Diffuse Parenchymal Lung Diseases Atlas
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Idiopathic pulmonary fibrosis (IPF) is characterized by a dominant pattern of reticular opacities and honeycombing, predominantly in the subpleural regions of the lower lobes. Ground-glass opacities and traction bronchiectasis may also be seen. The distribution demonstrates an apicobasal gradient. Nonspecific interstitial pneumonitis (NSIP) most commonly demonstrates a dominant ground-glass opacity pattern, distributed bilaterally and more prominent in the lower lobes without an apicobasal gradient. Cryptogenic organizing pneumonia (COP) appears as patches of consolidation and ground-glass opacity in the peripheral and subpleural regions of the middle and lower lung zones.
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Diffuse Parenchymal Lung Diseases Atlas
- 1. Diffuse Parenchymal Lung Diseases Dr Muhammad Shoyab FCPS (Part-II) March 10, 2015
- 2. Definition A heterogeneous group of conditions that affect the connective tissue interstitium as well as the alveolar epithelium and capillary endothelium of the lungs. Harrison
- 3. Previously called ILD • Involves not only interstitium • But all components of lung parenchyma • Hence renamed as DPLD Oxford | Cecil | Davidson
- 4. DPLD is an “Umbrella” Term • >200 disease entities • Varying aetiologies & pathogeneses (infections & malignancies not included, by convention) • Clinical, spirometric, imaging & histologic features are overlapping Nadel
- 5. A Diagnostic Challenge • Clinical, spirometric, imaging & histologic features are overlapping • 10% are normal on CXR & PFT • HRCT 90% sensitive Nadel
- 6. Role of Radiology • About 10 diseases account for 90% cases • Primary goal is to distinguish treatable from untreatable disease RA | Nadel
- 7. DPLDs are Disease for Everyone | Radiologist Pulmonologist Rheumatology Intensive / Palliative Care Geriatrics Occupational HealthEnvironmental Health Oncology Public Health
- 9. Different Classifications of DPLD Kumar & Clarke | Cecil | Nadel | Oxford Clinical Classification Histopathological Classification Inflammatory / Fibrotic Granulomatous Radiological Classification Reticular Upper zone dominant Nodular Lower zone dominant Ground-glass Cystic
- 10. ATS-ERS Classification – 2002 ILD Atlas
- 12. ILD Atlas The Task Ahead
- 13. ILD Atlas The Task Ahead
- 16. THE LUNG PARENCHYMA The Secondary Pulmonary Lobule
- 17. Lung Parenchyma ILD Atlas Structures distal to the terminal bronchiole, i.e. where gas exchange takes place
- 18. primary lobule SECONDARY LOBULE Purely conducting No gas exchange Gas exchange occurs LUNG PARENCHYMA The Bronchial Tree CT Lung
- 19. CT Lung | ILD AtlasSecondary Lobule Area supplied by a single lobular bronchiole.
- 20. CT Lung | ILD Atlas Smallest part of lung structure demarcated by septa. Secondary Lobule
- 21. CT Lung | ILD Atlas Structural unit of lung. Smallest part of lung structure demarcated by septa. Secondary Lobule
- 22. Layout of the Secondary Lobule Davidson
- 23. The Interstitium Septa and connecting tissue that support the bronchial and vascular trees. Felson | Davidson
- 24. The Interstitium • Axial • Peripheral • Parenchymal Felson | DavidsonDivisions
- 25. The Interstitium Felson | DavidsonDisease Patterns Centrilobular Perilymphatic Subpleural Intralobular
- 27. Clinical Features Persistent, Non- Productive Cough Progressive Exertional Dyspnoea • Haemoptysis • Chest Pain • Cyanosis
- 29. Radiographic Patterns Ground-Glass Opacity Consolidation Nodular Reticular RA | Felson Nodular Reticular
- 30. GROUND-GLASS
- 31. • Hazy increase in lung opacity • Vascular markings preserved CT LungGround-Glass
- 32. • Hazy increase in lung opacity • Vascular markings preserved CT LungGround-Glass
- 33. Ground-Glass CT Lung | DLD
- 34. • Increased lung opacity / density • Vascular markings preserved Ground-Glass CT Lung | RA
- 35. • Increased lung opacity / density • Vascular markings preserved Ground-Glass CT Lung
- 36. • Represents treatable disease Ground-Glass CT Lung
- 37. CONSOLIDATION
- 38. Consolidation Murray & Nadel | CT Lung | RA • Homogeneous increase of opacity / density • Obscure vascular markings Air bronchograms Silhouetting of adjacent structures
- 39. Murray & Nadel | CT LungConsolidation • Homogeneous increase of opacity / density • Obscure vascular markings • Air bronchograms • Silhouetting of adjacent structures
- 40. NODULAR
- 41. CXR GuideNodular Sharply defined areas of soft tissue opacity, upto 3 cm in diameter
- 42. Sharply defined areas of soft tissue density, upto 3 cm in diameter CT LungNodular
- 43. CT LungNodular
- 44. CT LungNodular : Size <7 mm : Micronodule <1 cm : Small Nodule >1 cm : Large Nodule
- 45. •Perilymphatic •Centrilobular • Tree-in-bud •Random Nodular : Distribution CT Lung | RA
- 46. Nodular : Centrilobular CT Lung | RA Nodules separated from pleura and septa by 5– 10 mm with no nodules in the subpleural spaces.
