SM 163a - Intro to DPLD Flashcards
What are the common findings of ILD on chest x-ray?
- Reticular or reticulonodular pattern of fibrosis
- Usually prominent in lung bases
- Hilar lymphadenopathy
- Calcified pleural plaques
What patterns are shown in this HRCT?
What disease is this consistent with?
- Ground glass opacities
- Reticulation
- Traction bronchiectasis
Consistent with Nonspecific Interstitial Pneumonia (NSIP)
What defines interstitial lung disease (aka diffuse parenchymal lung disease)?
Damage to the alveolar wall, including the interstitium
- May also involve the alveolar airspace and small conducting airways
- Causes a restrictive defect in lung function
Serologic testing for auto-antibodies is most helpful in diagnosing which forms of ILD?
Specific diagnoses of connective tissues disease - associated ILD
What is necessary to diagnose sarcoidosis?
All of the following:
- Compatible history
- Decreased cutaneous delayed-type hypersensitivity reactions
- Increase in Th1/Th17 responses at sites of disease
- Characteristic physical exam, labs, and imaging
- Biopsy of lung, lymph node, or extrapulmonary site that shows non-caseating, epithelioid, granulomatous inflammation without evidence of infection or other cause of the granuloma
What is sarcoidosis?
A multi-system disorder of unknown cause characterized by non-caseating granulomatous inflammation in affected tissues
- May be triggered by exogenous agents in genetically susceptible individuals
- Involves the lungs in 90% of cases
Which of the following cell types is most susceptible to injury?
- Type I alveolar epithelial cells
- Type II alveolar epithelial cells
- Pulmonary fibroblasts
a. Type I alveolar epithelial cells
These cells make up most of the structural components in the alveolar wall.
What autoimmune diseases are associated with ILD?
Sarcoidosis
Hypersensitivity pneumonitis
Scleroderma
Lupus
Which three ILDs can be diagnosed via bronchoscopy with transbronchial biopsy?
Sarcoidosis
Lymphangitic carcinoma
Pulmonary infections
(The sample is too small to diagnose others)
What cells will be abnormally abundant in the bronchoalveolar lavage of a patient with hypersensitivity pneumonitis?
Lymphocytes
What pattern is shown in this HRCT?
What disease might this indicate?
UIP pattern
- Lower-lobe, peripheral predominant reticular opacities
- Traction bronchiectasis
- Peripheral honeycombing
The UIP pattern is consistent with IPF. However, we cannot diagnose IPF unless we know for sure it is idiopathic
Describe the HRCT findings of acute/subacute Hypersensitivity Pneumonitis
- Ground glass opacities
- Centrilobular
- Usually upper-lobe predominant
- Signs of air-trapping
What is the usual treatment for pneumoconiosis or hypersensitivity penumonitis?
- Remove offending exposure
- Corticosteroids
- Anti-inflammatory agents
What are the symptoms of ILD?
Usually nonspecific
- Dyspnea
- Cough
- Fatigue
- Weight loss
- Fever
- Non-pulmonary symptoms/signs of connective tissue disease
What is the interstitium?
The potential space between alveolar epithelium and capillary endothelium
This x-ray shows a _________ pattern, characteristic of _________.
This X-ray shows a reticular** pattern, characteristic of **interstitial lung diseases.
Specifically, chronic fibrosing IIPs (this one specifically shows IPF)
Which HRCT shows a reticular opacity and which shows a nodular opacity?
Which diseases do these opacities represent?
What HRCT findings are consistent with IPF?
UIP pattern
- Reticular opacities
- Peripheral, lower-lobe predominant
- Traction bronchiectasis
- Enlargement of the airway due to abnormality in the parenchyma surrounding the airway
- Honeycombing
Describe the presentation of subacute/chronic Hypersenitivity Pneumonitis (HP)
- Dyspnea, cough, fatigue, weight loss
- Caused by prolonged or repeated antigen exposure
- Usually without history of acute HP
- Can progress to pulmonary fibrosis with respiratory failure