Diagnosis

• There is an average of 1-2 year delay from symptom onset to diagnosis^1-3
• The most common symptoms of IPF are dyspnea and dry cough^4,5
• Patients may present with digital clubbing^6-8
• Dry, inspiratory crackles are another common sign of IPF^4,9

COMMON SYMPTOMS SIGNIFICANTLY IMPACT QUALITY OF LIFE IN PATIENTS WITH IPF.¹⁰

• Depression: Patients may experience fear, worry, anxiety, and panic related to their poor prognosis^10,11
• Fatigue: Fatigue/exhaustion occurs frequently,¹⁰ and is exacerbated by further muscle deconditioning¹¹
• Dyspnea: Dyspnea is universal in patients with IPF¹¹ and is extremely distressing¹⁰
• Cough: Chronic cough causes significant impairment in patients’ lives¹⁰
• Dyspnea and physical function capacity are strong drivers of health-related quality of life¹²

DIFFICULTY WITH DIAGNOSIS IS COMMON IN PATIENTS WITH IPF.^1-3

• Difficulty with diagnosis is common, as the early symptoms of IPF are nonspecific and the disease itself is rare.^13,14
  • Symptoms may be attributed to more common ailments such as bronchitis, asthma, chronic obstructive pulmonary disease (COPD), emphysema, or heart disease¹⁴
• 38% of patients report seeing 3 or more physicians before receiving a diagnosis of IPF¹⁴
• >50% of patients are initially misdiagnosed¹⁴
• 1-2 year delay between onset of symptoms and diagnosis^1-3

THE DIAGNOSTIC PROCESS CAN TAKE AN AVERAGE OF 1-2 YEARS^1-3

• Bronchitis, asthma, COPD, emphysema, or heart disease are often considered first, as symptoms of IPF are common manifestations of these diseases¹⁴
• Delayed referral to subspecialty care center was associated with higher mortality rates in patients with IPF. Delay is defined as time from onset of dyspnea to time of initial evaluation at a tertiary care center¹⁵

DIAGNOSTIC TOOLS

There are several diagnostic tools for evaluating interstitial lung disease.

CLINICAL ASSESSMENT

• Comprehensive medical history^4,16,17
  • Past medical history^4,18
  • Current and past chronic medications^4,18
  • Social history^4,18
  • Family history^4,18,19
  • IPF is a diagnosis of exclusion, making a complete history essential to identifying potential causes of dyspnea and cough⁴
• Physical examination — Chest auscultation⁴
• Pulmonary function testing:^4,17 — FVC, forced expiratory volume in 1 second (FEV₁), diffusing capacity of the lung for carbon monoxide (DL_CO), lung volumes
  • IPF shows a restrictive defect²⁰
• 6-minute walk test (6MWT)⁴
• Pulse oximetry²¹
• Cardiac evaluation to rule out heart disease²¹
• Serologic testing to exclude connective tissue disease (CTDs)^4,16,22

RADIOLOGICAL ASSESSMENT

• Chest x-ray¹⁸
• HRCT^4,16
• Usual interstitial pneumonia pattern
  • Subpleural, basal predominance
  • Heterogeneous distribution
  • Honeycombing with/without traction bronchiectasis or bronchiolectasis

PROCEDURAL ASSESSMENT

• Bronchoscopy
• Surgical lung biopsy (if necessary)^4,16

MULTIDISCIPLINARY MEETING

• Team-based approach to evaluating all evidence in order to reach a consensus diagnosis¹⁶