Pulmonary Fibrosis Flashcards
Idiopathic Fibrosing Interstitial Pneumonia, formerly known as Idiopathic Pulmonary Fibrosis
Interstitial lung diseases (ILDs)
Idiopathic pulmonary fibrosis
Pulmonary Fibrosis
Sarcoidosis
ILDs causes
Most common causes r/t environmental/occupational exposures, esp to inorganic or organic dusts
ILDs often assoc c connective tissue dz: SLE, RA, scleredema
Presentation
Persistent dyspnea on exertion or persistent nonproductive cough
Abnormal CXR
Pulm s&s assoc c another dz, like connective tissue dz
Restrictive ventilatory pattern on office spirometry
Dx tests
routine blood tests, serologic studies chest radiograph pulmonary function testing arterial blood gas analysis computed tomography
History taking
Age Gender Smoking hx Duration of illness Prior medication use Family hx Occupational hx Environmental exposure
Symptoms
Dyspnea Cough Hemoptysis Wheezing Chest pain
Carefully record clinical findings consistent with connective tissue diseases
musculoskeletal pain weakness fatigue fever joint pains or swelling photosensitivity Raynaud phenomenon pleuritis dry eyes, and dry mouth.
PE
findings are nonspecific
cracles - less likely to be in sarcoidosis, can be present in absence of CXR abnormalities
Insp squeeks - common in bronchiolitis
Cor pulmonale - late stages of pulm fibrosis, with pulm HTN
cyanosis - uncommon in ILD, usually late manifestation
Clubbing
Labs
Usually not helpful but may include: LFTs Renal fxn CBC UA CK for myocytosis
CXR
ILD often initially suspected d/t abnormal CXR
CXR is normal in about 10% of ILD
Most common CXR abnormality is reticular patten
PFT
Measurement of fxn helpful in determining extent of ILD
Finding obstructive vs restrictive findings useful in narrowing DD
Lung Bx
Not required to make dx in all pts c ILD but often not possible to make clear dx and stage dz without assessing lung tissue
Role of bx:
To assess disease activity.
To exclude neoplastic and infectious processes that occasionally mimic chronic, progressive interstitial disease.
To identify a more treatable process than originally suspected.
To establish a definitive diagnosis and predict prognosis before proceeding with therapies which may have serious side effects.
Tx controversial
No randomized study has shown that any tx improves survival or QOL vs no tx
