Restrictive Lung Disorders
Dr. hameed
Restrictive Lung Diseases
• Characterized by:
– Reduced lung compliance
– More pressure needed to expand lungs
– Lungs are stiff
– Pulmonary function test:
• Low FEV1, Low FVC (the ratio FEV1/FVC is
normal)
Restrictive Lung Diseases
• Types:
– Chest wall abnormality (not primary lung)
• Deformities, kyphoscoliosis
• Neuromuscular disease
– Primary lung disease
• Acute: Acute Respiratory Distress Syndrome (ARDS)
• Chronic:
– Interstitial lung disease (interstitial pneumonia), Idiopathic
pulmonary fibrosis
– Occupational: Asbestosis, silicosis, coal worker pneumoconiosis
– Immune diseases: Sarcoidosis, SLE, RA
– Physical injury: : Radiation
– Drugs: Chemotherapy, methotrexate
Acute Lung Injury &
Acute Respiratory Distress Syndrome
(ARDS)
ARDS
• A clinical syndrome caused by diffuse alveolar capillary
and epithelial damage.
• Characterized by:
– Rapid onset of life-threatening respiratory insufficiency,
– Cyanosis
– Tachypnea
– Tachycardia
– Bilateral pulmonary infiltrates (non cardiogenic edema)
– Severe arterial hypoxemia that is refractory to oxygen therapy
– May progress to multisystem organ failure.
Acute Lung Injury – Acute Respiratory
Distress Syndrome ARDS
• Direct injury to lung:
Pneumonia, aspiration, pulmonary contusion, fat
embolism, drowning, inhalation injury, post-lung
transplant
• Indirect Lung injury:
Sepsis, shock, cadiopulmonary bypass, acute
pancreatitis, transfusion, uremia
ARDS: pathogenesis
• The alveolar-capillary membrane is formed by two
separate barriers: the microvascular endothelium
and the alveolar epithelium.
• In ARDS, The integrity of this barrier is compromised
by either endothelial or epithelial injury, or, more
commonly, both.
ARDS
• Increased vascular permeability and alveolar
flooding, loss of diffusion capacity, and
widespread surfactant abnormalities caused
by damage to type II pneumocytes
• lung injury is caused by an imbalance of pro-
inflammatory and anti-inflammatory
mediators.
ARDS: pathogesesis
Acute Lung Injury – Acute Respiratory
Distress Syndrome ARDS
• Morphology:
– Diffuse alveolar damage
1. Exudative phase
2. Proliferative phase
3. Fibrotic phase
Acute Lung Injury – Acute Respiratory
Distress Syndrome ARDS
• Exudative phase (0 – 7 days)
• Grossly Lung is dark, red, firm, heavy
• Capillary congestion
• Necrosis of epithelial cells
• Interstitial, alveolar edema, hemorrhage
• Collection of neutrophils
• Alveolar collapse (loss of surfactant)
• Fibrin thrombi
• Hyaline membrane lining alveolar ducts
Diffuse alveolar damage in acute lung injury and acute respiratory distress
syndrome. Some alveoli are collapsed; others are distended. Many are lined by bright
pink hyaline membranes (arrow).
Acute Lung Injury – Acute Respiratory
Distress Syndrome ARDS
• Proliferative phase
• 1-3 weeks
• Proliferation of type II pneumocytes
• Macrophages, removing cell debris
• Fibrotic phase
• Resolution with minimal fibrosis
• Repair/Fibrosis, thick alveolar walls
• Progressive fibrosis (honeycomb lung) Rare
Acute Lung Injury – Acute Respiratory
Distress Syndrome ARDS
• Clinical:
– Mortality was 100%
– Now 30-40% with good ICU support
– Poor prognosis: old age, multisystem failure, high
levels of IL-1 (proinflammatory cytokines)
Chronic Restrictive Lung
Diseases
Chronic Restrictive Lung Diseases
• Group of different diseases
• Similar clinical, pulmonary function test and
pathological findings
• Represent 15% of non-infectious diseases of lungs
• End-stage: diffuse interstitial pulmonary fibrosis
(Honeycomb lung)
Chronic Restrictive Lung Diseases
• Interstitial lung disease (interstitial pneumonia),
Idiopathic pulmonary fibrosis
• Occupational: Asbestosis, silicosis, coal worker
pneumoconiosis
• Immune diseases: Sarcoidosis, SLE, RA, Wegener
• Physical injury: : Radiation
• Drugs: Chemotherapy, methotrexate
Idiopathic pulmonary fibrosis
• also known as cryptogenic fibrosing alveolitis
– A pulmonary disorder of unknown etiology.
– Characterized by patchy but progressive bilateral
interstitial fibrosis
– Primarily affects elderly
– Male are more affected than female
– Limited to the lungs
– Poor prognosis
– No effective treatment
IPF: Pathogenesis
• “repeated cycles” of epithelial activation/injury by some
unidentified agent
• Results in interstitial edema and accumulation of
inflammatory cells (alveolitis).
IPF: Pathogenesis
IPF: Morphology
• honeycomp appearance (advanced stage)
IPF: clinical course
• Present insidiously with gradual onset of nonproductive
cough
• Progressive dyspnea
• Cyanosis
• Corpulmonale
• Peripheral edema
Thank you
