Approach To Diffuse Parenchymal Lung Diseases


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Approach To Diffuse Parenchymal Lung Diseases

  1. 1. Approach To Interstitial Lung Diseases or Diffuse Parenchymal Lung Diseases Gamal Rabie Agmy, MD, FCCP Professor of Chest Diseases, Assiut university ERS National Delegate of Egypt
  2. 2. Objectives • Review the spectrum of ILD or DPLD • Identify clues on presentation to make the diagnosis • Review common radiographic findings in ILD • Come up with an algorithm to make the diagnosis
  3. 3. What is the Pulmonary Interstitium? • Interstitial compartment is the portion of the lung sandwiched between the epithelial and endothelial basement membrane • Expansion of the interstitial compartment by inflammation with or without fibrosis – Necrosis – Hyperplasia – Collapse of basement membrane – Inflammatory cells
  4. 4. The Lung Interstitium The interstitium of the lung is not normally visible radiographically; it becomes visible only when disease (e.g., edema, fibrosis, tumor) increases its volume and attenuation. The interstitial space is defined as continuum of loose connective tissue throughout the lung composed of three subdivisions: (i) the bronchovascular (axial), surrounding the bronchi, arteries, and veins from the lung root to the level of the respiratory bronchiole (ii) the parenchymal (acinar), situated between the alveolar and capillary basement membranes (iii) the subpleural, situated beneath the pleura, as well as in the interlobular septae.
  5. 5. Secondary pulmonary lobular anatomy
  6. 6. The terminal bronchiole in the center divides into respiratory bronchioles with acini that contain alveoli. Lymphatics and veins run within the interlobular septa Centrilobular area in blue (left) and perilymphatic area in yellow (right)
  7. 7. Pulmonary lesions Focal Diffuse
  8. 8. Pulmonary lesions  Focal  Diffuse
  9. 9. Clinical Presentation • Dyspnea on exertion or a persistent non productive cough • Abnormal CXR • Pulmonary symptoms associated with another disease, such as CVD • PFT abnormalities
  10. 10. Approach to DPLD DPLD of known Cause Idiopathic Interstitial Pneumonias Exposure Drugs Hypersensitivity Pneumonitis CVD Pneumoconiosis IPF Granulomatous Lung Diseases (Sarcoidosis) IIP other than IPF Desquamative Interstitial Pneumonia Others LAM Histiocytosis X Malignancy Respiratory BronchiolitisInterstitial Lung disease IPF: 47-64% NSIP: 14 to 36% Toxic Inhalation Radiation Acute Interstitial Pneumonia Cryptogenic Organizing Pneumonia RBILD/DIP: 1017% COP: 4-12% AIP: 2% LIP: 2% Lymphocytic Interstitial Pneumonia Non Specific Interstitial Pneumonia
  11. 11. Incident Cases of ILD Sarcoidosis 8% Occupation 11% DILD 5% DAH 4% CTD 9% Other 11% Pulmonary Fibrosis 52% (Incidence of IPF=26-31 per 100,000) Coultas AJRCCM 1994; 150:967
  12. 12. Historical Classification of IIP UIP/IPF UIP DIP UIP-BO LIP Giant cell IP DIP UIP NSIP DIP-RBILD AIP RBILD 2002 ATS/ERS AIP Cellular NSIP Fibrotic 1970 1997 Liebow and Carington Katzenstein Adapted from Ryu JH, et al. Mayo Clin Proc. 1998;73:1085-1101. Adapted from ATS/ERS. Am J Respir Crit Care Med . 2002;165:277-304. COP LIP
  13. 13. Clinical Assessment • • • • History Physical Exam Chest Radiograph Pulmonary Function Testing – At Rest – Exercise • Serologic Studies • Tissue examination
  14. 14. History • • • • • • • • Age Gender Smoking history Medications Duration of symptoms Environmental exposure Occupational exposure Family history
  15. 15. History: Age and Gender Age Gender – LAM – Tuberous sclerosis – Pneumoconiosis
  16. 16. History: Smoking • All of the following • In Goodpasture’s DPLD are associated syndrome with smoking : – 100% of smokers vs. 20% a) b) c) d) e) IPF RBILD DIP Histiocytosis X Syndrome of IPF & emphysema of nonsmokers experience pulmonary hemorrhage • Individuals exposed to asbestos who smoke are more likely to develop asbestosis
  17. 17. History: Medications Schwartz, ILD text book, 4th edition
  18. 18. History: Occupational and Environmental INORGANIC
  19. 19. ORGANIC: Hypersensitivity Pneumonitis
  20. 20. Occupational ????
