Diffuse Alveolar Hemorrhage Flashcards
2 circulations in lung
Bronchial (high pressure, low volume)
Pulmonary (low pressure, high capacitance)
Bronchial circulation supplies __
Conducting airways (mainstem bronchi until terminal bronchioles)
Bronchial circulation arises from:
Aorta
Source of low-grade, chronic and diffuse alveolar hemorrhage
Pulmonary circulation
Bleeding in cystic fibrosis is due to:
Bronchiectasis
Infection presenting with hemoptysis
S. pneumoniae
S. aureus
Timeline of hemosiderin-laden macrophages
3 days: first appear
7-10 days: peak
2 months: resolution
Most common ANCA-associated vasculitis presenting with DAH from pulmonary capillaritis
MPA
Vessels affected by ANCA vasculitis
Small and medium
Necrotizing vasculitis of small- and medium-sized vessels with granulomatous inflammation
GPA
Clinical features are chronic rhinitis, sinusitis, OM, nasal cartilage destruction (saddle nose), salivary gland swelling, SG stenosis, tracheobronchial ulceration, parenchymal nodules that may cavitate, DAH
GPA
Antibodies in GPA
Anti-PR3 (c-ANCA pattern)
Gold standard for diagnosis of GPA
Biopsy: vasculitis and capillaritis with necrotizing granulomata, paucity of immune complexes
Antibodies in MPA
Anti-MPO (p-ANCA pattern)
Biopsy in MPA
PC with neutrophilic infiltration of small arterioles, venues, and capillaries with fibrinoid necrosis
Treatment of ANCA vasculitis
Induction with GC and cyclophosphamide for 3-6 months
Maintenance with prednisone, methotrexate, azathioprine for 1-2 years
Others:
Rituximab, IVIg, plasmapharesis
Vasculitis limited to lung and kidney
Anti-GBM disease
Diagnosis of anti-GBM
Anti-GBM antibodies in serum and basement membrane of lung and kidney
Treatment of anti-GBM
CS
Cyclophosphamide
PLASMAPHERESIS in all cases
PC with alveolar hemorrhage without renal or other systemic manifestations
IPC
Differentiates IPC from IPH
Lower Hgb, higher ESR in IPC
Treatment of IPC
Same as ANCA vasculitis
Autosomal dominant pulmonary hemorrhage syndrome
COPA Syndrome
Pathophysiology of COPA Syndrome
Dysfunctional autophagy
Th17 skewing and immune dysregulation
Chronic, relapsing and remitting alveolar hemorrhage
CT findings of COPA
Cystic disease
Biopsy of IPH
Bland alveolar hemorrhage, absence of inflammation
Treatment of IPH
CS
Steroid-sparing:
Hydroxychloroquine
Azathioprine
Others:
6-mercaptopurine
Cytologic finding in DAH
Hemosiderin-laden macrophages
Management of massive hemoptysis
High PEEP
Double lumen ET
Bronchial artery embolization