- 47. Nodular : Tree-in-Bud Gurney | RA | CT Lung Small nodules at the tips of peripheral thin branching opacities of different density
- 48. CT LungNodular : Perilymphatic • Subpleural • Septal
- 49. CT LungNodular : Perilymphatic • Characteristic location : subpleural • Most easily : Fissural
- 50. Not associated with any particular component of secondary lobule CT LungNodular : Random
- 51. Often bilateral, symmetrical, uniformly distributed throughout both lungs CT LungNodular : Random
- 52. • Numerous micronodules (1-3 mm) • All over both lungs • Indicates haematogenous spread of disease CT LungNodular : Random -- Miliary
- 53. Differential Diagnoses CT LungNodular : Random -- Miliary
- 55. CT LungLinear / Reticular Linear opacities across lung fields Caused by diffuse thickening of interstitium
- 56. CT LungLinear / Reticular : Septal Lines Caused by thickening of interlobular septa Form right angles with pleura
- 57. CT LungLinear / Reticular : Septal Lines Caused by thickening of interlobular septa Form right angles with pleura
- 58. CT LungLinear / Reticular : Intralobular • Lace or netlike appearance • Caused by thickening of connective tissue between acini (i.e. parenchymal interstitium)
- 59. CT LungLinear / Reticular : Irregular Small irregular linear opacities eg pulmonary fibrosis
- 61. CT LungBeaded Septal Lines Linear opacities + micronodules Caused by lymphatic spread of tumour eg lymphangitis carcinomatosa
- 62. CT LungBeaded Septal Lines Linear opacities + micronodules Caused by lymphatic spread of tumour eg lymphangitis carcinomatosa
- 63. CT LungCrazy Paving Combination of ground-glass and reticular
- 64. CT LungCrazy Paving : Differentials Alveolar proteinosis Sarcoidosis NSIP COP/BOOP Infection
- 65. CT LungHoneycombing Linear opacities with cystic changes
- 66. CT LungHoneycombing : Differentials • Represents irreversible / end-stage destruction • Primarily a feature of IPF
- 67. Steps of Evaluation by Imaging 1. Dominant pattern 2. Location : Centrilobular / perilymphatic / random 3. Distribution : Upper or lower; Central or peripheral; Unilateral or bilateral; Symmetic or asymmetric 4. Other findings : Effusion, lymph nodes, etc • C/F and other investigations
- 68. • >200 diseases • About 10 diseases cover 90% of cases • Characteristic HRCT features • Aim is to detect treatable disease ILD Atlas | RA
- 69. Idiopathic Pulmonary Fibrosis • Dominant pattern : Reticular & honeycombing • Additional pattern : Ground-glass & traction bronchiectasis • Distribution : Distinctly subpleural Tubaikh
- 70. Idiopathic Pulmonary Fibrosis Tubaikh • Dominant pattern : Reticular & honeycombing • Additional pattern : Ground-glass & traction bronchiectasis • Distribution : Distinctly subpleural
- 71. Idiopathic Pulmonary Fibrosis Tubaikh • Dominant pattern : Reticular & honeycombing • Additional pattern : Ground-glass & traction bronchiectasis • Distribution : Distinctly subpleural • Location : Basal + peripheral, reducing towards apex (apicobasal gradient)
- 72. Idiopathic Pulmonary Fibrosis Tubaikh • Dominant pattern : Reticular & honeycombing • Additional pattern : Ground-glass & traction bronchiectasis • Distribution : Distinctly subpleural • Location : Basal + peripheral, reducing towards apex (apicobasal gradient)
- 73. Idiopathic Pulmonary Fibrosis Tubaikh • Dominant pattern : Reticular & honeycombing • Additional pattern : Ground-glass & traction bronchiectasis • Distribution : Distinctly subpleural • Location : Basal + peripheral, reducing towards apex (apicobasal gradient) • No identifiable cause of fibrosis
