OVERVIEW
OVERVIEW
Pulmonary Langerhans cell histiocytosis is a chronic progressive lung disease characterized by peribronchiolar histiocytic infiltration and granulomatous destruction of Langerhans cells in the distal airways. It is relatively rare. The main manifestations are dry cough, progressive exertional dyspnea, and recurrent spontaneous pneumothorax.
Whether medical insurance
yes
Department
Respiratory Medicine
Clinical symptoms
The main manifestations are dry cough, progressive exertional dyspnea, and recurrent spontaneous pneumothorax.
Hazards
Repeated spontaneous pneumothorax, advanced pulmonary fibrosis, poor prognosis.
Examination
Blood routine, erythrocyte sedimentation rate, pulmonary function test, X-ray chest film, chest CT, bronchoscopy, lung biopsy, etc.
Diagnosis
Confirmation of diagnosis often relies on lung tissue biopsy.
Treatment principles
Smoking cessation, glucocorticoids or immunosuppressants, lung transplantation if necessary.
Curability
Aggressive treatment improves symptoms and quality of life. The prognosis for advanced disease is poor.
Dietary recommendations
Balanced nutrition and calcium supplementation.
Etiology
Etiology
The cause of the disease is unknown, and is mostly thought to be a disease caused by monoclonal histiocytosis, with immune regulation also playing a role. The disease is closely related to smoking, and more than 90% of patients are smokers.
Symptoms and Diagnosis
Typical symptoms
The main manifestations are dry cough, progressive exertional dyspnea, recurrent spontaneous pneumothorax, which may be accompanied by fever, night sweats, loss of appetite and emaciation.
Diagnostic basis
1. History of smoking.
2. Dry cough, progressive exertional dyspnea, recurrent spontaneous pneumothorax.
3. High-resolution CT examination shows nodular changes in the early stage, and then gradually progresses to vesicular changes or even honeycomb lung, with the lesions mainly involving the upper and middle lungs.
Treatment
Treatment guidelines
Smoking cessation, glucocorticoids or immunosuppressants, lung transplantation if necessary.
Drug therapy
Glucocorticoids or immunosuppressants
Prognosis
Aggressive treatment improves symptoms and quality of life. Prognosis is poor in advanced stages.
Nursing care
Daily care
1. Keep a good mood and eliminate bad emotions.
2. Give symptomatic treatment with medicines as prescribed by doctor.
3. Wash hands frequently, pay attention to hygiene and cleanliness, and strengthen oral hygiene.
4. If symptoms are not relieved after medication or abnormalities occur, consult a doctor promptly.
Dietary management
Eat more food with high calcium content and pay attention to the nutritional structure.