caa a22 4500 445883219 CHVBK 20180317145551.0 cr unu---uuuuu 170323e20110801xx s 000 0 eng 10.1007/s10157-011-0435-z doi (NATIONALLICENCE)springer-10.1007/s10157-011-0435-z Anti-glomerular basement membrane (anti-GBM) disease accompanied by vasculitis that was not positive for antineutrophil cytoplasmic antibodies to myeloperoxidase and proteinase 3: a report of two cases and the incidence of anti-GBM disease at one institution [Elektronische Daten] [Kimimasa Nakabayashi, Yasunori Fujioka, Yoshihiro Arimura, Toshihito Fukuoka, Tomohumi Marumo, Michiru Umino, Yasushi Kamiya, Takahiro Okai, Shigeru Tsurumaki, Toshihiko Nagasawa, Akira Yamada] Background: Anti-glomerular basement membrane (anti-GBM) disease is thought to be distinct from vasculitis. In contrast, there have been several papers suggesting the presence of angiitis in cases that were positive for anti-GBM antibody (Ab), as well as for either myeloperoxidase (MPO)- or proteinase 3 (PR3)-anti-neutrophil cytoplasmic antibody (ANCA) (Group I). We experienced four patients who had anti-GBM Abs, but not MPO- and PR3-ANCA (Group II), and two of these patients were found to have vasculitis. Therefore, we performed an in-depth study on these two patients. Methods: The patients with anti-GBM disease were isolated from 578 cases whose renal tissues were examined, and they were categorized into two groups. We have already published the data about Group I. We then proceeded to study two vasculitic patients in Group II clinically, pathologically, and serologically. The anti-GBM Ab and ANCA levels were detected by enzyme-linked immunosorbent assays. Renal specimens were studied by routine staining as well as immunohistochemical investigations of CD31 and type IV collagen. Results: The total number of patients with anti-GBM disease was 7 (7/578=1.2%), with 3 patients belonging to Group I and 4 patients belonging to Group II. Two patients in Group II were diagnosed to have vasculitis, but the remaining 2 patients did not. One vasculitic patient was complicated by pulmonary hemorrhage, while the other vasculitic patient displayed peripheral neuropathy as well as a small cavity lesion in the lung. The latter patient was found to be positive for perinuclear (p)-ANCA, but not for any other ANCA subsets. The renal pathology in the two vasculitic patients showed crescentic glomerulonephritis (CSGN) and immunoglobulin (Ig) G linear deposits along the glomerular capillary loops. The former patient showed fibrinoid angiitis in an afferent arteriole as well as peritubular capillaritis. The latter patient demonstrated peritubular capillaritis. These peritubular capillaritides were diagnosed by the loss of CD31 and type IV collagen staining, the blurred appearance of peritubular capillary walls by periodic acid-Schiff staining, and the pericapillary infiltration of inflammatory cells. Conclusion: The incidence of anti-GBM disease was very low, and our patients were categorized into two groups (Groups I and II) based on whether or not they were positive for MPO- or PR3-ANCA. Two patients in Group II were found to have vasculitis. According to our results, we concluded that the anti-GBM disease of Group II could also be associated with vasculitis. Japanese Society of Nephrology, 2011 Anti-GBM disease nationallicence Goodpasture's syndrome nationallicence ANCA nationallicence Peritubular capillaritis nationallicence Vasculitis nationallicence Nakabayashi Kimimasa First Department of Internal Medicine, Kyorin University School of Medicine, 6-20-2 Shinkawa, 181-8611, Mitaka, Tokyo, Japan aut Fujioka Yasunori Department of Pathology, Kyorin University School of