Analysis of multicentric Castleman’s disease with pulmonary involvement: A single center study

Abstract

Background: Multicentric Castleman’s disease (MCD) is a rare polyclonal lymphoproliferative disorder and often involves pulmonary lesions. An infection by a virus called human herpesvirus 8 (HHV-8) is associated with multicentric Castleman disease in about half of people with MCD. Whilst in Japan, most of the cases are associated with negative HHV-8. There is no standard therapy for MCD, and the literature consists mostly of single case reports and small case series. Aim: To analyze the clinical features and treatment options of MCD. Methods: The medical records of patients with MCD from 2008 to 2018 were retrospectively reviewed. Results: Seven patients (male: 2, female: 5) were diagnosed in those 11 years. All were non - HIV. The average age at diagnosis was 45 years. These 7 patients visited hospital because of: cough in 2, arthralgia in 1, chest pain in 1, fever in 1, anemia in 1, and finally chest X-ray abnormality in 1. Radiological findings revealed centrilobular nodules in 6 (86%), septal thickening in 6 (86%), multiple nodules in all (100%), ground glass attenuation in 5 (71%), and cysts in 4 (57%). Laboratory findings for almost all of the patients revealed anemia and elevated serum levels of CRP, IgG. Five patients were treated with steroid and tocilizumab and the remaining 2 had no treatment. Conclusion: The clinical course of MCD varies from stable to progressive. Long term treatment was needed and the combination of tocilizumab with steroid allowed the steroid to be tapered off safely and to successfully prevent the progression of MCD.