Download presentation
1
Interstitial Lung Disease
Julye N. Carew, M.D. January 12, 2007
2
Interstitial Lung Diseases
Definition Clinical Presentation and evaluation Radiographic Features Classification Treatment Prognosis
3
Definition “Interstitial lung disease”- a misnomer
Few cells in the interstitium of the normal lung Injury to basement membrane shared by epithelium and endothelium Increased alveolar permeability and spillage of serum contents into the alveolar space and recruitment of fibroblasts Collagen deposition Also injury to small airways=respiratory bronchioles, alveolar ducts and terminal bronchioles
4
Alveolar/Interstitial Space
Matrix Connective tissue Murray and Nadel, Text of Resp. Medicine
5
Pathogenesis Epithelial cell apoptosis with loss of basement membrane integrity: production of growth factors in response to alveolar epithelial injury with hyperplastic type II cells and myofibroblast recruitment Noble, et al. Clinics in Chest Med, Dec 2004
6
Pathogenesis UNKNOWN!!! Some understanding of the mechanism of injury
Initiating injuries are likely multiple= inhaled, sensitization to allergens, circulatory With continued injury, “repair” process continues with additional fibroproliferation that is unchecked
7
ILD 81 in 100,000 prevalence in men 67 in 100,000 in women
32.5 in 100,000 incidence in men 26 in 100,000 in women 200 in 100,000 incidence in age >75 30-40% of all ILD “IPF”
8
ILD Due to increasing numbers of cytotoxic drugs, increased detection of occupational lung disease and increasing life expectancy, as well as better imaging and diagnostic testing the incidence of these diseases is expected to rise
9
Evaluation of ILD EXTENSIVE HISTORY
AGE, GENDER, UNDERLYING COMORBIDITIES, DRUGS, SMOKING, OCCUPATIONAL HISTORY, HOBBIES, PETS, FAMHX DURATION OF SYMPTOMS PHYSICAL EXAM LABORATORIES IMAGING SPIROMETRY, LUNG VOLUMES AND DLCO
10
Laboratories LFTs, CBC ANA, RF, hypersensitivity panels, ANCA, anti-GBM ?CRP, ESR
11
Pulmonary function Spirometry Lung volumes Diffusion capacity
12
Spirometry UpToDate, 2007
13
Lung Volumes UpToDate, 2007
14
Diffusion capacity UpToDate, 2007
15
Diffusion Matrix Connective tissue
Murray and Nadel, Text of Resp. Medicine
16
Imaging PA/LAT CXR HRCT
17
GROUND GLASS Schwarz, ILD, 2003, RB
18
NODULAR GROUND GLASS Schwarz, ILD, 2003, HP
19
INTRALOBULAR SEPTAL THICKENING
Schwarz, ILD, 2003, LC
20
RETICULAR INFILTRATES
Schwarz, ILD, 2003, HP
21
HONEYCOMB LUNG Schwarz, ILD, 2003
22
HONEYCOMB LUNG
23
Clinical Classification
Connective Tissue Diseases Drug-induced Primary Unclassified Occupational Idiopathic Disorders
24
Clinical Classification-- CTD
Scleroderma Polymyositis-dermatomyositis Systemic lupus erythematosus Rheumatoid arthritis** Mixed connective tissue disease Ankylosing spondylitis Behcet’s Sjogren’s syndrome
25
RA
26
Rheumatoid Arthritis Interstitial fibrosis Male predominance 3:1
High titers of RF and rheumatoid nodules Occurs in 33% of all RA patients Usually patients are dyspneic, but also limited by joint disease Therapies may cause fibrosis (gold, methotrexate, penicillamine)
27
RA Schwarz and King, ILD 2003
28
Drugs Antibiotics--nitrofurantoin, sulfasalazine, ethambutol
Antiarrhythmics--amiodarone, tocainide, propranolol Anti-inflammatories--gold, penicillamine Anticonvulsants--dilantin Chemotherapeutic agents--mitomycin C, bleomycin, busulfan, cyclophosphamide, chlorambucil, methotrexate, azathioprine, BCNU [carmustine], procarbazine Therapeutic radiation Oxygen toxicity Narcotics Cocaine Paraquat
29
Primary- Unclassified
Sarcoidosis Eosinophilic granuloma Amyloidosis Lipoid pneumonia Lymphangitic carcinomatosis Broncholaveolar carcinoma Pulmonary lymphoma Gaucher's disease Niemann-Pick disease Hermansky-Pudlak syndrome Neurofibromatosis LAM Tuberous sclerosis Acute respiratory distress syndrome AIDS Bone marrow transplantation Postinfectious Eosinophilic pneumonia Alveolar proteinosis Diffuse alveolar hemorrhage syndromes Alveolar microlithiasis Metastatic calcification
30
Occupational Silicosis- tile, glass Asbestosis
Hard-metal pneumoconiosis Coal worker's pneumoconiosis Berylliosis-fluorescent bulbs, dental, electronics Aluminum oxide fibrosis-abrasives Talc pneumoconiosis Siderosis (arc welder)-iron foundry, welders Stannosis (tin)
