renal function at diagnosis. During the median follow-up of 52 months, 13 patients developed doubling of serum creatinine or end stage renal disease and they had a higher degree of GA and VA load (p = 0.03 and p = 0.042, respectively) as compared to the remaining patients. VA load, but not GA and RAPS grade, was associated with poor renal outcome (HR 3.016, 95% CI 1.45-6.25, p = 0.003). CONCLUSIONS: Baseline renal function is closely linked to the extent of AA amyloid deposit in renal parenchyma but only VA load was a predictor of renal outcome in AAA patients.

Bacillus Calmette-Guérin-induced perinuclear antineutrophil cytoplasmic antibodies associated vasculitis in bladder cancer.

Intravesical instillation of Bacillus Calmette-Guérin (BCG) immunotherapy remains the most effective adjuvant treatment for noninvasive bladder cancer. Systemic BCG-related complications are rare and usually related to infective agent or an immune-mediated reaction. We discussed a case with perinuclear antineutrophil cytoplasmic antibodies (p-ANCA) vasculitis, developing after instillation of BCG for non-invasive bladder cancer. A 68-year-old man presented with nephritic syndrome a few months after BCG instillations which was performed for his non-muscle-invasive bladder cancer adjuvant therapy. The renal function had declined slowly after the first instillation and urinary sediment reveals the new onset of nephritic proteinuria and hematuria. High titer of p-ANCA was present. His renal biopsy was consistent with acute renal vasculitis. The patient's creatinine level regressed with immunosuppressive therapy and he was clinically followed up without hemodialysis. Here, we presented a patient that diagnosed as p-ANCA related vasculitis occurred after BCG instillation.