Chest imaging II. Interstitial lung diseases

Transcription

1 Chest imaging II. Interstitial lung diseases Dávid L. Tárnoki MD, PhD Ádám D. TárnokiMD, PhD Department of Radiology Semmelweis University

2 Topics 1. Interstitial lung diseases 2. Occupational lung diseases 3. Pleura 2

3 Interstitial lung diseases 3

4 Anatomy Interstitial lung diseases Secondary lobule: 1-2,5 cm ø Centrilobular artery: 0,5-0,7 mm ø Centrilobular bronchiole (wall: 0,15 mm) bronchiolus terminalis terminal ducts acini alveoli Radiologyassistant; Webb WR, Radiology 2006;239 4

5 Interstitial lung diseases Anatomy Interstitium: Pulmonary arteries and veins Lymph vessels, lymph nodes Nerves Fibroblasts Collagen fibers Elastic fibers Lynch PJ, Creative Commons, 5

6 6 Interstitial lung diseases Pathogenesis - Overview Noxen (aerogenous, circulatory) Damage of the alveolar /from circulation/ endothelial membrane Remodeling Inflammation cells from circulation / interstitium (activation and recruitment) Granuloma Fibrosis

7 7 Interstitial lung diseases Pathogenesis - Overview Alveolar block (real diffusion disorder) Reduction of the alveolar space Reduction of the capillary diffusion area Reduction of the Diffusion constants (in anemia) GGO Reticulation Honeycombing

8 Definition Interstitial lung diseases Caused by chronic inflammation of the pulmonary interstitium with a scarred reconstruction of connective tissue proliferation due to different causes Over 150 triggers (allergens, Medicines, etc.) Half of the cases remain unclear Mostly affected alveoli and interstitium Airways and pulmonary vessels are often secondary affected 8

9 Interstitial lung diseases Clinics Unproductive cough Stress dyspnoea Drumstick fingers Clubbed (Watch glass) nails Basal crackling Weakness, weight loss Full history required: - Medicines - Job - Hobbies - "pleasure poisons" 9

10 Diagnostics Interstitial lung diseases Ideally by multidisciplinary ILD board: pneumologists, rheumatologists, radiologists and pathologists Laboratory: - Kidney functions, electrolytes, CRP - Differential blood counts - Antibodies Lung function (Restrictive ventilation fault) Imaging (Rö and HRCT) Bronchoscopy with transbronchial biopsy (TBB) and bronchoalveolar lavage (BAL) Open lung biopsy (VATS) 10

11 Interstitial lung diseases Clinics What kind of information the clinician needs from us? Diagnosis UIP (Usual interstitial pneumonia) biopsy is not necessary Other DD? biopsy is necessary Prognosis acute / cronic Comparison with previuos HRCT: progression / regression? Therapeutic options - Steroids - Lung transplantation Prognosis - Median survival after diagnosis years 11

12 12 Interstitial lung diseases Typical patterns of an ILD Ground glass opacity, consolidation Ground glass opacity (GGO) Ground glass opacities (Milk glass opacity - increased parenchyma decreasement) partially filled and / or collapsed alveoli e.g. active inflammation Consolidation - Consolidation completely filled and / or collapsed alveoli (Accumulation of exudate, transudate, or other tissue in the alveoli)

13 Interstitial lung diseases Ground glass opacity, consolidation Consolidation GGO 13

14 Interstitial lung diseases Idiopathic pulmonary fibrosis (heterogeneous entity) AIP (acute interstitial pneumonitis) UIP (usual interstitial pneumonitis) 70% DIP (desquamative interstitial pneumonia) RBILD (respiratory bronchiolitis ILD) NSIP (non specific interstial pneumonia) BOOP=COP (bronchiolitis obliterans organizing pneumonia = cryptogenic organizing pneumonia) Why do we need to subtype the idiopathic interstitial Pneumonias? Different prognosis which results different therapeutic approaches 14

15 Interstitial lung diseases X-Ray - Fine-stripping-reticular pattern that is superimposed by a diffuse transparency reduction - Increased reticulation with basal dominance NORMAL FIBROSIS 15

