Definition, classification and epidemiology

Transcription

1 Interstitial Lung Diseases Definition, classification and epidemiology Haluk Türktaş Professor of Pulmonary Medicine Gazi University Ankara

2 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.

3 THE LUNG INTERSTITIUM Webb WR, Müller NL, Naidich DP. High resolution CT of the lung New York Lippincott-Raven

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5 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

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7 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:

8 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

9 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:

10 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:

11 Survival for IIP Non IPF 60 IPF Others 40 NSIP 20 UIP Years Bjoraker JA et al. Am J Respir Crit Care Med. 1998;157:199.

12 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:

13 UIP

14 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:

15 Cellular NSIP

16 Fibrotic NSIP

17 Survival for UIP vs NSIP Cellular NSIP 80 NSIP % Alive Mixt NSIP 20 UIP 20 Fibrotic NSIP Years Months Daniil ZD et al. Am J Respir Crit Care Med. 1999;160:899.

18 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:

19 RB ILD DIP

20 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:

21 COP

22 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:

23 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:

24 Interstitial Lung Diseases / IPF Prevelance of DILD per Male Female Occupational / Enviromental ,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

25 Interstitial Lung Diseases Incidence and Prevalence of Idiopathic Pulmonary Fibrosis Ganesh Raghu et al, Am J Respir Crit Care Med 2006; 174:

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27 Interstitial Lung Diseases Incidence and andmortality of of idiopathic pulmonary fibrosis and andsarcoidosis in in the theuk J J Gribbin, Thorax 2006;61: IPF Incidence: 4.6 /100,000 Mean age 71 years, 62% male new cases each year Sarcoidosis Incidence: 5.0 /100,000 Mean age 47 years, 47% male new cases each year

28 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:

29 Interstitial Lung Diseases Incidence and andmortality of of idiopathic pulmonary fibrosis and andsarcoidosis in in the theuk J J Gribbin, Thorax 2006;61:

30 Interstitial Lung Diseases Incidence and andmortality of of idiopathic pulmonary fibrosis and andsarcoidosis in in the theuk J J Gribbin, Thorax 2006;61: Small cell lung cancer: Mesothelioma: Ovarian cancer: Kidney cancer: IPF: Sarcoidosis: cases each year

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32 THE SECONDARY LOBULE Webb WR, Müller NL, Naidich DP. High resolution CT of the lung New York Lippincott-Raven

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35 Granulomatous ILD Sarcoidosis Wegener Granulomatosis Churg-Strauss Syndrome Lymphamatoid granulomatosis Bronchocentric granulomatosis Well defined ILD Lymphangioleyomyomatosis LangerhansCell Histiocytosis Alveoler proteinosis Microlithiasis Eosinophilic pneumonia