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SEMINAR ON RESTRICTIVE LUNG DISEASE

Dr.Mohammed Hamza Baig

Pharm D

Restrictive lung disease

• Physiologically restrictive lung diseases are defined by reduced total lung capacity, vital capacity and functional residual capacity, but with preserved air flow.

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• Lung
• Interstitial Pulmonary
• Fibrosis (IPF):
• (Idiopathic)
• Occupational
• Collagenic
• Granulomatous
• Irradiation
• Resection
• Drug induced
• (Bleomycin, Methotrexate, Cyclophosphamide)

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Parenchymal

Extra-

Parenchymal

Restrictive Lung Diseases

Pleura

• Pleural effusion
• Pneumothorax
• Pleural fibrosis
• Pleural tumours
• Pleural thickening

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Chest Wall

• Trauma
• Kyphoscoliosis
• Ankylosing Spondylitis
• Neuromuscular Disease (Myasthenia/Guillain Barre)
• Morbid obesity
• Scleroderma

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Abdomen

Severe Distension

Restrictive - chest wall disorder

1.Kyphoscoliosis

2.Ankylosing spondilosis

3.Poliomyelitis

4.Severe obesity

5.Pleural diseases

Treatment:

• Each patient is individually assessed.
• Patients are treated if they have symptoms or progressive dysfunction on pulmonary function tests.
• Corticosteroids (Prednisone 1 mg/kg) is standard therapy.
• Prednisone dose is lowered over 6-8 weeks and continued at 15 mg for 1-2 years.
• Cyclophosphamide occasionally used.
• Antifibrotics such as colchicine may be used.
• Ancillary therapies such as oxygen, rehabilitation, psychosocial aspects are helpful.
• Surgical procedure for kyphoscoliosis is beneficial

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Pulmonary hypertension

• Pulmonary arterial hypertension (PAH) may be defined as a mean pulmonary artery pressure (PAPm) ≥25 mm Hg at rest with a pulmonary wedge pressure (also known as pulmonary artery occlusion pressure) ≤15 mm Hg measured by cardiac catheterization.

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Etiology

• Idiopathic (IPAH)
• Heritable
• Drug and toxin induced
• Associated with (APAH): Connective tissue diseases, HIV infection, Portal hypertension, Congenital heart diseases.
• Pulmonary hypertension owing to left-heart disease: Systolic dysfunction, Diastolic dysfunction and Valvular disease
• Pulmonary hypertension owing to lung diseases and/or hypoxemia: Chronic obstructive pulmonary disease, Interstitial lung disease, Other pulmonary diseases with mixed restrictive and obstructive pattern
• Pulmonary hypertension with unclear multifactorial mechanisms: Hematologic disorders: myeloproliferative disorders, splenectomy; Systemic disorders: sarcoidosis, neurofibromatosis, vasculitis; Metabolic disorders: glycogen storage disease, thyroid disorders;
• Others: tumoral obstruction, chronic renal failure on dialysis

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Clinical Presentation

Symptoms

• Exertional dyspnea, fatigue, weakness, complaints of general exertion intolerance, dyspnea at rest as the disease progresses, exertional chest pain, syncope, lower extremity edema
• Leg swelling, abdominal bloating and distension, anorexia, and more profound fatigue may develop as right ventricular dysfunction and tricuspid valve regurgitation evolve.

Signs

• Accentuated component of S₂ audible at the apex of the heart, early systolic ejection click, midsystolic ejection murmur, palpable left parasternal lift, right ventricular S₄ gallop, and a prominent "a" wave.

Signs of Advanced Disease

• Cyanosis (suggests right-to-left shunting), digital clubbing, rales, dullness, decreased breath sounds, accessory muscle use, wheezing, prolonged exhalation; peripheral venous insufficiency (suggests venous thrombosis or pulmonary thrombotic disease).

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Treatment

• Type 5A phosphodiesterase (PDE5A) is the target for sildenafil (Revatio®) and tadalafil (Adcirca®), which are also used to treat PAH. The side effects may include headache, nausea, mild hypotension, visual disturbances, and flushing of the skin.
• PGI2 analogs: Epoprostenol (PGI2, PGX, prostacyclin) (Flolan, Veletri) is marketed as an IV form of PGI2 for the treatment of pulmonary hypertension that does not respond to conventional therapies. Due to its short half-life (i.e., 3 to 5 mins), epoprostenol is administered by continuous IV infusion

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Calcium channel blockers

• Nifedipine extended release (Procardia XL, Nifedical XL, Adalat CC) Blocks the influx of extracellular calcium, causing vasodilation; more potent vasodilator than diltiazem.
• Diltiazem (Cardia XT, Cardizem CD, LA, SR, Taztia XT) Blocks the influx of extracellular calcium, causing vasodilation

Endothelin receptor antagonists

• Bosentan (Tracleer) Competitive antagonist for type A and B endothelin receptors, which mediate vasoconstriction and vasodilation. Lowers systemic vascular resistance, pulmonary vascular resistance, and mean pulmonary arterial pressure
• Ambrisentan (Letairis) Selective antagonist for type A endothelin receptors, which mediates vasoconstriction and vasodilation
• Lowers systemic vascular resistance, pulmonary vascular resistance, and mean pulmonary arterial pressure

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PATIENT COUNSELLING

Regarding disease:

• Restrictive lung diseases are characterized by reduced lung volumes, either because of an alteration in lung parenchyma or because of a disease of the pleura, chest wall, or neuromuscular apparatus.
• Chronic Obstructive Pulmonary Disease (COPD) is a preventable and treatable disease that makes it difficult to empty air out of the lungs.
• Pulmonary arterial hypertension (PAH) is a specific type of pulmonary hypertension where the tiny blood vessels in the lungs become scarred.
• Tuberculosis is a highly infectious disease that primarily affects the lungs caused by Mycobacterium tuberculi.

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Reference

• Leon shargel, Alan H, Paul F, Larry N. Comprehensive pharmacy review for naplex. 8^th edition. 2013.
• Joseph T. Dipiro, Barbara G. Wells, Celicy V. Dipiro. Pharmacotherapy handbook 7^th edition.
• Micromedex: Truven health analytics, 2017.
• Maffley RH: How to avoid complications of potent diuretics. JAMA 1976; 235(23K0):2526-2528.
• Tostmann A, Boeree MJ, Aarnoutse RE, et al: Antituberculosis drug-induced hepatotoxicity: concise up-to-date review. J Gastroenterol Hepatol 2008; 23(2):192-202.

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