RespiratoryTier 2Disease (DEADMAN)

Pulmonary vasculitides

Description

A group of ANCA-associated and other systemic vasculitides with characteristic pulmonary involvement - granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), and eosinophilic granulomatosis with polyangiitis (EGPA) are the principal ANCA-associated conditions affecting the lung

Epidemiology

Pulmonary involvement can be the presenting feature or develop later in the disease course; diffuse alveolar haemorrhage is a medical emergency across all three conditions

Aetiopathogenesis

Condition	ANCA pattern	Classic pulmonary feature
GPA	c-ANCA/PR3 (majority)	Cavitating nodules, tracheal/subglottic stenosis, alveolar haemorrhage
MPA	p-ANCA/MPO (majority)	Diffuse alveolar haemorrhage without granulomas
EGPA	p-ANCA/MPO (minority positive)	Asthma (often severe, preceding vasculitis by years) + eosinophilia + pulmonary infiltrates

Diagnosis

ANCA serology (with pattern/antigen specificity) + tissue biopsy (lung, kidney, or other affected organ) for histological confirmation
Pulmonary-renal syndrome (alveolar haemorrhage + glomerulonephritis) is a key presentation pattern shared with anti-GBM disease (Goodpasture's) - differentiate serologically

Management

Induction immunosuppression (high-dose corticosteroids + cyclophosphamide or rituximab) for severe/organ-threatening disease including alveolar haemorrhage
Maintenance therapy (lower-dose steroid + azathioprine/methotrexate/rituximab) thereafter
Plasma exchange considered for severe alveolar haemorrhage or rapidly progressive glomerulonephritis

Associations

Rapidly progressive glomerulonephritis, diffuse alveolar haemorrhage, asthma (EGPA), upper airway disease (GPA - saddle nose deformity, subglottic stenosis)

Natural history & complications

Untreated organ-threatening disease (alveolar haemorrhage, rapidly progressive GN) carries high short-term mortality; with modern immunosuppression, remission is achieved in the majority, though relapse is common and long-term monitoring is required

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