Restrictive Pulmonary Disease Flashcards
restrictive lung disease
difficulty fully expanding one’s lungs with air
what is restrictive lung disease often a result from
- condition causing stiffness (reduced compliance) in the lungs
- sometimes stiffness of the chest wall, weak muscles, damaged nerves
symptoms of restrictive lung disease
SOB
dry or non-productive cough
restrictive lung disease characterized by
- reduced lung volume: decreased total lung compliance, FEV1 remains unchanged, FEV1 to FVC preserved
- reduced compliance
internal causes of restrictive lung disease
- destruction or scarring of distal lung parenchyma due to infiltrates from inflammation, toxins, etc.
- idiopathic fibrotic disease
- CT disease
- drug-induced lung disease
- environmental exposure
- sarcoidosis
external causes of restrictive lung disease
- kyphoscoliosis
- pleural conditions like effusions, pleural scarring
- obesity
- neuromuscular disease
- ascites
- altered mechanical function induced by deformations of the rib cage
pathophysiology of restrictive lung disease
inflammation –> recruitment of fibroblasts –> pulmonary fibrosis –> sclerotic injury to alveolar walls –> imposing physical barrier to gas exchange –> decreases DLCO and ability of the alveoli to expand
obesity hypoventialtion syndrome
- pickwickian syndrome
- obese pt with sleeping disordered breathing
- hypoventilation
- daytime hypercapnia
- daytime hypoxemia
prognosis of restrictive lung disease
- varies depending on the etiology of pulmonary restriction
- pt’s diagnosed with interstitial pulmonary fibrosis have a median survival of 3-5 years after
- acute interstital pneumonia, 70% mortatlity and leads to ARDS
physical exam of restrictive lung disease
- lung sounds
- cyanosis
- digital clubbing
- cor pulmonale
- decreased tactile fremitus, dullness upon percussion, decreased intensity of breath sounds
medical management of restrictive lung disease
- symptom management
- corticosteroids
- anti-inflammatories
treatment for restrictive lung disease
- insuring adequate oxygen, maintaining an airway and obtaining maximal function
- surgical modification of MSK deformities
- heart-lung transplants
- not reversible
pneumoconiosis
- lung disease caused by breathing in certain kinds of dust particles that damage your lungs
- inflammation, coughing, fibrosis
- blood vessel and alveolar damage-interface becomes thicker and stiffer
interstitial fibrosis
inflammation and fibrosis of the pulmonary interstitium
interstitial lung disease
large group of disorders, most of which cause scarring of lung tissue
types of interstitial lung disease
- exposure related
- autoimmune related
- idiopathic
examples of pneumoconiosis
black lung disease
brown lung disease
asbestoses
silicosis
pneumoconiosis signs and symptoms
- range in severity
- cough
- phlegm
- SOB
- progressive respiratory failure
- lung cancer
- TB
- heart failure
treatment for chronic interstitial diseases
meds
treat complications
chest physiology
exercise
treatment for chronic interstitial diseases
meds
treat complications
chest physiology
exercise
relationship btw static lung volume and chronic interstitial disease
- decreased secondary to increased lung stiffness and decreased number of alveoli
honeycomb lung
wide spread fibrous
dilated and thickened terminal and respiratory bronchioles
why does a neuromuscular chest wall disorder result in restrictive lung disease
affects nerves that control respiration
why does skeletal deformities of the chest wall result in restrictive lung disease
osteoporosis
how does obesity result in restrictive lung disease
limited space for thoracic cavity to move
how does collagen vascular disease result in restrictive lung disease
connective tissue disease –> fibrosis –> restrictive
idopathic pulmonary fibrosis
- progressive interstitial pulmonary fibrosis and respiratory failure
- environmental factors: smoke
- genetic factors: smoking + gene variants
- age: rare under 50
how does idopathic pulmonary fibrosis begin
- insidiously with gradual increasing dyspnea on exertion and dry cough
- unpredictable progression
signs/symptoms of idiopathic pulmonary fibrosis
hypoxemia
cyanosis
clubbing
prognosis of idiopathic pulmonary fibrosis
median survival 3 yrs after dx
therapy for idiopathic pulmonary fibrosis
lung transplant
RA and pulmonary involvement
30-40% have pulmonary involvement
- chronic pleuritis, pneumonitis & fibrosis, pulmonary HTN
systemic sclerosis (scleroderma)
diffuse interstital fibrosis
systemic lupus and pulmonary involvement
- about 50% of peope with SLE will experience lung involvement during the course of their disease
- chronic, long term pneumonitis
- pulmonary HTN
pleuritis
inflammation of the pleura
pneumonitis
general term that refers to inflammation of the lung tissue - lung scarring
