De pathologie achter de CT

1. De pathologie achter de CT Bronkhorst colloquium 2013-2014 Interstitiële longziekten Katrien Grünberg, klinisch patholoog K.grunberg@vumc.nl

2. Voorbereiding • De opzet van de lezing is om u kennis te laten maken met een aantal histopathologische afwijkingen en patronen. Tijdens de cursus zal verder worden ingegaan op de vertaling van de histologie naar CT. • Om de histologische afwijkingen van de longen te kunnen herkennen, is kennis van de normale microscopische anatomie essentieel. Neem de volgende dia’s door als U uw kennis wilt opfrissen. • Vervolgens kunt U zelf aan de slag met 3 casus. Na enkele inleidende dia’s, vindt U de links naar gescande coupes.

3. Even opfrissen • Om de histologische afwijkingen van de longen te kunnen herkennen, is kennis van de normale microscopische anatomie essentieel. Neem de volgende dia’s door als U uw kennis wilt opfrissen.

4. Lungs are for gas exchange Alveolar surface area: 143 m2 Capillary surface area: 140 m2 90% Air-blood interface surface area: 126 m2 (70 times skin: 1.8 m2) Blood content 0.2 L RBC passes through capillary bed in 0.75 seconds

5. The alveolar-capillary membrane CO2 0.6 m O2

6. The alveolar-capillary membrane O2 CO2

7. Core business Gas exchange Infrastructure and Logistics Conducting airways Conducting vessels Innervation Defence Lungs

8. Alveolar duct – terminal bronchiole

9. Bronchioli alveoli airway smooth muscle epithelium lumen pulmonary artery Bronchovascular bundle

10. Airway epithelium 2 - 3 days

11. Alveoli

12. Surfactant producing cells:Type II pneumocytes and Clara cells Alveolar surface area: 143 m2 Barry R. Stripp et al. Am. J. Respir Cell Molec Biol. 2002;. 27: 170-8

13. Tracheobronchial lining Seromuc. Glands Goblet cell s surfactant

14. Lung vasculature Alveolar surface area: 143 m2 Capillary surface area: 140 m2 90% Air-blood interface surface area: 126 m2 Blood content 0.2 L RBC passes through capillary bed in 0.75 seconds

15. Lymph vessel Arteries and veins Axial artery Interlobular septum Vein Bronchiole

16. Arteries • Elastic artery (> 1 mm ) • bronchi • Muscular artery (50-1000 m) • bronchioli • Arterioles (< 50 m) • Alveolar parenchyma

17. Bronchus-associated lymphatic tissue (BALT)

18. Around p. arteries In interlobular septa Lymph vessels D-240

19. Interlobular septum The secondary lobule Pulm artery Vein Lymph vessel Airway

20. Inleiding in histopathologie van ILD

21. ILD is about patterns • Type of lesions • Fibrosis • Inflammation • Distribution

22. Fibrosis: distribution • Intersitial or airspace • Centrilobular or subpleural • Basal fields vs upper fields Lymphatic Centrilobular distribution

23. Inflammation • Composition • lymphocytic, plasmacell, neutro/eosinophilic • granulomatous • Distribution • Interstitial (centrilobular/diffuse) • Bronchiolitis • Vasculitis

24. Inflammation

25. Clues • Necrosis • Hyaline membranes (as in diffuse alveolar damage) • Pigmentation • Iron (bleeding) • Inhaled stuff • Funny looking cells and such

26. Case 1 • Fibrosis? • Distribution (centrilobular or subpleural?) • Inflammation? • Type? • Clues? • Descriptive diagnosis • Summary of pattern of fibrosis and inflammation, clues • DD

27. Case 1 pleura

28. Old and young fibrosis: temporal heterogeneity →fibroblast foci

29. Case 2 • M 71 yr. History of myocardial infarction. • Fibrosis? • Inflammation? • Distribution? • Airspaces? • Pattern • DD

30. Fibrosis. Where?

31. Nodular fibrosis. Where? And some infiltrate. Where?

32. Lymphocytic infiltrate in alveolar septa (interstitial pneumonitis) Nodular fibrosis in alveolar spaces: Masson bodies

33. Funny looking alveolar macrophages (foamy)

34. Pattern • Organizing pneumonia • Mild pneumonitis • Clue: foamy macrophages

35. General Differentials • Infection • Infection • Vasculitis • Collagen vascular disease / AID / IgG4 • Occupational/recreational toxic stuff (inhaled) • Drugs (iv, otherwise) • Idiopathic fibrosis • Other (inborn)

36. Clue • Patient used amiodarone • OP, pneumonitis, foamy macrophages all fit with amiodarone-induced ILD • http://www.pneumotox.com

37. Case 3 • M 75 yr • Fibrosis? (EvGthis staining shows fibrosis in red, elastin in black) • Specifics • Inflammation? • Specifics • Distribution? • Pattern • DD

38. Inflammation, bronchiolocentric

39. bronchiolocentric inflammation: lymphocytes (T cells)

40. Clues

41. Giant cells in poorly formed granulomas

42. Pattern • Minimal fibrosis • Bronchiolocentric interstitial pneumonia • Clue: poorly formed granulomas Differential Dx • …

43. Clue • Patient kept pigeons

44. Bird fancier's lung  Also called bird breeder's lung, pigeon breeder's lung, and poultry worker's lung Bagassosis Cephalosporium HP Cheese-washer's lung Chemical worker’s lung - Isocyanate HP Chemical worker's lung[9] - Trimellitic anhydride (TMA) HP Coffee worker's lung Compost lung Detergent worker's disease Familial HP Also called Domestic HP Farmer's lung Hot tub lung Humidifier lung Japanese summer house HP Also called Japanese summer-type HP Laboratory worker's lung Lycoperdonosis Malt worker's lung Maple bark disease Metalworking fluids HP Miller's lung Mollusc shell HP Mushroom worker's lung Peat moss worker's lung Pituitary snuff taker's lung Sauna worker's lung Sequoiosis Streptomyces HP Suberosis Tap water HP Thatched roof disease Tobacco worker's lung Wine-grower's lung Woodworker's lung EAA – hypersensitivity pneumonitis

45. How to bluff your way through interstitial lung disease • Extrinsic allergic alveolitis • Hypersensitivity pneumonitis • Usual interstitial pneumonia • Non-specific interstitial pneumonia • Smoking-related interstitial lung disease • (Cryptogenic) Organizing Pneumonia • (Bronchiolitis Obliterans) Organizing Pneumonia • Desquamative interstitial pneumonia • Respiratory bronchiolitis - ILD • Diffuse alveolar damage (≈ ARDS) EAA HP UIP NSIP SR-ILD (C)OP (BO)OP DIP RB-ILD DAD