243.1 🎓 醫孞生版

243.1.0.1 📌 䞀頁重點

243.1.0.1.1 Pathogen
  • Aspergillus species — A. fumigatus #1 cause (~ 85%), A. flavus, A. niger, A. terreus
  • Ubiquitous — soil, decaying vegetation, dust, water, construction sites
  • Spores (conidia) inhaled
  • 5 clinical forms based on host immunity + lung structure
243.1.0.1.2 5 Clinical Forms
243.1.0.1.2.1 1. Invasive Aspergillosis (IA)
  • Severe immunocompromise (neutropenia, anti-cancer chemo, HSCT, transplant, anti-CD20, advanced HIV with CD4 < 50)
  • Pulmonary (>90%) — most common; followed by disseminated + sinus + CNS
  • High mortality (30-50%)
  • “Halo sign” + “air crescent sign” on chest CT
  • Voriconazole first-line
243.1.0.1.2.2 2. Allergic Bronchopulmonary Aspergillosis (ABPA)
  • Asthma + cystic fibrosis (CF) patients
  • IgE-mediated allergic response to colonizing Aspergillus
  • Recurrent / persistent asthma + bronchiectasis (central) + mucous plugging
  • Elevated total IgE > 1000 IU/mL + specific Aspergillus IgE + Aspergillus precipitins (IgG)
  • Corticosteroids + itraconazole treatment
243.1.0.1.2.3 3. Aspergilloma (“Fungal Ball”)
  • Saprophytic colonization of pre-existing lung cavity (post-TB, sarcoidosis, COPD, lung abscess)
  • “Crescent sign” / “air-meniscus sign” on CXR/CT
  • Hemoptysis primary symptom (can be massive)
  • Stable but recurrent disease
  • Surgical resection for symptomatic + lobar disease (high-risk surgery — bleeding); embolization for hemoptysis
243.1.0.1.2.4 4. Chronic Pulmonary Aspergillosis (CPA)
  • Includes: chronic cavitary, chronic necrotizing, simple aspergilloma, chronic fibrosing
  • Underlying lung disease (COPD, post-TB, sarcoidosis)
  • Subacute / chronic course (months-years)
  • Cough, hemoptysis, weight loss, fatigue
  • Long-term itraconazole or voriconazole
  • Surgery for select
243.1.0.1.2.5 5. Cutaneous + Other Sites
  • Cutaneous: burn wounds, IV sites, traumatic inoculation
  • Sinus aspergillosis: invasive + chronic
  • Ocular: keratitis (trauma), endophthalmitis (post-surgical)
  • Otomycosis: external ear
243.1.0.1.3 Diagnosis
243.1.0.1.3.1 Imaging
  • Chest CT:
    • Halo sign (ground-glass around nodule) — early invasive
    • Air crescent sign — recovering invasive (neutrophil recovery)
    • Cavitary mass with intracavitary “fungal ball” — aspergilloma
    • Nodules, consolidation, infiltrates
  • MRI brain: for suspected CNS aspergillosis
  • Paranasal CT: for sinus disease
243.1.0.1.3.2 Biomarkers
  • β-D-glucan: elevated in IA, Candida, PJP (not specific)
  • Galactomannan (GM):
    • Aspergillus cell wall component
    • Serum GM screening in high-risk neutropenic
    • BAL GM more sensitive than serum for pulmonary
    • CSF GM for CNS
    • False positives: pip-tazo (lots, especially old), other molds, some lab issues
243.1.0.1.3.3 Microbiology
  • Sputum / BAL culture + microscopy
  • PCR of BAL fluid (emerging)
  • Tissue biopsy: definitive — hyphae 45° branching with septations in tissue (Aspergillus characteristic)
  • Aspergillus colonization vs invasion: tissue invasion is key
243.1.0.1.3.4 Pulmonary Function (ABPA)
  • Total IgE > 1000 IU/mL
  • Aspergillus-specific IgE elevated
  • Aspergillus precipitins (IgG)
  • Peripheral eosinophilia
  • Central bronchiectasis on CT
  • Mucous plugging
243.1.0.1.4 Treatment
243.1.0.1.4.1 Invasive Aspergillosis
  • First-line: Voriconazole 6 mg/kg IV q12h × 2 doses then 4 mg/kg IV q12h (or oral 200 mg bid)
  • TDM: trough 1-5.5 mg/L
  • Alternative: Isavuconazole (oral or IV — lower toxicity, linear PK)
  • Salvage: Liposomal Amphotericin B 3-5 mg/kg/d
  • Combination: voriconazole + echinocandin in severe (controversial; no clear mortality benefit)
  • Duration: ≥ 6-12 weeks; longer for cavitary / severe / CNS / disseminated
  • Reduce immunosuppression if possible
  • Surgical resection for localized cavity / persistent disease / hemoptysis
243.1.0.1.4.2 ABPA
  • Systemic corticosteroids (prednisone 0.5 mg/kg/d → taper)
  • Itraconazole (or voriconazole) 200 mg bid × 16 wk
  • Anti-IgE therapy (omalizumab) for recurrent / severe
  • Optimal asthma + CF management
243.1.0.1.4.3 Aspergilloma
  • Observation if asymptomatic
  • Surgical resection for hemoptysis + symptomatic + lobar disease (high-risk surgery)
  • Bronchial artery embolization for hemoptysis
  • Antifungal limited efficacy in established aspergilloma
243.1.0.1.4.4 Chronic Pulmonary Aspergillosis
  • Itraconazole or voriconazole long-term (months-years)
  • Posaconazole alternative
  • Surgical resection for symptomatic single cavity
  • Management of underlying lung disease
243.1.0.1.4.5 Cutaneous + Sinus
  • Voriconazole + surgical debridement
  • Source control critical
243.1.0.1.5 Resistance
  • Azole-resistant Aspergillus fumigatus (TR34/L98H mutation in CYP51A) — Netherlands first, now Asia + Europe + USA
  • Mechanism: agricultural azole fungicide use → environmental selection
  • Voriconazole MIC ≥ 4 mg/L = resistant
  • Alternative: liposomal AmB, isavuconazole (variable activity), olorofim (Phase 3)
  • Surveillance recommended

