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Approach To Interstitial Lung Diseases

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Approach To Interstitial Lung Diseases

  1. 1. بسم الله الرحمن الرحيم ”ق الوا سبحانك لا علم لنا إلا ما علمتنا إنك أنت العليم الحكيم ” ) صدقالله العظيم ) البقرة – 32
  2. 2. Approach To Interstitial Lung Diseases or Diffuse Parenchymal Lung Diseases
  3. 3. By: Dr Consultant Chest Physcian TB TEAM Expert - WHO Mansoura -Egypt
  4. 4. What is the Pulmonary Interstitium? Interstitial compartment is the area of the lung between the alveolar epithelial and capillary endothelial basement membranes
  5. 5. Nomenclature More than 200 diseases can result in Interstitial Lung Disease (ILD). The term interstitial is misleading since most of these disorders are also associated with extensive alterations of alveolar and airway architecture. Diffuse parenchymal lung diseases (DPLD) increasingly in favor worldwide as a generic term for these disorders.
  6. 6. Epidemiology o ILD is more frequent than previously recognized. o Incidence ranges from 3 to 26 per 100.000 per year. o The prevalence of preclinical and undiagnosed ILD in the community is 10 times that of clinically recognized. o Among these, IPF is the most common, representing at least 30% of the incident cases.
  7. 7. ILD presents a clinical conundrum as;  1st at least 150 clinical entities and situation are associated with ILD.  2nd difficulty to determine the best specific diagnostic approach.  3rd a conclusive cause cannot be ascertained (even after lung biopsy) in a significant portion of patients.  Finally even when a specific diagnosis is made, an effective therapeutic regimen is not available for many patients with ILD.
  8. 8. Table 1: Potential Causes /Categories of Interstitial Lung Disease Cause Categories Byssinosis Malt worker's lung Coffee worker's lung Bird fancier's lung Farmer's lung Bagassosis Occupational or other inhaled organic agents (EAA/HP) Talc pneumoconiosis Berylliosis Hard metal fibrosis Silicosis Asbestosis Coal worker's pneumoconiosis Occupational or other inhaled inorganic agents Ankylosing spondylitis Sjogrens syndrome Bechet`s disease Dermatopolymyositis SLE Rheumatoid arthritis Scleroderma Mixed CT disease Collagen vascular disease related Chemotherapeutics ( Bleomycin, Methotrexate, Busulfan) Drug induced Lupus (Phyenytoin, procainamide) Antiarrhythmics (Amiodarone) Antibiotics (Nitrofurantoin, sulfasalazine) Gold Drug related
  9. 9. Table 1: Potential Causes /Categories of Interstitial Lung Disease Cause Categories Radiation / Radiotherapy Paraquat toxicity Oxygen Physical agents & toxins Tuberous sclerosis Neurofibromatosis Niemann-Pick disease Sarcoidosis Amyloidosis Lymphangioleiomyomatosis Primary disease diagnosis Pulmonary Langerhans cell histiocytosis Bronchoalveolar carcinoma Lymphangitis carcinomatosis Neoplastic diseases Churg -Strauss syndrome Wegener`s granulomatosis Vasculitides Pulmonary lymphoma Chronic aspiration Alveolar protienosis Lipoid pneumonia Eosinophilic pneumonia Alveolar filling diseases Pulmonary edema Pulmonary veno-occlusive disease Disorders of circulation Tuberculosis Residue of active infection of any type Infection
  10. 10. Diffuse Parenchymal Lung Disease (DPLD) DPLD of known cause, eg, Drugs or association, eg, Collagen vascular disease Idiopathic interstitial pneumonias Granulomatou s DPLD, eg, Sarcoidosis ,HP Other forms of DPLD, eg, LAM, HX, etc Idiopathic pulmonary fibrosis IIP other than idiopathic pulmonary fibrosis Desquamative interstitial pneumonia Acute interstitial pneumonia Nonspecific interstitial pneumonia Respiratory bronchiolitis interstitial lung disease Cryptogenic organizing pneumonia Lymphocytic interstitial pneumonia ATS/ERS Consensus Statement. Am J Respir Crit Care Med. 2002.
