Clinical Syndrome IPF NSIP. AIP Diffuse GG attenuation/consolidation ARDS RB/ RB-ILD. DIP Diffuse GG attenuation Smoking OP (BOOP)

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1 pneumonia (): can we tell the difference on Idiopathic Pneumonia: ATS/ERS Classification Idiopathic Pulmonary Fibrosis () vs Non-Specific Interstitial Pneumonitis (): Can We Tell the Difference Clinical Syndrome Imaging Findings Peripheral basilar reticulation; traction bronchiectasis; honeycomb lung GG attenuation; peripheral reticulation; traction bronchiectasis Diff Dx asbestos CVD, drugs AIP Diffuse GG attenuation/consolidation ARDS RB/ RB-ILD Centrilobular GG nodules; patchy GG attenuation; mild reticulation Smoking DIP Diffuse GG attenuation Smoking OP (BOOP) GG attenuation/consolidation; basilar distribution (common) infection, fumes LIP Centrilobular GG nodules/ cysts Aids Idiopathic Pneumonia: ATS/ERS Classification Clinical Syndrome Imaging Findings Peripheral basilar reticulation; traction bronchiectasis; honeycomb lung Groundglass attenuation; peripheral reticulation; traction bronchiectasis Diff Dx asbestos CVD, drugs AIP Diffuse GG attenuation/consolidation ARDS RB/ RB-ILD Centrilobular GG nodules; patchy GG attenuation; mild reticulation Smoking DIP Diffuse GG attenuation Smoking OP (BOOP) GG attenuation/consolidation; basilar distribution (common) LIP Centrilobular GG nodules/ cysts Aids infection, fumes Idiopathic Pulmonary Fibrosis () Clinical Findings: Mean age 57; insidious onset; mortality rate 60-70%; mean survival 5 6 yrs; no response to steroids Pathologic Findings: Heterogeneous temporal appearance; fibrogenic foci distinctive i i features; fibroblastic bl i proliferation i with scant interstitial inflammation; microscopic leading to gross honeycombing CT Findings: Honeycombing (+++); peripheral reticulation (++); groundglass (+); consolidation (+); lower lobe predominance (+++) Ellis S. Eur Radiol 2002;12 Usual Interstitial Pneumonia (UIP) temporal and spatial inhomogeneity normal lung alveolar wall thickening and inflammation fibrosis (fibroblastic foci) Courtesy: Ric Webb, MD CT Symposium 1

2 pneumonia (): can we tell the difference on UIP (). 2 yrs later (UIP): CT-Pathologic Correlations Prospective, multi-center study of 91 pts suspected of having with clinical, physiologic, CXR, and CT features assessed by 4 radiologists with F/U histologic correlation Analyzed with uni- and multivariate logistic regression analysis to compare pts with and without documented 54 (59%) of 91 pts pathologic dx. Using multivariate analysis of specific HRCT features - only independent predictors of included: Honeycombing (odds ratio, 5.36) and upper lobe reticulation lung (odds ratio, 6.28) Using these features specific Dx of established: Sens = 74%; Spec = 81%; PPV = 85%. Hunninghake G. Chest 2003;124: : Spectrum of HRCT Findings Groundglass predominance Asymmetric disease Focal air-trapping mimics chronic c hypersensitivity s ty pneumonitis Pulmonary ossification Mediastinal/hilar adenopathy Acute exacerbation rapid development of diffuse alveolar damage (DAD) TB and Lung cancer Souza C. AJR 2005;185 CT Symposium 2

3 pneumonia (): can we tell the difference on Do HRCT Findings of UIP Obviate Biopsy? Pulmonologist s Views Questionnaire members of the ACCP whether HRCT could replace lung bx in 16 DILD s 67% of 230 responders accepted HRCT dx of /UIP despite awareness of guidelines recommending histologic diagnosis Majority would not accept HRCT diagnosis for most other diseases even when HRCT is characteristic. LAM (37%) or LCH (19%) Diette GB. Respiration 2005;72:127-8 : Consensus Statement American Thoracic Society Major Criteria Exclusion of other known etiologies Abnormal PFT S Abn HRCT > 6 mos TBBX/BAL excluding other etiologies Minor Criteria Age > 50 yrs Insidious onset DOE Diagnosis > 3 mos duration Bibasilar rales In absence of OLB: Dx requires all 4 major - 3/4 minor criteria Idiopathic Pneumonia: ATS/ERS Classification Clinical Syndrome Imaging Findings Peripheral basilar reticulation; traction bronchiectasis; honeycomb lung Groundglass attenuation; peripheral reticulation; traction bronchiectasis Diff Dx asbestos CVD, drugs AIP Diffuse GG attenuation/consolidation ARDS RB/ RB-ILD Centrilobular GG nodules; patchy GG attenuation; mild reticulation Smoking DIP Diffuse GG attenuation Smoking OP (BOOP) GG attenuation/consolidation; basilar distribution (common) LIP Centrilobular GG nodules/ cysts Aids infection, fumes Non-Specific Interstitial Pneumonitis Clinical Findings: Broad age range; dyspnea, cough and fever without clubbing: 60% idiopathic - minimum 5% mortality. Associated with autoimmune diseases (Hasimoto s thyroiditis; drug reaction) and collagen vascular disease Pathologic Findings: 2 Forms Cellular : homogeneous changes without fibrogenic foci, alveolar macrophages, or architectural distortion/honeycombing. Variable degrees of interstitial inflammation and fibrosis Fibrotic : diffuse fibrosis +/- honeycombing CT Findings: Predominantly GG attenuation admixed with reticulation of variable severity often sparing portions of the subpleural lung without honeycombing Nonspecific Interstitial Pneumonia () temporal and spatial homogeneity mild interstitial pneumonia with cellular infiltration cellular or fibrotic forms fibrosis relatively mild Courtesy of Kevin Leslie CT Symposium 3