16 Interstitial lung diseases HRCT Plays a central role in detection, diagnosis and differential diagnosis Sufficiency of assessability with a layer thickness of 1.25 mm or less Acut Alveolitis: GGO the location of optimal bronchoscopic tissue collection Chronic: Nodules, lines and bands, pleural thickening Final stadium: Honeycombing Beehive pattern" 16

17 Interstitial lung diseases Basic pattern in HRCT linear reticular pattern nodular pattern Structures with density reduction (Lesions with "less air") Structures with density increase (Lesions with more air") 17

18 Interstitial lung diseases ILD classification according to ATS / ERS 18

19 19 Interstitial lung diseases 1. AIP (acute interstitial pneumonia) Acute alveolitis: milk opacity (density increase with still recognizable bronchovascular structures) "idiopathic form of ARDS" / ARDS of unclear cause Acute and short prognosis HRCT image: Acute: - homogeneous GGO (alveolitis) - diffuse consolidation Chronic: honeycombing, fibrosis

20 Interstitial lung diseases 2. NSIP (Non-specific interstitial pneumonia) Chronic form Manifestation: 4. Life decade Not tobacco-associated HRCT Image: - GGO - Peripheral, basal, subpleural, symmetrical fine reticular condensation, traction bronchiolectasis - no honeycomb pattern (no honeycombing) Usually occurs as a pulmonary involvement in the context of collagenosis Importantly, the NSIP. can be treated with systemic glucocorticosteroids prognosis more favorable than with UIP 20

21 Interstitial lung diseases 3. UIP (Usual Interstitial Pneumonia) Simultaneity of inflammation, proliferation and fibrosis HRCT is very specific: - GGO (signs of active inflammation and fibroblast proliferation) - Honeycomb patterns (honeycombing), bronchiectasis as signs of fibrosis (not present in NSIP!) - subpleural and basally stressed localization - Reticular pattern - Lack of changes that are not associated with UIP are compatible. Probability a correct diagnosis of a UIP at nearly 100% with HRCT Prognosis: poor 21 manju-imagingxpert.blogspot.com

22 Interstitial lung diseases Severe pulmonary parenchymal changes Normal or pathologic image? Fibrosis ( 22

23 Interstitial lung diseases Honeycombing: subpleural localized, multi-rowed, small cystic changes 23

24 24 Interstitial lung diseases Regardless of the cause End stage: honeycomb lung Upper lobes: signs of activity (GGO, alveolitis) Lower lobes: honeycombing

25 Interstitial lung diseases Lung fibrosis: honeycombing pattern Patients with a typical UIP pattern in HRCT: does not need lung biopsy 25

26 Interstitial lung diseases DD: Fibrosis after radiation (due to breast cancer) 43 year old woman 26

27 HRCT Interstitial lung diseases 3D reconstructions Lung volumen Pattern softwares 27

28 Interstitial lung diseases UIP (Usual Interstitial Pneumonia) Diaphragm movement disorders - ultrasound M-mode Complication: shrinking lung 28

29 Interstitial lung diseases 4. Collagenoses and vasculitides Pulmonary manifestations: - Rheumatoid arthritis (RA) - Systemic Lupus Erythematosus (SLE) - Sjögren syndrome - Systemic sclerosis / scleroderma - Dermatopolymyositis - Wegener granulomatosis X-ray: less sensitive In pronounced cases: Basal-dominant reticulonodular pattern HRCT: Patological findings in 30% pleural thickening Late stage: fibrosis What will the clinician ask from the radiologist? Disease characterization and expansion. UIP or NSIP patterns? 29

30 Occupational lung diseases 30

31 Interstitial lung diseases ILD classification according to ATS / ERS 31

32 Occupational lung diseases New diseases due to occupational regulations Chronic inhalation of inorganic dusts (e.g., silicate) Long-time exposure (e.g., mineral workers) Alveolar phagocytosis of inhaled particles and interstitial deposition Interstitial reticulo-granuloma formation, sometimes massive fibrosis Therapy: Exposure stop I. DISEASES OF IMMUNOLOGICAL / UNCLEAR AETIOLOGY II. PNEUMOCONIOSIS (Inhaled Particles) X-Ray: Nodular herd often with calcifications Hilary / mediastinal lymph nodes with calcifications ("laryngeal calcification") HRCT: X-ray patterns + Micronodular lesions pulmonary fibrosis 32

