Yasuhiro Terasaki1, Soichiro Ikushima2, Shoko Matsui3, Akira Hebisawa4, Yasunori Ichimura5, Shinyu Izumi6, Masuo Ujita7, Machiko Arita8, Keisuke Tomii9, Yuko Komase10, Isoko Owan11, Tetsuji Kawamura12, Yasuo Matsuzawa13, Miho Murakami14,15, Hiroshi Ishimoto16, Hiroshi Kimura17, Masashi Bando18, Norihiro Nishimoto14,15, Yoshinori Kawabata19, Yuh Fukuda1, Takashi Ogura20. 1. Department of Analytic Human Pathology, Nippon Medical School, Tokyo, Japan. 2. Department of Respiratory Medicine, Japanese Red Cross Medical Center, Tokyo, Japan. 3. Health Administration Center, University of Toyama, Toyama, Japan. 4. Division of Clinical Pathology, NHO Tokyo National Hospital, Tokyo, Japan. 5. Department of Respirology, Graduate School of Medicine, Chiba University, Chiba, Japan. 6. Department of Respiratory Medicine, National Center for Global Health and Medicine, Tokyo, Japan. 7. Department of Radiology, Nishigunma National Hospital, Shibukawa, Japan. 8. Department of Respiratory Medicine, Kurashiki Central Hospital, Kurashiki, Japan. 9. Department of Respiratory Medicine, Kobe City Medical Center General Hospital, Kobe, Japan. 10. Department of Respiratory Medicine, St. Marianna University School of Medicine, Yokohama City Seibu Hospital, Yokohama, Japan. 11. Department of Respiratory Medicine, National Hospital Organization Okinawa National Hospital, Ginowan, Okinawa, Japan. 12. Department of Respiratory Medicine, National Hospital Organization Himeji Medical Center, Hyogo, Japan. 13. Department of Respiratory Medicine, Toho University Medical Center-Sakura Hospital, Chiba, Japan. 14. Department of Molecular Regulation for Intractable Diseases, Institute of Medical Science, Tokyo Medical University, Osaka, Japan. 15. Osaka Rheumatology Clinic, Osaka, Japan. 16. Department of Respiratory Medicine, University of Occupational and Environmental Health, Kitakyushu, Japan. 17. Department of Respiratory Medicine, Nara Medical University, Nara, Japan. 18. Department of Respiratory Medicine, Jichi Medical University, Shimotsuke, Tochigi, Japan. 19. Division of Diagnostic Pathology, Saitama Prefectural Cardiovascular and Respiratory Center, Saitama, Japan. 20. Division of Respiratory Medicine, Kanagawa Cardiovascular and Respiratory Center, Yokohama, Japan.
Abstract
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.
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.