4 pneumonia (): can we tell the difference on GGO; mild reticulation; traction bronchiectasis Cellular : Response to treatment CT Symposium 4

5 pneumonia (): can we tell the difference on UIP/ Post Steroids Reticular markings, traction bronchiectasis Groundglass Patchy distribution Subpleural sparing Honeycombing Basilar predominance Subpleural dx Fibrotic : Scleroderma vs : Diagnostic Accuracy HRCT Compared CT findings (n = 21) with UIP (n = 32) CT diagnosis of : Sensitivity = 70%; Specificity = 63% CT diagnosis of : Sensitivity = 63%; Specificity = 70% Predominance of groundglass attenuation the cardinal feature of (odds ratio: 1.04 for each 1% increase in proportion of GG attenuation) Of patients presenting with clinical diagnosis correctly diagnosed in 70% 33% of pts with had equivalent extent of GG attenuation and reticulation; 12% had predominant GG attenuation! Conclusion: Considerable overlap exists vs MacDonlad S. Radiology 2001;221 CT Symposium 5

6 pneumonia (): can we tell the difference on : CT Pathologic Subgroup Correlations 55 cases documented categorized into 4 grades: Grade 1 - inflammation to Grade 4 - predominant fibrosis CT Findings: Areas of groundglass attenuation and architectural distortion identified in all cases. Traction bronchiectasis seen in 94%; intralobular reticular opacities seen in 87% Extent of traction bronchiectasis and reticulation correlated with the histologic grade (p <.001 and.05, respectively) Honeycombing identified in 12 (43%) of 28 pts with grade 4 disease and 3 (11%) of the remaining 27 pts (p <.001) Conclusion: Extent and severity of bronchiectasis and reticulation increase with worsening fibrosis Johkoh T. Radiology 2002;225 (UIP) VS Chronic IIP Sumikawa H. Radiology 2006;241:258 Retrospective study 92 path proved cases IIP: UIP=20; cellular =16; fibrotic =16; RB-ILD=11; DIP=15; LIP=14. Excluded AIP and OP. Pts with classic CT findings UIP not biopsied Overall correct diagnosis 145 (79%) of 184 reads correctly dxed 63% of cases 9/15 cases with - misdiagnosed as (minimal or no honeycombing) 5 cases with misdiagnosed as (3/5 honeycombing and peripheral reticulation No statistical difference in extent of GGA, extent of traction bronchiectasis or peripheral distribution between and : Extensive GGO/ Mild reticulation Traction bronchiectasis CT Symposium 6

7 pneumonia (): can we tell the difference on Fibrotic and : Changes in Pattern Over Time Silva C. Radiology 2008;247: Retrospective study 48 pts 23 ; 25 followed for months HRCT findings reviewed pattern and distribution: groundglass attenuation (GGA); reticulation; traction bronchiectasis; i honeycombing initially correctly identified 18 (78%) of 23 pts 5 (28%) of 18 follow-up HRCT more consistent with marked decrease GGA; increase reticulation and likelihood of peripheral distribution (p <.05) No significant differences predictive of pattern of evolution based on initial HRCT appearances Non Specific Interstitial Pneumonitis Is a separate clinical entity? or an early form of UIP? If so: what are its characteristics? Clinical findings Radiologic/ HRCT appearances Pathologic characteristics: and How to establish the diagnosis? vs. UIP/ and chronic hypersensitivity pneumonitis : Report of an ATS Project Travis W.D. Am J Resp Crit Care Med 2008 (in press) Multidisciplinary study (pathology/radiology/clinical) 67 cases identified as idiopathic of 193 reviewed Cases a-priori excluded if known etiology: collagen vascular disease; drug exposure; airborne antigens 126 cases excluded including 80 possible ; 46 definitively not 3 major groups HP, UIP, Organizing Pneumonia Conclusion: is a distinct clinical entity occurring most often in middle aged, non-smoking women (67%); good prognosis : Report of an ATS Project Travis W.D. Am J Resp Crit Care Med 2008 (in press) HRCT findings: Most common lower lung zone involvement (92%); reticular pattern (87%); traction bronchiectasis (82%); volume loss (77%) HRCT findings: Less common peripheral distribution (46%); Groundglass attenuation (44%); subpleural sparing (21%) and peribronchial thickening (6.6%) Honeycombing identified in 4.9% only Pathologic diagnosis made in 104 cases (definite = 31; probable = 74): of these, consensus dx other than reached in 38 (37%) of cases 36 due to atypical HRCT findings - most often a pattern of subacute HP or COP. : Report of an ATS Project Travis W.D. Am J Resp Crit Care Med 2008 (in press) HRCT findings: Most common lower lung zone involvement (92%); reticular pattern (87%); traction bronchiectasis (82%); volume loss (77%) HRCT findings: Less common peripheral distribution(46%); Groundglass attenuation (44%); subpleural sparing (21%) and peribrochial thickening (6.6%) Honeycombing identified in 4.9% only Pathologic diagnosis made in 104 cases (definite = 31; probable = 74): of these, consensus dx other than reached in 38 (37%) of cases 36 due to atypical HRCT findings - most often a pattern of subacute HP or COP. CT Symposium 7

