Clinical Tools for the Primary Care Physician

1. Clinical Tools for the Primary Care Physician

2. Objectives • Raise the clinical index of suspicion for ILD in patients presenting with the hallmark signs and symptoms • Identify the importance of early referrals to pulmonologists for potential IPF patients

3. Current Definition of IPF A distinct type of chronic fibrosing interstitial pneumonia of unknown cause, limited to the lungs, and associated with a surgical lung biopsy showing a histologic pattern of UIP ATS/ERS Consensus Statement. Am J Respir Crit Care Med. 2000;161:646-664. ATS/ERS Consensus Statement. Am J Respir Crit Care Med. 2002;165:277-304.

4. US Demographics • Incidence: > 30,000 patients/year • Prevalence: > 80,000 current patients • Age of onset: 40 to 70 years • Two-thirds > 60 years old at presentation • Males > females • Caucasians > minorities ATS/ERS Consensus Statement. Am J Respir Crit Care Med. 2000;161:646-664. Weycker D, et al. Paper presented at: CHEST 2002, November 2-7, 2002; San Diego, California.

5. IPF Prognosis • Median length of survival from diagnosis: 2.5 to 5 years • 5-year survival rate from diagnosis: 30% to 50% • Factors associated with increased survival • Younger age • Female gender • Milder dyspnea (less functional impairment) • Response to therapy • Cigarette smoking at time of diagnosis (unexplained result) ATS/ERS Consensus Statement. Am J Respir Crit Care Med. 2000;161:646-664.

6. IPF Prognosis:A Comparative Analysis Disease5-Year Survival Lung Cancer (all) 15% IPF 30–50% CHF 50% Colorectal Cancer 62% Breast Cancer 87% Prostate Cancer 98% ATS/ERS Consensus Statement. Am J Respir Crit Care Med. 2000;161:646-664. American Lung Association. Lung Disease Data 2003. Available at: www.lungusa.org/dta/s2s03/ldd03.pdf. National Institutes of Health. Congestive Heart Failure Data Fact Sheet. Available at: www.nhlbi.gov/health/public/heart/other/CHF.htm.

7. Potential Risk Factors • Familial (genetic) • Smoking • Environmental factors (eg, occupational exposure to • wood dust or metal dust) • Chronic aspiration associated with gastroesophageal • reflux disease (GERD) • Infectious agents ATS/ERS Consensus Statement. Am J Respir Crit Care Med. 2000;161:646-664.

8. Diseases That Mimic IPF • IPF is often misdiagnosed or diagnosed at an advanced stage of the disease • Symptoms of other diseases that mimic IPF: • COPD • CHF • Connective tissue diseases (eg, RA, Sjögren’s, SLE) • Other lung diseases (asbestosis, hypersensitivity pneumonitis) ATS/ERS Consensus Statement. Am J Respir Crit Care Med. 2000;161:646-664. ATS/ERS Consensus Statement. Am J Respir Crit Care Med. 2002;165:277-304.

9. Clinical Evaluation • Patient History • Comorbid diseases • Environmental exposures (tobacco, alcohol, recreational drugs) • Medications (eg, antibiotics, chemotherapeutic agents, radiation) • Occupational exposures (dates, duration, detailed description of work activities) • Pets • Previous malignancy and treatment • Family history ATS/ERS Consensus Statement. Am J Respir Crit Care Med. 2000;161:646-664. ATS/ERS Consensus Statement. Am J Respir Crit Care Med. 2002;165:277-304.

10. Clinical Evaluation • Physical Exam • Age > 50 years • Gradual onset of symptoms • Progressive dyspnea on exertion • Usually present 6 months • Nonproductive paroxysmal cough • Usually unresponsive to antitussives • Bibasilar “Velcro-like” crackles on auscultation • Digital clubbing (25% to 50% of patients) ATS/ERS Consensus Statement. Am J Respir Crit Care Med. 2000;161:646-664. ATS/ERS Consensus Statement. Am J Respir Crit Care Med. 2002;165:277-304.

11. Referring Patients to a Pulmonologist • Consider referring if the following are noted: • Progressive dyspnea on exertion  3 months • Unexplained dry cough  3 months • Desaturation occurs on oximetry testing • – Drop in saturation • Chest radiograph is abnormal • – Inflammation • – Fibrosis • – Lower-lobe predominance ATS/ERS Consensus Statement. Am J Respir Crit Care Med. 2000;161:646-664. ATS/ERS Consensus Statement. Am J Respir Crit Care Med. 2002;165:277-304.

12. 6-Minute Walk Test for Interstitial Lung Disease • Baseline blood pressure, pulse, O2 saturation • Timed walk at any pace • As many stops as necessary • If oxygen saturation persistently less than • 88%, repeat test with supplemental oxygen • Primary endpoint is walk distance Enright PL. Respir Care. 2003;48:783-785 ATS. Am J Respir Crit Care Med. 2002;166:111-117.

13. Typical Features of IPF on Chest X-Ray Abnormal CXR Normal CXR Slide courtesy of Ganesh Raghu, MD.

14. Terms in Radiology ReportsThat Should Get Your Attention • Chronic changes • Increased interstitial markings • Prominent interstitial changes • Fibrosis Abnormal CXR CXR courtesy of Ganesh Raghu, MD.

15. Radiographic Features Slide courtesy of W. Richard Webb, MD.

16. Multidisciplinary Approach to IPF Pulmonologists Serologies History PFTs Biopsy PE PCPs Pathologists CXR HRCT Radiologists

17. Reasons for Early Referral • Confirm Dx (some ILDs treatable) • List for lung transplant • Recent data suggests early intervention may • improve outcomes • Several on-going trials for potential new • therapies

18. Idiopathic Pulmonary Fibrosis: Ongoing Clinical Trials N/A = not available

19. Additional Management Issues • Patients should enroll in a pulmonary physical rehabilitation program • Supplemental oxygen • Lung transplant • Patient education and support is imperative ATS/ERS Consensus Statement. Am J Respir Crit Care Med. 2000;161:646-664. Gold Scientific Committee. Am J Respir Crit Care Med. 2001;163:1256-1276.