Pulmonary alveolar proteinosis is a rare lung disease characterized by abnormal accumulation of lipoproteinaceous material in the alveoli. It was first diagnosed in 1958. The accumulation is caused by a defect in clearance by alveolar macrophages or increased surfactant production. Secondary causes include lung infections, inhalation of toxins, and hematological malignancies. Symptoms vary from asymptomatic to cough, dyspnea, and respiratory failure. Diagnosis involves imaging showing characteristic patterns and analysis of material obtained by bronchoscopy or biopsy showing accumulation of lipoproteins in the alveoli. Treatment options include steroids, whole lung lavage, and treating any underlying causes. Prognosis is generally good with treatment

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