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Restrictive Lung Diseases Slide 2 1. Adult respiratory distress syndrome 2. Sarcoidosis 3. Asbestosis 4. Neonatal respiratory distress syndrome 5. Idiopathic Pulmonary fibrosis Restrictive Lung Diseases Slide 3 Acute respiratory distress syndrome 1.Def: Diffuse damage primarily to type 1 pneumocytes + capillary: followed by injury of type 2 cells and endothelium. 2.Cause: gm - ve Sepsis, trauma, lung infection etc. 3.Pathogenesis: 1.Diffuse alveolar damage: DAD 2.In E coli infection this DAD is mediated by free radicals from Neutrophils. Slide 4 Respiratory membrane Alveolar sac Slide 5 Hyaline membrane Slide 6 ARDS Morphology: Gross: lung is diffusely firm, airless, and rubbery. Micro: Hyaline membrane Clinical: acute dyspnea, cyanosis, hypoxemia. X ray: white out : bilateral lung opacity Unresponsive to 100% oxygen treatment. Mortality is between 30-40%. Slide 7 Intra alveolar hyaline membrane: fibrin and cell debris reduced oxygen diffusion. Whiteout End Slide 8 Neonatal respiratory distress syndrome Reduced surfactant due to defective pneumocytes 2. Clinical similar to ARDS Slide 9 Sarcoidosis Slide 10 Sarcoidosis Definition: disease with non caseating granulomas, can involve any organ. Most common presentation. Bilateral hilar lymphadenopathy and /or lung involvement. Hyper-calcemia is present ( due to high 1,25- dihydroxyvitamin D by sarcoid macrophage). Slide 11 Morphology: Micro Microscopy : Non caseating epithelioid cell granulomas in all effected organs with giant cells. Hilar node: These lympnnode may undergo calcification: egg shell calcification. Slide 12 Asteroid bodies Schuamann body Giant cells contain these structures Concentric calcification Slide 13 Clinical Course of Sarcoidosis Asymptomatic, discovered incidentally (many cases). Dry cough/ night sweat/ fever. Eye involvement: Mikulicz syndrome: corneal opacity, blindness. Skin involvement: erythema nodosum (painful erythematous plaques or rash). Slide 14 Sarcoidosis : diagnosis Final diagnosis is by lung LN biopsy or biopsy of the skin or eye lesion. Helpful: CD4/CD8 ratio >2.5 ( in bronchoalveolar lavage lymphocytes ) PPD negative. End Slide 15 Asbestosis Cause: Asbestos fibers Morphology of lung: Early: diffuse interstitial fibrosis in lower lobe and fibrous pleural plaque [parietal pleura]. Late stage: honeycomb lung. Complications: bronchogenic carcinoma, mesothelioma. Slide 16 Asbestos fibers : morphology Asbestos body or Ferruginous body which is positive with Prussian blue. Amphibole (thin and straight) and serpentine (curly. Flexible, common ). Present in extracellular tissue in lung interstitium (initial ingested by macrophage that eventually dies off). Slide 17 Asbestosis : morphology Ferruginous body which is positive with Prussian blue. They arise when macrophages attempt to phagocytose asbestos fibers. Slide 18