Premium
Comparison of clinical and pathological features of lung lesions of systemic IgG4‐related disease and idiopathic multicentric Castleman's disease
Author(s) -
Terasaki Yasuhiro,
Ikushima Soichiro,
Matsui Shoko,
Hebisawa Akira,
Ichimura Yasunori,
Izumi Shinyu,
Ujita Masuo,
Arita Machiko,
Tomii Keisuke,
Komase Yuko,
Owan Isoko,
Kawamura Tetsuji,
Matsuzawa Yasuo,
Murakami Miho,
Ishimoto Hiroshi,
Kimura Hiroshi,
Bando Masashi,
Nishimoto Norihiro,
Kawabata Yoshinori,
Fukuda Yuh,
Ogura Takashi
Publication year - 2017
Publication title -
histopathology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.626
H-Index - 124
eISSN - 1365-2559
pISSN - 0309-0167
DOI - 10.1111/his.13186
Subject(s) - pathology , medicine , igg4 related disease , fibrosis , lung , vasculitis , pathological , lesion , infiltration (hvac) , stromal cell , disease , physics , thermodynamics
Aims The lung lesion [immunoglobulin (Ig)G4‐L] of IgG4‐related disease (IgG4‐ RD ) is a condition that occurs together with IgG4‐ RD and often mimics the lung lesion [idiopathic multicentric Castleman's disease ( iMCD ‐L)] of idiopathic multicentric Castleman's disease ( iMCD ). Because no clinical and pathological studies had previously compared features of these diseases, we undertook this comparison with clinical and histological data. Methods and results Nine patients had IgG4‐L (high levels of serum IgG4 and of IgG4 + cells in lung specimens; typical extrapulmonary manifestations). Fifteen patients had iMCD ‐L (polyclonal hyperimmunoglobulinaemia, elevated serum interleukin‐6 levels and polylymphadenopathy with typical lymphadenopathic lesions). Mean values for age, serum haemoglobin levels and IgG4/IgG ratios were higher in the IgG4‐L group and C‐reactive protein levels were higher in the iMCD ‐L group. All IgG4‐ RD lung lesions showed myxomatous granulation‐like fibrosis (active fibrosis), with infiltration of lymphoplasmacytes and scattered eosinophils within the perilymphatic stromal area, such as interlobular septa and pleura with obstructive vasculitis. All 15 lung lesions of iMCD , however, had marked accumulation of polyclonal lymphoplasmacytes in lesions with lymphoid follicles and dense fibrosis, mainly in the alveolar area adjacent to interlobular septa and pleura without obstructive vasculitis. Conclusions Although both lesions had lymphoplasmacytic infiltration, lung lesions of IgG4‐ RD were characterized by active fibrosis with eosinophilic infiltration within the perilymphatic stromal area with obstructive vasculitis, whereas lung lesions of iMCD had lymphoplasmacyte proliferating lesions mainly in the alveolar area adjacent to the perilymphatic stromal area. These clinicopathological features may help to differentiate the two diseases.