Clinical outcomes and survival in AA amyloidosis patients / Desfechos clínicos e sobrevida em pacientes com amiloidose AA

Abstract Aim Amyloid A amyloidosis is a rare complication of chronic inflammatory conditions. Most patients with amyloid A amyloidosis present with nephropathy and it leads to renal failure and death. We studied clinical characteristics and survival in patients with amyloid A amyloidosis. Methods: A total of 81 patients (51 males, 30 females) with renal biopsy proven amyloid A amyloidosis were analyzed retrospectively. The patients were divided into good and poor outcomes groups according to survival results. Results: Most of the patients (55.6%) had nephrotic range proteinuria at diagnosis. Most frequent underlying disorders were familial Mediterranean fever (21.2%) and rheumatoid arthritis (10.6%) in the good outcome group and malignancy (20%) in the poor outcome group. Only diastolic blood pressure in the good outcome group and phosphorus level in the poor outcome group was higher. Serum creatinine levels increased after treatment in both groups, while proteinuria in the good outcome group decreased. Increase in serum creatinine and decrease in estimated glomerular filtration rate of the poor outcome group were more significant in the good outcome group. At the time of diagnosis 18.5% and 27.2% of all patients had advanced chronic kidney disease (stage 4 and 5, respectively). Median duration of renal survival was 65 ± 3.54 months. Among all patients, 27.1% were started dialysis treatment during the follow-up period and 7.4% of all patients underwent kidney transplantation. Higher levels of systolic blood pressure [hazard ratios 1.03, 95% confidence interval: 1-1.06, p = 0.036], serum creatinine (hazard ratios 1.25, 95% confidence interval: 1.07-1.46, p = 0.006) and urinary protein excretion (hazard ratios 1.08, 95% confidence interval: 1.01-1.16, p = 0.027) were predictors of end-stage renal disease. Median survival of patients with organ involvement was 50.3 ± 16 months. Conclusion Our study indicated that familial Mediterranean fever constituted a large proportion of cases and increased number of patients with idiopathic amyloid A amyloidosis. Additionally, it was observed that patient survival was not affected by different etiological causes in amyloid A amyloidosis.

Resumo Objetivo: A amiloidose AA é uma complicação rara de condições inflamatórias crônicas. A maior parte dos pacientes com amiloidose AA apresenta nefropatia, que leva à insuficiência renal e à morte. Estudaram-se as características clínicas e a sobrevida em pacientes com amiloidose AA. Métodos: Analisaram-se retrospectivamente 81 pacientes (51 homens, 30 mulheres) com amiloidose AA comprovada por biópsia renal. Os pacientes foram divididos em grupos de desfecho bom e ruim de acordo com os resultados de sobrevida. Resultados: A maior parte dos pacientes (55,6%) tinha proteinúria na faixa nefrótica no momento do diagnóstico. Os distúrbios subjacentes mais frequentes foram a febre familiar do Mediterrâneo (FFM, 21,2%) e a artrite reumatoide (10,6%) no grupo de desfecho bom e a malignidade (20%) no grupo de desfecho ruim. Somente a pressão arterial diastólica no grupo de desfecho bom e o nível de fósforo no grupo de desfecho ruim foram mais elevados. Os níveis séricos de creatinina aumentaram após o tratamento em ambos os grupos, enquanto a proteinúria diminuiu no grupo de desfecho bom. O aumento na creatinina sérica e a diminuição na TFGe do grupo de desfecho ruim foram mais significativos no grupo de desfecho bom. No momento do diagnóstico, 18,5% e 27,2% de todos os pacientes tinham doença renal crônica avançada (estágios 4 e 5, respectivamente). A duração média da sobrevida renal foi de 65 ± 3,54 meses. Entre todos os pacientes, 27,1% iniciaram tratamento de diálise durante o período de seguimento e 7,4% de todos os pacientes foram submetidos a transplante renal. Níveis elevados de pressão arterial sistólica [taxas de risco (HR) 1,03, intervalo de confiança (IC) de 95%: 1 a 1,06, p = 0,036], creatinina sérica (HR 1,25, IC 95%: 1,07 a 1,46, p = 0,006) e excreção urinária de proteínas (HR 1,08, IC 95%: 1,01 a 1,16, p = 0,027) foram preditores de doença renal terminal. A mediana da sobrevida de pacientes com comprometimento de órgãos foi de 50,3 ± 16 meses. Conclusão: O presente estudo indicou que a FFM constituiu uma grande proporção de casos e crescente quantidade de pacientes com amiloidose AA idiopática. Adicionalmente, observou-se que a sobrevida do paciente não foi afetada pelas diferentes causas etiológicas na amiloidose AA.

Key advances in the histopathologic classification to ANCA-associated glomerulonephritis / 医学研究生学报

Anti-neutrophil cytoplasmic antibody ( ANCA)-associated glomerulonephritis is the most common cause of rapidly progressive glomerulonephritis worldwide , and the renal biopsy is not only the gold standard for establishing the diagnosis ,but also help-ful to judge the prognosis in clinical practice .In 2010,Berden et al presented a pathologic classification which proposed four general categories of lesions:Focal, crescentic, mixed, and sclerotic.And then, several validation studies proved the prognostic value of this classification.However, the difference of renal survival is large in various pathological types among different studies , so it needs more studies to develop its prognostic value .This review mainly focuses on clinical significance , definition of pathological feature , and vali-dation study about this classification .