  21. 21. History: Duration of Illness 1. Acute Diseases (Days to weeks) • DAD (AIP), EP, Vasculitis/DPH, Drug, CVD ________________________________________________________________________________________________________________ 2. Subacute Diseases (weeks to months) • HSP, Sarcoid, Cellular NSIP, Drug, “Chronic” EP, Bronchiolitis/ SAD __________________________________________________________________________________________________________________ 3. Chronic Diseases (months to years) • UIP, Fibrotic NSIP, Pneumoconioses, CVD-related, Chronic HSP Smoking (RBILD and PLCH)
  22. 22. Modified Liebow classification of the idiopathic interstitial pneumonias (Katzenstein) • Acute • Acute interstitial pneumonia (AIP) • Subacute • • • • Nonspecific interstitial pneumonia Lymphocytic Interstitial Pneumonia Cryptogenic Organizing Pneumonia Desquamative interstitial pneumonia/ Respiratory bronchiolitis-associated interstitial lung disease (NSIP) (LIP) (COP) (DIP) (RBILD) • Chronic • Usual interstitial pneumonia (UIP)
  23. 23. Physical Findings • • • • • • Resting Tachypnea Shallow breathing Dry crackles Digital clubbing Pulmonary HTN Non-pulmonary findings
  24. 24. Laboratory
  25. 25. ILD: Evaluation • Rdiographic – CXR – HRCT • Physiologic testing – PFT – Exercise test • Lung Sampling – BAL – Lung biopsy: (TBBx, Surgical)
  26. 26. CXR: LlMITATIONS • CXR is normal: – in 10 to 15 % of symptomatic patients with proven infiltrative lung disease – 30% of those with bronchiectasis – ~ 60 % of patients with emphysema • CXR has a sensitivity of 80% and a specificity of 82% percent for detection of DPLD • CXR can provide a confident diagnosis in ~ 23 % of cases
  27. 27. A normal CXR does not rule out the presence of DPLD
  28. 28. CXR CLUES Alveolar Filling • Air-bronchograms • Acinar rosettes • Diffuse consolidation • Nodule like, poor boarder definition • Silhouetting: obliteration of normal structures
  29. 29. CXR CLUES Interstitial Infiltrates • Nodular • Linear or reticular • Mixed • Honeycomb • Cysts and traction bronchiectasis • GGO
  30. 30. 4 Radiographic patterns     Reticular pattern Ground glass pattern Nodular pattern Cystic pattern
  31. 31. Ground glass pattern Interstitial Alveolar
  32. 32. Cystic pattern
  33. 33. Nodular pattern
  34. 34. Reticular pattern [ Interlacing linear shadows appearing as a mesh or net] Interstitial lung disease Usual interstitial pneumonia Desquamative interstitial pneumonia Acute interstitial pneumonia Non specific interstitial pneumonia Interstitial pulmonary edema Idiopathic pulmonary fibrosis Collagen vascular diseases Drug induced lung diseases Radiation induced lung diseases
  35. 35. Interstitial peumonia 50 Y F, with cough and fever
  36. 36. Radiological findings Peribronchial cuffing [bronchial wall thickening] Septal lines [short lines perpendicular to the pleura] Honeycombing [Cystic abnormalities =multiple peripheral cysts, mm-cm, thick walls] Traction bronchiectasis
  37. 37. Interstitial lung disease Other findings Spider appearance of the interlobular vessels due to interstitial opacities around the vessels Thickened interlobar fissures Sub-pleural lines [curvilinear arc lines parallel to the pleura] Ground glass density
  38. 38. Idiopathic pulmonary fibrosis
  39. 39. Interstitial fibrosis
  40. 40. Honeycombing
  41. 41. F 78Y Diabetic and hypertensive presented with severe dyspnea suspected to pulmonary embolism , treated with anticoagulants with mild improvement
  42. 42. Sarcoidosis • Multi system granulomatous disease • Unknown etiology • 90% of patients with sarcoidosis have chest changes • • • • Bilateral hilar and mediastinal adopathy Interstitial disease  lymph nodes Alveolar pattern simulating acute inflammatory disease] Cavitation, atelectasis, effusion (rare)
  43. 43. Sarcoidosis Nodal and Interstitial patterns
  44. 44. F 45Y Lymphadenopathy Sarcoidosis
  45. 45. Lymphangitis carcinomatosa
  46. 46. Lymphangitis carinomatosa F 59Y, with radical mastectomy
  47. 47. Drug induced lung diseases Immunologic reaction to drugs • Interstitial pattern similar to interstitial edema which progresses to alveolar pattern [busulfan, bleomycin, cytoxan,..] • Alveolar in filtrates similar to pulmonary edema [penicillin, sulfonamides,..] • Pleural and pericardial effusion + basal infilterates [isonaizid,…] • Hilar adenopathy [antionvulsant,..] Busulfan interstitial lung disease