  11. 11. Diffuse Parenchymal Lung Disease (DPLD) DPLD of known cause, eg, Drugs or association, eg, Collagen vascular disease Idiopathic interstitial pneumonias Granulomatou s DPLD, eg, Sarcoidosis ,HP Other forms of DPLD, eg, LAM, HX, etc Idiopathic pulmonary fibrosis IIP other than idiopathic pulmonary fibrosis Desquamative interstitial pneumonia Acute interstitial pneumonia Nonspecific interstitial pneumonia Respiratory bronchiolitis interstitial lung disease Cryptogenic organizing pneumonia Lymphocytic interstitial pneumonia ATS/ERS Consensus Statement. Am J Respir Crit Care Med. 2002.
  12. 12. Diffuse Parenchymal Lung Disease (DPLD) DPLD of known cause, eg, Drugs or association, eg, Collagen vascular disease Idiopathic interstitial pneumonias Granulomatou s DPLD, eg, Sarcoidosis ,HP Other forms of DPLD, eg, LAM, HX, etc Idiopathic pulmonary fibrosis IIP other than idiopathic pulmonary fibrosis Desquamative interstitial pneumonia Acute interstitial pneumonia Nonspecific interstitial pneumonia Respiratory bronchiolitis interstitial lung disease Cryptogenic organizing pneumonia Lymphocytic interstitial pneumonia ATS/ERS Consensus Statement. Am J Respir Crit Care Med. 2002.
  13. 13. Idiopathic Interstitial Pneumonia (IIP) RB-ILD: Respiratory Bronchiolitis Interstitial Lung Disease DIP: Desquamative Interstitial Pneumonia LIP: Lymphocytic Interstitial Pneumonia AIP: Acute Interstitial Pneumonia COP: Cryptogenic Organizing Pneumonia IPF: Idiopathic Pulmonary Fibrosis (IPF) NSIP: Nonspecific Interstitial Pneumonia
  14. 14. ATS/ERS Classification of Idiopathic Interstitial Pneumonias Histologic Pattern Clinical/Radiologic/Pathologic Diagnosis Usual interstitial pneumonia Idiopathic pulmonary fibrosis/cryptogenic fibrosing alveolitis Nonspecific interstitial pneumonia Nonspecific interstitial pneumonia Organizing pneumonia Cryptogenic organizing pneumonia Diffuse alveolar damage Acute interstitial pneumonia Respiratory bronchiolitis Respiratory bronchiolitis interstitial lung disease Desquamative interstitial pneumonia Desquamative interstitial pneumonia Lymphoid interstitial pneumonia Lymphoid interstitial pneumonia
  15. 15. 2002 International Consensus Classification of the Idiopathic Interstitial Pneumonias • Acute –Acute interstitial pneumonia (AIP) • Subacute o Nonspecific interstitial pneumonia (P) (NSIP) o Cryptogenic Organizing Pneumonia (COP) o Desquamative interstitial pneumonia (DIP) o Respiratory bronchiolitis-associated / (RBILD) interstitial lung disease o Lymphoid interstitial pneumonia (LIP) • Chronic • Usual interstitial pneumonia (UIP)
  16. 16. Clinical IPF has “Usual Interstitial Pneumonia (UIP) pathologically. . . UIP ≈ IPF
  17. 17. 2002 UIP ? Fibrotic NSIP Prognostic Classification of ILD ? COP Drug reaction Connective tissue disease Cellular NSIP Hypersensitivity Pneumonitis Good Bad
  18. 18. Diagnostic Criteria for IPF in the Absence of a Surgical Lung Biopsy Major Criteria Exclusion of other known causes of ILD Evidence of restriction and/or impaired gas exchange HRCT: bibasilar reticular abnormalities with minimal ground glass opacities TBB or BAL that does not support an alternative diagnosis Minor Criteria Age > 50 years Insidious onset of otherwise unexplained dyspnea on exertion Duration of illness > 3 months Bibasilar, inspiratory, Velcro® crackles All major criteria and at least 3 minor criteria must be present to increase the likelihood of an IPF diagnosis ATS/ERS. Am J Respir Crit Care Med. 2000;161:646-664.