- 74. Idiopathic Pulmonary Fibrosis Tubaikh • Dominant pattern : Reticular & honeycombing • Additional pattern : Ground-glass & traction bronchiectasis • Distribution : Distinctly subpleural • Location : Basal + peripheral, reducing towards apex (apicobasal gradient) • No identifiable cause of fibrosis
- 75. Idiopathic Pulmonary Fibrosis Tubaikh • Ground-glass opacities represent pre-fibrotic change, i.e. treatable (steroid-responsive) • Honeycombed areas represent established / advanced fibrosis, i.e. irreversible / end-stage parenchymal destruction
- 76. Idiopathic Pulmonary Fibrosis Tubaikh • Ground-glass opacities represent pre-fibrotic change, i.e. treatable (steroid-responsive) • Extensive ground-glass opacities / ground-glass opacities other than adjacent to fibrotic areas suggest alternative diagnosis
- 77. Idiopathic Pulmonary Fibrosis Tubaikh • Ground-glass opacities represent pre-fibrotic change, i.e. treatable (steroid-responsive) • Extensive ground-glass opacities / ground-glass opacities other than adjacent to fibrotic areas suggest alternative diagnosis • Absence of apicobasal gradient, relative sparing of subpleural zone suggest alternative diagnosis
- 78. Idiopathic Pulmonary Fibrosis Tubaikh • Mild mediastinal lymphadenopathy present in 70% cases
- 79. Idiopathic Pulmonary Fibrosis Tubaikh • Mild mediastinal lymphadenopathy present in 70% cases • L/N > 15 mm or >2 nodal stations suggests associated malignancy
- 80. Idiopathic Pulmonary Fibrosis Tubaikh • Mild mediastinal lymphadenopathy present in 70% cases • L/N > 15 mm or >2 nodal stations suggests associated malignancy • Bronchogenic carcinoma is 10-20 times more common in IPF always look for a mass
- 81. Idiopathic Pulmonary Fibrosis Tubaikh • Mild mediastinal lymphadenopathy present in 70% cases • L/N > 15 mm or >2 nodal stations suggests associated malignancy • Bronchogenic carcinoma is 10-20 times more common in IPF always look for a mass • Pleural effusion also suggests malignancy (5% false +ve)
- 82. Idiopathic Pulmonary Fibrosis Tubaikh • Dominant pattern : Reticular & honeycombing • Additional pattern : Ground-glass & traction bronchiectasis • Distribution : Distinctly subpleural • Location : Basal + peripheral, reducing towards apex (apicobasal gradient)
- 83. Idiopathic Pulmonary Fibrosis Tubaikh • Dominant pattern : Reticular & honeycombing • Additional pattern : Ground-glass & traction bronchiectasis • Distribution : Distinctly subpleural • Location : Basal + peripheral, reducing towards apex (apicobasal gradient)
- 84. Idiopathic Pulmonary Fibrosis Tubaikh
- 85. Idiopathic Pulmonary Fibrosis Tubaikh
- 86. Idiopathic Pulmonary Fibrosis Tubaikh
- 87. Nonspecific Interstitial Pneumonitis • Dominant pattern : Patchy ground-glass opacities
- 88. Nonspecific Interstitial Pneumonitis • Dominant pattern : Patchy ground-glass opacities • Additional pattern : Reticular opacities, bronchiectasis, “microcystic” honeycombing
- 89. Nonspecific Interstitial Pneumonitis • Dominant pattern : Patchy ground-glass opacities • Additional pattern : Reticular opacities, bronchiectasis, “microcystic” honeycombing • Distribution : Bilateral, subpleural, more in lower lobes
- 90. Nonspecific Interstitial Pneumonitis • Dominant pattern : Patchy ground-glass opacities • Additional pattern : Reticular opacities, bronchiectasis, “microcystic” honeycombing • Distribution : Bilateral, subpleural, more in lower lobes • No apicobasal gradient