  48. 48. Air space filling disease Replacement of alveolar air by fluid, cells, other material Represents an ongoing potentially treatable lesion Ground glass density [geographic distribution] morphologic changes below the resolution of CT due to Ground glass pattern Interstitial Alveolar
  49. 49. HISTOLOGIC CORRELATIONS IN GGO a) granulomata beyond special resolution b) thickening of the interstitium c) partial filling of the alveoli (associated with cellular phase (cellular phase OR fibrosis) at BAL) d) increased blood volume e) combination of all the above ERS 2008
  50. 50. GROUND GLASS OPACITIES CT-pathologic correlation variety of interstitial, alveolar and vascular diseases below the threshhold of spatial resolution of HRCT •Partially filled alveoli •Active interstitial inflammation •Fine fibrotic process •Hyperemia RULE OUT FINE FIBROSIS: traction bronchiectasis Vessel caliber TO FURTHER FOCUS DD TIMING CLINICAL SETTING BAL ERS 2008 Leung AN, Miller RR, Muller NL. Radiology 1993;188:209 –214
  51. 51. DIP • • • • • 90% of patients with DIP smoked or had smoked cigarettes onset of symptoms : ~ 40 yrs dyspnoea and cough male predominance: 2>1 inspiratory crackles : 60% digital clubbing :50% RARE DISEASE Hartman et al Radiology 1993 (n=22 from 5 centers) -in children DIP it is probably a different disease not related to smoking -DIP also occurs in non-smokers (of 40 cases of Carrington et al: 10%) -association with systemic disorders or infections -DIP element (focal pigmented macrophage accumulation) histologically ERS 2008 in all smokers - “DIP-like reaction”
  52. 52. GROUND GLASS: PREVAILING FEATURE ERS 2008 GGO in: Outpatients with Slowly Progressive Dyspnea
  53. 53. DIP Typically: subpleural /lower lung zones Reticulation seen in ~40-50% Honeycombing NOT significant ERS 2008
  54. 54. DIP ERS 2008
  55. 55. Outpatients with Slowly Progressive Dyspnea EAA 1. Centilobular nodules • Ill defined (unlike sarcoidosis) 2. Patchy or diffuse GGO 3. Superimposition of (1) and (2) 4. Geographic low density areas on inspiratory HRCT 5. Regional air trapping on expiratory HRCT ERS 2008
  56. 56. ERS 2008
  57. 57. Ground glass pattern [ Increased attenuation of the lung with preserved broncho vascular marking ]  Patients with AIDS, ground glass opacities= P.carinii pneumonia  Patients ground with lung glass transplant opacities= cytomegalovirus pneumonia or rejection P.carinii pneumonia in an AIDS patient
  58. 58. Air bronchogram sign Air filled bronchi passing through opaque lung parenchyma Pulmonary lesion Alveolar pathology Consolidation
  59. 59. Bilateral lower lobe pneumonia
  61. 61. Pulmonary edema
  62. 62. Pulmonary edema, 2 cases
  63. 63. Diffuse pulmonary hemorrhage Hemoptysis, anemia and air space opacities Appear rapidly and clear within few days Spare the lung apex and peripheral zones Bilateral, may be asymmetric, air bronchogram Repeated attacks → pulmonary fibrosis Pulmonary hemorrhage (normal heart) [3 days, 6 days, one month]
  64. 64. Pulmonary hemorrhage in SLE
  65. 65. Bronchoalveolar carcinoma Other causes: Lymphoma, pulmonary edema, some types of pneumonia [obstructive, lipoid] 6-10% of primary lung cancer Cough, sputum, weight loss, hemoptysis, bronchorrhea Radiographic patterns : Single or multiple pulmonary nodules [ Air bronchogram] Segmental or lobar consolidation. Diffuse air space disease . CT angiogram (non specific) Visualization of pulmonary vessels within airless lung
  66. 66. Alveolar cell Carcinoma
  67. 67. Broncho aleveolar carcinoma
  68. 68. Brocho-alveolar cell Carcinoma
  69. 69. Pneumonia versus bronchoalveolar carcinoma
  70. 70. F 72 Y with chest pain dyspnea and frothy expectoration
  71. 71. Alveolar proteinosis Alveolar filling by proteinaceous material Male : female 4:1 Possible causes:  Idiopathic  Occupational (silica)  Drug- induced  Immune compromise Geographic distribution of areas of ground glass opacities + thickened interlobular septa within  crazy paving appearance Air bronchogram is uncommon
  72. 72. Photograph of a pavement street in Buenos Aires, Argentina (left), drawings of the lungs (center) and lung tissue (top right), and close-up high-resolution CT scan (bottom right) show the crazy-paving pattern.
