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. 2000161646-664. ATS/ERS Consensus
Statement. Am J Respir Crit Care Med.
2002165277-304.
4
US Demographics

• Incidence gt 30,000 patients/year
• Prevalence gt 80,000 current patients
• Age of onset 40 to 70 years
• Two-thirds gt 60 years old at presentation
• Males gt females
• Caucasians gt minorities

ATS/ERS Consensus Statement. Am J Respir Crit
Care Med. 2000161646-664. 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. 2000161646-664.
6
IPF PrognosisA Comparative Analysis
Disease 5-Year Survival Lung
Cancer (all) 15 IPF 3050 CHF 50
Colorectal Cancer 62 Breast
Cancer 87 Prostate Cancer 98
ATS/ERS Consensus Statement. Am J Respir Crit
Care Med. 2000161646-664. 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/healt
h/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. 2000161646-664.
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, Sjögrens,
  SLE)
• Other lung diseases (asbestosis,
  hypersensitivity
• pneumonitis)

ATS/ERS Consensus Statement. Am J Respir Crit
Care Med. 2000161646-664. ATS/ERS Consensus
Statement. Am J Respir Crit Care Med.
2002165277-304.
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. 2000161646-664. ATS/ERS Consensus
Statement. Am J Respir Crit Care Med.
2002165277-304.
10
Clinical Evaluation

• Physical Exam
• Age gt 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. 2000161646-664. ATS/ERS Consensus
Statement. Am J Respir Crit Care Med.
2002165277-304.
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. 2000161646-664. ATS/ERS Consensus
Statement. Am J Respir Crit Care Med.
2002165277-304.
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. 200348783-785
• ATS. Am J Respir Crit Care Med. 2002166111-117.

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. 2000161646-664.
• Gold Scientific Committee. Am J Respir Crit Care
  Med. 20011631256-1276.