243.1.0.2 1⃣ Microbiology + Epidemiology

243.1.0.2.1 Species
  • A. fumigatus (#1 cause invasive aspergillosis, ~ 85%)
  • A. flavus (sinus, cutaneous; mycotoxin aflatoxin)
  • A. niger (otomycosis common)
  • A. terreus (rare invasive; intrinsic AmB resistance)
  • A. nidulans (chronic granulomatous disease association)
  • 250+ species; few cause human disease
243.1.0.2.2 Habitat
  • Ubiquitous in environment
  • Soil, decaying vegetation, dust, water
  • Construction sites (high spore release)
  • Air conditioning systems
  • Aspergillus spores ubiquitous in air — most healthy people don’t develop disease
243.1.0.2.3 Spore Inhalation
  • Conidia (spores) 2-3 µm diameter — fine enough to reach alveoli
  • Most cleared by alveolar macrophages
  • Disease when:
    • Immunocompromise
    • Pre-existing lung disease (cavity, bronchiectasis)
    • Allergic predisposition (asthma, CF, atopy)
    • Mucous obstruction
243.1.0.2.4 Pathogenesis
  • Tissue invasion in immunocompromise
  • Angioinvasion → vascular damage + thrombosis + infarction + hemorrhage
  • Hyphae morphology: 45° branching with septations (Aspergillus)
  • Cell wall components: galactomannan, β-glucan (used for diagnosis)

243.1.0.3 2⃣ Invasive Aspergillosis (IA)

