Interstitial Lung Diseases Definition, classification and epidemiology Haluk Türktaş Professor of Pulmonary Medicine Gazi University Ankara
Interstitial Lung Diseases Definition of ILD A diverse group of pulmonary disorders with variable degrees of pulmonary inflammation and fibrosis that are classified together because of similar clinical, roentgenographic, physiologic or pathologic manifestations.
THE LUNG INTERSTITIUM Webb WR, Müller NL, Naidich DP. High resolution CT of the lung New York Lippincott-Raven
Interstitial Lung Diseases Exogenous and endogenous stimuli Dust Fume Cigarette smoke Autoimmune conditions Injury Drugs Infections Radiation Other diseases Activation of the inflammatory and repair response
Interstitial Lung Diseases Classification of DILD DPLD of known cause or association e.g: drugs. Collagen dis. Idiopathic Interstitial Pneumonias Granulomatous ILD e.g.: Sarcoidosis Other forms of IP e.g. LAM, HX,eosinophilic pneumonias... Idiopathic Pulmonary Fibrosis/ UIP IP other than Idiopathic Pulmonary fibrosis Desquamative Respiratory Bronchiolitis ILD Acute Cryptogenic organising pneumonia Non-spesific Lymphocytic Am J Respir Crit Care Med 2002;165:277-304
Inhaled inorganic dusts Coal Coalworkers pneumoconiosis pneumoconiosis Aspestosis Aspestosis Silicosis Silicosis Talc Talcpneumoconiosis Aluminium Aluminiumoxide oxidefibrosis Berylliosis Berylliosis Heavy Heavymetal fibrosis fibrosis Siderosis Siderosis Stannosis Stannosis Inhaled organic dusts Bird Birdbreeder s breeder slung lung Farmer s Farmer slung Bagassosis Bagassosis Coffee Coffeeworker s worker slung Mushroom Mushroomworker s worker slung Tea Teagrower s grower slung Malt Malt worker s worker slung Tobacco Tobaccogrower s grower slung Collagen Vascular Diseases Drugs Systemic Systemiclupus lupuseritematosis Ankylozing Ankylozingspondylitis Rheumatoid Rheumatoidartritis artritis Mixed Mixedcollagen Scleroderma Scleroderma Primary PrimarySjögren SjögrenSyndr. Syndr. Polimiyositis Polimiyositis Behçet Behçet Syndrome Syndrome Dermatomiyositis Dermatomiyositis Goodpasture GoodpastureSyndrome Amiodorone Amiodorone Bleomycin Bleomycin Propranolol Propranolol Busulfan Busulfan Tocainide Tocainide Cyclophosphamide Nitrofrantoin Nitrofrantoin Chlorambucil Chlorambucil Sulfasalazine Sulfasalazine Melphalan Melphalan Cephalasporinler Cephalasporinler Methotrexate Methotrexate Gold Gold Azothioprine Azothioprine Penicilamine Penicilamine Cytosine Cytosinearabiniside Phenytoin Phenytoin Carmustine Carmustine Mitomycine Mitomycine Lomustine Lomustine Antidepresants Antidepresants
Interstitial Lung Diseases Classification of DILD DPLD of known cause or association e.g: drugs. Collagen dis. Idiopathic Interstitial Pneumonias Granulomatous ILD e.g.: Sarcoidosis Other forms of IP e.g. LAM, HX,eosinophilic pneumonias... Idiopathic Pulmonary Fibrosis/ UIP SarcoidosisIP Lymphangioleyomyomatosis other than Idiopathic Wegener Pulmonary Granulomatosis Langerhans fibrosis Cell Histiocytosis Churg-Strauss Alveolar Syndrome proteinosis Lymphamatoid Alveolar granulomatosis microlithiasis Respiratory Bronchiolitis ILD Bronchocentric Eosinophilic granulomatosis pneumonia Desquamative Acute Cryptogenic organising pneumonia Non-spesific Lymphocytic Am J Respir Crit Care Med 2002;165:277-304
Interstitial Lung Diseases Classification of DILD DPLD of known cause or association e.g: drugs. Collagen dis. Idiopathic Interstitial Pneumonias %40-50 Granulomatous ILD e.g.: Sarcoidosis Other forms of IP e.g. LAM, HX,eosinophilic pneumonias... Idiopathic Pulmonary Fibrosis/ UIP IP other than Idiopathic Pulmonary fibrosis Desquamative Respiratory Bronchiolitis ILD Acute Cryptogenic organising pneumonia Non-spesific Lymphocytic Am J Respir Crit Care Med 2002;165:277-304
Survival for IIP 100 80 Non IPF 60 IPF Others 40 NSIP 20 UIP 0 0 2 4 6 8 10 12 14 16 18 Years Bjoraker JA et al. Am J Respir Crit Care Med. 1998;157:199.
