OVERVIEW
Alveolar Proteinosis
Alveolar proteinosis is a disease of unknown etiology characterized by the abnormal deposition of large amounts of phospholipoprotein-like material in the alveoli.
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Department
Respiratory medicine
Clinical symptoms
Progressive post-activity shortness of breath, which may be accompanied by a slight cough or white mucoid sputum, with increased sputum volume secondary to infection. Cyanosis and pestle fingers are usually seen in patients with severe hypoxia.
Hazards
Easy to combine with pulmonary infections, especially opportunistic infections, and a few patients die of respiratory failure.
Complications
Pulmonary infection, respiratory failure, etc.
Examination
Blood routine examination, chest X-ray, chest CT examination, sputum examination, bronchoalveolar lavage, lung function and other tests.
Diagnosis
Symptoms are atypical, and diagnosis mainly relies on imaging as well as BAL or transbronchial lung biopsy or open chest lung biopsy.
Treatment principle
None of the drug treatment is effective. Transbronchoscopic segmental bronchoalveolar lavage, whole-lung lavage, GM-CSF therapy or gene therapy may be chosen.
Curability
7.9% to 29% of patients can remit on their own, treatment can accelerate the remission and improve the condition.
Dietary advice
Normal diet, pay attention to nutritional balance.
Etiology
Epidemiology
There are more males than females, with a male to female ratio of 2:1. The disease can develop at any age, from infancy to 70 years of age, but it is more common in middle-aged people between 30 and 50 years of age, accounting for about 80% of all cases. 7.9% to 29% of patients can resolve on their own. =
Etiology
The etiology is unknown. It may be related to (1) defective alveolar macrophage function, (2) secondary to other diseases or factors, and (3) genetic mutation.
Symptoms and diagnosis
Typical symptoms
The onset of the disease may be rapid or slow. Exercise intolerance is the most common first manifestation, and if undiagnosed, it may be characterized by progressive dyspnea and cough. It may be accompanied by fever, weakness, weight loss, chest pain, hemoptysis and loss of appetite. Respiratory symptoms in infants and young children are more insidious, most often manifested by growth retardation, with vomiting and diarrhea as the first symptom. When secondary infection occurs, sputum may be yellow and purulent. Cyanosis and severe shortness of breath may occur as the disease progresses. Signs are rare. There are only a few scattered wet rales or pleural friction sounds.
Other symptoms
The volume of sputum is increased in secondary infections. Cyanosis and mortar and pestle fingers are usually seen in patients with severe hypoxia.
Diagnostic basis
Diagnosis is based on imaging and a BAL or transbronchial lung biopsy or open lung biopsy. Chest imaging is suggestive of PAP if it is characterized by the presence of typical signs such as ground-glass shadows, map sign, and paving-stone sign, as well as a typical milky appearance of the BAL fluid, which can be delaminated on resting, and is diagnosed in combination with characteristic light microscopic manifestations of the pathology section.
Treatment
Treatment guideline
Bronchoscopic segmental alveolar lavage or whole lung lavage, and GM-CFS can be applied at the same time.
Drug therapy
Drug therapy is ineffective.
Surgical treatment
Transbronchoscopic segmental alveolar lavage or total lung lavage under local or general anesthesia. Most patients require whole-lung lavage.
Other treatments
GM-CSF therapy. There are clinical applications of subcutaneous injection of GM-CSF, or nebulized inhalation.
Prognosis.
The disease resolves spontaneously in 7.9% to 29% of patients. The average survival rate of patients who undergo whole-lung lavage is significantly higher than that of those who do not. The average five-year survival rate for patients who undergo total lung lavage is 92% to 96%.
Questions you may be concerned about
How to maintain alveolar proteinosis
Maintenance of alveolar protein deposition disease is mainly, pay attention to rest, avoid colds, and supply of nutrition.
1. The main clinical manifestations of alveolar proteinosis are shortness of breath, coughing, coughing up sputum, fatigue, emaciation, etc. Patients must pay attention to rest, ensure a good sleep, live a regular life, and avoid excessive exertion.
2. Alveolar proteinosis is likely to worsen when the weather is cold. It is important to increase or decrease the clothing according to the weather changes in a timely and appropriate manner to avoid catching a cold, colds and other conditions that may aggravate the cough and asthma.
3. Pay attention to the balanced supply of nutrients in the diet, eat more vegetables and fruits and high-quality protein foods (such as fish, chicken, etc.), which will help patients with alveolar protein deposition disease to recover.
Seek medical advice promptly if you feel unwell.
Nursing care
Daily care
To avoid or reduce the inhalation of dust and smoke, and avoid active and passive smoking, so as not to aggravate lung damage. To prevent and promptly treat colds, lower respiratory tract and lung infections.
Dietary regimen
Eat a normal diet and pay attention to balanced nutrition.