1. Restrictive lung disease
Dr. Rehab Farrag Gwada

2. Objectives Define the restrictive lung disease
Explains the causes & pathogenesis of RLD
Identify risk factors & symptoms of RLD
Outline the diagnosis in addition to MEDICAL CARE AND TREATMENT
Identify the prognosis

3. Definition
RLD are a category of extra-pulmonary, pleural, or parenchymal respiratory diseases that restrict lung expansion, resulting in:-
A- A decreased lung volume
B- An increased work of breathing
C- Inadequate ventilation and/or oxygenation.
Pulmonary function test demonstrates a decrease in the forced vital capacity but FEV1/FVC is normal.

4. Classification of Restrictive lung diseases based on anatomical structures:
Intrinsic lung diseases (diseases of the lung parenchyma)
Extrinsic disorders (extra-parenchymal diseases)

5. Intrinsic Lung Diseases cause either
Inflammation and/or scarring of lung tissue (interstitial lung disease) or
Fill the air spaces with exudate and debris (pneumonitis).
These diseases are classified further according to the etiological factor.

6. Extrinsic Disorders
The chest wall, pleura and respiratory muscles are the components of respiratory pump.
Disorders of these structures will cause lung restriction and impair ventilatory function.
These are grouped as:
Non-muscular diseases of the chest wall.(e.g: kyphoscoliosis )
Neuromuscular disorders. E.g:Poliomyelitis

7. Pathophysiology

8. Pathophysiology Intrinsic lung diseases:
Diffuse parenchymal disorders cause reduction in all lung volumes.
This is produced by excessive elastic recoil of the lungs.
Expiratory flows are reduced in proportion to lung volumes.
Arterial hypoxemia is caused by ventilation/perfusion mismatch.
Impaired diffusion of oxygen will cause exercise-induced desaturation.
Hyperventilation at rest secondary to reflex stimulation.

9. Extrinsic Disorders
Diseases of the pleura, thoracic cage, decrease compliance of respiratory system.
There is reduction in lung volumes.
Secondarily, atelectasis occurs leading to V/Q mismatch and hypoxemia.
The thoracic cage and neuromuscular structures are a part of respiratory system.
Any disease of these structures will cause restrictive disease and ventilatory dysfunction.

10. Extrinsic Disorders
Obesity is becoming a major cause of restrictive lung disease in the developed world. Over 30% of American adults are classified as obese, with a BMI greater than 30.
There is an inverse relationship between BMI and lung volumes.
Jones et al[2006] showed that FRC and ERV can even be significantly reduced in the overweight, with BMI of

11. Risk factors
Exposure to dust, metals, organic solvents, and agricultural employment is associated with increased risk of IPF
Race
US prevalence of sarcoidosis is estimated to be times higher among African Americans compared to white Americans
Sex
Men are more likely to have pneumoconiosis because of occupational exposure, IPF, and collagen-vascular diseases (eg, rheumatoid lung).
-sarcoidosis is slightly more common in women.
Age
IPF is rare in children. Most patients with IPF are older than 50 years.
Some intrinsic lung diseases present in patients aged years. These include sarcoidosis, collagen-vascular–associated diseases.
Sarcoidosis, also called sarcoid, is a disease involving abnormal collections of inflammatory cells (granulomas) that can form as nodules in multiple organs.[1] The granulomas are most often located in the lungs or its associated lymph nodes, but any organ can be affected.[2] Sarcoidosis seems to be caused by an immune reaction to an infection or some other trigger (called an antigen) that continues even after the initial infection or other antigen is cleared from the body.
Pneumoconiosis is an occupational lung disease and a restrictive lung disease caused by the inhalation of dust
Idiopathic pulmonary fibrosis (IPF) is a chronic and ultimately fatal disease characterized by a progressive decline in lung function.[2][3] The term pulmonary fibrosis means scarring of lung tissue and is the cause of worsening dyspnea (shortness of breath). Fibrosis is usually associated with a poor prognosis.[2][3][4]
IPF belongs to a large group of more than 200 lung diseases known as interstitial lung diseases (ILDs), characterized by the involvement of lung interstitium.[3] The interstitium, the tissue between the air sacs in the lung, is the primary site of injury in ILDs