243.1.0.3.1 Risk Factors
243.1.0.3.1.1 Major Immune Compromise
  • Neutropenia (< 500/µL, especially prolonged > 10 days)
  • Anti-cancer chemotherapy
  • HSCT (especially allogeneic, prolonged engraftment)
  • Solid organ transplant (especially lung; less heart, kidney, liver)
  • Severe COPD on high-dose inhaled corticosteroids
  • Anti-CD20 therapy (rituximab, ocrelizumab)
  • Anti-TNF therapy
  • High-dose corticosteroids ≥ 20 mg prednisone for weeks
  • Advanced HIV (CD4 < 50)
  • Chronic granulomatous disease (CGD — genetic; A. nidulans + fumigatus)
  • Severe influenza + COVID-19 (post-viral immunosuppression — increased recognition)
243.1.0.3.2 Clinical Forms
243.1.0.3.2.1 Pulmonary IA (> 90%)
  • Most common form
  • Fever, cough, chest pain, hemoptysis
  • Often initial nonspecific
  • Severe: respiratory failure
243.1.0.3.2.2 Sinus IA
  • Rhinocerebral mucormycosis-like presentation
  • Facial pain, nasal discharge, swelling
  • May extend to brain
  • DM controlled cases + immunocompromise
243.1.0.3.2.3 CNS Aspergillosis
  • Hematogenous spread from lung
  • Direct extension from sinuses
  • Cerebral abscess, infarct
  • Stroke-like presentation
  • High mortality
243.1.0.3.2.4 Disseminated
  • Hematogenous spread
  • Skin, kidney, heart, liver, multiple organs
  • Severe immune compromise
243.1.0.3.2.5 Cutaneous
  • Catheter site
  • Burn wound
  • Traumatic inoculation
  • Nodular lesions
243.1.0.3.3 Imaging — Chest CT (Diagnostic)
243.1.0.3.3.1 Halo Sign
  • Ground-glass opacity around solid nodule
  • Reflects angioinvasion + surrounding hemorrhage
  • Early invasive aspergillosis (especially neutropenic)
  • Sensitivity 60-90% in early IA
243.1.0.3.3.2 Air Crescent Sign
  • Crescent of air between dying parenchyma + cavity wall
  • Appears days-weeks later
  • Often during neutrophil recovery
  • Distinctive for invasive aspergillosis
243.1.0.3.3.3 Other CT Findings
  • Nodules + cavitations
  • Wedge-shaped peripheral infarcts
  • Consolidation
  • Pleural-based lesions
  • Halo sign + air crescent sign = pathognomonic combination
243.1.0.3.4 Diagnosis
243.1.0.3.4.1 Galactomannan (GM)
  • Aspergillus cell wall component (mannan)
  • Serum GM screening in high-risk neutropenic (q3-5 days during neutropenia)
  • BAL GM more sensitive than serum (especially in non-neutropenic)
  • CSF GM for CNS aspergillosis
  • Cutoff: serum index > 0.5 or BAL > 1.0
  • False positives: piperacillin-tazobactam (older lots; now less of an issue), Penicillium, Histoplasma, blastomycosis, other molds, lab issues
  • False negatives: posaconazole prophylaxis, voriconazole therapy, mild disease
243.1.0.3.4.2 β-D-Glucan
  • Elevated in IA + Candida + PJP (not specific for Aspergillus)
  • Adjunctive
243.1.0.3.4.3 PCR
  • BAL Aspergillus PCR
  • Emerging diagnostic
  • High sensitivity + specificity in research settings
243.1.0.3.4.4 Microbiology + Histology
  • BAL culture (Aspergillus growth)
  • Sputum less reliable (colonization vs invasion)
  • Tissue biopsy: definitive — hyphae 45° branching with septations (Aspergillus characteristic; differential from Mucor — broader, irregular, less septate, 90° branching)
243.1.0.3.5 Treatment
243.1.0.3.5.1 First-Line
  • Voriconazole:
    • 6 mg/kg IV q12h × 2 doses then 4 mg/kg IV q12h (loading + maintenance)
    • Or oral 200 mg bid
    • TDM: trough 1-5.5 mg/L
    • Adjust dose based on trough
243.1.0.3.5.2 Alternative First-Line
  • Isavuconazole (oral or IV):
    • 200 mg loading × 6 doses then 200 mg/day
    • Linear pharmacokinetics (less TDM)
    • Lower hepatotoxicity than voriconazole
    • Less drug interactions
    • Less photosensitivity
    • Comparable efficacy (SECURE trial)
243.1.0.3.5.3 Salvage / Alternative
  • Liposomal Amphotericin B 3-5 mg/kg/day
  • For voriconazole intolerance / failure
  • Higher dose (5 mg/kg) for severe disease
243.1.0.3.5.4 Combination Therapy
  • Voriconazole + Echinocandin (caspofungin, anidulafungin)
  • Controversial — no clear mortality benefit (some trials negative)
  • May be considered for severe / progressing disease
  • Long-term outcomes unclear
243.1.0.3.5.5 Duration
  • ≥ 6-12 weeks minimum
  • Until:
    • Clinical resolution
    • Radiographic improvement
    • Galactomannan trends negative
    • Resolution of immune compromise
  • Longer for:
    • Cavitary disease (months)
    • CNS aspergillosis
    • Disseminated disease
    • Persistent immunosuppression
243.1.0.3.5.6 Surgical
  • Resection for:
    • Localized disease with persistent imaging finding
    • Hemoptysis (cavitary)
    • Failure of medical management
    • Pulmonary aspergilloma
    • Approach near large vessels (close monitoring)
243.1.0.3.5.7 Reduce Immunosuppression
  • Critical adjunct
  • Recovery from neutropenia
  • Reduce corticosteroids
  • Hold chemotherapy when feasible
  • Granulocyte transfusion historic (rare now)
243.1.0.3.6 Prognosis
  • Mortality 30-50% in invasive
  • Reduced with voriconazole + earlier diagnosis
  • Worse outcomes:
    • Persistent neutropenia
    • CNS involvement
    • Disseminated disease
    • High initial galactomannan
    • Delayed treatment

