Interstitial lung disease Flashcards
What is the definition of Interstitial Lung Diseases (ILD)?
A heterogeneous group that includes over 200 separate conditions affecting the lung parenchyma, causing inflammation and potential fibrosis
Also referred to as diffuse parenchymal lung diseases.
What are common symptoms of ILD?
Nonspecific symptoms include:
* Dyspnea on exertion
* Chronic dry cough
* Fatigue
Chest pain is not commonly associated with most ILDs.
What medications are commonly associated with causing ILD?
Common medications include:
* Amiodarone
* Nitrofurantoin
* Anti-neoplastic agents (bleomycin)
* NSAIDs
* Immune-modulating drugs (e.g., azathioprine, methotrexate)
Previous radiation exposure can also contribute.
What are common physical exam findings in patients with ILD?
Common findings include:
* Fine inspiratory basilar ‘Velcro’ crackles
* Clear lung fields in nonfibrotic disease
* Digital clubbing
IPF typically presents with lower lobe findings.
What laboratory studies should be obtained in the workup of ILD?
Lab studies may include:
* Complete blood counts
* Comprehensive metabolic panel
* Autoimmune labs (e.g., ESR, CRP)
It’s unclear if a broad CTD/autoimmune workup is cost-effective in ILD.
What are the characteristic HRCT findings in ILD?
Characteristic findings include:
* Basilar predominant peripheral reticular pattern
* Nodular infiltrates
* Ground glass and alveolar opacities
* Honeycombing
Honeycombing indicates advanced fibrosis and poor prognosis.
What is the typical pulmonary function test (PFT) pattern in ILD?
Most ILDs show a restrictive lung pattern characterized by decreased FVC and DLCO
A 6-minute walking test may also be used to assess exertional hypoxemia.
What are the major biopsy patterns in ILD?
Major patterns include:
* Usual interstitial pneumonia (UIP)
* Nonspecific interstitial pneumonia (NSIP)
* Organizing pneumonia
* Granulomatous pattern
Each pattern can guide the diagnosis of specific ILDs.
What is the treatment approach for ILD?
Treatment may include:
* Removal from exposures
* Immunosuppressive therapies
* Antifibrotic drugs
* Supportive therapy
* Lung transplantation
Correct diagnosis is critical for determining appropriate treatment.
What is idiopathic pulmonary fibrosis (IPF)?
The most common ILD of unknown cause, typically presenting in the 5th-6th decade of life, often in men with a history of smoking
IPF has a poor prognosis with about 50% survival at 3-5 years.
What are common HRCT findings in IPF?
Typical HRCT findings include:
* UIP pattern
* Peripheral reticulation
* Honeycombing
Histopathology also shows the UIP pattern.
What characterizes sarcoidosis?
A multisystem disorder often presenting with lung involvement, especially in young black women
Common findings include bilateral hilar lymphadenopathy and noncaseating granulomas on histopathology.
What is the cause of hypersensitivity pneumonitis?
Occurs due to mixed type III/IV hypersensitivity reactions to various antigen exposures
Common antigens include thermophilic actinomycetes and Aspergillus.
True or False: Steroids are the first-line treatment for idiopathic pulmonary fibrosis (IPF).
False
Steroids may worsen mortality in IPF.
What is bird fancier’s lung?
A hypersensitivity pneumonitis with highly variable presentation depending on antigens and exposure time course.
What are the CT findings in subacute bird fancier’s lung?
Ground glass opacities with centrilobular nodules.
What are the CT findings in chronic bird fancier’s lung?
Subpleural reticulations with traction bronchiectasis and subpleural honeycombing.
What does histopathology show in bird fancier’s lung?
Poorly defined non-caseating granulomas near small airways.
What is the primary treatment for bird fancier’s lung?
Antigen avoidance.
What happens if chronic antigen exposure occurs in bird fancier’s lung?
Can lead to irreversible fibrosis.
What role do steroids play in bird fancier’s lung?
May be useful for symptom control if avoidance is not possible.
What type of opacities are typically present on CT for occupational lung diseases?
Rounded or linear opacities.
What is asbestosis?
A lung disease resulting from chronic exposure to asbestos.
What are some occupations linked to asbestosis?
Shipbuilding, plumbing, roofing.
What are the complications associated with asbestosis?
- Pulmonary fibrosis
- Pleural disease
- Bronchogenic carcinoma
- Mesothelioma
What is a specific CT finding for asbestosis?
Ivory white calcified supradiaphragmatic and pleural plaques.
Which lung lobes are primarily affected by asbestosis?
Lower lung lobes.
In which industries is berylliosis commonly found?
Aerospace and manufacturing.
What is the histopathology pattern of berylliosis?
Granulomatous (noncaseating) pattern.
Which lung lobes are primarily affected by berylliosis?
Upper lung lobes.
What is coal worker’s pneumoconiosis also known as?
Black lung disease.
What does imaging typically show in coal worker’s pneumoconiosis?
Small, rounded, nodular opacities in the upper lobes.
What is silicosis?
A lung disease resulting from silica exposure.
In which industries is silicosis commonly found?
Sandblasting, mining, stonecutting, foundries.
What are the CT findings in silicosis?
Crazy paving pattern with diffuse ground-glass densities and thickened septa.
What is a characteristic CXR finding in silicosis?
Eggshell calcifications of hilar lymph nodes.
Why are Pt’s w/ silicosis at a higher risk of TB infection?
Silica causes dysfunction of alveolar macrophages
What treatment may provide relief in silicosis?
Whole lung lavage.
What are some specific connective tissue diseases that may cause ILD?
- Systemic sclerosis
- Rheumatoid arthritis
- Dermatomyositis/polymyositis
- Systemic lupus erythematosus
- Sjogren syndrome.
What medication has been shown to slow disease progression in patients with idiopathic pulmonary fibrosis (IPF)?
Nintedanib
Ms. Raines is a 28 year old black female who is seeing you with a complaint of dry cough and dyspnea. Her chest x-ray shows bilateral hilar lymphadenopathy. You review her recent metabolic panel which is remarkable for a moderate elevation of calcium. What is the most likely diagnosis?
Sarcoidosis
Mrs. Walker is a 51 year old female with a previous history of intersitial lung disease who is seeing you to review the results of her recent high resolution CT scan. Which of the following findings on CT is consistent with the poorest prognosis?
Honeycombing
What is the histopathology pattern on lung biopsy most consistent w/ Dx of sarcoidosis?
Granulomatous pattern
D. Areas of fibrosis and honeycombing that are interspersed w/ areas of normal lung
IPF - fibrotic lesions are posterior and basal (peripheral lesions that need surgical biopsy as opposed to bronchoscopy)
B. Peripheral edema
Cor pulmonale secondary to hypoxemia and pulmonary HTN
E. Nitrofurantoin
B. Connective tissue disease
A. Obstructive pattern (decreased FEV1/FCV ratio)
B. Restrictiv pattern (decreased TLC)
D. Decreased DCLO
