Presentation on theme: "Radiology of Connective Tissue Disease associated Interstitial Lung Disease John Murchison."— Presentation transcript:
1 Radiology of Connective Tissue Disease associated Interstitial Lung Disease John Murchison
2 Why do HRCT?Superior to CXR and conventional CT at showing parenchymal abnormalitiesWith MDCT all CT chests are effectively HRCT
3 Indications for HRCT Is lung disease present? What is the nature of the abnormality?Are changes acute or chronic?Follow up to assist managementSelection of biopsy site
4 Indications for HRCT Is lung disease present? Much more sensitive at assessing lung parenchma than CXRSuperimposed structures? Normal ? AbnormalPFT abnormalities and apparently normal CXRIs further management required?Or no need eg emphysema
5 Indications for HRCT What is the nature of the abnormality? Sometimes able to give a specific diagnosisBronchiectasis, UIP, emphysemaNarrows the differential diagnosisAppropriate selection of further tests
6 Indications for HRCT Are changes acute or chronic? Chronic eg fibrosisAcute ground glassFollow up to assist managementHas disease progressed / improved?Selection of biopsy siteMany diffuse lung diseases have a patchy distribution. Select active diseaseAvoid end stage fibrosis
8 Scanning Variants Prone scan- supine dependant changes often seen particularly at lung basesIf concern that may be obscuring early fibrosis or that changes may not be genuine do prone scanprone
9 Scanning Variants Prone scan- supine dependant changes often seen particularly at lung basesIf concern that may be obscuring early fibrosis or that changes may not be genuine do prone scanprone
10 Scanning Variants Inspiration Expiratory scan Expiration Demonstrates air trappingIf concern re obstructive lung disease eg PFTsHyperlucency on HRCTIf particularly looking for conditions where air trapping likely eg Bronchiolitis obliteransIn normal patients HU increases uniformly on expirationIf air trapping HU remains lowExpiration
11 Patterns of air space opacification consolidationground glass
12 Ground Glass ground glass attenuation Ground glass attenuation may be correlate witha) evidence of interstitial inflammation with airspace filling by macrophagesb)patchy fibrosis orc) a combination of above.
13 HRCT features of fibrosis, Intra-lobular and inter-lobular septal thickening, walled cysts representing honeycombing,may be associated traction bronchiectasis
14 DPLD IPF / CFA NSIP COP AIP RB-ILD DIP LIP Diseases of known causeor associationGranulomatous diseasesIdiopathic interstitial pneumoniasOthersConnective tissue diseasesDrug-induced diseasesAsbestosisPneumoconiosisSarcoidosisUIPNSIPAIPLIPCOPRB-ILDDIPEosinophilicdiseasesRare diseasesIPF / CFAHypersensitivity pneumonitis
15 CXR-UIPInitially ill-defined or ground-glass opacities, peripheral reticular opacities,As disease progresses reticular pattern becomes coarser, most marked at bases, often volume loss, end stage diffuse honeycombing.
16 HRCT1) features of fibrosis, Intralobular septal thickening, walled cysts representing honeycombing, may be associated traction bronchiectasis2)ground glass attenuation common but usually less than reticular abnormalities. Ground glass attenuation may be correlate with a) evidence of interstitial inflammation with airspace filling by macrophages b)patchy fibrosis or c) a combination of above.3)characteristically a peripheral basal distribution
17 Radiological differential diagnosis in ‘IPF’ An HRCT that predominantly shows bibasal honeycombing is virtually 100% specific for UIP.The HRCT pattern of UIP found in IPF can be indistinguishable from that seen in asbestosis, collagen vascular disease or as a response to drugs.Patients with chronic hypersensitivity pneumonitis or with end-stage sarcoidosis can uncommonly develop a CT pattern similar to UIPIPFDrugsCTDAsbestosisSarcoidEAAUIPNSIPDIP RBILDCOP
18 CXR NSIPbilateral pulmonary infiltrates. Lower lung zones more frequently involved.
19 HRCT NSIP1) ground glass predominant finding in most cases and sole finding ~50%.2) Irregular linear or reticular opacities seen about 50% cases. May be traction bronchiectasis.3) Honeycombing and consolidation relatively infrequent4) Bilateral symmetrical basal predominance
20 NSIP radiological differential diagnosis Depends on the predominant pattern exhibited.Experienced radiologist found it indistinguishable fromUIP 32%Hypersensitivity pneumonitis 20%Organising pneumonia 14%Other diagnosis 12% Radiology 2000 vol 217
21 Extent and Distribution of disease UIP /NSIP Feature NSIP UIP p valueDisease extent (%) / /Ground glass (%) / / <.005Coarseness score (max 15) / /Subpleural distribution (%)Basal distribution (%)Bronchocentric distribution53 patientsMacdonald et al Radiology 2001: 221
22 COP radiologyPatchy non-segmental, unilateral or bilateral areas of air space consolidation.Often vary in site and configuration over time.May be irregular reticular opacities. Rarely a major feature.Small nodular opacities usually with other features but occasionally on their own
23 COP HRCT findings 1 Bilateral Air-space Consolidation 80% 2 Ground glass opacities 60%3 Subpleural and/or peribronchovascular distribution4 Bronchial wall thickening, dilatation in abnormal areas5 Small nodular opacities often peribronchiolar (30-50% of cases)6 May get irregular reticular opacities7 Combination of findings in 1 and 2
27 Scleroderma.(PSS) High prevalence of pulmonary involvement HRCT patternsPulmonary arterial hypertension( 50%)ILD ( 80%) usually NSIPPleural thickening /effusionOesophageal dilatation.
28 CXR and CT cluesJoint abnormalities particularly AC and shoulder joints with Rheumatoid arthritisDilated oesophagus suggest scleroderma or variantPulmonary artery enlargement out of proportion to lung parenchymal changes may reflect vaculopathy especially scleodermaSoft tissue calcification –dermatomosytis or sclerodermaMultiple compartments think RA
29 Radiology of Idiopathic ILD The lung has a limited number of patterns of response to injury and there is often a lack of correlation between aetiological insult and radiological appearance of the lungWe need to recognise that in many cases there is not a clear cut match between the ‘clinical syndrome’ and the ‘radiological pattern’ of diseaseThe latter may be more important in determining prognosisPatients are managed in a multi-disciplinary manner in order to reach a final clinical diagnosis
30 Drug treatmentGold usually diffuse alveolar infiltrates- can look like OPMethotrexate-can produce sub-acute hypersensitivity- centilobular ground glassPneumonitis –more likely if pre-existing ildD-penacillamine- constrictive bronchiolitisNon -steroidals – hypersensitivitySalicylates OD pulmonary oedemaOpportunist infectionsIncreased risk lymphoma and lung cancer