  19. 19. Approach to Diagnosing IPF Clinical Evaluation: History, PE, CXR, PFTs, 6MWT Not IIP Potential IIP HRCT Diagnostic of IPF or other diffuse lung disease Diagnosis uncertain Surgical lung biopsy Transbronchial Bx or BAL Diagnostic Nondiagnostic Not IPF IPF Adapted from ATS/ERS Consensus Statement. Am J Respir Crit Care Med. 2002.
  20. 20. Approach to the Diagnosis of ILD Clinical • History • Physical • Laboratory • PFTs Primary care physicians Radiology • Chest X-ray • HRCT Pathology • Surgical lung biopsy Pulmonologists Radiologists Pathologists Multidimensional and multidisciplinary
  21. 21. Clinical Assessment • History • Physical Exam • CXR - HRCT • Pulmonary Function Testing – At Rest – Exercise • Serologic Studies • Tissue examination
  22. 22. Making the Diagnosis in ILD • HISTORY, HISTORY
  23. 23. History 1) Age 2) Gender 3) Smoking history 4) Medications 5) Duration of symptoms 6) Environmental exposure 7) Occupational exposure 8) Family history
  24. 24. HISTORY 1. AGE: Some of the ILDs are more common in certain age groups: Age 20-40 years - Sarcoidosis - CTD - LAM - EG > 50years - Idiopathic pulmonary fibrosis (cryptogenic fibrosing alveolitis)
  25. 25. HISTORY 2. GENDER: - Premenopausal female: LAM (lymphangioleiomatosis) - Female predominant: ILD associated with CTD. - Male predominant: ILD associated with RA Pneumoconiosis
  26. 26. HISTORY 3.SMOKING: Diseases associated with smoker: - EG (histocytosis X) - Desquamative interstitial pneumonitis - Respiratory broncholitis ILD Diseases less likely to be seen in smoker: - Hypersensditivity pneumonitis -Sarcoidosis
  27. 27. History: Smoking • All of the following DPLD are associated with smoking except: a) IPF b) RBILD c) DIP d) HP e) Histiocytosis X • In Goodpasture’s syndrome – 100% of smokers vs. 20% of nonsmokers experience pulmonary hemorrhage • Individuals exposed to asbestos who smoke are more likely to develop asbestosis
  28. 28. HISTORY 4. Drugs
  29. 29. Drug-Induced Interstitial Lung Disease Antibiotics Nitrofurantoin, Sulfsalazine Anti-inflammatory agents Aspirin Gold Pencillamine Chemotherapeutic agents Bleomycin sulfate Mitomycin C Busulfan Cyclophosphamide Chlorambucil Melphalan Azathioprine Cytosine arabinoside Methotrexate Miscellaneous O2 Drugs inducing pulmonary infiltrates and eosinophilia Radiation L-tryptophan Drug-induced SLE Procainamide hydrochloride Isoniazid Hydralazine hydrochloride The hydantoins Pencillamine Illicit drugs Heroin Methadone hydrochloride Propoxyphene hydrochloride (Darvon)
  30. 30. HISTORY 5.DURATION: i. Insidious over months or years (e.g. IPF, Sarcoidosis) ii. Acute (less than 3 weeks) (e.g. drug reaction, acute hypersensitivity pneumonitis, chemical exposure) iii. Subacute: 3-12 weeks (e.g. BOOP)
  31. 31. History 6. OCCUPATIONAL HISTORY AND ENVIROMENTAL EXPOSURES • Each of the following requires specific exposure: 1. Pneumoconiosis 2. Hypersensitivity pneumonitis (HP) • The occupational history should begin with the patient’s first job and continues chronologically. • The patient should be asked to describe the exact duties at each job.
  32. 32. Occupational ????
