interstitial lung dz Flashcards
interstitial lung disease is also known as
pulmonary fibrosis
in interstitial lung disease, the lung is affected in what 3 ways
- lung parenchyma is damaged
- walls of alveoli become inflamed
- scarring (fibrosis) begins and lungs become stiff
interstitial lung disease has what primary clinical presentation
- progressive dyspnea on exertion and nonproductive cough
- wheezing and CP are uncommon
PE is consistent with
- crackles at lung bases
- inspriatory squeaks
- cor pulmonale
- cyanosis and digital clubbing (advanced dz)
interstitial lung disease
what abnormal CXR findings are consistent with interstitial lung disease
- ground-glass appearance: often early finding
- bilateral opacities, reticular “net-like” (most common)
- honeycombing indicated poor prognosis
pulmonary function tests reveal that most interstitial lung disease are . TLC will be? FEV1/FVC ratio?
- restrictive
- TLC decreased
- FEV1 and FVC decreased but ratio is normal
contrary to other interstitial lung diseases, sarcoidosis has what pattern on PFT
obstructive
expected diffusing capacity of lung for CO (DLCO) in interstitial lung disease
- reduced
- ability of gas to cross from air -> interstitium -> blood is diminished due to inflammation of alveolar wall
expected ABG in interstitial lung disease
- may be normal
- may show hypoxemia or respiratory alkalosis
- increased RR -> decreased PaCO2
what is bronchoalveolar lavage
- extension of bronchoscopy
- allows for cellular analysis
- may help narrow DDx, define stage of disease, assess progress or response to therapy
what is the gold standard to diagnose interstitial lung disease
lung biopsy
provide indications when lung biopsy is indicated
- atypical or progressive symptoms
- age >50 yo
- fever, weight loss, hemoptysis
- ILD symptoms with normal or atypical CXR
list complications of interstitial lung disease
- pulmonary HTN -> Cor pulmonale (rt ventricular hypertrophy) -> Rt heart failure
- pneumothorax (alveoli become so stiff, they rupture)
- elevated CA risk
- progressive respiratory insufficiency
what is often the initial procedule of choice for lung biopsy
transbronchial lung biopsy
- less invasive, less tissue for analysis
known occupation and environmental causes of interstitial lung disease
- inorganic dust (asbestos, silica, hard metals)
- organic dust (bacteria, animal proteins)
- gases, fumes
known drug cause of interstitial lung disease
- chemo
- abx: macrobid
- radiation treatment
interstitial lung disease is associated with what diseases
- sarcoidosis
- connective tissue or autoimmune
- scleroderma
- SLE
- RA
- polymyositis
- systemic vasculitis (granulomatosis with polyangitis)
what is Pneumoconiosis
- any disease of the respiratory tract due to inhalation of dust particles
- asbestosis, silicosis
when does asbestosis present
- due to chronic inhalation of asbestos fibers
- usually presents 10-15 yrs of exposure
- age 40-75 yo
- m>f
asbestos is linked to causing what conditions
- bronchogenic (lung) CA
- smoking increases risk
- malignant mesothelioma
What is mesothelioma
- form of CA almost always associated with asebestos exposure
- CA in mesothelium (protective lining that covers most organs)
- most common in pleura
What CXR findings are consistent with asbestosis
- opacities in lower lungs, thickened pleura, pleural plaques
how is asbestosis diagnosed?
- consistent hx, PE, symptoms and CXR
- open lung bx will provide definitive diagnosis but usually not indicated
asbestosis will have what findings on PFT
- restrictive
- decreased TLC
- FEV1/FVC normal
asbestosis management
- consult pulmonology
- smoking cessation
- no effective therapy
- vaccinate: influenza, pneumovax
- male
- 50ish
- pipefitter
- smoker
- CXR: pleural plaques
asbestosis
prognosis of asbestosis
- lung damage is irreversible
- smoking + asbestosis = 59 x more likely to get lung CA
which type of pneumoconiosis is characterized by fibronodular lung disease
silicosis
what causes silicosis
inhalation of silica dust
- occupations: mining, construction, granite cutting, pottery making
presentation
- 10-12 years exposure
- may be asymptomatic
- non-progressive once exposure elimiated
- hilar node calcification (eggshell pattern)
- small round opacities on CXR
chronic simple silicosis
presentation
- >20 years exposure
- progressive even after exposure eliminated
- tachypnea, prolonged expiration, rhonchi, wheezing
- digital clubbing (uncommon)
- cyanosis (advanced)
- cor pulmonale (advanced)
- CXR: enlarging opacities that can cavitate
- PFT: restrictive
chronic complicated silicosis
management of chronic complicated silicosis
- consult pulmonologist
- smoking cessation
- no specific therapy
- vaccinate: influenza, pneumovax
which condition is associated with noncaseating granulomas, predominantly in the lungs, but can affect heart, liver, spleen, joints, bone
sarcoidosis
sarcoidosis normally affects what patient population
- african american
- 20-40 yo
- slight female predominance
clinical presentation
- fever, anorexia, arthralgias (45% of people)
- dyspnea on exertion, cough, CP
- arthritis, cranial nerve palsies, visual disturbances, erythema nodusum
- PFT: restrictive
sarcoidosis
what is the most common pattern of lymphadenopathy in sarcoidosis
- bilateral symmetric hilar and right paratracheal mediastinal adenopathy
increased serum ACE is associated with what ILD condition
sarcoidosis
managment of sarcoidosis
- consult pulmonologist
- 75% require only symptomatic -> NSAIDs
- corticosteroids: mainstay of therapy
what is granulomatosis with polyangitis
- immune-mediated systemic vasculitis
which ILD condition is associated with necrotizing granulomas of the upper and lower respiratory tract and glomerulonephritis
granulomatosis with polyangitis
granulomatosis with polyangitis usually affects what patient population
- white
- male
- presents 40-50s
clinical presentation
- upper airway
- rhinorrhea, purulent/bloody nasal dx
- oral/nasal ulcers
- lower airway
- dyspnea, cough, pleuritic pain, hemoptysis
- PFT: restrictive or obstructive; decreased DLCO
- CT: blood vessels leading to nodules and cavities
granulomatosis with polyangitis
which condition is associated with a + ANCA: antineutophil cytoplasmic antibody, usually C-ANCA
granulomatosis with polyangitis
managment of granulomatosis with polyangitis
- consult rheumatologist
- tx: cyclophosphamide (immunosuppressant) and glucocorticoid
what is the most common idiopathic interstitial pneumonias
idiopathic pulmonary fibrosis
how is idiopathic pulmonary fibrosis diagnosed
- diagnosis made after excluding other causes of ILD
- lung biopsy
what is interstitial pneumonitis
another name for idiopathic pulmonary fibrosis
what patient population is associated with idiopathic pulmonary fibrosis
- male
- 60s
- a common man
- smoker
managment of idiopathic pulmonary fibrosis
- consult pulmonologist
- eval for lung transplant
