Does pulmonary histiocytosis matter?

summary

Pulmonary histiocytosis is a group of rare diseases of unknown origin. The name of the disease was proposed by Lichtenstein in 1953. The disease mostly occurs in children, the incidence, clinical symptoms and the scope of lesions vary greatly, but there is a common pathological feature in pathology, that is, abnormal proliferation of histiocytes. Does pulmonary histiocytosis matter?

Does pulmonary histiocytosis matter?

Langerhans histiocytosis can occur at any age. Multiorgan and multisystem Langerhans histiocytosis usually occurs in infants and children, but pulmonary involvement is usually not the main clinical manifestation. On the contrary, lung Langerhans histiocytosis only involving the lung mostly occurred in the age group of 20-40 years old, and there was no significant gender difference.

The clinical manifestations of pulmonary Langerhans histiocytosis vary greatly. About 25% of the patients have no clinical symptoms and are only found by chance during physical examination. Some patients are found during X-ray examination after pneumothorax or respiratory symptoms. The most common symptoms were dry cough (56% - 70%) and dyspnea (40%). Other clinical symptoms included chest pain (10% - 20%), fatigue (30%), weight loss (20% - 30%) and fever (15%). About half of the patients had a history of rhinitis before onset. Hemoptysis is rare, less than 5%. About 25% of patients may have recurrent pneumothorax.

4% - 20% of the patients may have cystic lesions of the bone. Patients usually have focal bone pain or pathological fracture. A few patients (about 15%) may have central nervous system involvement and indicate poor prognosis. Physical examination: no obvious abnormality was found“ "Crackle" and "clubbing finger" are not common. Secondary pulmonary hypertension can be seen, but it is often ignored. Pulmonary heart disease can occur in the late stage of the disease.

matters needing attention

The clinical course of pulmonary histiocytosis is very different. About 50% of the patients can subside naturally or after glucocorticoid treatment. 10% - 20% of the patients show progressive progress, recurrent spontaneous pneumothorax, and finally death due to respiratory failure and pulmonary heart disease. There are several factors related to the prognosis of the patients. They include younger or older age of onset, persistent systemic symptoms, recurrent pneumothorax, extrapulmonary manifestations, diffuse cystic changes and significant pulmonary dysfunction in the early stage of onset.