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INTERSTITIAL LUNG DISEASE

INTERSTITIAL LUNG DISEASE. SPEAKER-DR.SAGAR DNB MED.RES. INTRODUCTION.

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INTERSTITIAL LUNG DISEASE

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  1. INTERSTITIAL LUNG DISEASE SPEAKER-DR.SAGAR DNB MED.RES

  2. INTRODUCTION • ILDs represent a large number of conditions that involve the parenchyma of the lung—the alveoli, the alveolar epithelium, the capillary endothelium, and the spaces between these structures, as well as the perivascular and lymphatic tissues. • Also called Diffuse Parenchymal Lung Disease

  3. INTRODUCTION • Heterogeneous group of disorders have similar clinical, roentgenographic, physiologic, or pathologic manifestations. • Often associated with considerable morbidity and mortality, and there is little consensus regarding the best management of most of them.

  4. CLASSIFICATION One useful approach to classification is to separate the ILDs into two groups based on the major underlying histopathology: those associated with predominant inflammation and fibrosis, and those with a predominantly granulomatous reaction in interstitial or vascular areas Each of these groups can be further subdivided according to whether the cause is known or unknown. For each ILD there may be an acute phase, and there is usually a chronic one as well. Rarely, some are recurrent, with intervals of subclinical disease.

  5. Major Categories of Alveolar and Interstitial Inflammatory Lung Disease • ON LUNG RESPONSE- • Alveolitis, Interstitial Inflammation, and Fibrosis • Granulomatous

  6. Alveolitis, Interstitial Inflammation, and Fibrosis • Known Cause- • Asbestos • Fumes, gases • Drugs (antibiotics, amiodarone, gold) and chemotherapy drugs • Radiation • Aspiration pneumonia • Residual of adult respiratory distress syndrome

  7. UNKNOWN CAUSES • Idiopathic interstitial pneumonias (IIP)- •  Idiopathic pulmonary fibrosis (usual interstitial pneumonia) (UIP) • Desquamative interstitial pneumonia(DIP) •  Respiratory bronchiolitis-associated interstitial lung disease (RB-ILD) •  Acute interstitial pneumonia (diffuse alveolar damage) •  Cryptogenic organizing pneumonia (bronchiolitisobliterans with organizing pneumonia) (COD) •  Nonspecific interstitial pneumonia • Connective tissue diseases- •  Systemic lupus erythematosus, rheumatoid arthritis, ankylosingspondylitis, systemic sclerosis, Sjögren's syndrome, polymyositis-dermatomyositis • Pulmonary hemorrhage syndromes • Goodpasture's syndrome, idiopathic pulmonary hemosiderosis, isolated pulmonary capillaritis

  8. CONT… • Pulmonary alveolar proteinosis • Lymphocytic infiltrative disorders (lymphocytic interstitial pneumonitis associated with connective tissue disease) • Eosinophilic pneumonias • Lymphangioleiomyomatosis(LAM) • Amyloidosis • Inherited diseases- •   Tuberous sclerosis, neurofibromatosis, Niemann-Pick disease, Gaucher's disease, Hermansky-Pudlak syndrome • Gastrointestinal or liver diseases -(Crohn's disease, primary biliary cirrhosis, chronic active hepatitis, ulcerative colitis) • Graft-vs.-host disease- (bone marrow transplantation; solid organ transplantation)

  9. Granulomatous • Known Cause- • Hypersensitivity pneumonitis (organic dusts) • Inorganic dusts: beryllium, silica

  10. Unknown Cause(GRANULOMATOUS) • Sarcoidosis • Langerhans' cell granulomatosis (eosinophilicgranuloma of the lung) • Granulomatousvasculitides-- •  Wegener's granulomatosis, allergic granulomatosis of Churg-Strauss • Bronchocentricgranulomatosis • Lymphomatoidgranulomatosis

  11. Incident Cases of ILD (Incidence of IPF=26-31 per 100,000)

  12. 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

  13. Clinical Assessment • History • Physical Exam • Chest Imaging • Pulmonary Function Testing • At Rest • Exercise • Serologic Studies • Tissue examination