  73. 73. Alveolar proteinosis [crazy- paving]
  74. 74. Nodular pattern [ multiple rounded opacities 110mm] Miliary [1-2mm], the size of millet seeds TB Metastases Pneumoconiosis Sarcoidosis Alveolar cell carcinoma     Hematogenous dissemination Innumerable fine nodules Uniform distribution Mild thickening of the interstitial lung markings Miliary TB
  75. 75. Miliary TB
  76. 76. Sarcoidosis with miliary nodules and lymph nodes M 57 Y
  77. 77. Miliary deposits of breast cancer
  78. 78. Silicosis Inhalation of high concentrations of silicon dioxide • • Fine interstitial opacities with B Kerley’s lines (early) Multiple nodular shadows scattered in the lungs (classic) • Sparing apex and base • Calcification may occur
  79. 79. Progressive massive fibrosis   Nodules enlarge and coalesce to form masses Bilateral, almost symmetrical • Almost always in the upper ½ of the lungs • The more the fibrosis, the less apparent nodules
  80. 80. Silicosis
  81. 81. Pulmonary alveolar microlithiasis Innumerable tiny calcific particles are diffusely distributed in the alveoli • • • • Most patients are asymptomatic Dense sharply defined nodules The density is greatest in the lung bases Black pleura sign [unaffected pleura between lung and ribs]
  82. 82. Alveolar microlithiasis
  83. 83. Cystic pattern [ multiple thin walled air containing lesions 1cm or more ] Histeocytosis Lymphangioleiomyomatosis Lymphocytic interstitial pneumonia Emphysema Cystic bronchiectasis Tuberous sclerosis
  84. 84. Lung Cysts Differential Diagnosis Pulmonary fibrosis (Honeycombing) Lymphangliomyomatosis Langerhans cell histiocytosis Lymphocytic Interstitial Pneumonia (LIP)
  85. 85. UIP UIP or NSIP Rough Reticular Fine Reticular Traction Bronchiectasis and Interface sign Honey combing
  86. 86. Usual Interstitial Pneumonia UIP HRCT Findings Reticular opacities, thickened intra- and interlobular septa Irregular interfaces Honey combing and parenchymal distorsion Ground glass opacities (never prominent) Basal and subpleural predominance
  87. 87. Basal and subpleural distribution UIP
  88. 88. The Many ‘HRCT Faces’ of NSIP Honeycombing not a prominent feature !!!!
  89. 89. Lymphangioleiomyomatosis (LAM) HRCT Morphology Thin-walled cysts (2mm - 5cm) Uniform in size / rarely confluent Homogeneous distribution Chylous pleural effusion Lymphadenopathy in young women
  90. 90. Lymphangioleiomyomatosis (LAM)
  91. 91. Tuberous Sclerosis (young man)
  92. 92. Langerhans Cell Histiocytosis HRCT Findings Small peribronchiolar nodules (1-5mm) Thin-walled cysts (< 1cm), Bizarre and confluent Ground glass opacities Late signs: irreversible / parenchymal fibrosis Honey comb lung, septal thickening, bronchiectasis
  93. 93. Langerhans Cell Histiocytosis 1 year later Peribronchiolar Nodules Cavitating nodules and cysts
  94. 94. Langerhans Cell Histiocytosis
  95. 95. Langerhans Cell Histiozytosis Key Features Upper lobe predominance Combination of cysts and noduli Characteristic stages Increased Lung volume Sparing of costophrenic angle S M O K I N G
  96. 96. Langerhans Cell Histiocytosis
  97. 97. Langerhans Cell Histiocytosis Differential Diagnosis Only small nodules Sarcoidosis, Silicosis Only cysts idiopathic Fibrosis LAM Destructive emphysema
  98. 98. A professional diver..........