8 pneumonia (): can we tell the difference on DILD: Diagnostic Algorithm Hx, Physical; CXR; PFT s, HRCT, BAL DILD: Diagnostic Algorithm Hx, Physical; CXR; PFT s, HRCT, BAL DILD Known Etiology: Drugs; CVD Idiopathic interstitial pneumonia Granulomatous disease: Sarcoid; HP Miscellaneous: diseases Chronic HP;EG DILD Known Etiology: Drugs; CVD Idiopathic interstitial pneumonia Granulomatous disease: Sarcoid; HP Miscellaneous: diseases Chronic HP;EG Definite UIP/ Adapted: Bhalla M. JTI 2003 Surgical biopsy in absence of confident diagnosis ; COP (BOOP); AIP; RB-ILD; DIP; LIP Definite Diagnosis UIP/ (60% Clinical + HRCT) Adapted: Bhalla M. JTI 2003 Surgical biopsy in absence of confident diagnosis ; COP (BOOP); AIP; RB-ILD; DIP; LIP DILD: Diagnostic Algorithm Hx, Physical; CXR; PFT s, HRCT, BAL DILD: Diagnostic Algorithm Hx, Physical; CXR; PFT s, HRCT, BAL DILD Known Etiology: Drugs; CVD Idiopathic interstitial pneumonia Granulomatous disease: Sarcoid; HP Miscellaneous: diseases Chronic HP;EG DILD Known Etiology: Drugs; CVD Idiopathic interstitial pneumonia Granulomatous disease: Sarcoid; HP Miscellaneous: diseases Chronic HP;EG Clinical; HRCT Diagnosis + Surgical biopsy in absence of confident diagnosis Clinical; HRCT Diagnosis + Surgical biopsy in absence of confident diagnosis Adapted: Bhalla M. JTI 2003 ; COP (BOOP); AIP; RB-ILD; DIP; LIP Adapted: Bhalla M. JTI 2003 Where Specific Dx of requires Path-HRCT correlation Cellular vs Fibrosing IIP s and Prognosis Churg A, Muller N. Chest 2006;130: Good Prognosis: Path - Pure cellular process/ +/- Organizing pneumonia HRCT- GG attenuation (GGA)/airspace consolidation Intermediate Prognosis Path linear fibrosis (follows alveolar walls without architectural distortion HRCT- GGA/ consolidation + reticulation (< 25%) Poor Prognosis Path UIP fibrosis/architectural distortion/microscopic honeycomb HRCT- extensive reticulation/honeycombing Cellular vs Fibrosing IIP s and Prognosis Churg A, Muller N. Chest 2006;130: Good Prognosis: Cellular, RBILD-most DIP, Subacute HP, COP/BOOP, cellular drug reactions/cvd Intermediate Prognosis Fibrotic (including -like HP and CVD Some cases DIP Poor Prognosis UIP (including UIP like HP, CVD and drug reaction) Rare DIP Adds HP; Excludes AIP and LIP CT Symposium 8

9 pneumonia (): can we tell the difference on Chronic HP: CT Features Comparison with Pathologic Evidence of Fibrosis and Survival Retrospective study 26 pts open lung bx Data base 4000 pts pts histologic evidence of fibrosis These more likely l to have: Traction bronchiectasis (p =.015) honeycombing (p =.007) and UIP pattern on CT (p =.007) (subpleural reticulation/lower zone predominance) Sahin H. Radiology 2007;244: Cellular vs Fibrosing IIP s: Diagnostic Approach Churg A, Muller N. Chest 2006;130: Good Prognosis: = TBBx/BAL - Empirical Rx Path - Pure cellular process/ +/- Organizing pneumonia HRCT- GG attenuation (GGA)/airspace consolidation Intermediate Prognosis = Surgical Lung Bx Path linear fibrosis (follows alveolar walls without architectural distortion HRCT- GGA/ consolidation + reticulation (< 25%) Poor Prognosis = Conservative Management Path UIP fibrosis/architectural distortion/microscopic honeycomb HRCT- extensive reticulation/honeycombing HRCT Diagnosis of Basal honeycombing visible on HRCT has a high predictive value for UIP In the absence of a known disease or exposure, is very likely the diagnosis Lung biopsy is unlikely to be performed Poor prognosis; treatment of little value Not all shows honeycombing CT Symposium 9