12. Symptoms of intrinsic diseases
Progressive exertional dyspnea is the predominant symptom.
A dry cough is common. A productive cough is an unusual sign in most patients with diffuse parenchymal lung disorders.
Hemoptysis or grossly bloody sputum occurs in patients with diffuse alveolar hemorrhage syndromes and vasculitis.
Wheezing is an uncommon manifestation but can occur in patients with chronic pneumonia, and respiratory bronchiolitis.
Chest pain is uncommon in most instances of the disease, but pleuritic chest pain can occur in patients with rheumatoid arthritis, and some drug-induced disorders

13. Symptoms of extrinsic disorders
Nonmuscular diseases of the chest wall affect patients with kyphoscoliosis.
Patients younger than 35 years tend to be asymptomatic, whereas middle-aged patients
develop dyspnea
decreased exercise tolerance
respiratory infections.

14. Symptoms of extrinsic disorders
Patients with neuromuscular diseases develop significant respiratory muscle weakness and may demonstrate:-
Fatigue
Dyspnea upon exertion, followed by dyspnea at rest.
Impaired control of secretions.
Recurrent lower respiratory tract infections.
NB: Acute and chronic respiratory failure, pulmonary hypertension, and cor pulmonale eventually ensue.

15. Diagnosis History Physical examination. Chest radiograph
CT scan of the lungs.
Pulmonary function test (Spirometry) [ FEV1,  FVC, FEV1/FVC normal]
Gas exchange (Arterial PaO2 and PaCO2 are reduced, pH normal. On exercise PaO2 decreases dramatically)
Bronchoscopy

16. MEDICAL CARE AND TREATMENT
Treatment depends on the specific diagnosis
Intrinsic Disorders
Supplemental oxygen is used to support the patient with pulmonary disease. Oxygen is
used as a drug to treat low oxygen levels below 90%. O2 helps to reduce the work of breathing, and decrease cardiac work load.
Corticosteroids

17. MEDICAL CARE AND TREATMENT
Cytotoxic therapy
immunosuppressive cytotoxic agents may be considered for patients who do not respond to steroids, experience adverse effects, or have contraindications to high-dose corticosteroid; therapy will reduce inflammation
Antifibrotic therapy helps to slow progression of fibrotic tissue damage
Pulmonary rehabilitation program
Lung transplant is a surgical procedure that replaces a diseased lung with a healthy lung from a donor.

18. MEDICAL CARE AND TREATMENT
Extrinsic Disorders
Treatment of disorder to minimize impairment may consist of treatment of the underlying cause of the chest wall disorder.
Non-invasive positive pressure ventilation (NPPV).
It decreases the work of breathing and thereby improves alveolar ventilation while simultaneously resting the respiratory musculature
Mechanical ventilation via artificial airway is used to treat advanced restrictive lung disease.
Oral /Nasal suctioning
cough-assist
Treatment for massive obesity consists of weight loss
Surgical procedures
Pulmonary rehabilitation program

19. Prognosis
The mortality and morbidity from various causes of restrictive lung disease is dependent on the underlying case of the disease process.
The median survival time for patients with idiopathic pulmonary fibrosis(IPF) is of 3-6 years after diagnosis.
Approximately 15% of Pulmonary sarcoidosis patients develop pulmonary fibrosis and disability.
Factors that predict poor outcome include :-
older age, male sex,
severe dyspnea, history of cigarette smoking,
severe loss of lung function,
appearance and severity of fibrosis on radiologic studies,
lack of response to therapy, and prominent fibroblastic foci on
evaluation.

20. Thank you