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Pulmonary function in familial amyloidosis with polyneuropathy.
Acta Medica Scandinavica
|January 1, 1981
Summary
Familial amyloidosis with polyneuropathy affects respiratory muscles, causing low lung pressures. Long-term disease may lead to reduced diffusing capacity due to amyloid deposits.
Area of Science:
- Pulmonary Medicine
- Neurology
- Genetics
Background:
- Familial amyloidosis with polyneuropathy (FAP) is a progressive, inherited disorder.
- Neuropathy typically affects peripheral nerves, leading to sensory and motor deficits.
- Respiratory muscle involvement in FAP is not fully characterized.
Purpose of the Study:
- To evaluate pulmonary function in patients with familial amyloidosis with polyneuropathy.
- To assess the impact of FAP on respiratory muscle strength and lung mechanics.
- To investigate potential mechanisms for pulmonary dysfunction in FAP.
Main Methods:
- Pulmonary function tests were conducted on 14 patients diagnosed with FAP.
- Measurements included maximum expiratory and inspiratory pressures, spirometry, and diffusing capacity.
- Arterial oxygen tension was assessed at rest.
Main Results:
- All patients with advanced polyneuropathy exhibited reduced maximum expiratory and inspiratory pressures, indicating respiratory muscle weakness.
- Spirometry suggested a mild restrictive pattern in several patients, with reduced expiratory and inspiratory flows in two.
- Reduced diffusing capacity was observed in patients with long-standing FAP, suggesting an alveolo-capillary block from amyloid infiltration.
Conclusions:
- Familial amyloidosis with polyneuropathy involves respiratory muscles, impacting lung function.
- Amyloid deposition in the lungs may cause impaired gas exchange in advanced stages of FAP.
- Pulmonary monitoring is crucial for patients with FAP to detect and manage respiratory complications.