De pathologie achter de CT

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1 De pathologie achter de CT
Bronkhorst colloquium Interstitiële longziekten De pathologie achter de CT 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. 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
1.6 mL/1m2! 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
CO2

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

8 Alveolar duct – terminal bronchiole

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

10 Airway epithelium days

11 Alveoli Illustrate pores of cohn

12 Surfactant producing cells: Type II pneumocytes and Clara cells
Figure 1. Transmission electron microscopic appearance of Clara cells in terminal bronchioles of wild-type (A) and CCSP-/- mice (B). The apex of the cell projected into the airway lumen and the nucleus (N) was located in the basal portion of the cell. Electron dense secretory granules were found near the apical cytoplasmic membrane in WT mice (arrow), but were absent among Clara cells from CCSP-/- mice (magnification: bar = 1.0 µm). 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 Arteries and veins Axial artery Interlobular septum Lymph vessel Vein
Plaatjes van bronch arterie en interlob vene Vein Bronchiole

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

17 Bronchus-associated lymphatic tissue (BALT)

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

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

20 Inleiding in histopathologie van ILD

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

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

23 Inflammation Composition Distribution
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? Inflammation? Clues? Descriptive diagnosis DD
Distribution (centrilobular or subpleural?) Inflammation? Type? Clues? Descriptive diagnosis Summary of pattern of fibrosis and inflammation, clues DD A93 (uip pattern) centrilobular, possibly LCH Similar case A78

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 A82 amiodarone Consult Spaane Ziekenhuis (S12-20), longen: Uitgebreide en gevorderde organiserende pneumonie van beide longen, met slechts een focaal en gering diffuus alveolair schade beeld. Het beeld is niet specifiek is maar kan goed passen bij Amiodarone gebruik. Verminderd beoordeelbaar t.a.v. vaatpathologie, onvoldoende argumenten voor hypoxische - of congestieve vasculopathie.

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 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 M 75 yr Fibrosis? (EvG this staining shows fibrosis in red, elastin in black) Specifics Inflammation? Distribution? Pattern DD A80-79 EAA Consult Kennemer Gasthuis (T ), chirurgische longbiopten (I ROK, II RBK): Complex beeld van overwegend bronchiolocentrisch georiënteerde interstitiële fibrose, pneumonitis en granulomen waarbij in eerste plaats een extrinsieke allergische alveolitis overwogen wordt. Voor besprekingen verdere overwegingen zie microscopie.

38 Inflammation, bronchiolocentric

39 bronchiolocentric inflammation: lymphocytes (T cells)

40 Clues

41 Giant cells in poorly formed granulomas

42 Differential Dx … 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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