Restrictive Lung Disease Flashcards
how does restrictive lung disease impact lung volumes?
decrease in RV compared to normal
overall, total lung volume also decreases
what is the overall impact of restrictive lung diseases on the lungs?
- reduced total lung volume
- decrease compliance
- decrease inspiratory reserve capacity
- hypoxemia
- associated with conditions that reduce cell wall movement
how are restrictive lung diseases treated?
- symptom management
- corticosteroids
- ensure adequate oxygenation, maintain an airway, and obtain max physical function
- sometime surgical modification
- heart-lung transplants
Restrictive lung diseases can be put into what 3 categories?
- Chronic Interstital diseases
- Chest wall disorders
- Pulmonary involvement in autoimmune diseases
List restrictive lung diseases that are chronic interstitial diseases
- Pneumoconiosis
- Honeycomb lung
- Pulmonary fibrosis
what is Pneumoconiosis?
a disease caused by breathing in certain kinds of dust particles that damage the lungs
often called occupational lung disease
results in interstitial fibrosis
describe the pathology of Pneumoconiosis
usually takes years to develop
inflammation from particulate matter leads to pulmonary scarring
blood vessels and alveolar damage-become thicker and stiffer
What are the s/s of Pneumoconiosis?
- cough
- phlegm
- SOB contributing to reduced physical functional capacity
- progressive respiratory failure
- lung cancer
- TB
- heart failure (cor pulmonale)
Give some examples of conditions that are a type of Pneumoconiosis
- Black lung disease (coal dust)
- Brown lung disease (agricultural dust)
- Asbestoses
- Silicosis (silica)
how is Pneumoconiosis treated?
nonreversible
treat complications
some meds
chest physiology → mobilize and remove secretions
exercise
what is honeycomb lung?
widespread lung fibrous
dialted and thickened terminal and respiratory bronchioles
what is pulmonary fibrosis?
refers to a variety of disorders in which ongoing epithelial damage or chronic inflammation of lung tissue leads to progressive scarring of the lung resulting in respiratory failure
what are some causes of pulmonary fibrosis?
- Idiopathic (66% of the time)
- scar tissue resulting from recovery from active diseases like:
- TB
- ARDs
- RA, SLE (CT diseases)
- XRT and chemotherapy
describe the clinical course for idiopathic pulmonary fibrosis
- begins insidiously w/gradual increasing exertional dyspnea and dry cough
- unpredictable progression
- hypoxemia, cyanosis and clubbing
- decreased diffusing capacity of the alveolocapillary membrane leading to hypoxemia
- median survival ~3 years after dx
what is the only definitive therapy for idiopathic pulmonary fibrosis?
lung transplantation
list categories/types of chest wall disorders that can be considered restrictive lung diseases
- Neuromuscular
- ALS, polio, MD, Guillian-Barre
- Skeletal deformities
- kyphosis, scoliosis, chest wall injury or deformity
- Postsurgical status
- abdominal and thoracic surgeries
- Obesity (impact diaphragm descending)
- Collagen vascular diseases
- scleroderma, SLE, RA
List some autoimmune diseases that impact pulmonary function and result in restrictive lung diseases
- Systemic sclerosis (scleroderma)
- RA
- SLE
what is scleroderma?
an autoimmune disease of CT characterized by excessive collagen deposition in the skin and internal organs, particularly the kidneys and lungs
how does scleroderma impact/damage organs?
inflammation
severe thickening and obstruction of vessels
cutaneous fibrosis occurs
how does RA impact the lungs?
30-40% of RA pts have pulmonary involvement
can increase infection risk and it impacts CT leading to fibrosis → leads to various lung related pathologies like chronic pleuritis, pneumonitis and fibrosis and pulmonary HTN
what is a characterstic sign of SLE?
a facial rash that resembles the wings of a butterfly unfolding across both cheeks
how many SLE patients experience pulmonary related issues and what are the 2 types of pulmonary issues?
~50%
acute lupus pneumonitis
chronic lupus pneumonitis
what is acute lupus pneumonitis?
an acute infection of the lungs in a patient with lupus
this is a serious condition requiring immediate attention
can cause lung scarring
what is chronic lupus pneumonitis?
long term pneumonitis
slowly developing infection that results in scarring and pulmonary HTN
T/F: SLE is a multi-system disease
TRUE
impacts kidneys, lungs, CNS, blood vessles, and heart
chest wall trauma that impact lungs function can be broadly described as _______________________
anything that disrupts normal thoracic wall integrity and thus movement
blunt force trauma, rib/sternal injuries, surgical injury (thoracotomy)
