IPF Flashcards
(26 cards)
Describe Cor pulmonale
Right heart failure, an enlargement of the right ventricle due to disease within the lungs and/or pulmonary blood vessels
How do IPF patients typically present to physicians? Describe early and late phase symptoms, compare
Early Phase ~ dyspnea, dry cough, Velcro like crackles, fatigue
Late Phase ~ cyanosis, Cor pulmonale, clubbing of fingers, accentuated pulmonic second sound, peripheral edema
Describe accentuated pulmonic second sound
Occurs when the pulmonary valve closure is louder than usual due to increased pressure in the pulmonary artery
How common is clubbing of the fingers? Is clubbing an early sign or late sign?
Between 25% and 50% of patients with IPF eventually develop clubbing, so it signifies late stage IPF.
What is cyanosis?
It is a bluish discoloration of the skin and mucous membranes due to hypoxia (lack of oxygen).
Describe the suspected pathophysiology of IPF. Please be sure to include the importance ofepithelial injury, fibrotic growth and proliferation, honeycombing, and loss of alveolar cell function.
IPF pathophysiology is not completely understood.
IPF is thought to arise following injury to the alveolar epithelium.
The overall pathogenic mechanism can be divided into 3 general stages:
Epithelial cell injury
Abnormal repair responses
Progressive fibrosis
Injury to the alveolar epithelium promotes normal tissue repair and scar formation (fibrosis), which is unchecked in IPF.
Patients with IPF appear to have abnormal wound-repair mechanisms in the lung.
Lungs damaged by IPF are characterized by the presence of large spaces and resemble a honeycomb structure.
Honeycombing in IPF occurs as a result of alveolar collapse, which increases traction on airway walls and causes dilation of the surrounding bronchi.
Is the pathophysiology of IPF well understood?
no, scientists are still researching the pathophysiology of IPF.
What are the 3 stages of the pathogenic mechanism?
epithelial cell injury, abnormal repair responses, progressive fibrosis
What is the scar formation called?
fibrosis
What is honeycombing?
large spaces in the lungs visible macroscopically that are caused by alveolar collapse
Define acute exacerbations and why they are clinically meaningful.
Acute exacerbations are clinically significant deteriorations or events of unidentifiable cause
The sudden worsening of respiratory symptoms over one month or less, worsening hypoxemia, new infiltrates detectable on chest x-ray or high-resolution computed tomography (HRCT), and the absence of apparent infection or heart disease
Such events may occur at any time during the course of disease
For some patients, exacerbations may be the presenting manifestation of their disease
Acute exacerbations are meaningful because these episodes precede nearly half of the deaths attributed to IPF
They occur in up to 20% of patients within 3 years of diagnosis
They can occur even with mild disease
They have a hospital mortality rate of 50%
They have a median survival time of only 4.2–15.5 months, even when patients survive the acute hospitalization
After an acute exacerbation, patients do not return to prior disease status, and are usually left more compromised
What are some of the clinical signs of an acute exacerbation?
worsening hypoxemia, new infiltrates on chest x-ray or HRCT, and the absence of an infection or heart disease.
What percentage of patients might experience an acute exacerbation in the first 3 years of diagnosis?
up to 20%
What is the hospital mortality rate for patients having an acute exacerbation?
50%
Describe the long-term outlook for patients who have experienced an acute exacerbation?
patients are left more compromised following an acute exacerbation
Walk us through the diagnostic process described in the ATS/ERS guidelines.
3 main criteria for diagnosis of IPF:
Exclusion of other known causes of interstitial lung disorder (ILD) such as domestic and occupational environmental exposures, connective tissue disease, or drug toxicity
The presence of a usual interstitial pneumonia pattern (IPP) on HRCT in patients not subjected to surgical lung biopsy
Specific combinations of HRCT and surgical lung biopsy
Participant may wish to sketch out the algorithm:
What are some of the causes of ILD that need to be excluded for an IPF diagnosis?
domestic and occupational environmental exposures, connective tissue disease, or drug toxicity
What types of procedures and scans are necessary for diagnosis?
HRCT scan and/or lung biopsy
What finding is necessary from an HRCT in order to diagnose IFP?
Presence of a usual interstitial pneumonia pattern (UIP)
What follows a lung biopsy in the diagnostic algorithm when possible/probable UIP is found?
MDD, multidisciplinary discussion between physician team members
Who is involved in the MDD?
Name the MDD involved
What role do pulmonary function tests (PFTs) play in the diagnosis of IPF?
PFTs do not play a role in the diagnosis of IPF, as they do not provide any differential information to other ILDs; some might answer that PFTs play a role in the assessment of disease/impairment severity.
Describe the co-morbidities often seen in IPF and why they are important for physicians to recognize.
Participants should list these co-morbidities:
- Gastroesophageal reflux (GERD)
- Pulmonary hypertension
- Emphysema
- Lung cancer
- Obstructive sleep apnea (OSA)
Reasons these co-morbidities are important:
GERD (where stomach acid refluxes into the esophagus) coexists with IPF in up to 90% of patients.
Pulmonary hypertension has been reported in 32%–84% of patients with IPF.
Pulmonary hypertension may impact eligibility for lung transplantation.
Emphysema frequently coexists with IPF, most likely due to a shared association with cigarette smoking.
The prevalence of emphysema in IPF has not been established, but a number of studies suggest approximately one-third of IPF patients may be affected by both diseases
Lung cancer can occur in approximately 10% to 15% of patients with IPF, which results in a very poor prognosis.
Obstructive sleep apnea (OSA) is prevalent in patients with IPF and may be under-recognized by physicians. In one study, 20% of patients with IPF had mild OSA and 68% had moderate-to-severe OSA; only 12% had a normal apnea-hypopnea index (AHI).
Can you think of any other comorbidities associated with IPF?
GERD/PH/emphysema/lung cancer/OSA—whichever ones not mentioned in their answer—goal is to prompt them to list ALL of the important comorbidities