  99. 99. .......after cessation of smoki
  100. 100. LIP = Lymphocytic Interstitial Pneumonia Benign lymphoproliferative disorder Diffuse interstitial infiltration of mononuclear cells Not limited to the air ways as in follicular Bronchiolitis
  101. 101. Sjögren: LIP
  102. 102. LIP = Lymphocytic Interstitial Pneumonia Rarely idiopathic In association with: Sjögren’s syndrome Immune deficiency syndromes, AIDS Primary biliary cirrhosis Multicentric Castlemean’s disease
  103. 103. Sjoegren disease Dry eye and dry mouth Fibrosis, bronchitis and bronchiolitis LIP Up to 40 x increased risk for lymphoma (mediastinal adenopathy) and 2 x times increased risk for neoplasma Overlap Sarcoid, DM/PM, MXCT SLE, RA (pleural effusion)
  104. 104. Young woman LAM Dry mouth LIP Smoker Histiocytosis
  105. 105. Emphysema Fibrosis (UIP
  106. 106. Wegener‘s disease
  107. 107. Rheumatoid Arthritis
  108. 108. Outline Typical HRCT patterns of lung diseases with cysts Mosaic pattern and its differential Emphysema Atypical HRCT patterns Quiz
  109. 109. Where is the pathology ??????? in the areas with increased density meaning there is ground glass in the areas with decreased density meaning there is air trapping
  110. 110. Pathology in black areas Airtrapping: Airway Disease Bronchiolitis obliterans (constrictive bronchiolitis) idiopathic, connective tissue diseases, drug reaction, after transplantation, after infection Hypersensitivity pneumonitis granulomatous inflammation of bronchiolar wall Sarcoidosis granulomatous inflammation of bronchiolar wall Asthma / Bronchiectasis / Airway diseases
  111. 111. Airway Disease what you see…… In inspiration sharply demarcated areas of seemingly increased density (normal) and decreased density demarcation by interlobular septa In expiration ‘black’ areas remain in volume and density ‘white’ areas decrease in volume and increase in density INCREASE IN CONTRAST DIFFERENCES AIRTRAPPING
  112. 112. Bronchiolitis obliterans
  113. 113. Early Sarcoidosis
  114. 114. Chronic EAA
  115. 115. Hypersensitivity pneumonitis Extr. Allerg. Alveolitis (EAA) HRCT Morphology acute - subacute acinar (centrilobular) unsharp densities ground glass (patchy - diffuse) chronic: fibrosis Intra- / interlobular septal thickening Irregular interfaces Traction bronchiectasis
  116. 116. Pathology in white Areas Alveolitis / Pneumonitis Ground glass desquamative intertitial pneumoinia (DIP) nonspecific interstitial pneumonia (NSIP) organizing pneumonia In expiration both areas (white and black) decrease in volume and increase in density DECREASE IN CONTRAST DIFFERENCES
  117. 117. DI P
  118. 118. Cellular NSIP
  119. 119. Mosaic Perfusion Chronic pulmonary embolism LOOK FOR Pulmonary hypertension idiopathic, cardiac disease, pulmonary disease
  120. 120. CTEPH = Chronic thrombembolic pulmonary hypertension
  121. 121. Outline Typical HRCT patterns of lung diseases with cysts Mosaic pattern and its differential Emphysema Atypical HRCT patterns Quiz
  122. 122. Emphysema histopathological definition …..permanent abnormal enlargement of airspaces distal to the bronchioles terminales and …...destruction of the walls of the involved airspaces
  123. 123. Centrilobular Emphysema
  124. 124. Panlobular Emphysema
  125. 125. CLE and PLE in one Patient
  126. 126. Fibrosis and Emphysema
  127. 127. CT findings: • Relatively well-defined, low attenuation areas with very thin (invisible) walls, surrounded by normal lung parenchyma. • As disease progresses: – Amount of intervening normal lung decreases. – Number and size of the pulmonary vessels decrease. – +/- Abnormal vessel branching angles (>90o), with vessel bowing around the bullae.
  128. 128. Emphysema •Curved arrow: area of low attenuation. •Solid arrow: zones of vascular disruption. •Open arrow: area of lung destruction.
