RT Journal Article
SR Electronic
T1 Analysis of multicentric Castleman's disease (MCD) with pulmonary lesions
JF European Respiratory Journal
JO Eur Respir J
FD European Respiratory Society
SP P5123
VO 42
IS Suppl 57
A1 Soichiro Ikushima
A1 Yasuhiro Terasaki
A1 Akira Hebisawa
A1 Takeshi Johko
A1 Fumikazu Sakai
A1 Norihiro Nishimoto
A1 Takashi Ogura
A1 Yasunori Ohta
A1 Haruyuki Ishii
A1 Shoko Matsui
A1 Hiroshi Yamamoto
A1 Yasunori Ichimura
A1 Masuo Ujita
A1 Tetsuharu Kawamura
A1 Masashi Bando
A1 Hiroshi Ishimoto
A1 Shinyu Izumi
A1 Norihiko Hata
A1 Keisuke Tomii
A1 Isoko Owan
A1 Hiroshi Kimura
A1 Yuko Komase
A1 Yasuo Matsuzawa
A1 Machiko Arita
A1 Akira Shiraki
YR 2013
UL http://erj.ersjournals.com/content/42/Suppl_57/P5123.abstract
AB BackgroundMCD is a systemic lymphoproliferative disease that often involves pulmonary lesions. Few studies have combined analyses of clinical, radiological, and pathological (CRP) data relating to this rare disease.AimWe aimed to identify the CRP features of this disease via a multicenter retrospective study.MethodsWe enrolled 40 patients who had MCD with pulmonary involvement throughout Japan.ResultsThe average age at diagnosis was 47 years (men, 13; women, 12). The patients were admitted with symptoms of cough (48%), dyspnea (24%), and fever (18%). Laboratory findings revealed elevated serum levels of IL-6 (24.1 pg/mL), KL6 (736.7 U/mL), CRP (6.68 mg/dL) and IgG4 (394.1 mg/dL). Common radiologic findings included presence of ground glass attenuation (96%), centrilobular nodules (92%), septal thickening (72%), bronchovascular thickening (64%), and cysts (64%).From the pathological point of view, all specimens showed lesions characteristic of marked lymphoplasmacytic accumulation with lymphoid follicles and lymphatic distribution of foci of dense fibrosis. Small cystic lesions were found in 4 lung samples, but no obstructive vasculitis was observed. The mean IgG4+/IgG+ plasma cell ratio was 22%. A pulmonary function test revealed progressive obstructive airflow limitation. Steroid therapy was successful in preventing progression in just 33% of cases, but tocilizumab was successful in 67%. Three patients died due to respiratory failure with progression of diffuse cystic lesions.ConclusionThe clinical course of pulmonary involvement of MCD varies from stable disease to progressive obstructive impairment. Tocilizumab appears to be more effective than steroid therapy in controlling the disease.