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