  129. 129. Emphysematous Bullae
  130. 130. Quantitative CT: • Spirometically triggered images at 10% and 90% vital capacity (VC) have been reported to be able to distinguish patients with chronic bronchitis from those with emphysema. – Patients with emphysema had significantly lower mean lung attenuation at 90% VC than normal subjects or patients with chronic bronchitis. – Attenuation was the same for normal subjects and those with chronic bronchitis.
  131. 131. Asthma: Marc Gosselin, MD
  132. 132. HRCT findings: • Bronchial wall thickening • Mucoid impaction • Mosaic lung attenuation with air trapping – Findings may be reversible with pharmacologic treatment. • Centrilobular thickening
  133. 133. Asthma: Marc Gosselin, MD
  134. 134. Most frequent CT findings of bronchiactasis: Most frequent Less frequent • Lack of tapering of the bronchial lumen • Bronchial wall thickening • Bronchial dilatation • Visualized peripheral bronchi • Mucus plugging
  135. 135. Bronchiectasis Radiology 2002; 225: 663-672 Arrows demonstrating various grades of bronchial wall thickening, with lack of tapering of the bronchial wall lumen.
  136. 136. Cystic Bronchiectasis
  137. 137. Bronchiectasis Signet ring? or Solitaire ring? Radiology 1999; 212: 67-68 “Signet ring” sign “Question Dogma” …Marc Gosselin, MD
  138. 138. Cystic Changes and Decreased Density Quiz What is Your Diagnosis ?
  139. 139. Emphysema Fibrosis LAM
  140. 140. Emphysema LCH
  141. 141. LCH Bronchiolitis
  142. 142. … holes…… Clues to Diagnosis Is there a wall ? What is the shape and size ? Smoker ? Other signs (e.g., bronchiectasis, pulmonary hypertension)
  143. 143. Reversed Halo Sign on High-Resolution CT of Cryptogenic Organizing Pneumonia Kim et al AJR 2003; 180:1251-1254 90% of their pts! reversed halo signs (central ground-glass opacity and surrounding air-space consolidation of crescentic and ring shapes) Voloudaki et al GGO ring : ERS 2008 septal inflammation cellular debris organising pneumonia
  144. 144. Bullous lung disease  Uncommon cause of respiratory distress in young males  Patients have history of significant cigarette smoking  Multiple large bullae impair the pulmonary mechanics 50Y M Primary bullous disease – Vanishing lung syndrome
  145. 145. Reticular pattern Interstitial lung disease Usual interstitial pneumonia Desquamative interstitial pneumonia Acute interstitial pneumonia Non specific interstitial pneumonia Interstitial pulmonary edema Idiopathic pulmonary fibrosis Collagen vascular diseases Drug induced lung diseases Radiation induced lung diseases
  148. 148. Nodular pattern [ multiple rounded opacities 1-10mm] Milliary [1-2mm], the size of millet seeds • TB • Metastases • Pneumoconiosis Milliary TB
  150. 150. Cystic pattern [ multiple thin walled air containing lesions 1cm or more ] Histeocytosis Lymphangioleiomyomatosis Lymphocytic interstitial pneumonia Emphysema Cystic bronchiectasis Tuberous sclerosis
  151. 151. Clinical HISTORY & EXAMINATION Tuberous sclerosis Emphysema IMAGING Histeocytosis Lymphangioleiomyomatosis Emphysema Cystic bronchiectasis
  152. 152. HRCT: Radiographic Pattern
  153. 153. Radiographic Patterns in ILD Pleural Involvement Lymphangitic Carcinomatosis LAM Drug Induced Radiation Pneumonitis Asbestosis Effusion Thickening Plaques Mesothelioma Collagen vascular disease Adenopathy Sarcoidosis Lymphoma Lymphangitic CA LIP Amyloidosis Berylliosis Silicosis Kerley B lines Chronic LV failure Lymphangitic CA Lymphoma LAM Veno-occlusive disease Acute Eosinophilic Pneumonia
  154. 154. PFT: Lung Volumes Restrictive Disease VC VC VC TLC TLC RV Normal TLC RV RV ILD NM Disease
  155. 155. Probability of Histologic Diagnosis of Diffuse Diseases Transbronchial Biopsy Surgical Biopsy 1. Granulomatous diseases 2. Malignant tumors/lymphangitic 3. DAD (any cause) 4. Certain infections Often 5. Alveolar proteinosis 6. Eosinophilic pneumonia 7. Vasculitis 8. Amyloidosis 9. EG/HX/PLCH Sometimes 10. LAM 11. RB/RBILD/DIP 12. UIP/NSIP/LIP COP 13. Small airways disease 14. PHT and PVOD Courtesy of Kevin O. Leslie, MD. Never
