Pulmonary alveolar proteinosis

PAP

1. PULMONARY ALVEOLAR
PROTEINOSIS
Dr.Annapurna

2. •
Abnormal intra alveolar accumulation of floccular
lipoproteinaceous material derived from surfactant
phospholipids and lipoproteins

• first diagnosed in 1958
• may be congenital-2% (thymic alymphoplasia)
idiopathic-90%
secondary-5-10%

3. Etiopathogenesis:
• alveoli filled with lipoproteinaceous material
similar to normal surfactant containing lipids &
proteins A,B,C,D
• defect in clearance by alveolar macrophages
• increased production

4. •
Secondary causes include:
lung infections-PCP,Nocardiosis
inhalation of silica,TiO2,Al,Indium,insecticides

haematological malignancies
HIV/AIDS
DMARD-Leflunomide
GM-CSF Signalling defects

5. Clinical features
• 20-50 years males, smokers
•1/3 asymptomatic
• 1/3: persistent dry cough
progressive dyspnoea
fatigue,malaise,weight loss,failure to thrive
intermittent low grade fever,night sweats
pleuritic chest pain,hemoptysis
• 1/3 land into PHTN & corpulmonale
• Clubbing,cyanosis,fine end inspiratory crackles

6. Work up
• IMAGING:
CXR – bilateral perihilar infiltrates with consolidation
bat wing (apices,cp angle sparing)
reticulonodular shadows
HRCT – CRAZY PAVING PATTERN
(patchy ground glass opacities
interlobular septal thickening
intralobular interstitial thickening
polygonal pattern

9. Differential diagnosis
• cardiogenic pulmonary edema
• p.jiroveci pneumonia
• sarcoidosis
• hypersensitivity pneumonitis

10. •
flexible bronchoscopy with bal
appearance of BAL is milky white
increased proteins-SP A,D & in serum
increased serum & BAL autoantibody against
GM-CSF
increased LDH

12. •
lung biopsy
• transbronchial biopsy
• alveoli filled with non foamy lipo proteinaceous
material

13. treatment:
• steroids
• mucolytics
• aerosol proteinases
• treating underlying cause
• whole lung lavage
•Prognosis: poor in neonates
• adults- mortality rate is less than 10%

14. THANK YOU