343.4 📋 章末速記 Summary

343.4.1 🔑 一句話總結

Renal vascular disease 涵蓋：(1) renal artery stenosis (RAS) — atherosclerotic ARAS (elderly, often bilateral) vs fibromuscular dysplasia FMD (young women, “string of beads”)；CORAL trial 2014 確立 ARAS 用 OMT first-line + stent for refractory；FMD 用 balloon angioplasty cure；(2) renal vein thrombosis (RVT) — nephrotic syndrome (esp MN with albumin < 2.5), hypercoagulable, tumor extension (RCC) — LMWH or DOAC；(3) thrombotic microangiopathy (TMA) spectrum：TTP (ADAMTS13 < 10%, anti-ADAMTS13 antibodies) → plasma exchange + steroids + caplacizumab (HERCULES 2019) + rituximab refractory + avoid platelets；HUS — typical STEC (E. coli O157:H7, post-bloody diarrhea, supportive, avoid antibiotics) vs atypical (aHUS, complement dysregulation CFH/CFI/MCP/CFB/C3, eculizumab or ravulizumab)；drug-induced TMA (calcineurin inhibitors, ICI, VEGF inhibitors, quinine, mitomycin, gemcitabine); HELLP + pregnancy TMA; (4) atheroembolic disease (cholesterol embolism) — post-arterial procedure (livedo + blue toes + eosinophilia + Hollenhorst plaques)；(5) vasculitis — large/medium/small vessel；hypertensive nephropathy = chronic HTN → glomerulosclerosis + arteriolar hyalinosis；BP < 130/80 + ACE/ARB；malignant HTN (BP > 180/120 + end-organ damage + TMA) → IV antihypertensives ICU；scleroderma renal crisis → ACE inhibitor (captopril) lifesaving + avoid prednisone；APOL1 risk genotype in African ancestry hypertensive nephropathy.

343.4.2 💊 治療精要

• ARAS：OMT (ACE/ARB + statin + ASA + BP control) first-line per CORAL 2014; stent for refractory HTN/flash pulmonary edema/progressive CKD
• FMD：balloon angioplasty alone (no stent typically); 50-60% HTN cure
• RVT：anticoagulation (LMWH for cancer; DOAC otherwise) + treat underlying nephrotic
• TTP：daily plasma exchange + corticosteroids + caplacizumab (HERCULES, FDA 2019) + rituximab for relapsing/refractory + avoid platelets
• aHUS：eculizumab (Soliris) or ravulizumab (Ultomiris) anti-C5 + meningococcal vaccination pre-treatment
• STEC HUS：supportive only; avoid antibiotics (may worsen Shiga toxin release)
• drug-induced TMA：stop offending agent + supportive
• cholesterol embolism：no specific antidote + statins + ASA + avoid further procedures
• HTN nephropathy：BP < 130/80 (KDIGO 2021) + ACE/ARB first-line + multi-drug + SGLT2i emerging
• malignant HTN emergency：IV labetalol/nicardipine/clevidipine/nitroprusside; reduce SBP 20-25% in first hour (then gradual)
• scleroderma renal crisis：ACE inhibitor (captopril titrated up) — lifesaving + ARB if intolerant + avoid prednisone

343.4.3 🎯 盧醫師的考前提醒

1. CORAL trial (2014) changed ARAS practice：stent + OMT not superior to OMT alone for most → OMT first-line + stent reserved for refractory HTN, flash pulmonary edema, progressive CKD
2. FMD vs ARAS 鑑別：FMD = young women + “string of beads” + medial type 80-90% + balloon angioplasty cure 50-60%; ARAS = elderly atherosclerosis + bilateral often
3. renal vein thrombosis (RVT) risk in nephrotic：MN with albumin < 2.5 → highest risk → consider prophylactic anticoagulation (LMWH or DOAC)
4. TTP diagnostic triad/pentad：MAHA + thrombocytopenia + (renal + neurologic + fever); ADAMTS13 activity < 10% + anti-ADAMTS13 antibodies confirmatory; PLASMIC score for pre-treatment probability
5. HERCULES (2019) caplacizumab for TTP：anti-vWF nanobody + reduces recovery time + relapses; FDA 2019; adjunct to PEX + steroids
6. aHUS vs typical STEC HUS：aHUS = complement dysregulation (CFH, CFI, MCP, CFB, C3 mutations) → eculizumab / ravulizumab; STEC HUS = E. coli O157:H7 post-bloody diarrhea → supportive only, avoid antibiotics (controversy)
7. eculizumab requires meningococcal vaccination pre-treatment (C/Y conjugate + B vaccines 2 weeks before); strict prophylaxis if urgent
8. cholesterol embolism post-arterial procedure：livedo reticularis + blue toes + eosinophilia + Hollenhorst plaques (retinal) + multi-organ; no specific antidote
9. scleroderma renal crisis：rapid + severe HTN + AKI + TMA; ACE inhibitor (captopril titrated up) lifesaving + avoid prednisone (may precipitate) + recovery often takes months
10. malignant HTN treatment principle：reduce SBP 20-25% in first hour, then gradual (avoid precipitous drop → cerebral / renal ischemia); IV labetalol or nicardipine first-line