test 7.2 Flashcards
Definition of restrictive lung disease
Characterized by reduction in lung volume. (TLC, VC, or resting lung volume)
What are the two groups of restrictive lung disease?
Lung parenchyma (intrinsic): air spaces fill with exudates and debris Extraparenchyma (extrinsic): dz of chest wall, pleura, and respiratory muscles
Types of intrinsic restrictive lung disease
Sarcoidosis
Idiopathic pulmonary fibrosis
Interstitial lung disease (asbestosis, pneumoconiosis, meds, drugs, radiation, hypersensitivity pneumonitis, rheumatoid arthritis, lupus, scleroderma)
ARDS, IRDS
Types of extrinsic restrictive lung disease
Myasthenia gravis
Guillain Barre
Kyphosis and chest wall deformities
Dz that restricts lower throacic or abdomen volume (obesity, hernia, ascites)
True or false: Restrictive lung dz preserves airflow and resting lung volume
True
How do you measure airflow and resting lung volume?
functional residual capacity (FRC)
Which step is passive, lung inflation or lung deflation?
Deflation
Volume of air in the lungs with respiratory muscles fully relaxed so no airflow is present
FRC
Multisystem inflammatory disease of unknown etiology (esp.lungs, lymph nodes, skin)
sarcoidosis
How does sarcoidosis present?
Non-necrotizing granulomas in lungs and intrathroacic lymph nodes. Exaggerated immune response from T cells
What race has greater prevalence and severity of sarcoidosis?
African Americans
Typically age of onset of sarcoidosis
24-45
fever, weight loss, arthralgia, DOE, crackles, skin lesions, uveitis, lupus pernio
Sarcoidosis
How is sarcoidosis staged?
CXR
Bilateral hilar lymphadenopathy stage
1
BHL & infiltrates stage
2
Infiltrates stage
3
Fibrosis stage
4
What do non-caseating granulomas secrete?
Vit D like hormone (causes hypercalcemia and hypercalciuria), ACE (ACE levels are high)
What will pulmonary function tests show?
Decreased VC and DLCO, pulm. htn, O2 drops w/ 6. min of walking
Measures the ability of lungs to transfer gas from inhaled air to the RBCs in pulmonary capillaries
DLCO
Mainstay of therapy for sarcoidosis
Corticosteroids
Sarcoidosis and can’t tolerate steroids
Methotrexate
Given for cutaneous lesions, hypercalcemia, neruological, bone lesions of sarcoidosis
Chloroquine
Qualifications for lung transplant with sarcoidosis
Stage 4 with VC <40%
Follow up of sarcoidosis
CXR and PFT every 6-12 months
Annual slit lamp eye exam
Specific form of chronic, progressive interstitial scarring of unknown cause
Idiopathic pulmonary fibrosis
Prognosis of IPF
Poor, 2-5 year survival from time of dx
Epithelial-fibroblastic dz that causes diffuse epithelial cell disorganization
IPF pathophys