  156. 156. Pracical Aproach to Interstitial Lung Diseases
  157. 157. Patterns of Interstitial Lung Disease
  158. 158. Linear Pattern A linear pattern is seen when there is thickening of the interlobular septa, producing Kerley lines. Kerley A lines Kerley B lines Kerley A lines The interlobular septa contain pulmonary veins and lymphatics. The most common cause of interlobular septal thickening, producing Kerley A and B lines, is pulmonary edema, as a result of pulmonary venous hypertension and distension of the lymphatics. Kerley B lines
  159. 159. DD of Kerly Lines: Pulmonary edema is the most common cause Mitral stenosis Lymphangitic carcinomatosis Malignant lymphoma Congenital lymphangiectasia Idiopathic pulmonary fibrosis Pneumoconiosis Sarcoidosis
  160. 160. b. Reticular Pattern A reticular pattern results from the summation or superimposition of irregular linear opacities. The term reticular is defined as meshed, or in the form of a network. Reticular opacities can be described as fine, medium, or coarse, as the width of the opacities increases. A classic reticular pattern is seen with pulmonary fibrosis, in which multiple curvilinear opacities form small cystic spaces along the pleural margins and lung bases (honeycomb lung)
  161. 161. This 50-year-old man presented with end-stage lung fibrosis PA chest radiograph shows medium to coarse reticular B: CT scan shows multiple small cysts (honeycombing) involving predominantly the subpleural peripheral regions of lung. Traction bronchiectasis, another sign of end-stage lung fibrosis.
  162. 162. c. Nodular pattern  A nodular pattern consists of multiple round opacities, generally ranging in diameter from 1 mm to 1 cm  Nodular opacities may be described as miliary (1 to 2 mm, the size of millet seeds), small, medium, or large, as the diameter of the opacities increases  A nodular pattern, especially with predominant distribution, suggests a specific differential diagnosis
  163. 163. Disseminated histoplasmosis and nodular ILD. CT scan shows multiple bilateral round circumscribed pulmonary nodules.
  164. 164. Hematogenous metastases and nodular ILD. This 45-yearold woman presented with metastatic gastric carcinoma. The PA chest radiograph shows a diffuse pattern of nodules, 6 to 10 mm in diameter.
  165. 165. Differential diagnosis of a nodular pattern of interstitial lung disease SHRIMP Sarcoidosis Histiocytosis (Langerhan cell histiocytosis) Hypersensitivity pneumonitis Rheumatoid nodules Infection (mycobacterial, fungal, viral) Metastases Microlithiasis, alveolar Pneumoconioses (silicosis, coal worker's, berylliosis)
  166. 166. d. Reticulonodular pattern results A reticulonodular pattern results from a combination of reticular and nodular opacities. This pattern is often difficult to distinguish from a purely reticular or nodular pattern, and in such a case a differential diagnosis should be developed based on the predominant pattern. If there is no predominant pattern, causes of both nodular and reticular patterns should be considered.
  167. 167. How To Approach a Practical Diagnosis?
  168. 168. Rule no. 1 An acute appearance suggests pulmonary edema ,miliary TB,DAD or pneumonia
  169. 169. Disseminated histoplasmosis and reticulonodular ILD. A: PA chest radiograph, close-up of right upper lung, shows reticulonodular ILD. B: CT scan shows multiple circumscribed round pulmonary nodules, 2 to 3 mm in diameter.
  170. 170. Rule no. 2 Reticulonodular lower lung predominant distribution with decreased lung volumes suggests: (APC) 1. Asbestosis 2. Aspiration (chronic) 3. Pulmonary fibrosis (idiopathic) 4.Collagen vascular disease
  171. 171. Asbestos-related pleural disease and asbestosis
  172. 172. Pulmonary fibrosis and rheumatoid arthritis.
  173. 173. Systemic sclerosis. A: PA chest radiograph shows a bibasilar and subpleural distribution of fine reticular ILD. The presence of a dilated esophagus (arrows) provides a clue to the correct diagnosis. B: CT scan shows peripheral ILD and a dilated esophagus (arrow).
