Restrictive pulmonary syndrome: cause, what symptoms?

What is a restrictive syndrome?

The restrictive syndrome is characterized by restriction of total lung capacity, often associated with a decrease in vital capacity, leading to a reduction in mobilizable lung volume during inspiration and expiration. Most often it is detected during respiratory function tests. “The restrictive syndrome is opposed to obstructive syndrome which results in obstruction of expiratory flows and which is linked to asthma or a pathology related to smoking such as COPD. Pathologies that can cause a restrictive syndrome are fibrosing pulmonary pathologies making the lung more rigid (more difficult to inflate), pleural pathologies, neuromuscular diseases, obesity and chest wall/chest deformities. The result is alveolar hypoventilation which leads to an accumulation of C02 in the blood.“, specifies Dr Maxens Decavèle, resuscitator at the Pitié-Salpêtrière hospital.

What are the causes of a restrictive syndrome?

Many pathologies can cause a restrictive syndrome:

• Neuromuscular diseases that affect the diaphragm (main ventilation muscle) which then contracts less well, resulting in a restriction of lung volume: multiple sclerosis, amyotrophic lateral sclerosis, myopathies, myotonias, myasthenias, sGuillain-Barré syndrome ;
• Osteo-articular pathologies affecting the thoracic spine (kypho-scoliosis);
• Damage to the chest wall (sequelae of radiotherapy, burns) which becomes more rigid. ;
• Pathologies of the pleura: pleural calcifications, thoracoplasty, pleurisy which can make fibrin by healing and stiffening the envelope of the lung.
• Lung diseases: pneumonectomy, sequelae posttuberculosis, interstitial pneumopathy, idiopathic pulmonary fibrosis, sequelae of radiotherapy, lymphangitis and sarcoidosis.
• Pulmonary fibrotic diseases: idiopathic pulmonary fibrosisautoimmune diseases with lung damage such as rheumatoid arthritis, Goujeron-Sjögren syndrome, inflammatory myopathies.
• Obesity.

What are the symptoms of a restrictive syndrome?

“The restrictive syndrome is mainly manifested by dyspnea (unpleasant and agonizing feeling of lack of air, difficulty in breathing)) with effort then with the slightest effort then at rest when the disease progresses, orthopnea (shortness of breath when lying down), repeated lung infections, morning headaches, sleep disorders and severe fatigue“, indicates the resuscitator doctor.

The diagnosis of restrictive syndrome is based on spirometry, a test to assess the functioning of the lungs. More specifically, spirometry aims to measure the amount of air in the lungs (lung volumes) and the airflows during exhalation. This examination is part of the functional respiratory tests (EFR).

What treatment to treat a restrictive syndrome?

The management of restrictive pulmonary syndrome is based on the treatment of the cause (acute neuromuscular disease, pulmonary involvement of autoimmune diseases, etc.). “Sometimes, respiratory involvement may progress to chronic respiratory failure and require the start oflong-term oxygen therapy or even non-invasive ventilation using an artificial home ventilator“, adds Dr. Maxens Decavèle.

Thanks to Dr Maxens Decavèle, resuscitator at the Pitié-Salpêtrière hospital