Abstract
Pulmonary Langerhans cell histiocytosis (PLCH) is a disease of the reticulohistiocytic system of unknown etiology, characterized by primary proliferation of atypical histiocytes and the formation of histiocytic granulomas in the lungs, also in other organs and tissues.
Aim: to evaluate the effectiveness of various therapy regimens for patients with PLCH.
Materials and Methods: 167 people with PLCH have been observed, aged mean 34.5 ± 13.1 years. The diagnosis was established clinically and radiographically, confirmed histologically in 75% of cases. Therapy regimens were used: 1) monotherapy of SCS in 27 patients; 2) the combination of low doses SCS with cytostatic therapy (azathioprine/endoxan) in 123 patients; 3) chemotherapy (cladribine/etoposide) with a progressive course or consistency of the disease in 17 people. The effectiveness of therapy was assessed on the results of CT imaging of the chest organs (no increase in the number and size of cysts, reduction or regression of foci), pulmonary functional tests (stabilization of indicators with combined bronchodilatory therapy) for the first year after therapy every 6 months, then once a year. The duration of follow-up was from 1 to 17 years.
Results:
Lung transplantation was performed in two patients in 2018.
Conclusion: The use of a combination of SCS+cytostatic drug is more effective than monotherapy with SCS. Chemotherapy can improve the prognosis of the course of the disease in patients with rapid progression.
Footnotes
Cite this article as: European Respiratory Journal 2019; 54: Suppl. 63, OA2142.
This is an ERS International Congress abstract. No full-text version is available. Further material to accompany this abstract may be available at www.ers-education.org (ERS member access only).
- Copyright ©the authors 2019