  33. 33. CHEMICAL AGENTS ASSOCIATED WITH ILD CHEMICAL AGENT SOURCE OF EXPOSURE Nitrogen dioxide Agriculture (Silo-Filler’s disease) Nitrogen oxide Electrical arc welding Chlorine Accidental spills Sulfur dioxide Manufacturing: sulfites, sulfates, fumigants, commercial refrigerants Oxygen Mechanical ventilation
  34. 34. HISTORY 7. FAMILY HISTORY: Occasionally helpful. ¨ Autosomal dominant pattern: - IPF - Sarcoidosis - Neurofibromatosis ¨ Autosomal recessibe pattern: Gauchen’s disease
  35. 35. Physical examination of the respiratory system is rarely helpful in the diagnostic evaluation of interstitial lung diseases.
  36. 36. INTENSITY OF SYMPTOMS: i. Minimal symptoms in the presence of grossly abnormal chest radiograph (e.g., Sarcoidosis, histocytosis X). ii. Severe symptoms in the presence of mild radiograph abnormalities (e.g., IPF, HP). iii. Sudden worsening of dyspnea (particularly if assocaites with pleural pain) may indicate a spontaneous pneumothorax.
  37. 37. Diagnostic approach: Clinical picture • Typically present with progressive exertional dyspnoea and/or persistent nonproductive cough. • Other unusual chest symptoms: – Haemoptysis  e.g. alveolar hemorrhage syndromes, pulmonary vascular diseases – Pleuritic chest pain  e.g. collagen vascular illness. – Wheezing  e.g. Churg -Strauss syndrome & EAA. • Dry bibasilar crepitations / Squeaks. • Clubbing (most common in IPF) • Cyanosis. • Signs of right heart failure. Late in advanced disease
  38. 38. • New onset of hemoptysis in a patient with known ILD suggests a complicating malignancy. • Fever: IPF, RA are almost never associated with fever.
  39. 39.  Diffuse crackles: presence or absence adds little. May be present in the presence of normal c-xray.  “Velcro rales” are common in most forms of ILD. They are less likely to be heared in sarcoidosis.  Inspiratory squeaks typical of Boop.  Clubbing: most commonly seen in IPF but non-specific. Rare in EG, sarcoidosis, HP.
  40. 40. • Among patients with ILD clubbing is found in 25-50% of patients with IPF and 50% of patients with DIP and 75% of patients with ILD from rheumatoid arthritis.
  41. 41. Extra thoracic manifestations : directive but not diagnostic. i. Nasal discharge or other upper airway symptoms that suggest Wegener’s granulomatosis. ii Arthritis: CVD, Sarcoidosis or granulomatous vasculitides. iii. IPF: arthralgias but never true synovitis. iv. Skin rashes: common to Sarcoidosis, CVD and granulomatous vasculitis.
  42. 42. DPLD: Evaluation
  43. 43. DPLD: Evaluation • Rdiographic – CXR – HRCT • Physiologic testing – PFT – Exercise test • Lung Sampling – BAL – Lung biopsy: (TBBx, Surgical)
  44. 44. Radiological Patterns of ILD • Interstitial lung disease may result in four patterns of abnormal opacity on chest radiographs and CT scans: 1. Linear 2. Reticular 3. Nodular 4. Reticulonodular • These patterns are more accurately and specifically defined on (HRCT) scan - key investigation
  45. 45. Patterns of Interstitial Lung Disease
  46. 46. CHEST RADIOGRAPH  ILD is often suspected on the basis of an abnormal chest x-ray.  Review all previous films to assess the rate of change in disease activity.  Remember, chest radiograph is normal in 10% of patients with ILD (particularly those with HP).