Interstitial Lung Diseases Classification of DILD DPLD of known cause or association e.g: drugs. Collagen dis. Idiopathic Interstitial Pneumonias %40-50 Granulomatous ILD e.g.: Sarcoidosis Other forms of IP e.g. LAM, HX,eosinophilic pneumonias... Idiopathic Pulmonary Fibrosis/ UIP %47-64 Desquamative IP other than Idiopathic Pulmonary fibrosis Respiratory Bronchiolitis ILD Acute Cryptogenic organising pneumonia Non-spesific Lymphocytic Am J Respir Crit Care Med 2002;165:277-304
UIP
Interstitial Lung Diseases Classification of DILD DPLD of known cause or association e.g: drugs. Collagen dis. Idiopathic Interstitial Pneumonias %40-50 Granulomatous ILD e.g.: Sarcoidosis Other forms of IP e.g. LAM, HX,eosinophilic pneumonias... Idiopathic Pulmonary Fibrosis/ UIP %47-64 Desquamative IP other than Idiopathic Pulmonary fibrosis Respiratory Bronchiolitis ILD Acute Cryptogenic organising pneumonia %14-36 Non-spesific Lymphocytic Am J Respir Crit Care Med 2002;165:277-304
Cellular NSIP
Fibrotic NSIP
Survival for UIP vs NSIP 100 100 Cellular NSIP 80 NSIP 80 60 60 % Alive 40 40 Mixt NSIP 20 UIP 20 Fibrotic NSIP 0 0 1 2 3 4 5 6 7 0 0 20 40 60 80 100 Years Months Daniil ZD et al. Am J Respir Crit Care Med. 1999;160:899.
Interstitial Lung Diseases Classification of DILD DPLD of known cause or association e.g: drugs. Collagen dis. Idiopathic Interstitial Pneumonias %40-50 Granulomatous ILD e.g.: Sarcoidosis Other forms of IP e.g. LAM, HX,eosinophilic pneumonias... Idiopathic Pulmonary Fibrosis/ UIP %47-64 Desquamative IP other than Idiopathic Pulmonary fibrosis %10-17 Respiratory Bronchiolitis ILD Acute Cryptogenic organising pneumonia %14-36 Non-spesific Lymphocytic Am J Respir Crit Care Med 2002;165:277-304
RB ILD DIP
Interstitial Lung Diseases Classification of DILD DPLD of known cause or association e.g: drugs. Collagen dis. Idiopathic Interstitial Pneumonias %40-50 Granulomatous ILD e.g.: Sarcoidosis Other forms of IP e.g. LAM, HX,eosinophilic pneumonias... Idiopathic Pulmonary Fibrosis/ UIP %47-64 Desquamative IP other than Idiopathic Pulmonary fibrosis %10-17 Respiratory Bronchiolitis ILD Acute Cryptogenic organising pneumonia %4-12 %14-36 Non-spesific Lymphocytic Am J Respir Crit Care Med 2002;165:277-304
COP
Interstitial Lung Diseases Classification of DILD DPLD of known cause or association e.g: drugs. Collagen dis. Idiopathic Interstitial Pneumonias %40-50 Granulomatous ILD e.g.: Sarcoidosis Other forms of IP e.g. LAM, HX,eosinophilic pneumonias... Idiopathic Pulmonary Fibrosis/ UIP %47-64 Desquamative IP other than Idiopathic Pulmonary fibrosis %10-17 Respiratory Bronchiolitis ILD <%2 Acute Cryptogenic organising pneumonia %4-12 %14-36 Non-spesific Lymphocytic <%2 Am J Respir Crit Care Med 2002;165:277-304
LUNG INJURY Age Genetic factors Environmental factors Nature of the injury -etiologic agent -recurrent vs single -endothelial vs epithelial HISTOPATHOLOGICAL PATTERN DIP RB-ILD LIP COP NSIP AIP UIP INFLAMMATION FIBROSIS Thannickal VJ Ann Rev Med 2004;53:395-417
Interstitial Lung Diseases / IPF Prevelance of DILD per 100 000 Male Female Occupational / Enviromental 20.8 0,6 Drugs / Radiation 1,2 2,2 Collagen Vascular Diseases 7,7 11 Idiopathic Pulmonary Fibrosis 20,2 13,2 Sarcoidosis 8,3 8,8 Coultas DG. Am J Respir Crit Carre Med. 1994
Interstitial Lung Diseases Incidence and Prevalence of Idiopathic Pulmonary Fibrosis Ganesh Raghu et al, Am J Respir Crit Care Med 2006; 174: 810-816
Interstitial Lung Diseases Incidence and andmortality of of idiopathic pulmonary fibrosis and andsarcoidosis in in the theuk J J Gribbin, Thorax 2006;61:980-985 IPF Incidence: 4.6 /100,000 Mean age 71 years, 62% male 4.000 new cases each year Sarcoidosis Incidence: 5.0 /100,000 Mean age 47 years, 47% male 3.000 new cases each year
Interstitial Lung Diseases Incidence and andmortality of of idiopathic pulmonary fibrosis and andsarcoidosis in in the theuk DILD Tanımı J J Gribbin, Thorax 2006;61:980-985
Interstitial Lung Diseases Incidence and andmortality of of idiopathic pulmonary fibrosis and andsarcoidosis in in the theuk J J Gribbin, Thorax 2006;61:980-985
Interstitial Lung Diseases Incidence and andmortality of of idiopathic pulmonary fibrosis and andsarcoidosis in in the theuk J J Gribbin, Thorax 2006;61:980-985 Small cell lung cancer: 6.000 Mesothelioma: 1.800 Ovarian cancer: 5.600 Kidney cancer: 4.300 IPF: 4.000 Sarcoidosis: 3.000 cases each year
THE SECONDARY LOBULE Webb WR, Müller NL, Naidich DP. High resolution CT of the lung New York Lippincott-Raven
Granulomatous ILD Sarcoidosis Wegener Granulomatosis Churg-Strauss Syndrome Lymphamatoid granulomatosis Bronchocentric granulomatosis Well defined ILD Lymphangioleyomyomatosis LangerhansCell Histiocytosis Alveoler proteinosis Microlithiasis Eosinophilic pneumonia