Summary of GN prognosis
- 2021–2025通用更新: SGLT2i已成為多數蛋白尿性GN(尤其IgAN、FSGS、Alport、DM nephropathy)之標準支持治療(DAPA-CKD 2020（eGFR 25–75、UACR 200–5000 mg/g）、EMPA-KIDNEY 2022（eGFR 20–<45 不論蛋白尿；eGFR 45–<90 需 UACR≥200 mg/g）)→改善腎生存; 下表預後數據多為pre-SGLT2i時代
1.Type of GN#
| Type of GN | Prognosis |
|---|---|
| MCD | 1.Spontaneously remission - Early remission(within 3M):5-10% only - 50% by 18M - 70% by 36M 2.After Tx - >70%在4M內CR - Child: 50% responds within 2W and near all within 1-2M - Adult 50% response within 4W, 其他多到4M(CR(所以初始劑量才要維持3-4M)), 共有80-95%CR; 但50-75%relapse, 10-25% frequent relapse, 25-30% steroid dependence, 5-10% steroid resistance(需要renal biopsy(若治療前沒做)) - Complete remission快速(abrupt); 如果有response到CR多2-3W, partial response少到(MCD is almost always an all-or-none disease)(如果PR要懷疑是不是診斷錯誤, EX:FSGS) |
| MCD/FSGS variant-Idiopathic mesangial proliferative GN | 1.Isolated hematuria預後很好 2.Nephrotic syndrome者有些像MCD, 有些像FSGS, 預後不一 |
| MCD/FSGS variant-IgM | 1.Spontaneous remission? 2.After steroid - Responsive:dependent:resistant=1:1:1(所以prognosis比MCD差很多) - 20-35% ESRD in 5-15yrs - 血清高C3及低IgG/C3預測較差腎預後〔PMID 16282696〕 |
| MCD/FSGS variant-C1q nephropathy | 1.Prognosis分型(Vizjak JASN 2008; PMID 18650484)： - Podocytopathy型(MCD樣者77% CR，FSGS樣者33%進展至ESRD) - 增生型GN(57%腎功能穩定，多表現為CKD)〔PMID 18650484〕 |
| FSGS | 1.預後指標 - Proteinuria - Nephrotic syndrome - Spontaneous remission: <10% - More spontaneous remission in non-nephrotic range proteinuria and normal GFR - Average 5yrs renal survival:60-90%; 10yrs renal survival:30-55%(PR:75%, no remission:35%; 見下面response to Tx) - If Tx no response with proteinuria >10g/day, 幾乎5年內進展成ESRD - No nephrotic syndrome - If normal renal function at first - 10yrs renal survival:85% - Initial renal function - if Cre>1.3 - 10yrs renal survival 27% - Histology - Worse:Interstitial fibrosis, collapsing variant, cellular variant - Collapsing variant renal survival - 1yr:75%, 3yrs:33%(vs. others:85% and 67%) - Better:tip lesions(more likely to respond to steroid therapy or to spontaneously remit)=>CR60%, PR15% to steroid, 有些series追蹤起來不治療也有一大部分會spontaneous remission(所以才覺得像MCD variant) - Response to Tx - PR/CR者:10 yrs renal survival:80% - Non-responder: 10 yrs renal survival:<50% 2.Primary FSGS(after Tx) - Mean to remission:12-14M(很久, 要有耐心...) - vs. MCD >70%在4M內CR - CR, PR, no remission:20, 40, 40% (所以60% response to immunosuppressors)(=不治療的MGN, 雖然姜醫師的經驗是MGN不治不太會(remission) - CR者35%relapse, PR者50%relapse - PR有可能還是會變成ESRD |
| Primary MGN | 1.不治療 - 不治療也有20% CR, 40%PR(i.e.↓>50% proteinuria with daily urine protein<3.5g/d)(=治療的FSGS)(雖然Dr.姜文智:<10% spontaneous remission, 所以都要治療) - 15-30% relapse - CR者和正常人一樣每年約↓1ml/min GFR, PR者每年約↓2ml/min GFR, no remission者每年約↓10ml/min GFR - 不論是Tx之後的remission or spontaneous remission的outcomes是一樣的 - ESRD - 15% at 5yrs, 35% at 10yrs, 40% at 15 yrs - 所以平均renal survival > 15yrs - Risk:>50y/o, non-Asian(所以亞洲人預後比較好?)(回思LN是白人預後比較好), nephrotic range proteinuria(尤其>8g/day), increased Cre at presentation, severe TIN 2.若有治療 in iMN - Endoxan group - 40%CR, 30-50% PR(注:CR/PR/no remission由20%, 40%, 40%變成40%, 40%, 20%) - 25-30% relapse - CNI group - The same remission rate - 40% relapse |
| IgAN/HSP | 1.歷史數據: Overall 50% slow progression to ESRD over 20-25yrs (Risk factors: Proteinuria >1g/d、Elevated Cre、HTN、MEST-C 高分/IFTA) 2.當代目標(KDIGO 2025; PMID 40975525): 經最佳支持治療(RAASi+SGLT2i)+標靶治療，控制蛋白尿 <0.5 g/d(理想<0.3 g/d) 且 eGFR 穩定者，長期 20 年 ESRD 風險大幅降低至遠低於歷史 50% 數據 3.未達目標或高風險者年 eGFR 下降可達 1-3 ml/min/1.73m²/yr 以上 |