  47. 47. A normal CXR does not rule out the presence of DPLD
  48. 48. – CXR is normal in 10 % of ILD - May be mistaken for heart failure
  49. 49. Patterns of Interstitial Lung Disease
  50. 50. Linear Pattern •A linear pattern is seen when there is thickening of the interlobular septa, producing Kerley 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 Kerley B lines Kerley A lines
  51. 51. DD of Kerly Lines • Pulmonary edema is the most common cause • Mitral stenosis • Lymphangitic carcinomatosis • Malignant lymphoma • Congenital lymphangiectasia • Idiopathic pulmonary fibrosis • Pneumoconiosis • Sarcoidosis
  52. 52. Reticular Pattern • A classic reticular pattern is seen with Idiopathic pulmonary fibrosis, in which multiple curvilinear opacities form small cystic spaces along the pleural margins and lung bases (honeycomb lung) • Asbestosis • Connective tissue disease • Hypersensitivity pneumonitis
  53. 53. Peripheral Reticular pattern
  54. 54. Classical idiopathic Pulmonary Fibrosis
  55. 55. This 50-year-old man presented with end-stage lung fibrosis PA chest radiograph shows medium to coarse reticular opacities CT scan shows multiple small cysts (honeycombing) involving predominantly the subpleural peripheral regions of lung. Traction bronchiectasis, another sign of end-stage lung fibrosis.
  56. 56. IPF is a progressive disease. Successive computed tomography (CT) lung images show reticular infiltrates with honeycombing in the basilar and peripheral portions of the lung. The patient's disease was progressive and she died from respiratory failure at the age of 61.
  57. 57. 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
  58. 58. Nodular pattern in sarcoidosis. Posteroanterior chest radiograph shows numerous bilateral nodules involving mainly the upper and middle lung zones. Also note evidence of right paratracheal lymph node enlargement (arrow). The patient was a 37-year-old woman with sarcoidosis.
  59. 59. Nodular Pattern. Innumerable tiny nodules are randomly scattered throughout both lungs. Some of them also involve the fissures (arrowheads), however not insistently. A few relatively spared areas with less nodules show reduced vascularization [this was a miliary reactivation of an old tuberculosis (TB)].
  60. 60. Disseminated histoplasmosis and nodular ILD. CT scan shows multiple bilateral round circumscribed pulmonary nodules.
  61. 61. Hypersensitivity pneumonitis.
  62. 62. Differential diagnosis of a nodular pattern of interstitial lung disease SHRIMP 1. Sarcoidosis 2. Histiocytosis (Langerhans cell histiocytosis) 3. Hypersensitivity pneumonitis 4. Rheumatoid nodules 5. Infection (mycobacterial, fungal, viral) 6. Metastases 7. Microlithiasis, alveolar 8. Pneumoconioses (silicosis, coal worker's, berylliosis)
  63. 63. Reticulonodular pattern  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.
  64. 64. Reticulonodular pattern
  65. 65. Reticulonodular pattern
  66. 66. A 71-year-old white man with rheumatoid arthritis (RA) and no pulmonary complaints. A and B, Posteroanterior (PA) and lateral chest radiographs demonstrate a bilateral reticulonodular pattern with a basal predominance
  67. 67. Computed tomography demonstrating diffuse reticular-nodular densities and bilateral groundglass opacities.
  68. 68. Drug Reaction
  69. 69. A single cut of the high-resolution computed tomography scan showing diffuse ground glass and reticulonodular opacities, suggestive of interstitial pneumonitis.
  70. 70. fine reticulonodular opacities throughout . both lungs. (b) High-resolution CT scan obtained the same day shows ground-glass attenuation, intralobular interstitial thickening, and ill-defined small nodular hyperattenuating area ©2001 by Radiological Society of North America
  71. 71. Thorax CT scans are showing the bilateral interstitial and peribronchial reticulonodular infiltrations
  72. 72. How To Approach a Practical Diagnosis?
  73. 73. Rule no. 1 An acute appearance suggests  edema or  pneumonia. An acute appearance suggests pulmonary edema or pneumonia
  74. 74. 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
  75. 75. Pulmonary fibrosis and rheumatoid arthritis.
  76. 76. 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).
  77. 77. Asbestosis
  78. 78. 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
  79. 79. 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
  80. 80. 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).
