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De pathologie achter de CT Bronkhorst colloquium 2013-2014 Interstitiële longziekten Katrien Grünberg, klinisch patholoog

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Presentatie over: "De pathologie achter de CT Bronkhorst colloquium 2013-2014 Interstitiële longziekten Katrien Grünberg, klinisch patholoog"— Transcript van de presentatie:

1 De pathologie achter de CT Bronkhorst colloquium Interstitiële longziekten Katrien Grünberg, klinisch patholoog

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.opfrissen Vervolgens kunt U zelf aan de slag met 3 casus. Na enkele inleidende dia’s, vindt U de links naar gescande coupes. inleidende dia’sgescande 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 Alveolar surface area: 143 m 2 Capillary surface area: 140 m 2 90% Air-blood interface surface area: 126 m 2 (70 times skin: 1.8 m2) Blood content 0.2 L RBC passes through capillary bed in 0.75 seconds Lungs are for gas exchange

5 The alveolar-capillary membrane CO 2 O2O2 0.6  m

6 The alveolar-capillary membrane O2O2 CO 2

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

8 Alveolar duct – terminal bronchiole

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

10 Airway epithelium days

11 Alveoli

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

13 Tracheobronchial lining Seromuc. Glands Goblet cell s surfactant

14 Lung vasculature Alveolar surface area: 143 m 2 Capillary surface area: 140 m 2 90% Air-blood interface surface area: 126 m 2 Blood content 0.2 L RBC passes through capillary bed in 0.75 seconds

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

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

17 Bronchus-associated lymphatic tissue (BALT)

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

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

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

27 Case 1 Case 1 pleura

28 → fibroblast foci Old and young fibrosis: temporal heterogeneity

29 Case 2 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 Nodular fibrosis in alveolar spaces: Masson bodies Lymphocytic infiltrate in alveolar septa (interstitial pneumonitis)

33 Funny looking alveolar macrophages (foamy)

34 Pattern Organizing pneumonia Mild pneumonitis Clue: foamy macrophages

35 General Differentials 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

37 Case 3 Case 3 M 75 yr Fibrosis? (EvG this staining shows fibrosis in red, elastin in black )EvG –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 EAA – hypersensitivity pneumonitis 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

45 How to bluff your way through interstitial lung disease EAA HP UIP NSIP SR-ILD (C)OP (BO)OP DIP RB-ILD DAD ═ 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)

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