  14. History: Age and Gender Age Gender • FEMALES- • LAM • Tuberous sclerosis • MALES- • Pneumoconiosis

  15. History: Medications www.pneumotox.com Schwartz, ILD text book, 4th edition

  16. History: Occupational and Environmental INORGANIC

  17. ORGANIC: Hypersensitivity Pneumonitis

  18. Occupational ????

  19. History: Duration of Illness • 1. Acute Diseases (Days to weeks) • AIP, EP, Drugs, Hypersensitivity • ________________________________________________________________________________________________________________ • 2. Subacute Diseases (weeks to months) • Sarcoid, Drug, COP • __________________________________________________________________________________________________________________ • 3. Chronic Diseases (months to years) • IPF, Pneumoconioses,LCH,Sarcoidosis

  20. PRESENTATION • Dyspnea is a common and prominent complaint in patients with ILD. • Non-Productive Cough • Fatigue,Weight loss • Symptoms associated with other diseases • Clubbing ,cyanosis

  21. EXAMINATION • Late inspiratory ‘Velcro’ crackles unaltered by coughing at lung bases are heard. • Features of RV failure • Loud P2 present.

  22. Physical Findings • Resting Tachypnea • Shallow breathing • Dry crackles • Digital clubbing • Pulmonary HTN • Non-pulmonary findings

  23. Laboratory

  24. ILD: Evaluation • Radiographic • CXR • HRCT • Physiologic testing • PFT • Exercise test • Lung Sampling • BAL • Lung biopsy: (TBBx, Surgical)

  25. CXR CLUES Interstitial Infiltrates • Nodular • Linear or reticular • Mixed • Honeycomb • Cysts and traction bronchiectasis • GGO

  26. CXR CLUES Alveolar Filling • Air-bronchograms • Acinar rosettes • Diffuse consolidation • Nodule like, poor boarder definition • Silhouetting: obliteration of normal structures

  27. Adenopathy Sarcoidosis Lymphoma Lymphangitic CA Amyloidosis Berylliosis Silicosis Radiographic Patterns in ILD Pleural Involvement Kerley B lines • Lymphangitic Carcinomatosis • LAM • Drug Induced • Radiation Pneumonitis • Asbestosis • Effusion • Thickening • Plaques • Mesothelioma • Collagen vascular disease Chronic LV failure Lymphangitic CA Lymphoma LAM Veno-occlusive disease Acute Eosinophilic Pneumonia

  28. IPF: CXR Reduced lung volume Basal and peripheral reticulation Images courtesy of W. Richard Webb, MD.

  29. 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

  30. A normal CXR does not rule out the presence of ILD

  31. HRCT • 2 essential technical factors: • Narrow collimation • Use of a high spatial frequency reconstruction algorithm • Does not use contrast • Prone and supine • Inspiratory and expiratory

  32. Characteristic HRCT findings • Bibasilar interstitial and intralobular reticular opacities • Interlobular septal thickening • Subpleural honeycomb changes • Traction bronchiectasis in the lower lobes. • There may also be a variable amount of ground-glass opacity.

  33. HRCT Conventional Supine Prone

  34. 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)

  35. HRCT Clues • What is the dominant HR-pattern: • Reticular • Nodular • High attenuation (ground-glass, consolidation) • Low attenuation (emphysema, cystic) • Where is it located within the secondary lobule (centrilobular, perilymphatic or random) • Is there an upper versus lower zone? • Central versus peripheral predominance • Are there additional findings (pleural involvement, lymphadenopathy, traction bronchiectasis)

  36. HRCT: Radiographic Pattern

  37. HRCT Findings in Late IPF

  38. Honeycomb lung

  39. Classic IPF HRCT Basal and subpleural predominance Reticular opacities Traction bronchiectasis Honeycombing Image courtesy of W. Richard Webb, MD.

  40. Ground Glass Pattern • HP • PCP pneumonia • DIP • NSIP • PAP • DAH • Fluid

  41. Cysts or Cyst Like Bronchiectasis LAM EG E

  42. TLC VC TLC VC TLC VC RV RV RV PFT: Lung VolumesRestrictive Disease ILD NM Disease Normal

  43. DLCO AND ABG • DLCO reduced but nonspecific. • Does not correlate with severity of disease • ABG- • Normal to severe hypoxemia • Retained CO2 rare ..in end stage disease

  44. FiberopticBronchoscopy and BronchoalveolarLavage (BAL) • In selected diseases (e.g., sarcoidosis, hypersensitivity pneumonitis, DAH syndrome, cancer, pulmonary alveolar proteinosis) • Useful in narrowing D/D • Usefulness of BAL in the clinical assessment and management remains to be established

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