  81. 81. Langerhans Cell Histiocytosis (Eosinophilic Granuloma)
  82. 82. o The presence of cystic images within the parenchyma raises the possibilities of three major cystic ILD LAM, Tuberous sclerosis and Langerhans cell granulomatosis o In LAM and Tuberous sclerosis, the cysts are numerous, thin walled, typically less than 2 mm in diameter and distributed throughout the pulmonary parenchyma. o In Langerhans cell granulomatosis cysts are bizar
  83. 83. Rule no. 4 Associated lymphadenopathy suggests : 1.Sarcoidosis 2.Neoplasm (lymphangitic carcinomatosis, lymphoma, metastases) 3. Infection (viral, mycobacterial, or fungal) 4. Pneumoconiosis ( Silicosis , Berylliosis )
  84. 84. 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
  85. 85. Rule no. 5 Associated pleural thickening and/or calcification suggest asbestosis.
  86. 86. Rule no. 6 Associated pleural effusion suggests : 1.pulmonary edema 2.lymphangitic carcinomatosis 3.lymphoma 4.collagen vascular disease (RA ,SLE)
  87. 87. 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.
  88. 88. Rule no. 7 Associated pneumothorax suggests 1. lymphangioleiomyomatosis 2. Langerhans Cell Histiocytosis LCH. 3. IPF
  89. 89. 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 thin-walled cysts and a loculated right pneumothorax (P).
  90. 90. 1. Acute •P.Edema •Pneumonia 2. Pleural effusion •1.pulmonary edema •2.lymphangitic carcinomatosis •3.lymphoma 3.Pneumothorax •4.collagen vascular disease •LAM •LCH 4.Predominantly Below with reduced volume 1. Asbestosis 2. Aspiration (chronic) 3. Pulmonary fibrosis (idiopathic) 4.Collagen vascular disease
  91. 91. 5. A middle or upper lung predominant 1. Mycobacterial or fungal disease 2. Silicosis 3. Sarcoidosis 4. Langerhans Cell Histiocytosis 6. Associated lymphadenopathy 1.Sarcoidosis 2.neoplasm (lymphangitic carcinomatosis, lymphoma, metastases) 3. infection (viral, mycobacterial, or fungal) 4. Silicosis , Berylliosis 7. Pleural Thickening and or Calcification •Asbestosis
  92. 92. Helpful Radiographic Patterns in the Differential Diagnosis of Interstitial Lung Disease Upper zone predominance  Granulomatous disease Sarcoidosis Pulmonary histiocytosis X (eosinophilic granuloma) Chronic hypersensitivity pneumonitis Chronic infectious diseases (e.g.TB, histoplasmosis)  Pneumoconiosis Silicosis Berylliosis Coal miners’ pneumoconiosis Hard metal disease  Miscellaneous Rheumatoid arthritis (necrotic nodular form) Ankylosing spondylitis Radiation fibrosis
  93. 93. Helpful Radiographic Patterns in the Differential Diagnosis of Interstitial Lung Disease • Peripheral lung zone predominance Bronchiolitis obliterans with organzing pneumonia Eosinophilic pneumonia IPF • Lower zone predominance Idiopathic pulmonary fibrosis Rheumatoid arthritis (associated with UIP) Asbestosis
  94. 94. Peripheral Location COP IPF
  95. 95. Helpful Radiographic Patterns in the Differential Diagnosis of Interstitial Lung Disease • Lung consolidation Lobar/Segmental Infiltrates Chronic or acute eosinophilic pneumonia Bronchiolitis obliterans with organizing pneumonia Aspiration (lipid pneumonia) Alveolar carcinoma Lymphoma Alveolar proteinosis • Isolated lung cysts Pulmonary histiocytosis X Lymphangioleiomyomatosis Chronic PCP
  96. 96. Helpful Radiographic Patterns in the Differential Diagnosis of Interstitial Lung Disease • Haze or ground glass attenuation Hypersensitivity pneumonitis Desquamative interstitial pneumonia Respiratory bronchiolitis-ILD Drug toxicity Pulmonary hemorrhage • In acute hypersensitivity pneumonitis HRCT show multifocal diffuse ground glass attenuation despite a normal chest radiograph. • Smokers with symptomatic RBILD typically have patchy ground glass attenuation on HRCT.