243.1.0.4 3⃣ Allergic Bronchopulmonary Aspergillosis (ABPA)

243.1.0.4.1 Background
  • IgE-mediated allergic response to Aspergillus colonization of airways
  • Asthma + cystic fibrosis (CF) primary host populations
  • ~ 1-2% asthma; ~ 7-10% CF
243.1.0.4.2 Pathogenesis
  • Aspergillus colonizes bronchi (especially central)
  • Persistent antigenic stimulation
  • Mixed Type I + Type III hypersensitivity
  • Eosinophilic + IgE-mediated inflammation
  • Mucous plugging + bronchiectasis (central)
243.1.0.4.3 Clinical
  • Worsening / recalcitrant asthma
  • Recurrent pulmonary infiltrates
  • Cough + wheeze + dyspnea
  • Sometimes severe with respiratory failure
  • Chronic productive cough (mucus plugs — brown/black)
  • Mucous plug “casts” expectorated
243.1.0.4.4 Diagnosis (ISHAM 2024 Criteria)
243.1.0.4.4.1 Required
  • Asthma OR cystic fibrosis
  • Aspergillus IgE positive + Total IgE > 1000 IU/mL
243.1.0.4.4.2 Major Criteria (≥ 2)
  • Aspergillus IgG positive (precipitins)
  • Peripheral eosinophilia
  • Central bronchiectasis on CT (cylindrical, varicose, cystic)
  • Mucous plugging
  • Pulmonary infiltrates on CXR
  • Reversible bronchoconstriction
  • Mucoid impaction on CT
243.1.0.4.4.3 Stages
  1. Acute: above criteria + active disease
  2. Remission: ≥ 6 months without symptoms/lab changes
  3. Exacerbation: relapse with above features
  4. Steroid-dependent: requires chronic steroid
  5. Fibrocavitary: end-stage with fibrosis + cavities
243.1.0.4.5 Treatment
243.1.0.4.5.1 Corticosteroids
  • Prednisone 0.5 mg/kg/day × 2 weeks → taper over 3-6 months
  • Mainstay for active disease
  • Severe / refractory: higher initial dose
243.1.0.4.5.2 Itraconazole
  • 200 mg PO bid × 16 weeks standard
  • Voriconazole alternative
  • Reduces fungal burden → reduces antigenic stimulation
  • TDM for itraconazole (variable absorption)
243.1.0.4.5.3 Anti-IgE (Omalizumab)
  • For severe / steroid-dependent ABPA
  • Especially in CF
  • Reduces IgE levels + steroid requirement
  • Off-label for ABPA but commonly used
243.1.0.4.5.4 Optimal Asthma + CF Management
  • Long-acting bronchodilators
  • Inhaled corticosteroids
  • CF: airway clearance, pancreatic enzymes
  • Manage exacerbations
243.1.0.4.6 Monitoring
  • Total IgE (reflects disease activity)
  • Imaging
  • Symptoms
  • Eosinophilia

243.1.0.5 4⃣ Aspergilloma (“Fungal Ball”)

