Interstitial Lung Disease - Clinical Flashcards
What are the signs and symptoms of interstitial lung diseases?
Dyspnea, especially with exertion
Dry cough
Hypoxemia
Clubbing of digits -> from growth factors released in fibrosis
Extra signs / symptoms related to specific diseases
What is the best way to check for interstitial lung disease in a patient if you suspect it and why?
Look at old imaging
- > usually a slow progression over the years
- > patients don’t remember when symptoms started
What should you generally think about if the disease is more acutely onset than interstitial lung diseases?
Rule out other diseases like infection or pulmonary edema
What interstitial lung diseases follow an acute pattern?
Acute interstitial pneumonitis (AIP)
Interstitial lung diseases manifesting with alveolar hemorrhaging
- >
- Churg Strauss (allergic granulomatous angiitis)
- >
- Microscopic polyangiitis (leukocytoclastic vasculitis)
- >
- granulomatous pulmonary angiitis (GPA)
1&2 here we’ve studied before and are associated with p-ANCA
What are the clinical manifestations of AIP and what is the prognosis?
Acute pulmonary fibrosis which looks like ARDS
Very poor prognosis, does not respond to therapy and requires lung transplant
What are the major risk factors for IPF (idiopathic pulmonary fibrosis, also called UIP)?
Tobacco smoking and acid reflux (GERD refluxes into airways)
Can also be familial
What are the signs of IPF on physical exam?
Bilateral fine inspiratory crackles at lung bases - Velcro sign
How is probable diagnosis of IPF made?
Typical findings of IPF on high resolution CT, exclusion of other diseases which can cause pulmonary fibrosis
Clinical symptoms match - i.e. progressive, slowly worsening dyspnea and cough
How is definitive diagnosis of IPF made and is this recommended?
Open lung biopsy to exclusive other diseases which can cause pulmonary fibrosis
NOT recommended for routine biopsy if imaging is typical
-> quite invasive surgery
What are the imaging findings of IPF?
Honeycombing - especially under the pleura and in lower lobes
Fine reticular / reticulonodular opacities - i.e. cystic spaces and ground glass appearance, due to thickening of the interstitium and dilation of airspaces
Traction bronchiectasis
What is traction bronchiectasis / why does it occur?
Airways being held open by fibrotic contracture of lung tissue
-> remember UIP / IPF is characterized by fibrosis of pleura and bibasilar lung spaces, which can drag some airways open in a semi-obstructive pattern
What is the gold standard measurement in PFTs for restrictive lung disease, and what does well of suggesting pulmonary fibrosis?
Gold standard - decreased total lung capacity (TLC)
Pulmonary fibrosis - more likely with decreased diffusion capacity (DLCO)
What is the treatment for IPF and what is definitiely not effective? Prognosis?
Treatment - anti-fibrosis agents such as Nintedanib and Pirfenidone
Prognosis - poor, median survival 3 years, may progress to AIP
What does CT scan show for NSIP and what treatment is best?
Ground glass opacities - hazy appearance to lungs
Better chance to respond to steroids -> more inflammatory process, less fibrosis
Who does sarcoidosis typically affect and what are the hallmark pathologic features?
African American females
Non-caseating granulomas
Calcified hilar lymph nodes
Can also affect laryngx, trachea, and bronchi
What other pulmonary disease can some of the symptoms of sarcoidosis mimic?
Common asthma - can cause bronchial hyperreactivity
What are the possible ocular, and skin manifestations of sarcoidosis?
Ocular - anterior and posterior uveitis
Skin - lupus pernio (skin lesions looking like lupus butterfly rash), erythema nodosum (usually on shins, like due to endemic mycoses)
What are the possible nervous system and heart manifestations of sarcoidosis?
Nervous system: **Bell’s palsy, neuropathy, hypothalamic and pituitary lesions which can lead to SIADH or diabetes insipidus
Heart involvement: Sudden death due to complete heart block
What are the common imaging findings of sarcoidosis? How are PFTs affected?
Hilar lymph nodes, and peribronchial distribution favoring the upper lobes
-> PFTs may be normal or have restrictive pattern
How is diagnosis of sarcoidosis made?
Clinical suspicion + definitive biopsy of non-caseating granulomas (which is actually important in this case)
What is the main treatment for sarcoidosis and when do we treat?
Steroids, or methotrexate if not responsive (immunomodulator)
Treat in two scenarios:
- Active / progressive lung disease
- Extrapulmonary symptoms
What is Cryptogenic Organizing Pneumonia and how is diagnosis typically suspected?
Idiopathic disease affecting middle age NONsmokers with subacute pattern over 6-8 weeks
Diagnosis made after failure to improve with antibiotics
What will PFTs and imaging show with cryptogenic organizing pneumonia (COP)?
PFTs - usually restrictive pattern
Imaging - bilateral and peripheral (bronchiolar) asymmetric patchy infiltrates, with or without air bronchograms
->alveoli often show ground glass appearance and reversed halo sign (ground glass with opacity on outside)
What is the treatment and prognosis for COP?
Prolonged tapering dose of steroids
-> prognosis is very good
What is the CT findings of respiratory bronchiolitis interstitial lung disease and treatment?
Centrilobular / ground glass opacities with bronchial wall thickening
Very related to DIP (desquamative interstitial pneumonia) -> very good prognosis with smoking cessation
What are the features of pulmonary histiocytosis and the treatment?
Formation of nodular opacities and thick-wall pulmonary cysts which may cause pneumothorax
Treatment is to quit smoking