| PSGN/IRGN | 1.PSGN - Diuresis多begin within 1-2W(所以才說2W仍PD要biopsy), Cre returns to baseline by 3-4W, C3 normal within 4-8W(<2M; C3沒回復要biopsy), hematuria resolves within 3-6M(<1yr), proteinuria比較慢回復可能要好幾年(尤其initial nephrotic syndrome者) - 小孩子的預後很好, >90%會recover; 大人可能有30-50%不會完全回復 - 但基本上之後都還是有secondary FSGS的可能性, 導致幾十年後ESRD(longterm prognosis沒有以前想的那麼好) - 很少recurrence, 可能是因為persistence of Ab to potential antigens 2.S.A associated GN - C3一樣應該要在2M內回復(under appropriate Abx) - Worse than PSGN, especially in DM Pts - 只有25-50% complete remission, 若(Elderly or DM or underlying CKD)則預後更差 - why?因為Pt狀況本來就差? 3.IE related GN - 20% died, 10% ESRD, 40% CKD, 30% complete remission |
| MPGN | DDD預後較差, 比較多crescent, biopsy時較多cTIN, clinical remission也少, 若DDD發生在大人預後更差, 大多10 yrs內ESRD |
| Cryoglobulinemia | 看type與underlying: - Type I(MGUS/lymphoproliferative):看血液腫瘤預後 - Type II-III(HCV相關):DAA 治癒 HCV 後多數腎病改善, 預後大幅改善; 治療反應不佳/重症者較差 |
| TMA | 看etiologies...(詳見TMA(TTP-aHUS and secondary)) |
| LN | 1.Type III/IV - 10-30% ESRD 2.Pure type V - 35% develop concurrent proliferative disease within 10yrs - 10 yr renal survival:72-98% (10年約10% ESRD) 3.Relapse - 多within 5yrs after induction Tx, average 8%/yr(depends on maintenance Tx and CR/PR); but 50%(!!!) proliferative LN who initially achieve CR會relapse after tapering and DC maintenance Tx (故 KDIGO 2024 明訂誘導＋維持總療程應 ≥36 個月 以遏止復發〔PMID 38182286〕) |
| AAV | 1.MPA/GPA - Untreated - 90% mortality in 2 yrs - Treated - Remission rate:70-90%; but relapse after remission:10-60%(40%) - Mortality:12-44% in 4-10 yrs(但是以前的data現在可能好很多) - 主因為immunosuppressive Tx造成的infection and ANCA vasculitis導致的CV, pulmonary, renal failure - For renal outcomes - 15-30% <5 yrs ESRD - Risk factors:Cre很高者 or elderly or resistant disease(60-70% ESRD in months) or relapse or biopsy:FSGS/IFTA - 一旦renal failure需要RRT,40-70%經治療可off dialysis至少>3yrs!!! 2.EGPA - Untreated - 50% mortality in 3M - Mortality 10-30% after 5 yrs - 主因為CV(佔50%), status asthmaticus, CNS, renal failure, GI bleeding |
| Anti-GBM disease | 1.不治療>90%會死亡或ESRD 2.Dialysis dependent AKI者很難recovery(來勢洶洶), 僅<10%會recover - 也因此已經洗腎且看起來不會回復又沒有其他indication(AAV, lung involvement)者, 不太做Tx(但也不一定) - 和AAV不同! 3.Remission者不太會recurrence(2%) |
| Congo red and thioflavine-T negative-nonamyloid-fibrillar glomerular deposition diseases | 約50% 2-6 yrs後ESRD |
| Fibronectin glomerulopathy | 約20-40 y/o開始proteinuria, microscopic hematuria and HTN, then progress to ESRD over 15-20 yrs(所以進展不快) |
| Collagenofibrotic glomerulopathy | progressive to ESRD around 10 yrs after diagnosis |
| Nail-patella syndrome | 預後好:腎臟侵犯約 30-50%(蛋白尿/血尿), 其中 ~5-10% 進展至 ESRD(多在成年後); 不會復發於移植腎 |
| Amyloidosis | Renal failure(20% when diagnosed)多non-reversible, 雖然median Cre at diagnosis:1.1, 但最後常ESRD... |
| MIDD | LCDD - Renal failure(nearly all)多non-reversible(所以預後差...) |
| Cast nephropathy | 50% reversible of renal failure(if <6M), mean response time:2M |
| TBMD/Alport's syndrome | 1.Alport's syndrome - 1.X-linked和AR表現類似, 多16-35y/o ESRD - AD則損失renal function較慢(多45-60y/o ESRD), deafness/ocular abnormalities較罕見且較晚 2.TBMD(現歸為AD Alport光譜，非必然良性，若合併蛋白尿、高血壓或CKD具進展風險〔PMID 33094278〕) 3.Fabry's disease(classical 男性):腎臟累及多於 30-40 歲進展至 ESRD; ERT/agalsidase beta 可延緩但無法逆轉 |