31
Idiopathic Acute interstitial pneumonitis (Hamman-Rich syndrome)
Idiopathic pulmonary fibrosis “IPF” Familial idiopathic pulmonary fibrosis Desquamative interstitial pneumonitis Respiratory bronchiolitis Cryptogenic organizing pneumonia Nonspecific interstitial pneumonitis Lymphocytic interstitial pneumonia (Sjögren's syndrome, connective tissue disease, AIDS, Hashimoto's thyroiditis) Autoimmune pulmonary fibrosis (inflammatory bowel disease, primary biliary cirrhosis, idiopathic thrombocytopenic purpura, autoimmune hemolytic anemia)
32
Idiopathic ILD Diagnosis is made based upon clinical history, radiographic findings and pathology Some pathologic findings are pathognomonic for a particular disease, while others are suggestive in the correct clinical setting
33
IPF Major Criteria Minor Criteria
• Exclusion of other known causes of ILD, such as certain drug toxicities, environmental exposures, and connective tissue diseases • Abnormal pulmonary function studies that include evidence of restriction (reduced VC often with an increased FEV1 /FVC ratio) and impaired gas exchange [increased AaPo2 with rest or exercise or decreased DLCO ] • Bibasilar reticular abnormalities with minimal ground glass opacities on HRCT scans • Transbronchial lung biopsy or bronchoalveolar lavage (BAL) showing no features to support an alternative diagnosis Minor Criteria • Age > 50 yr • Insidious onset of otherwise unexplained dyspnea on exertion • Duration of illness 3 mo • Bibasilar, inspiratory crackles (dry or “Velcro” type in quality) IPF consensus statement, 2000, AJRCCM
34
Idiopathic pulmonary fibrosis
Pathology=UIP Schwarz, ILD, 2003
35
Treatment Very limited literature, NO RANDOMIZED TRIALS
ATS recommendations: Prednisone 60 mg daily tapering or 20 mg plus Cytoxan mg/day Retrospective case review (n=78 with OLB), UIP=38, NSIP=28, RB/DIP=13 At 6 months, 11% UIP, 29% NSIP, 80% RB/DIP improved Nicholson, AJRCCM, 2000
36
Treatment of IPF Azathioprine
37
Treatment of IPF Colchicine and 2000 studies (Douglas et al., AJRCCM) compared colchicine to prednisone and other combinations and showed no difference in survival in any of the groups but with fewer side effects with Colchicine NAC
38
Treatment of IPF Gamma interferon
Th-1 cytokine that induces CD4 cells to the Th1 phenotype, inhibits the proliferation of fibroblasts and downregulates TGF-β Administered subcu three times weekly Flu-like symptoms Mixed results with this drug in trials, but may be due to the fact that it needs to be used earlier in disease
39
Treatment of IPF Pirfenidone
Pyridone molecule that inhibits TGF-β-stimulated collagen production Ameliorates bleomycin-induced fibrosis in hamsters Undergoing stage III trials
40
IPF King, et al., AJRCCM, 2001
41
DIP Clinical classification=pathology DISEASE OF SMOKERS!!
4th-5th decades Subacute cough and dyspnea CT shows diffuse GG infiltrates Smoking cessation
42
DIP Schwarz, ILD, 2003
43
DIP Leslie, Clinics in Chest Med, 2006
44
RB-ILD SMOKING DISEASE!!
Similar to DIP, but pathology slightly different, in peribronchiolar distribution Smoking cessation +/- steroids
45
RB-ILD Schwarz, ILD, 2003
46
LIP Characterized by monotonous sheets of polyclonal lymphocytes
Clinical=pathology Uncommon Associated with Sjogren’s and AIDS May remit with steroids and other ISD, progress to fibrosis, lead to infectious complications, or develop lymphoma
47
LIP Leslie, Clinics in Chest Med, 2006
48
NSIP Originally represented “difficult to characterize”
Clearly represents a separate disease from IPF Primary and secondary forms (HP, drugs, CVD, AIDS and transplants) Second most common diagnosis Middle-aged, majority smoker’s Surgical biopsy required to make diagnosis HOMOGENEOUS ?? Early UIP
49
NSIP Leslie, Chest, 128:5, 2005
50
Interstitial pneumonias
American Thoracic Society/European Respiratory Society classification of the idiopathic interstitial pneumonias Histologic pattern Clinico-radiologic-pathologic UIP IPF NSIP NSIP Organizing pneumonia COP Diffuse alveolar damage (DAD) AIP Respiratory bronchiolitis pattern RBILD Desquamative pattern DIP Lymphoid pattern LIP
Similar presentations
© 2025 SlidePlayer.com. Inc.
All rights reserved.