  174. 174. Rule no. 3 A middle or upper lung predominant distribution suggests: (Mycobacterium Settle Superiorly in Lung) 1. Mycobacterial or fungal disease 2. Silicosis 3. Sarcoidosis 4. Langerhans Cell Histiocytosis
  175. 175. Complicated silicosis. PA chest radiograph shows multiple nodules involving the upper and middle lungs, with coalescence of nodules in the left upper lobe resulting in early progressive massive fibrosis
  176. 176. Sarcoidosis. CT scan shows nodular thickening of the bronchovascular bundles (solid arrow) and subpleural nodules (dashed arrow), illustrating the typical perilymphatic distribution of sarcoidosis.
  177. 177. Langerhan cell histiocytosis. This 50-year-old man had a 30 pack-year history of cigarette smoking. A: PA chest radiograph shows hyperinflation of the lungs and fine bilateral reticular ILD. B: CT scan shows multiple cysts (solid arrow) and nodules (dashed arrow).
  178. 178. Rule no. 4 Associated lymphadenopathy suggests : 1.Sarcoidosis 2.neoplasm (lymphangitic carcinomatosis, lymphoma, metastases) 3. infection (viral, mycobacterial, or fungal) 4. Silicosis 5.Congestive heart failure with congestive lymphadenopathy.
  179. 179. Simple silicosis. A: CT scan with lung windowing shows numerous circumscribed pulmonary nodules, 2 to 3 mm in diameter (arrows). B: CT scan with mediastinal windowing shows densely calcified hilar (solid arrows) and subcarinal (dashed arrow) nodes.
  180. 180. Rule no. 5 Associated pleural thickening and/or calcification suggest asbestosis.
  181. 181. Rule no. 6 Associated pleural effusion suggests : 1.pulmonary edema 2.lymphangitic carcinomatosis 3.lymphoma 4.collagen vascular disease 5.LAM
  182. 182. Cardiogenic pulmonary edema. PA chest radiograph shows enlargement of the cardiac silhouette, bilateral ILD, enlargement of the azygos vein (solid arrow), and peribronchial cuffing (dashed arrow).
  183. 183. Lymphangitic carcinomatosis. This 53-year-old man presented with chronic obstructive pulmonary disease and large-cell bronchogenic carcinoma of the right lung. CT scan shows unilateral nodular thickening (arrows) and a malignant right pleural effusion.
  184. 184. Rule no. 7 Associated pneumothorax suggests lymphangioleiomyomatosis or LCH.
  185. 185. Lymphangioleiomyomatosis (LAM). A: PA chest radiograph shows a right basilar pneumothorax and two right pleural drainage catheters. The lung volumes are increased, which is characteristic of LAM, and there is diffuse reticular ILD. B: CT scan shows bilateral thinwalled cysts and a loculated right pneumothorax (P).
  186. 186. Tell me the rules again?
  187. 187. 1. Acute •P.Edema •Pneumonia •.Miliary TB •.DAD 2. Pleural effusion •1.pulmonary edema •2.lymphangitic carcinomatosis •3.lymphoma •4.collagen vascular disease 3.Pneumothorax •lymphangioleiomyom atosis •LCH 4.Predominantly Below with reduced volume 1.Asbestosis 2. Aspiration (chronic) 3. Pulmonary fibrosis (idiopathic) 4.Collagen vascular disease
  188. 188. 5. A middle or upper lung predominant 1. 2. 3. 4. Mycobacterial or fungal disease Silicosis Sarcoidosis Langerhans Cell Histiocytosis 6. Associated lymphadenopathy 7. Pleural Thickening and or Calcification •Asbestosis 1.Sarcoidosis 2.neoplasm (lymphangitic carcinomatosis, lymphoma, metastases) 3. infection (viral, mycobacterial, or fungal) 4. Silicosis 5.CHF
  189. 189. Approach to the ILD Patient Patient with Suspected ILD Hx, PE, CXR, PFT, Labs Dx likely by bronch? Yes Yes Is bronch diagnostic? No STOP HRCT Hx and HRCT consistent with IPF Hx and HRCT Dx of other ILD Suspected other ILD STOP Dx likely by bronch? STOP Atypical clinical or CT features of IPF Yes Is bronch diagnostic? No No Yes STOP VATS UIP NSIP RBILD DIP DAD OP LIP Non IIP Martinez F, Flaherty K. Available at: