Bronkhorst colloquium 2013-2014 Interstitiële longziekten De pathologie achter de CT Katrien Grünberg, klinisch patholoog K.grunberg@vumc.nl Preparing: introduction and 3 cases The introduction on microscopic anatomy of the lungs was A. Helpful / clear B. Unnecessary C. Not helpful / Confusing The cases & questions were A. Easy B. Challenging C. Tough 1
Should we treat ground glass opacity? Yes (in most cases) 2
Groundglass opacity in HRCT Wiki: ground glass opacity (GGO) is a nonspecific finding on HRCT that indicates a partial filling of air spaces by exudate or transudate, as well as interstitial thickening or partial collapse of lung alveoli. Thickening of alveolar septa Edema Inflammation Granulomas Fibrosis 3
Edema Exudate Neutrophils Eosinophils Hyalin membranes Alveolar macrophages Organizing pneumonia Granulomas Hemorrhage Alveolar macrophages Inhaled substances PVOD Alveolar filling Sarcoidosis Bacterial infection Wegeners Left heart failure Early infection PVOD DAD Atypical pneumonia Bacterial Infection More infection Eosinophilic Churg-Strauss pneumonia Churg-Strauss Asthma Parasitic infection Fungal infection DAD (1000 causes) Late phase infection Occupational exposure Smoking Left heart failure Diffuse Patchy Peripheral Focal Halo sign Collins & Stern Appl Radiol 1998 4
ILD diagnostics is about patterns Honeycombing Interlobular septa? (alveolar filling) Alveolar septa Fibrosis inflammation Cyst Septal lines Ground glass opacity Parenchyma Honeycombing Reticular Fibrosis Construction of the Tower of Babel by Pieter Brueghel the Elder (1526/1530 1569) 5
ILD is about patterns Histology Type of lesions Fibrosis, honeycombing Inflammation Fibrin/exudate/Edema Nodules Clues Distribution Bronchiolocentric Angiocentric Alveolar Interstitial Lymphatic (sub)pleural Radiology Pattern of radio-opacity Hypo- or hyperdensity Ground glass Reticulation Honeycombing Nodules Distribution Upper-mid-lower Centrilobular (angio-/bronchio) Diffuse Lymphatic (sub)pleural Fibrosis: distribution Intersitial or airspace Centrilobular or subpleural Lymphatic Basal / mid/hilar / upper fields Lymphatic Centrilobular distribution 6
Fibrosis: distribution Case 1 Case 3 Inflammation Composition neutro/eosinophil T cell Histiocyte, granulomatous B-cell, plasmacell Distribution Interstitial (centrilobular/diffuse) Bronchiolitis Vasculitis 7
Neutrophils Inflammation B cells 64% Bronchitis Follicular bronchitis Histiocytes T cells Granuloma Pneumonitis Pneumonitis Case 3 8
Hyaline membranes Diffuse alveolar damage Congestion/hemorrhage Pulmonary veno-occlusive disease 9
Clues Necrosis Pigmentation Iron (bleeding) Inhaled stuff Funny looking cells and such Pigmentation DIP Iron, calcium salts in blood vessel pseudopneumoconiosis 90% 10
Pigmentation Plexogenic pulmonary arteriopathy in enzyme-substituted Gaucher s disease. A case report. Den Bakker et al. Histopathology 2012 Inhaled stuff Plant Anthracosis Asbestos 11
Funny looking cells Langerhanscell histocytosis CD1a Histopathology patterns Type of lesions Fibrosis, honeycombing Inflammation Fibrin/exudate/Edema Clues Distribution Bronchiolocentric Angiocentric Alveolar Interstitial Lymphatic (sub)pleural 12
Case 2 M 71 yr. History of myocardial infarction. Fibrosis. Where? 13
Nodular fibrosis. Where? And some infiltrate. Where? 14
Funny looking alveolar macrophages (foamy) Seek the difference Masson body (Intra-alveolar fibrous plug) Organizing pneumonia Fibroblast focus (part of the alveolar wall) UIP Case 2 Case 1 15
Case 2 Fibrosis? Yes, plugs in alveolar spaces Inflammation? Yes, lymphocytes (T cells) in alveolar interstitium Descriptive diagnosis? Organizing pneumonia DD? Infection, toxic/medication, CVD,, cryptogenic Case 3 M 75 yr 16
Inflammation, bronchiolocentric bronchiolocentric inflammation: lymphocytes (T cells) 17
Clues Clues (detail) 18
Case 3 Fibrosis? Hardly Inflammation? Yes, lymphocytes (T cells), pneumonitis, bronchiolocentric distribution Clues? Poorly formed granulomas Descriptive diagnosis? Bronchiolocentric pneumonitis with poorly formed granulomas DD? Hypersensitivity pneumonia (EAA) Should What explains we What treat Treatment? is the the ground diagnosis? glass glass opacity? 19
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