Exam 2: Interstital Lung Disease Flashcards
What is the pathophysiology of ILD?
A process of fibrosis and aberrant healing response, sometimes inflammatory but not infectious
A patient presents with a progressive DOE and a persistent non-productive cough. There is no wheezing or chest pain. Patient has crackles at the lung bases and digital clubbing. What are you suspicious of?
ILD
Erythema nodosum is common in what disease?
Sarcoidosis
Gottrons papules are common in what disease?
Dermatomyositis
What is the gold standard of diagnosis of ILD?
Tissue biopsy
What CXR finding indicates poor prognosis?
Honey combing
What are the common CXR findings of ILD?
- Ground glass appearance
- reticular (most common), nodular, or mixed pattern of opacities
If the distribution of disease is in the upper lung on CT, what does that indicate?
Inhalation related diseases
If the distribution of disease is in the lower portion of the lungs on CT, what does that indicate?
IPF, connective tissue, disease, asbestosis
What may be the only finding in early ILD?
Reduced Diffusing capacity of lungs for carbon monoxide
What might ABGs show in ILD?
Hypoxemia and respiratory alkalosis
What is the one contraindication to lung biopsy?
Honey combing on imaging because prognosis is already poor and biopsy wont change management
What is a trans bronchial biopsy?
Done during bronchoscopy, biopsy forceps passed through bronchoscope. Helpful to biopsy central locations, not periphery
What are the two options for surgical biopsy?
Video assisted thoracoscopic surgery or thoracotomy
What is the most common interstitial lung disease?
Idiopathic pulmonary fibrosis
What is the histopathology pattern seen with idiopathic pulmonary fibrosis?
Usual interstitial pneumonia (UIP)
What are the two pharmacologic treatment options for idiopathic pulmonary fibrosis?
Nintedanib: suppresses multiple signaling receptors involved in pathogenesis of fibrosis
Pirfenidone: reduces lung fibrosis though down regulation of production of growth factors and procollagens
What is sarcoidosis?
A multisystem inflammatory disease that forms non-caseating granulomas
What do the non- caseating granulomas in sarcoidosis secrete?
- 1,25 dihydroxyvitamin D (elevated calcium)
- ACE
A young female presents with DOE, chest pain, and systemic complaints. She has erythema nodosum, lupus pernio, and granulomatous uveitis. What are you suspicious of?
Sarcoidosis
What labs will be elevated in sarcoidosis?
- Elevated serum ACE
- hypercalcemia
- elevated alkaline phosphatase
What is often seen on CXR in sarcoidosis?
Hilar adenopathy
What is the staging based on CXR of sarcoidosis?
Stage 0: Normal
Stage 1: Hilar adenopathy
Stage 2: Hilar adenopathy and diffuse infiltrates
Stage 3: Only diffuse parenchymal infiltrates
Stage 4: Pulmonary fibrosis
What is silicosis?
A fibronodular lung disease characterized by inhalation of silica dust
What kind of pattern do PFTs show for silicosis and coal workers pneumonconiosis?
Restrictive
What is seen on CXR in patients with chronic simple silicosis and coal workers pneumonconiosis?
- Hilar node calcification (eggshell pattern)
- Small round nodular opacities
What is seen on CXR is asbestosis?
Opacities in the lower lungs, thickened pleura, and pleural plaques
What kind of pattern do PFTs show in asbestosis?
Restrictive pattern
What is mesothelioma almost always associated with?
Asbestosis
What is hypersensitivity pneumonitis?
An inflammatory syndrome of the lung caused by repetitive inhalation of antigens in a susceptible host
Is hypersentivity pneumonitis chronic?
No, it is a reversible process that is temporally related to exposure
What disease is characterized by necrotizing granulomas?
Granulomatosis with polyangiitis (GPA)
What disease is an immune mediated systemic vasculitis of small-medium vessels
GPA
What disease is saddle most deformity associated with?
GPA
What is the initial treatment for GPA?
Cyclophosphamide and corticosteroids
