PULMONARY ALVEOLAR PROTEINOSIS Dr.Annapurna

• 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%

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

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

HIV/AIDS DMARD-Leflunomide GM-CSF Signalling defects

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

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

Differential diagnosis

• cardiogenic pulmonary edema• p.jiroveci pneumonia• sarcoidosis• hypersensitivity pneumonitis

• 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

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

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

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