- 91. IPF vs NSIP • Dominant pattern : Patchy ground-glass opacities • Additional pattern : Reticular opacities, bronchiectasis, “microcytic” honeycombing • Distribution : Bilateral, subpleural, more in lower lobes • No apicobasal gradient • Most commonly due to connective tissue diseases • Dominant pattern : Reticular & honeycombing • Additional pattern : Ground-glass & traction bronchiectasis • Distribution : Distinctly subpleural • Location : Basal + peripheral, reducing towards apex (apicobasal gradient) • Idiopathic
- 92. Nonspecific Interstitial Pneumonitis • Dominant pattern : Patchy ground-glass opacities • Additional pattern : Reticular opacities, bronchiectasis, “microcytic” honeycombing • Distribution : Bilateral, subpleural, more in lower lobes • No apicobasal gradient
- 93. Nonspecific Interstitial Pneumonitis • Dominant pattern : Patchy ground-glass opacities • Additional pattern : Reticular opacities, bronchiectasis, “microcytic” honeycombing • Distribution : Bilateral, subpleural, more in lower lobes • No apicobasal gradient
- 95. Cryptogenic Organising Pneumonia • Patches of consolidation ± ground-glass opacities • Peripheral / subpleural zones of middle & lower lung fields Gurney | Tubaikh
- 96. Cryptogenic Organising Pneumonia • Patches of consolidation ± ground-glass opacities • Peripheral / subpleural zones of middle & lower lung fields Gurney | Tubaikh
- 97. Cryptogenic Organising Pneumonia • Patches of consolidation ± ground-glass opacities • Peripheral / subpleural zones of middle & lower lung fields • Reticulonodular • Bronchiectasis Gurney | Tubaikh
- 98. Cryptogenic Organising Pneumonia • Patches of consolidation ± ground-glass opacities • Peripheral / subpleural zones of middle & lower lung fields • Reticulonodular • Bronchiectasis Gurney | Tubaikh
- 99. Cryptogenic Organising Pneumonia • Patches of consolidation ± ground-glass opacities • Peripheral / subpleural zones of middle & lower lung fields • Reticulonodular • Bronchiectasis • May look like pleural / fissural mass, with speculated margin biopsy needed Gurney | Tubaikh
- 100. Asbestosis Gurney | Path Atlas 20 – 30 yrs H/O asbestos exposure Male predominant (due to occupational exposure)
- 101. Asbestosis Gurney . • Dominant pattern : fibrosis • Additional pattern : reticulonodular • Distribution : Posterobasilar, subpleural Fibrotic bands projecting from pleura Pleural plaques (80%) ± calcification (15%) Pleural effusion / thickening
- 102. Asbestosis Gurney . • Dominant pattern : fibrosis • Additional pattern : reticulonodular • Distribution : Posterobasilar, subpleural Fibrotic bands projecting from pleura Pleural plaques (80%) ± calcification (15%) Pleural effusion / thickening
- 103. Asbestosis Gurney . • Dominant pattern : fibrosis • Additional pattern : reticulonodular • Distribution : Posterobasilar, subpleural • Fibrotic bands projecting from pleura • Pleural plaques (80%) ± calcification (15%) • Pleural effusion / thickening
- 104. Silicosis Gurney | Path Atlas Occurs 20 years after >20 years of silica exposure Male predominant (due to occupational exposure)
- 105. Silicosis : Simple PneumoconiosisGurney . • Dominant pattern : micronodules • Distribution : • Centrilobular & subpleural • Upper posterior lung fields • Bilateral, Right > Left Subpleural nodules mimic pleural plaques (pseudoplaques) 3% nodules calcify 20% L/N, 5% calcify
- 106. Silicosis : Simple PneumoconiosisGurney . • Dominant pattern : micronodules • Distribution : • Centrilobular & subpleural • Upper posterior lung fields • Bilateral, Right > Left • Subpleural nodules mimic pleural plaques (pseudoplaques) • 3% nodules calcify • 20% L/N, 5% calcify