  97. 97. Extrinsic Allergic Alveolitis (Hypersensitivity Pneumonitis)
  98. 98. Desquamative Interstitial Pneumonia
  99. 99. • HRCT scans are more sensitive and have a greater ability to detect anatomic abnormalities than do chest radiograph. • It helps the surgeon to identify areas of non-fibrotic, active disease and relatively unaffected areas to guide appropriate site selection for biopsy.
  100. 100. • HRCT helps in identifying "active and reversible inflammation" (ground glass attenuation) and irreversible fibrotic manifestations (traction bronchiectasis and honeycombing). • Extensive fibrotic changes suggest end or advanced stage disease with limited potential for both invasive diagnostic and therapeutic approaches which could be toxic.
  101. 101. Pulmonary Function  Regardless of the cause, a restrictive lung defect and decreased diffusing capacity (DLco) are the predominant physiological abnormalities seen in ILD.  Exercise affords the most sensitive diagnostic and physiological test for ILD. The degree of arterial hypoxemia induced by exercise and the alveolar-arterial difference in P02 (PAO2 – PaO2 gradient) correlate well with the degree of pulmonary fibrosis.
  102. 102. Pulmonary Function • Usually a restrictive abnormality – Normal FEV1/FVC ratio – Reduced VC and TLC – Impaired diffusing capacity • Arterial hypoxemia at rest / exercise. • Mixed PFT abnormality if DPLD is superimposed on COPD • Isolated abnormality in diffusion – early interstitial pneumonia
  103. 103. Pulmonary Function  Most of the ILD have a restrictive defect.  Mixed pattern: - Sarcoidosis - Hypersensitivity Pneumonitis (HP) - Histocytosis X - Lymphangioleiomyomatosis (LAM) - Wegener’s granulomatosis - Broncholitis obliterans organizing pneumonia (BOOP) rarely present with mixed pattern
  104. 104. Bronchoalveolar lavage (BAL) • Narrow the differential diagnosis. • Diagnostic: infectious causes, occupational exposures, malignancy & pulmonary alveolar proteinosis. • Differential cell count of BAL: – Sarcoidosis: lymphocytosis / ++ T-helper cells & high CD4/CD8 ratio : > 2. – HP: marked T lymphocytosis/ CD4: CD8 < 1. – IPF: increases in neutrophils and eosinophils.
  105. 105. Bronchoalveolar lavage (BAL) The value of differential cell count of BAL in the diagnosis of DPLD has been extensively explored & found to be less helpful.
  106. 106. "Bronchoalveolar lavage"
  107. 107. Transbronchial Lung Biopsy (TBX) • Minimally invasive • Performed at the same time as BAL. • Useful in the diagnosis of some ILDs e.g. Sarcoidosis, infection or lymphangitis carcinomatosis .  Unfortunately, TBX is of limited value in the diagnosis IIPs  small amount of tissue obtained.
  108. 108. Surgical lung biopsy • Surgical lung biopsy remains the “gold standard” for diagnosis. • The size of specimens, site of biopsy, expertise of pathologists are factors that may preclude a conclusive diagnosis. • The site of the biopsy should be chosen on the basis of HRCT findings and ideally be at the interface of involved and less involved lung tissue. • A biopsy of more than one site in the lung is more helpful.
  109. 109. Surgical lung biopsy - Video-assisted Thoracoscopic (VATS) biopsy -Open lung biopsy VATS lung biopsy is the preferred method of obtaining lung tissue. Indication for biopsy - Inflammatory cause - Young patient (might require many years of immunosuppression) - Diagnosis nor clear •If HRCT shows classical pattern of IPF, then biopsy is not required
  110. 110. Probability of Histologic Diagnosis of DOLD Surgical Biopsy 1. Granulomatous diseases 2. Malignant tumors/lymphangitic 3. DAD (any cause) 4. Certain infections 5. Alveolar proteinosis 6. Eosinophilic pneumonia 7. Vasculitis 8. Amyloidosis 9. EG/HX/PLCH 10. LAM 11. RB/RBILD/DIP 12. UIP/NSIP/LIP COP 13. Small airways disease 14. PHT and PVOD Transbronchial Biopsy Often Sometimes Never Courtesy of Kevin O. Leslie, MD.

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