Key advances in the histopathologic classification to ANCA-associated glomerulonephritis / 医学研究生学报

Anti-neutrophil cytoplasmic antibody ( ANCA)-associated glomerulonephritis is the most common cause of rapidly progressive glomerulonephritis worldwide , and the renal biopsy is not only the gold standard for establishing the diagnosis ,but also help-ful to judge the prognosis in clinical practice .In 2010,Berden et al presented a pathologic classification which proposed four general categories of lesions:Focal, crescentic, mixed, and sclerotic.And then, several validation studies proved the prognostic value of this classification.However, the difference of renal survival is large in various pathological types among different studies , so it needs more studies to develop its prognostic value .This review mainly focuses on clinical significance , definition of pathological feature , and vali-dation study about this classification .

Clinical Analysis Of Iga Nephropathy In Patients Over 50 Years Of Age / 대한내과학회지

BACKGROUND: IgA nephropathy is recognized as a disease affecting primarily young men under 30 years of age but it is relatively uncommon over 50 years of age. Findings on clinical and histological presentation and outcome of over 50 years of age have rarely published in Korea. METHODS: Between Febrary 1994 and July 2003, one hundred and thirty nine IgAN patients were recruited over 8 years. Nineteen patients over age 50 were compared to one hundred and twenty patients under age 50 clinical, histological findings, 5-Yr renal survival rate. Mean post-biopsy follow-up month was 23.8 +/- 23.5 months. RESULTS: Both group of patients were similar to baseline for gross hematuria, Male and female ratio. But older patients had a higher incidence of daily for 24 hour urine protein (p=0.010), systolic and diastolic blood pressure (p=0.010, p<0.01), serum C3 (p=0.001) and serum C4 (p=0.003). Albumin (p=0.011), creatinine clearance (p<0.01) were significantly lower in the older patients at the time of renal biopsy. Histologic grade IV was more common in the older patients (p=0.001). Moderate to severe mesangial proliferation (p=0.001) and crescent formation (p=0.043), arteriolosclerosis (p=0.006) were more common in older patients. Mesangial small deposition of IgA (p=0.007) and glomerular peripheral deposition of IgG, IgA, C1q (p=0.024, p=0.014, p=0.009) were more common in older patients than in under 50 years of age. Mesangial small electron dense deposits were more common in older patients than in younger patients (p=0.031). CRF (Ccr < 60 mL/min) was confirmed 55.6% over age 50 and 27.4% under age 50 (p=0.017). Renal replacement therapy was done 15.8% over age 50 and 4.3% under age 50 (p=0.048). 5-Year renal survival curves showed that kidney survival rate was 44.4% over age 50 and 72.6% under age 50 (p=0.0248). CONCLUSION: Poor prognostic factors were more common over age 50 than under age 50. CRF and renal replacement therapy were more common over age 50. Analysis of renal survival curves shows that the probability of developing ESRF increase after age 50. But, prolonged prospective follow-up is necessary to confirm this trend.

Long-term Prognostic Factors in Pediatric Focal Segmental Glomerulosclerosis

PURPOSE: Efforts to predict long-term outcome of focal segmental glomerulosclerosis(FSGS) have been made but have yielded conflicting results. Reports are rare especially in pediatric patients. In this study, we reviewed the predictable prognostic factors in patients of FSGS. METHOD: Fifty children who diagnosed as biopsy-proven FSGS at depart- ment of pediatrics at Yonsei university were studied retrospectively. Based on medical records, response to treatment and pathologic slides, we compared normal renal function group and decreased renal function group, assessed the factors affecting renal survival and progression to renal failure. RESULTS: The mean age at onset was 8 1/12 years, sex ratio was 2.3 : 1, and the mean duration of follow-up was 7 1/12 years. The overall renal survival rate was 34% at 5 years, 8% at 10 years. Five-year survival rate was 74% in normal renal function group and 27% in decreased renal function group. Between the two groups, there were no significant differences in age at onset, sex ratio, amount of proteinuria, incidence of hematuria and hypertension, mesangial hypercellularity. Decreased renal function group showed higher serum creatinine level, poor response to treatment, higher percent of glomeruli with sclerosis, moderate to severe tubulointerstitial change and vascular change(p<0.05). The prognostic factors of renal survival rate were same as above and incidence of hypertension also affected renal survival(p<0.05). The progression rate to renal failure did not show statistically significant factor. CONCLUSION: We reviewed the factors affecting long-term outcome of FSGS. Serum creatinine level, steroid responsiveness, and the degree of glomerulosclerosis were significant prognostic factors.

Prognostic factors and renal survival rates in IgA nephropathy

A retrospective study of 223 patients with IgA nephropathy (IgAN) was performed to clarify the prognostic factors and the renal survival rates of the disease. One hundred twenty-two patients were followed-up for more than 6 months after their renal biopsy (mean follow-up duration: 43.0 months), and 20 of them (16.4%) had progressed to end-stage renal disease (ESRD). Using univariate analysis, 8 risk factors (2 clinical and 6 histopathological findings) for developing ESRD were identified: renal insufficiency at initial presentation (serum creatinine > or = 1.5 mg/dl); heavy proteinuria(> or = 3.5 gm/day); moderate to severe histopathologic findings such as class IV/V lesions by W.H.O. classification, mesangial hypercellularity, glomerular sclerosis, interstitial infiltration, interstitial fibrosis, and tubular atrophy. In multivariate regression analysis, class IV/V lesions and renal insufficiency at initial presentation were the independent prognostic factors of IgAN. The renal survival rates were 100% at 1 year, 97.0% at 3 years, and 78.9% at 5 years. In conclusion, it seems that about 20% of IgAN patients have a risk to progress to ESRD within 5 years, and a careful follow-up is recommended especially in patients who have either renal insufficiency at the time of presentation or severe renal pathology (class IV/V lesions).