- 107. Silicosis : Complicated PneumoconiosisGurney . • Mass-like aggregates of micronodules • Irregularly elliptical in shape • ± Calcification, Cavitation • Produce emphysema in distal lung • May lead to pneumothorax • Synonym : progressive massive fibrosis
- 108. Silicosis : Complicated PneumoconiosisGurney . • Mass-like aggregates of micronodules • Irregularly elliptical in shape • ± Calcification, Cavitation • Produce emphysema in distal lung • May lead to pneumothorax • Synonym : progressive massive fibrosis
- 109. DPLD in Rheumatoid Arthritis Gurney . • Pleural thickening / effusion • Reticulonodular, honeycombing, bronchiectasis • Lower zone predominant Rheumatoid nodule (5%)
- 110. DPLD in Rheumatoid Arthritis Gurney . • Pleural thickening / effusion • Reticulonodular, honeycombing, bronchiectasis • Lower zone predominant • Rheumatoid nodule (5%) • Subpleural • Wax & Wane • May cavitate
- 111. DPLD in Rheumatoid Arthritis Gurney . • Middle-aged female • History & other features of RA • Erosion of distal clavicle
- 112. Lung Changes in SLE Gurney . • Pleural thickening / effusion 50% : small, uni / bilateral • Bibasilar • Reticular • Nodular • Centrilobular • Ground-Glass • Bronchiectasis • Honeycombing
- 113. Lung Changes in SLE Gurney • Pleural thickening / effusion 50% : small, uni / bilateral • Bibasilar • Reticular • Nodular • Centrilobular • Ground-Glass • Bronchiectasis • Honeycombing
- 114. Lung Changes in SLE Gurney • Pleural thickening / effusion 50% : small, uni / bilateral • Bibasilar • Reticular • Nodular • Centrilobular • Ground-Glass • Bronchiectasis • Honeycombing
- 115. Langerhans Cell HistiocytosisMurray & Nadel • Centrilobular nodules and cysts • Most prominent in the upper lobes • Occasionally accompanied by pneumothorax
- 116. Langerhans Cell HistiocytosisMurray & Nadel • Centrilobular nodules and cysts • Most prominent in the upper lobes • Occasionally accompanied by pneumothorax
- 117. Langerhans Cell HistiocytosisMurray & Nadel • Centrilobular nodules and cysts • Most prominent in the upper lobes • Occasionally accompanied by pneumothorax
- 118. Murray & NadelLymphangioleiomyomatosis • Rounded, thin-walled cysts throughout • Pneumothorax or pleural effusion (chylous) may accompany
- 119. Murray & NadelSarcoidosis • hilar & mediastinal L/N • nodules deposited along bronchovascular bundles and interlobular septa • air space filling due to lymphocyte-macrophage alveolitis • linear densities secondary to fibrotic scarring
- 120. ILD AtlasSarcoidosis : Stage 1 Bilateral hilar lymphadenopathy
- 121. ILD AtlasSarcoidosis : Stage 2 Bilateral hilar lymphadenopathy with diffuse pulmonary infiltration
- 122. ILD AtlasSarcoidosis : Stage 3 Diffuse pulmonary infiltration without lymphadenopathy
- 123. Sarcoidosis : Stage 4 ILD Atlas Fibrocystic, bullous, and emphysematous changes
- 124. Other Modalities • Gallium-67 • Selectively accumulates in areas of infection, inflammation, and neoplasm • Assessment of the extent of sarcoidosis ILD Atlas
Editor's Notes
- ILD represent a heterogeneous group of conditions that involve the parenchyma of the lung—the alveoli, the alveolar epithelium, the capillary endothelium, and the spaces between those structures—as well as the perivascular and lymphatic tissues. this heterogeneous group are classified together because of similar clinical, roentgenographic, physiologic, or pathologic manifestations (H-18).
