Restrictive Lung Disease

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Original Editor - Lucinda hampton

Top Contributors - Lucinda hampton, Kim Jackson and Vidya Acharya  

Introduction

Restrictive lung diseases are a heterogeneous set of pulmonary disorders defined by restrictive patterns on spirometry. These disorders are characterized by a reduced distensibility of the lungs, compromising lung expansion, and, in turn, reduced lung volumes, particularly with reduced total lung capacity (TLC).

Etiology

Its aetiology can be highly variable ranging from

• Intrinsic - with lung parenchymal involvement ie Interstitial Lung Disease: an umbrella term that encompasses a large number of disorders that are characterised by diffuse cellular infiltrates in a periacinar location. The spectrum of conditions included is broad, ranging from occasional self-limited inflammatory processes to severe debilitating pulmonary fibrosis.
• Extrinsic to the lung -
  • obesity
  • neuromuscular disorders
  • chest wall deformities
    • scoliosis: can lead to extrinsic restrictive lung disease from the diminution of lung volume under the convex rib hump and on the concave side, where the ribs impinge on the lung 4
    • excessive kyphosis
    • kyphoscoliosis

Those with a restrictive lung disease pattern can often have decreased lung volumes, an increased work of breathing, and inadequate ventilation and/or oxygenation. Pulmonary function tests often demonstrate a decrease in the forced vital capacity (FVC).

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