308.4 📋 章末速記 Summary

308.4.1 🔑 一句話總結

Bronchiectasis = 永久不可逆 bronchial dilation + 慢性 inflammation + impaired mucociliary clearance；分 CF (Ch308) vs non-CF；non-CF 病因多：post-infectious (30%, TB, NTM, pertussis)、immunodeficiency (CVID)、ABPA、autoimmune (RA, IBD)、PCD/Kartagener (situs inversus + sinusitis + bronchiectasis)、α1-AT、aspiration、idiopathic；HRCT = tram tracks + signet ring + bronchial wall thickening；common organisms：H. influenzae、Pseudomonas (predicts worse outcomes)、S. aureus、NTM (MAC, “Lady Windermere syndrome”)；治療：airway clearance + chronic azithromycin (EMBRACE, BAT, BLESS) + treat underlying + inhaled tobramycin for Pseudomonas + brensocatib (ASPEN 2024) — first disease-modifying；MAC 治療 = macrolide + ethambutol + rifampin × 12 個月。

308.4.2 💊 治療精要

• airway clearance：chest PT、OPEP (Acapella, Flutter)、HFCWO vest、hypertonic saline 7% nebulized、mannitol
• acute exacerbation antibiotics：cover known colonizers；amoxicillin-clavulanate、azithromycin、doxycycline；Pseudomonas → ciprofloxacin / IV cefepime / pip-tazo
• chronic suppressive azithromycin 250 mg/d 或 500 mg 3x/wk（EMBRACE, BAT, BLESS — ↓ exacerbations 50%）
• inhaled antibiotics for Pseudomonas：tobramycin (TOBI)、colistin、aztreonam
• 2024 NEW：brensocatib (DPP-1 inhibitor) — ASPEN trial positive，FDA expected 2024-2025
• MAC：macrolide (azithromycin) + ethambutol + rifampin × 12 個月 after culture conversion；inhaled amikacin for refractory
• M. abscessus：complex, multi-drug IV + PO ≥ 12 個月

308.4.3 🎯 盧醫師的考前提醒

1. bronchiectasis etiology workup 必須：immunoglobulins (CVID)、CFTR (CF adult)、α1-AT、ABPA workup (IgE + Aspergillus)、ANCA/RF/autoimmune、NTM、PCD if suspicious — 30% post-infectious、idiopathic up to 50%
2. HRCT 三大徵象：tram tracks (parallel airways)、signet ring sign (bronchus > artery)、bronchial wall thickening；patterns = cylindrical / varicose / cystic
3. Pseudomonas colonization = bad prognosis marker：↑ exacerbations + ↑ lung decline + ↑ mortality；inhaled antibiotics needed
4. azithromycin chronic 三大試驗：EMBRACE, BAT, BLESS 都顯示 ↓ exacerbations 50%；但 QT、hearing、resistance 是 trade-off
5. brensocatib ASPEN 2024：第一個 disease-modifying therapy for bronchiectasis（DPP-1 inhibitor 抑制 neutrophil serine proteases）— 2024-2025 FDA approval
6. Kartagener syndrome：PCD + situs inversus + sinusitis + bronchiectasis + male infertility；nasal NO 低 + ciliary EM/genetic
7. Lady Windermere syndrome：elderly thin women + MAC infection + right middle lobe / lingula bronchiectasis (suppression of cough hypothesis)
8. NTM diagnosis (ATS/IDSA)：clinical + radiographic + 2 sputum positive OR 1 BAL positive；長療程 + specialty referral
9. MAC treatment：azithromycin + ethambutol + rifampin × 12 months after culture conversion（不只 12 個月 total）；inhaled amikacin for refractory
10. diffuse panbronchiolitis (DPB)：Asian populations (Japan, Korea, China)；HLA-Bw54 association；erythromycin low-dose long-term dramatically effective — 鑑別點