33 Occupational lung diseases I. DISEASES OF IMMUNOLOGICAL / UNCLEAR AETIOLOGY 1. Organising pneumonia (OP) Idiopathic form: Cryptogen-organizing pneumonia (COP) Non-infectious inflammation that may occur in collagenous diseases, chronic eosinophilic pneumonia, exogenous allergic alveolitis, infections or even as a medication reaction Symptoms: chronic subfebrile temperatures, dyspnoea and cough HRCT: Band-shaped, subpleural and peribronchial stressed focal consolidations with basal predominance - migrating consolidations Atoll sign: by a ring-like consolidation with central milk glass Prosch H. Journal für Pneumologie 2015; 3 (1),

34 Occupational lung diseases I. DISEASES OF IMMUNOLOGICAL / UNCLEAR AETIOLOGY 2. Exogen Allergic Alveolitis, EAA ( Farmer lung, Hypersensitivity pneumonitis, HP) Immune reaction of the alveoli and bronchioles Inhalation of organic dusts (actinomycetes, aspergilli, excrements, flour), chemotherapeutic agents Anamnesis! X-ray: normal in the acute / subacute stage HRCT: Acute / subacute stage: Milk glass infiltrates (centrilobular) reticular pattern bronchial wall thickening Chronic stage: Fibrosis (X-Ray) Prosch H. Journal für Pneumologie 2015; 3 (1),

35 3. Silikosis Occupational lung diseases II. PNEUMOCONIOSIS (Inhaled Particles) Inhalation of mineral dusts (e.g., SiO2, coal) CT: - Nodules - Hiliary and mediastinal lymphadenopathy Eggshell calcifications - Complicated pneumoconiosis: fibrosis

36 4. Asbestosis Occupational lung diseases II. PNEUMOCONIOSIS (Inhaled Particles) Inhalation of asbestos fibers Manifestation years after exposure CT: - interlobular septal thickening - Honeycomb pattern (honeycombing) - Pleural plaques Malignisation: pleural mesothelioma, bronchial carcinoma 36

37 37 Asbestosis Occupational lung diseases Community Houses

38 Pleura 38

39 Ultrasound Pleura Artefacts: B Lines In ILD patients pleural effusion is not typical. Pleura irregularities! Pleura lines 39

40 40 Pleura Mesothelioma % of all asbestosis cases develop with a latency of years - High-risk groups: construction workers, carpenters, electricians, vehicle workshops - Clinical: chest pain, dyspnoea, weight loss, sometimes fever - Infiltrations of the thorax wall and the ribs come before survival is an average of 18 to 28 months - X-Ray, CT: elongated or glandular pleural thickening, thickness over 10 mm pleural calcifications X-Ray CT US

41 Summary For the characterization of interstitial changes, the Computed tomography (HRCT) is the method of choice None of the samples presented is specific for a disease (UIP only) The suspicious diagnosis results from the morphology, the distribution of findings, the dynamics and the clinic discussion with the clinicans (!) 41

42 Summary 1. What kind of information needed for the clinicians? - Diagnosis of the ILDs, biopsy, alveolitis or fibrosis patterns 2. X-Ray: Fine-striated reticular pattern, basal dominance 3. HRCT-Signs: Acute inflammation (alveolitis): GGO Chronic: Honeycombing 4. Difference between NSIP and UIP: NSIP: no honeycombing UIP is very specific: honeycombing 42

43 Summary 5. Consequences of asbestosis: pleural mesothelioma, bronchial carcinoma 6. X-ray and CT signs of sarcoidosis: Bilateral hilar and mediastinal lymphnodes, intrapulmonary changes. 43

44 44 You only see what you know (Johann Wolfgang von Goethe) Thank you! Thank you for the images: Dr. Monostori und Dr. Judit Pápay