- ILD represent a heterogeneous group of conditions that involve the parenchyma of the lung—the alveoli, the alveolar epithelium, the capillary endothelium, and the spaces between those structures—as well as the perivascular and lymphatic tissues. this heterogeneous group are classified together because of similar clinical, roentgenographic, physiologic, or pathologic manifestations (H-18).
- ILD represent a heterogeneous group of conditions that involve the parenchyma of the lung—the alveoli, the alveolar epithelium, the capillary endothelium, and the spaces between those structures—as well as the perivascular and lymphatic tissues. this heterogeneous group are classified together because of similar clinical, roentgenographic, physiologic, or pathologic manifestations (H-18).
- ILD represent a heterogeneous group of conditions that involve the parenchyma of the lung—the alveoli, the alveolar epithelium, the capillary endothelium, and the spaces between those structures—as well as the perivascular and lymphatic tissues. this heterogeneous group are classified together because of similar clinical, roentgenographic, physiologic, or pathologic manifestations (H-18).
- ILD represent a heterogeneous group of conditions that involve the parenchyma of the lung—the alveoli, the alveolar epithelium, the capillary endothelium, and the spaces between those structures—as well as the perivascular and lymphatic tissues. this heterogeneous group are classified together because of similar clinical, roentgenographic, physiologic, or pathologic manifestations (H-18).
- ILD represent a heterogeneous group of conditions that involve the parenchyma of the lung—the alveoli, the alveolar epithelium, the capillary endothelium, and the spaces between those structures—as well as the perivascular and lymphatic tissues. this heterogeneous group are classified together because of similar clinical, roentgenographic, physiologic, or pathologic manifestations (H-18).
- ILD represent a heterogeneous group of conditions that involve the parenchyma of the lung—the alveoli, the alveolar epithelium, the capillary endothelium, and the spaces between those structures—as well as the perivascular and lymphatic tissues. this heterogeneous group are classified together because of similar clinical, roentgenographic, physiologic, or pathologic manifestations (H-18).
- Distal to terminal bronchioles, i.e. take part in gas exchange
- secondary pulmonary lobule or parts of it can be seen by CT, even when lung is normal. That is why the secondary pulmonary lobule is the ideal unit of subsegmental lung organisation with which the CT and pathologic abnormality can be correlated. At the same time, HRCT is the only radiological method that can visualize details of the secondary pul-monary lobule, which is why it is the most useful imaging modality for ILD.
- septa penetrate deeply as incomplete partitions from the subpleural space between lung segments and subsegments but also between secondary pulmonary lobules and Acini . components of sec lob are not separated by septa but only supported by fibrous strands.
- septa penetrate deeply as incomplete partitions from the subpleural space between lung segments and subsegments but also between secondary pulmonary lobules and Acini . components of sec lob are not separated by septa but only supported by fibrous strands.
- D/D by pattern of interstitial involvement * Parenchymal : Elastic & collagen fibres that provide a supporting framework for the alveolar capillaries * peripheral connective tissue includes the sub-pleural space and the lung septa that penetrate deeply as incomplete partitions from the subpleural space into the lung not only between lung segments and subsegments but also between secondary pulmonary lobules and acini (Weibel1979). So the pleura is in anatomic continuity with the different lung septa. * AXIAL : originates at the hilum, surrounds the bronchovascular structures and extends peripherally upto at the centre of the acini
- D/D by pattern of interstitial involvement * Parenchymal : Elastic & collagen fibres that provide a supporting framework for the alveolar capillaries * peripheral connective tissue includes the sub-pleural space and the lung septa that penetrate deeply as incomplete partitions from the subpleural space into the lung not only between lung segments and subsegments but also between secondary pulmonary lobules and acini (Weibel1979). So the pleura is in anatomic continuity with the different lung septa. * AXIAL : originates at the hilum, surrounds the bronchovascular structures and extends peripherally upto at the centre of the acini
- Identification of these patterns is an important step in the evaluation of HRCT chest, followed by consideration of the location of involvement and patient data (ct lung)
- Also known Acinar nodules (dr swajal)
- may near centre of lobule, near interlobular septa / parenchyma in between, also in relation to pleura and arterial branches but have no consistent or predominant relationship with any of these structures.
- may near centre of lobule, near interlobular septa / parenchyma in between, also in relation to pleura and arterial branches but have no consistent or predominant relationship with any of these structures.