ILD Talk培训讲义.pptVIP

BOOP, RB-ILD, COP, UIP, and DIP; The ABCs of Interstitial Lung Disease (ILD) Andrew R. Fischer, MD, FCCP Pulmonary and Critical Care Division, Sigma Medical Group Clinical Case HF is a 68 year old male. Smoked 1ppd from age 18-50. Retired accountant. Married. Pt noted DOE when walking up/down hills on vacation in Rome. Denies prior DOE—wife notes pt “not keeping up” during walks w/ group over past year or more. No recent CP, productive cough, F/C/Sweats. Neg family hx of ILD. Meds: Simvastatin, ASA Exam: Appears well ~ stated age. Vitals Nl except RR=22 HEENT: Nl; Neck: Nl; Chest: Dry inspiratory crackles over lower 1/3 bilaterally. Cor: Nl; Abdo: Soft Obese; Ext: No Edema, early clubbing, no cyanosis Questions to think about….. Which form(s) of interstitial lung disease (ILD) are strongly associated with cigarette smoking? What is the median survival of Idiopathic Pulmonary Fibrosis (IPF) from the time of diagnosis? What are some of the FDA approved treatment(s) for IPF? Hallmarks of ILD Nonmalignant Noninfectious Often insidious in onset Primary Disease vs. Significant component of a multi-system illness Classically Described as of known vs. unknown etiology Common Symptoms Signs of ILDs on Presentation Dyspnea on exertion Cough, most often dry Dry inspiratory crackles on chest exam Digital Clubbing Evidence of cor pulmonale Idiopathic Interstitial PneumoniasUnderstanding the Alphabet Soup IPF—Idiopathic Pulmonary Fibrosis (UIP histology) NSIP—Nonspecific Interstitial Pneumonia RB-RB/ILD-DIP family GIP-Giant Cell Interstitial Pneumonitis AIP-Acute Interstitial Pneumonitis—aka Haman Rich Syndrome IIP Mimic UIP Autoimmune UIP/IPF NSIP RB-ILD/DIP AIP RA Scleroderma DM/PM Sjorgren’s MCTD Idiopathic Pulmonary Fibrosis (IPF) Histology: UIP Older people ~ +60 Family clusters exist (may be younger) Males:Females ~ 3:1 Associated with smoking IPF Subpleural, Lower Lung Predominance Idiopathic Pulmonary Fibrosis (IPF) Treatment-No FDA approved