02a: Interstitial Lung Disease Flashcards
T/F: Most ILDs are of unknown cause.
True
List four key clinical features of ILDs.
- Dyspnea
- Tachycardia
- Cyanosis
- Crackles
T/F: Pulmonary HT and CHF are common clinical consequences of ILDs.
True
Often the first pulm function test to be abnormal in ILDs is (increase/decrease/not change) in (X).
Decrease;
DLCO
You would expect lung compliance to (increase/decrease/not change) in ILDs since elastic recoil is (increased/decreased/not changed).
Decrease;
Increased
Regardless of cause, the earliest common manifestation of most ILDs is:
Alveolitis (accumulation of inflammatory/immune effector cells within alveolar walls/spaces)
IPF (Idiopathic Pulm Fibrosis) will show (X) histology pattern. Briefly describe what you would see.
X = UIP (usual interstitial pneumonia)
- Predominantly fibrosis along interlobular septa (esp subpleural and lower lobes)
- Temporal heterogeneity
Temporal heterogeneity: areas of ongoing fibrosis, aka (X), can be seen near areas of (Y).
X = fibroplastic foci Y = normal lung or established fibrosis
T/F: Fibrosis/tissue damage in IPF (Idiopathic Pulm Fibrosis) is a result of inflammation.
False! Doesn’t respond to anti-inflammatory drugs
Result of “wound-healing” due to unidentified agent
T/F: IPF can eventually lead to bronchiectasis, metaplasia, and smooth muscle hyperplasia.
True
NSIP (Non-specific Interstitial Pneumonia) clinical course differs from UIP in which way(s)?
- Responds to steroids
2. Much better prognosis
(NSIP/UIP) tends to occur in younger patients.
NSIP
T/F: “Ground glass” opacifications/honeycombing is seen in UIP, but not NSIP.
False
T/F: NSIP will present histologically similar to infectious pneumonia.
False (not evenly distributed)
The least specific pattern of lung injury is (X). When no etiology is identified the term (Y) is applied.
X = Organizing Pneumonia (OP) Y = Cryptogenic Organizing Pneumonia (COP)
T/F: Cryptogenic Organizing Pneumonia (COP) and Desquamative Interstitial Pneumonia (DIP) respond super well to steroid therapy.
True
(UIP/NSIP/COP/DIP) has a clear association with smoking and is characterized by large collections of (X) cells in airspaces.
DIP (Desquamative Interstitial Pneumonia)
X = (“smoker’s”) macrophages (filled with brown pigment)
T/F: DIP is minimally (if at all) associated with fibrosis.
True
T/F: Fibrosis is prominent feature of COP.
False
DIP: Alveolar walls may be thickened due to:
WBCs (lymphocytes, plasma cells, eosinophils)
An immunologically-mediated predominantly interstitial lung disease caused by
prolonged exposure to inhaled organic dusts and antigens.
Hypersensitivity Pneumonitis
“Farmer’s lung” and “air conditioner lung” are examples of which type of pulm interstitial diseases?
HP (Hypersensitivity Pneumonitis)
T/F: Reactions of HP (Hypersensitivity Pneumonitis) occur in interstitium, esp lobar septa.
False - in alveoli (“allergic alveolitis”)
Respiratory Bronchiolitis (RB) ILD is similar to (X) disease, but with (more/less) pronounced symptoms.
X = DIP (Desquamative Interstitial Pneumonia)
Less
List some clinical symptoms/patient history that would make you suspicious for DIP (Desquamative Interstitial Pneumonia).
- Smoker
- Insidious onset of cough, dyspnea
- Digital clubbing (50%)
Presence of circulating immune complexes and small non-caseating granulomas is characteristic of (X) interstitial lung disease.
X = HP (hypersensitivity pneumonitis)
(X) diseases are lung reactions to inhaled mineral dusts (coal dust, silica, asbestos,
beryllium, iron oxides).
X = pneumoconiosis
Non-caseating granulomas and multi-nucleated giant cells in lung make you suspicious of (UIP/NSIP/DIP/Sarcoidosis).
Sarcoidosis
Most prevalent chronic occupational disease in the world.
Silicosis (ILD)
Anthracosis refers to disease in which (X) occurs. At its mildest, it presents with (Y) symptoms.
X = inhaled carbon pigment is engulfed by alveolar and interstitial macrophages (a pneumoconiosis) Y = no
Patients with UIP typically present with (sudden/gradual) increase dyspnea during (exertion/rest/sleep). This is accompanied by (productive/non-productive) cough.
Gradual (insidious);
Exertion;
Non-productive
Orthopnea is:
Shortness of breath (dyspnea) while lying flat
ILDs: PCO2 is typically (high/normal/low), except for advanced disease, when it (rises/drops) relative to normal.
Low or normal (mild respiratory alkalosis);
Rises
Unlike normal individuals, patients with ILDs increase Alv ventilation during exercise by increasing:
Predominantly RR (can’t increase TV)
The majority of ILD patients will have which findings on CXR at initial evaluation.
Nodulolinear Infiltrates (bilateral) with lower lobe, peripheral predominance
T/F: Some patients with ILDs may present with completely normal CXR.
True (but under 10%)
Prominent hilar and mediastinal adenopathy on CXR are common in which disease states?
Sarcoidosis, tumor, infectious granulomatous disease
Peripheral infiltrates with central sparing is seen in (X) disease. The opposite is seen in (Y) disease.
X = chronic eosinophilic pneumonia Y = pulmonary edema
ILDs: Serological studies primarily help in the diagnosis of (X) diseases as the cause for pulm fibrosis.
X = CVD (Collagen Vascular Diseases)
Ex: Lupus, RA
ILDs: while elevated levels of anti-nuclear Ab or Rheumatoid factor may be suggestive of (X) diseases, low levels of these is suggestive of (Y) disease.
X = Lupus or RA; Y = IPF (low levels seen in 30-40% of patients)
IPF: CXR shows (X), CT shows (Y), and biopsy shows (Z).
X = increase in linear markings (esp at bases) Y = patchy peripheral process with honeycombing Z = fibroblastic foci
(UIP/NSIP/HP/DIP) is typically associated with (X) conditions such as scleroderma.
NSIP;
X = rheumatologic
T/F: Both UIP and DIP patterns can be found (simultaneously) in different areas of the same lung.
True
T/F: Diagnosis of UIP can be done by VAT (Video-Assisted Thoroscopy) or transbronchial biopsy.
False - VAT is procedure of choice (biopsy is ok too)
Bronchoscopy/transbronchial biopsy not possible in UIP due to heterogeneous pattern (samples too small, sampling error too great)
New treatment options for IPF include:
Pirfenidone and Nintedanib.
T/F: Management of IPF includes long-term treatment with corticosteroids.
False - doesn’t respond to steroids!!
T/F: Antioxidants have proven to be beneficial for IPF treatment.
False
Nintedanib has been FDA approved for treatment of (X). What’s its mechanism of action?
X = UIP/IPF
Oral Tyrosine Kinase Inhibitor that interferes with multiple receptors (FGF, PDGF, VEGF)
Pirfenidone has been FDA approved for treatment of (X). What’s its mechanism of action?
X = UIP/IPF
We don’t know…
How might unmonitored oxygen therapy accelerate disease progression of IPF?
Increased production of oxygen radicals
“Ground glass” appearance on CT is caused by:
Alveolar inflammation
Signet ring sign on CT is (X) and indicative of which disease complication?
X= dilated airway (diameter larger than plum a it travels with)
Bronchiectasis
