Jeopardy Flashcards
Characteristics of Rare Disease
Chronic, Progressive, Degenerative and life threatening
Challenges of rare diseases for the patients include:
limited scientific understanding of the disease
inequities and difficulties in accessing treatment and care
substantial economic impact for patients and their families
Challenges of rare diseases for the HCP
healthcare provider’s unawareness of rare diseases and their inability to provide optimal care
• treatments not available, or when available, may be costly
lack of clinical guidelines for vast majority of rare diseases
Orphan Drug Act 1983
7 year patent and tax credits
In the United States, a disease or disorder is considered rare when it
affects fewer than 200,000 people in the country at any given time
Patients with rare diseases comprise 1 out of every _______ Americans and _________ of the European Union (EU) population.
10; 6% to 8%
ILD can be caused by HP (Hypersensitivity pneumonitis)
True
Primary function of Resp System
Obtain O2 remove Co2
Cell type responsible for gas exchange
Type 1 alveolar epithelial cells
Healthy lungs
High lung capacity
Obstructive Lung Dx
Asthma, COPD, Chronic Bronchitis, Cystic Fibrosis
What % of IIP (Idiopathic Interstitial Pneumonia) have IPF (Idiopathic Pulmonary Fibrosis)
50%
forced vital capacity (FVC)
which is the volume of air that can be forcefully exhaled from the lungs after taking the deepest breath possible
The pathogenesis of interstitial lung disease
believed to initiate with an injury to structural cells in the lung that is not properly repaired.
The pathogenesis of interstitial lung disease
results in repetitive injury to alveolar epithelial cells
Which cells produce ECM
myofibroblasts
Simplest PFT
Spirometry
Low DLCO can indicate
poor gas exchange
HRCT can be interpreted by
Radiologist
According to the 2018 ATS/ERS/JRS/ALAT guidelines, the HRCT features most commonly seen in UIP (Usual Interstitial Pneumonia) are:
honeycombing, reticulation, traction bronchiectasis, and traction bronchiolectasis.
IPF (Idiopathic Pulmonary Fibrosis) Radiologic you will see
UIP Pattern
IPF (Idiopathic Pulmonary Fibrosis) Symptoms
Dyspnea, cough, bilateral crackles
As the name interstitial lung disease implies, the microscopic changes that occur in the lungs of patients with ILD typically begin within
pulmonary interstitium
a type of transbronchial biopsy that uses a frozen probe to extract lung tissue and has the advantage of removing a larger piece of lung tissue with a higher percentage of alveolar tissue
Cryobiopsy
Probable UIP (Usual Interstitial Pneumonia) Distribution
Subpleural and basal predominant
Bronchoalveolar Lavage (BAL)
BAL cell analysis may be useful in excluding other diagnoses, such as hypersensitivity pneumonitis
Rheumatologist
a physician, specifically an internist or pediatrician, with additional training in the diagnosis and treatment of musculoskeletal diseases and systemic autoimmune conditions, including CTDs (connective tissue diseases) which are referred to as rheumatic diseases.
the 2013 ATS/ERS classification system groups idiopathic interstitial pneumonias into 3 main categories
- major IIPs
- rare IIPs
- unclassifiable IIPs
Underlying Conditions are INDEPENDENT of progressive fibrosis in PF-ILD
True
The following three factors impact how well pulmonary gas exchange by diffusion can occur
- partial pressure gradients of O2 and CO2
- physical properties of the respiratory membrane
- ventilation-perfusion coupling
The classic example of Fibrosing ILD
IPF (Idiopathic Pulmonary Fibrosis)
exposure to an HP( Hypersensitivity Pneumonitis)-inducing environmental agent, such as one of many different types of bacteria, fungi, animal proteins, and chemicals. How to treat?
Remove the agent
TGF-β, PDGF, and FGF are
profibrotic cytokines whose signaling cascades are believed to play an important role in the pathogenesis of ILD.
myofibroblasts lay down ECM that is primarily composed of
dense collagen fibers
