Slide 1

SPEAKER-DR.SAGAR DNB MED.RES

Slide 2

ILDs represent a large number of conditions that involve the parenchyma of the lungthe 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

Slide 3

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.

Slide 4

One useful approach to classification is to separate the ILDs into two groups based on the major underlying histopathology: (1)those associated with predominant inflammation and fibrosis, and (2) 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.

Slide 5

ON LUNG RESPONSE- Alveolitis, Interstitial Inflammation, and Fibrosis Granulomatous

Slide 6

Known Cause- Asbestos Fumes, gases Drugs (antibiotics, amiodarone, gold) and chemotherapy drugs Radiation Aspiration pneumonia Residual of adult respiratory distress syndrome

Slide 7

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 (bronchiolitis obliterans with organizing pneumonia) (COD) Nonspecific interstitial pneumonia Connective tissue diseases- Systemic lupus erythematosus, rheumatoid arthritis, ankylosing spondylitis, systemic sclerosis, Sjgren's syndrome, polymyositis-dermatomyositis Pulmonary hemorrhage syndromes Goodpasture's syndrome, idiopathic pulmonary hemosiderosis, isolated pulmonary capillaritis

Slide 8

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)

Slide 9

Known Cause- Hypersensitivity pneumonitis (organic dusts) Inorganic dusts: beryllium, silica

Slide 10

Sarcoidosis Langerhans' cell granulomatosis (eosinophilic granuloma of the lung) Granulomatous vasculitides-- Wegener's granulomatosis, allergic granulomatosis of Churg-Strauss Bronchocentric granulomatosis Lymphomatoid granulomatosis

Slide 11

(Incidence of IPF=26-31 per 100,000)

Slide 12

Slide 13

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

Slide 14

Slide 15

Slide 16

History Physical Exam Chest Imaging Pulmonary Function Testing At Rest Exercise Serologic Studies Tissue examination

Slide 17

FEMALES- LAM Tuberous sclerosis MALES- Pneumoconiosis AgeGender

Slide 18

www.pneumotox.com Schwartz, ILD text book, 4 th edition

Slide 19

History: Occupational and Environmental INORGANIC

Slide 20

ORGANIC: Hypersensitivity Pneumonitis

Slide 21

Slide 22

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

Slide 23

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

Slide 24

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

Slide 25

Resting Tachypnea Shallow breathing Dry crackles Digital clubbing Pulmonary HTN Non-pulmonary findings

Slide 26

Slide 27

Slide 28

Slide 29

Slide 30

Radiographic CXR HRCT Physiologic testing PFT Exercise test Lung Sampling BAL Lung biopsy: (TBBx, Surgical)

Slide 31

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

Slide 32

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

Slide 33

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

Slide 34

Images courtesy of W. Richard Webb, MD. Basal and peripheral reticulationReduced lung volume

Slide 35

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

Slide 36

Slide 37

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

Slide 38

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.

Slide 39

Supine Prone Conventional HRCT

Slide 40

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)

Slide 41

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)

Slide 42

Slide 43

Slide 44

Honeycomb lung

Slide 45

Image courtesy of W. Richard Webb, MD. Reticular opacitiesTraction bronchiectasis Honeycombing Basal and subpleural predominance

Slide 46

HP PCP pneumonia DIP NSIP PAP DAH Fluid

Slide 47

LAM Bronchiectasis E EG

Slide 48

TLC RV VC TLC RV VC TLC RV VC Normal ILD NM Disease

Slide 49

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

Slide 50

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

Slide 51

Most effective method in confirming diagnosis and assessment of disease May identify a more treatable process than originally suspected Avoids confusion and anxiety later in the clinical course if the patient does not respond to therapy or suffers serious side effects from it.

Slide 52

Fiberoptic bronchoscopy initial procedure of choice. If specific diagnosis not made. Then VATS or Surgical biopsy can be done

Slide 53

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 Often Sometimes Never Transbronchial Biopsy Courtesy of Kevin O. Leslie, MD.

Slide 54

VATS is the preferred procedure for obtaining a lung biopsy High diagnostic accuracy Less morbidity and mortality than open lung biopsy BAL and TBBx limited to excluding other IPF mimickers Ideal biopsy Two or more surgical wedge biopsies with areas of normal lung Samples should measure 3 5 cm in length and 2 3 cm in depth Outpatient thoracoscopic lung biopsy can be a safe and effective procedure for patients with interstitial or focal lung disease

Slide 55

Permanent removal of the offending agent, when known Early identification and aggressive suppression of the acute and chronic inflammatory process thereby reducing further lung damage

Slide 56

Hypoxemia (Pa O2 < 55 mmHg) at rest and/or with exercise should be managed by supplemental oxygen. If cor pulmonale develops, diuretic therapy and phlebotomy may occasionally be required

Slide 57

Glucocorticoid therapy is recommended- for symptomatic ILD patients with eosinophilic pneumonias, COP, CTD, sarcoidosis, acute inorganic dust exposures, acute radiation pneumonitis, DAH, and drug- induced ILD In organic dust disease, glucocorticoids are recommended for both the acute and chronic stages.

Slide 58

A common starting dose is prednisone, 0.51 mg/kg in a once-daily oral dose for 412 weeks. If the patient is stable or improved, the dose is tapered to 0.250.5 mg/kg for an additional 4 12 weeks depending on the course. Rapid tapering or a shortened course of glucocorticoid treatment can result in recurrence. If the patient's condition continues to decline while on glucocorticoids, a second agent added and the prednisone dose is lowered to or maintained at 0.25 mg/kg per day.

Slide 59

Cyclophosphamide and azathioprine (12 mg/kg lean body weight per day), with or without glucocorticoids in IPF, vasculitis, and other ILDs. An objective response usually requires at least 812 weeks to occur Other agents, including methotrexate, colchicine, penicillamine, and cyclosporine, have been tried. However, their role in the treatment of ILDs remains to be determined

Slide 60

Many cases of ILD are chronic and irreversible despite the therapy discussed above, and lung transplantation may then be considered

Slide 61

No therapy effective in the management of acute exacerbations of IPF Often mechanical ventilation is required but is usually not successful, with a hospital mortality rate of up to three-fourths of the patients. In those that survive, a recurrence of acute exacerbation is common and usually results in death at those times.

Slide 62

DIP AIP Idiopathic BOOP.

Slide 63

Slide 64

Unlike patients with IPF (UIP), Majority of patients with NSIP have a good prognosis (5-year mortality rate estimated at