Medicine, 6-20-2 Shinkawa, 181-8611, Mitaka, Tokyo, Japan aut Arimura Yoshihiro First Department of Internal Medicine, Kyorin University School of Medicine, 6-20-2 Shinkawa, 181-8611, Mitaka, Tokyo, Japan aut Fukuoka Toshihito First Department of Internal Medicine, Kyorin University School of Medicine, 6-20-2 Shinkawa, 181-8611, Mitaka, Tokyo, Japan aut Marumo Tomohumi First Department of Internal Medicine, Kyorin University School of Medicine, 6-20-2 Shinkawa, 181-8611, Mitaka, Tokyo, Japan aut Umino Michiru Department of Pathology, Kyorin University School of Medicine, 6-20-2 Shinkawa, 181-8611, Mitaka, Tokyo, Japan aut Kamiya Yasushi First Department of Internal Medicine, Kyorin University School of Medicine, 6-20-2 Shinkawa, 181-8611, Mitaka, Tokyo, Japan aut Okai Takahiro Department of Internal Medicine, Kawakita General Hospital, 1-7-3 Asagayakita, Suginami, 166-8588, Tokyo, Japan aut Tsurumaki Shigeru Division of Immunoserology in SRL Inc., 51 Komiya, 192-8535, Hachioji, Tokyo, Japan aut Nagasawa Toshihiko First Department of Internal Medicine, Kyorin University School of Medicine, 6-20-2 Shinkawa, 181-8611, Mitaka, Tokyo, Japan aut Yamada Akira First Department of Internal Medicine, Kyorin University School of Medicine, 6-20-2 Shinkawa, 181-8611, Mitaka, Tokyo, Japan aut Clinical and Experimental Nephrology Springer Japan 15/4(2011-08-01), 504-513 1342-1751 15:4<504 2011 15 10157 https://doi.org/10.1007/s10157-011-0435-z text/html Onlinezugriff via DOI 1 research-article jats NATIONALLICENCE 856 40 https://doi.org/10.1007/s10157-011-0435-z text/html Onlinezugriff via DOI NATIONALLICENCE 700 1- Nakabayashi Kimimasa First Department of Internal Medicine, Kyorin University School of Medicine, 6-20-2 Shinkawa, 181-8611, Mitaka, Tokyo, Japan aut NATIONALLICENCE 700 1- Fujioka Yasunori Department of Pathology, Kyorin University School of Medicine, 6-20-2 Shinkawa, 181-8611, Mitaka, Tokyo, Japan aut NATIONALLICENCE 700 1- Arimura Yoshihiro First Department of Internal Medicine, Kyorin University School of Medicine, 6-20-2 Shinkawa, 181-8611, Mitaka, Tokyo, Japan aut NATIONALLICENCE 700 1- Fukuoka Toshihito First Department of Internal Medicine, Kyorin University School of Medicine, 6-20-2 Shinkawa, 181-8611, Mitaka, Tokyo, Japan aut NATIONALLICENCE 700 1- Marumo Tomohumi First Department of Internal Medicine, Kyorin University School of Medicine, 6-20-2 Shinkawa, 181-8611, Mitaka, Tokyo, Japan aut NATIONALLICENCE 700 1- Umino Michiru Department of Pathology, Kyorin University School of Medicine, 6-20-2 Shinkawa, 181-8611, Mitaka, Tokyo, Japan aut NATIONALLICENCE 700 1- Kamiya Yasushi First Department of Internal Medicine, Kyorin University School of Medicine, 6-20-2 Shinkawa, 181-8611, Mitaka, Tokyo, Japan aut NATIONALLICENCE 700 1- Okai Takahiro Department of Internal Medicine, Kawakita General Hospital, 1-7-3 Asagayakita, Suginami, 166-8588, Tokyo, Japan aut NATIONALLICENCE 700 1- Tsurumaki Shigeru Division of Immunoserology in SRL Inc., 51 Komiya, 192-8535, Hachioji, Tokyo, Japan aut NATIONALLICENCE 700 1- Nagasawa Toshihiko First Department of Internal Medicine, Kyorin University School of Medicine, 6-20-2 Shinkawa, 181-8611, Mitaka, Tokyo, Japan aut NATIONALLICENCE 700 1- Yamada Akira First Department of Internal Medicine, Kyorin University School of Medicine, 6-20-2 Shinkawa, 181-8611, Mitaka, Tokyo, Japan aut NATIONALLICENCE 773 0- Clinical and Experimental Nephrology Springer Japan 15/4(2011-08-01), 504-513 1342-1751 15:4<504 2011 15 10157 Metadata rights reserved Springer special CC-BY-NC licence nationallicence BK010053 XK010053 XK010000 NATIONALLICENCE NATIONALLICENCE NL-springer