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
88% of IPF pts have this
Obstructive sleep apnea (OSA)
How does IPF present?
DOE, non-productive cough, weight loss, low grade fevers, fatigue, arthralgias
IPF PE
Inspiratory crackles
PH
Digital clubbing
Symptoms of PH
RV heave
Increasd JVP
Pedal edema
Tricuspid regurgitation
IPF CXR findings
Reticular opacities, predominantly at lung bases (honeycombing)
HIgh-res CT shows patchy reticular opacities
IPF treatment
Treat comorbidities Smoking cessation Oxygen therapy Vaccination (flu and pneumococcal) Lung transplant?
Specific form of chronic, progressive interstitial scarring of known cause
Interstitial Lung Disease
Asbestosis, silicosis, pneumoconiosis cause what type of lung dz?
ILD
Medication, drug, radiation caused scarring is what type of disease?
ILD
Hypersensitivity pneumonitis is a cause for what type of disease?
ILD
Collagen vascular diseases like rheumatoid arthritis, lupus, and scleroderma cause what type of scarring lung disease?
ILD
Result of ILD where increased interstitial tissue replaces normal capillaries and alveoli?
Decreased perfusion
What are the four leading coal producing states?
Wyoming
West Virginia
Kentucky
Pennsylvania
Coal mining can give you what disease?
pneumoconiosis
What medications can cause ILD?
Nitrofurantoin ASA Methotrexate Amiodarone Propanolol
What drugs can cause ILD?
Heroin
Methadone
Cocaine
Propoxyphene
What is cause of hypersensitivity pneumonitis that can cause ILD?
Bird-breeder’s lungs (bird dropping)
Farmer’s lungs (moldy hay)
Humidifier lungs (hot tubs, air conditioners)
Chemical worker’s lungs (resins, plastics, paints)
Connective tissue diseases that cause ILD
scleroderma, rheumatoid arthritis, SLE, Sjogren’s syndrome, polymyositis
S/Sx of ILD
DOE non-productive cough Decreased DLCO Diffuse inspiratory rales/wheezing Fever, chills, malaise
ILD CXR findings
Interstitial opacities (GROUND GLASS)
ILD Treatment
Corticosteroids Immunosuppressive (cyclosporine) Oxygen therapy Pulmonary rehab Transplant? Vaccines: pneumococcal, influenza
Characterized by bilateral pulmonary infiltrates and severe hypoxemia with diffuse alveolar injury leading to respiratory failure
ARDS
What are the two types of ARDS?
Direct (toxic inhalation)
Indirect (sepsis)
What is the mortality rate of ARDS if 3 organ systems fail?
99%
Pathophys of ARDS
Increased permeability of alveolar-capillary barrier, Main sites of injury are vascular endothelium (sepsis) or alveolar epithelium (aspiration). promotes pulmonary edema
What % of ARDS pts have no identifiable risk factor?
20
Aspiration of gastric contents, sepsis, pancreatitis, multiple long bone fx, burns, transfusions, CABG sx, and drug OD are risk factors for what?
ARDS
ARDS presentation
Critically ill, multi-system organ failure
Illness develops 12-28 hrs after event
PEEP and CPAP acronyms
PEEP: Positive end expiratory pressure
CPAP: Continuous positive airway pressure
ABG in ARDS
initially resp. alkalosis with progression to acidosis
CXR in ARDS
bilateral pulmonary infiltrates (evolve rapidly, most severe in first 3 days)
ARDS treatment
No drugs proven beneficial.
Ventilator support
PEEP, VTE, stress ulcer prophylaxis
Hyaline membrane disease
IRDS
Impaired surfactant synthesis, leading to atelectasis, hypoventilation and hypoxemia
IRDS
True or False: IRDS is related to prematurity (26-32 weeks) of infants
True
IRDS risk factors
prematurity maternal diabetes C section delivery Perinatal asphyxia FH of IRDS
S/Sx of IRDS
Cyanosis, apnea, rapid breathing, grunting, nasal flaring, decreased urine output
Diagnosis of IRDS
ABGs, CXR, lab tests (r/o infx)
IRDS treatment
CPAP
Surfactant
Warm, moist oxygen
Maintain ideal body temp, calm setting, carefully manage nutrition and fluids, treat infx right away
Rare autoimmune disorder where antibodies form against acetylcholine receptors at neuromuscular junction of skeletal muscles
Myasthenia gravis
How does myasthenia gravis cause restrictive lung dz?
Respiratory muscle weakness (extrinsic)
Autoimmune d/o when immune system attacks myelin sheath that surrounds peripheral nerves causing weakness
Guillain Barre syndrome–
Respiratory muscle weakness causes extrinsic restrictive lung dz
Pickwickian syndrome
Obesity hypoventilation syndrome
Obesity effects on breathing
Daytime hypercapnia
Extrinsic restrictive lung dz
High diaphragm