243.1.0.5.1 Background
  • Saprophytic colonization of pre-existing lung cavity
  • Aspergillus hyphae + cellular debris form ball within cavity
  • No tissue invasion
  • Often A. fumigatus
243.1.0.5.2 Predisposing Conditions
  • Post-tuberculosis cavity
  • Sarcoidosis (especially fibrocystic)
  • COPD/Emphysema bullae
  • Lung abscess cavity
  • Pneumoconiosis (silicosis cavities)
  • Cystic fibrosis bronchiectasis
  • Bronchiectasis secondary to other causes
243.1.0.5.3 Clinical
  • Hemoptysis primary symptom (10-95%; can be massive + life-threatening)
  • Cough
  • Chest pain
  • Sometimes asymptomatic
  • Stable disease often
243.1.0.5.4 Imaging
  • CXR / CT:
    • Crescent sign (air-meniscus): air-fluid crescent between fungal ball + cavity wall
    • Movement of fungal ball with patient position change
    • Cavity within lung tissue
    • Pre-existing cavity background
243.1.0.5.5 Diagnosis
  • Imaging (characteristic appearance)
  • Aspergillus IgG positive (precipitins) — most cases
  • Sputum culture (variable; doesn’t prove invasive)
  • No invasion → no IA workup typically
243.1.0.5.6 Treatment
243.1.0.5.6.1 Observation
  • Asymptomatic stable aspergilloma → observe + monitor
243.1.0.5.6.2 Surgical Resection
  • For:
    • Symptomatic (hemoptysis)
    • Massive hemoptysis episode
    • Lobar / segmental disease
  • High-risk surgery (bleeding, lung function loss, complications in fibrotic background)
  • Lobectomy / wedge resection
243.1.0.5.6.3 Bronchial Artery Embolization
  • For acute massive hemoptysis
  • Effective short-term
  • Sometimes used as bridge or alternative to surgery
243.1.0.5.6.4 Antifungal Therapy
  • Limited efficacy in established aspergilloma (fungal ball doesn’t penetrate well)
  • Itraconazole / voriconazole sometimes used pre-surgery or with embolization
  • Not curative typically
243.1.0.5.6.5 Direct Cavity Instillation
  • Amphotericin B paste or solution into cavity (rare)
  • Historical for refractory disease

243.1.0.6 5⃣ Chronic Pulmonary Aspergillosis (CPA)

243.1.0.6.1 Background
  • Subacute / chronic Aspergillus lung disease
  • Underlying lung disease common (COPD, post-TB, sarcoidosis, bronchiectasis)
  • Borderline invasion (less aggressive than IA)
  • ~ 3 million globally
243.1.0.6.2 Subtypes
243.1.0.6.2.1 Simple Aspergilloma
  • Single cavity with fungal ball
  • Stable disease
243.1.0.6.2.2 Chronic Cavitary Pulmonary Aspergillosis (CCPA)
  • Multiple cavities expanding
  • Symptomatic course
  • Loss of lung function
243.1.0.6.2.3 Chronic Fibrosing Pulmonary Aspergillosis
  • Progressive fibrosis
  • Lung function decline
  • Mimics IPF
243.1.0.6.2.4 Chronic Necrotizing Pulmonary Aspergillosis (CNPA / Semi-Invasive)
  • Locally invasive
  • Borderline IA
  • Mild-moderate immune compromise
243.1.0.6.3 Clinical
  • Chronic cough
  • Hemoptysis
  • Weight loss
  • Fatigue
  • Months-years course
  • Mimics TB
243.1.0.6.4 Diagnosis
  • Imaging (CT): cavitations, infiltrates, fibrosis
  • Aspergillus IgG positive
  • BAL culture sometimes
  • Differential from IA, TB, malignancy
243.1.0.6.5 Treatment
  • Long-term itraconazole or voriconazole (months-years)
  • Posaconazole alternative
  • TDM essential
  • Surgical resection for select (localized cavitary disease, hemoptysis)
  • Manage underlying lung disease
  • Smoking cessation

243.1.0.7 6⃣ Resistance + Newer Diagnostics

243.1.0.7.1 Azole-Resistant Aspergillus
  • TR34/L98H mutation in CYP51A
  • First identified Netherlands 2007
  • Now widespread Europe, Asia, USA
  • Caused by agricultural azole fungicides (mancozeb, others; environmental selection)
  • Voriconazole MIC ≥ 4 mg/L = resistant
  • Other mutations: TR46/Y121F/T289A, others
243.1.0.7.2 Treatment of Azole-R Aspergillus
  • Liposomal Amphotericin B
  • Isavuconazole (variable activity — some isolates)
  • Combination (echinocandin + voriconazole + liposomal AmB)
  • Olorofim (Phase 3) — promising for resistant Aspergillus
243.1.0.7.3 Surveillance
  • AST in clinical isolates (especially in high-risk patient)
  • Environmental surveillance
  • WHO + ECDC monitoring
  • Implementation challenges + access issues
243.1.0.7.4 Newer Diagnostics
  • PCR of BAL (commercial assays emerging)
  • Lateral flow device (LFD) for galactomannan — rapid POC
  • Plasma Aspergillus PCR in development
  • Multi-marker panels combining BG + GM + PCR