Interstitial Lung Disease Flashcards
(29 cards)
What is Pulmonary Fibrosis?
thickening of the interstitial septum of the lung between the arteriolar space and alveolus interfering with gas exchange in both directions
What are commonly associated caused of Pulmonary Fibrosis?
Idiopathic interstitial pulmonary fibrosis
Radiation
Bleomycin, Busulfan
Amiodarone, Methysergide
Nitrofurantoin, Methotrexate
How does pulmonary fibrosis present?
Dyspnea worse on exertion
Fine rales or crackles
Loud P2 heart sound
Clubbing of the fingers
What is Coal worker’s Pneumoconiosis associated with?
Coal
What is Silicosis associated with?
Sandblasting
Rock mining
Tunneling
What is Asbestosis associated with?
Shipyard workers
pipe fitting
insulators
What is Byssinosis associated with?
Cotton
What is Berylliosis associated with?
Electronic manufacturing
What is Bagassosis associated with?
Moldy sugar cane
What is the best initial test for Pulmonary Fibrosis?
CXR
What is the most accurate test for Pulmonary Fibrosis?
Lung Biopsy showing granulomas in Berylliosis
What is seen on PFTs in Pulmonary Fibrosis?
Restrictive pattern
DLCO decreased in proportion to severity of thickening
What are the treatment options for Interstitial lung diseases?
Prednisone for WBC or inflammatory infiltrate
Azathioprine long-term if response to steroids
Pirfenidoen: antifibrotic agent inhibits collagen synthesis
Nintedanib: TK-inhibitor blocks fibrogenic growth factors and inhibits fibroblasts
What is the Mechanism of Pirfenidone?
Antifibrotic: inhibits collagen synthesis
What is the Mechanism oF Nintedanib?
Tyrosine-Kinase Inhibitor: blocks fibrogenic growth factors and inhibits fibroblasts
What is Sarcoidosis?
Idiopathic inflammatory disorder predominately of the lungs most commonly in African American Women
How does Sarcoidosis commonly present?
Young African American Women with SOB on exertion and occasional fine rales without wheezing
What is seen on CXR for sarcoidosis?
Hilar Lymphadenopathy
What are other common symptoms of sarcoidosis?
Erythema Nodosum
Parotid gland enlargement
Facial Palsy
Heart block and restrictive cardiomyopathy
CNS involvement
Uveitis and Iritis
What is seen on CBC in Sarcoidosis?
Polycythemia from chronic hypoxia
What is the most accurate test for Sarcoidosis?
Right heart or Swans-Ganz Catheter
What are the treatment options for Sarcoidosis?
Prostacyclin Analogues (PA vasodilators)
Endothelin Antagonist
PDE-I
cGMP stimulators
CCB
Oxygen to slow progression
Lung transplant
What Prostcyclin analogues are commonly used in sarcoidosis?
Epoprostenol
Treprostinil
Iloprost
Beraprost
Selexipag
What Endothelin Anatagonists are commonly used for Sarcoidosis?
Bosentan
Ambrisentan
Macitentan