Antineutrophil cytoplasmic antibody-negative granulomatosis with polyangiitis localized to the lungs

Patients with granulomatosis with polyangiitis (GPA), formerly known as Wegener's granulomatosis, sometimes exhibit no clinical features. Here, we describe a case of antineutrophil cytoplasmic antibody (ANCA)-negative GPA presenting with only lung granuloma. A 55-year-old woman with a right upp...

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Main Authors: Yuichi Ohteru, Kazuki Hamada, Keiji Oishi, Junki Suizu, Misa Harada, Keita Murakawa, Ayumi Chikumoto, Kazuki Matsuda, Sho Uehara, Shuichiro Ohata, Yoriyuki Murata, Yoshikazu Yamaji, Kenji Sakamoto, Maki Asami-Noyama, Nobutaka Edakuni, Tsunahiko Hirano, Tomoyuki Kakugawa, Tomoyuki Murakami, Tamiko Takemura, Kazuto Matsunaga
Format: Article
Language:English
Published: Elsevier 2022-01-01
Series:Respiratory Medicine Case Reports
Subjects:
Online Access:http://www.sciencedirect.com/science/article/pii/S2213007122000223
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author Yuichi Ohteru
Kazuki Hamada
Keiji Oishi
Junki Suizu
Misa Harada
Keita Murakawa
Ayumi Chikumoto
Kazuki Matsuda
Sho Uehara
Shuichiro Ohata
Yoriyuki Murata
Yoshikazu Yamaji
Kenji Sakamoto
Maki Asami-Noyama
Nobutaka Edakuni
Tsunahiko Hirano
Tomoyuki Kakugawa
Tomoyuki Murakami
Tamiko Takemura
Kazuto Matsunaga
author_facet Yuichi Ohteru
Kazuki Hamada
Keiji Oishi
Junki Suizu
Misa Harada
Keita Murakawa
Ayumi Chikumoto
Kazuki Matsuda
Sho Uehara
Shuichiro Ohata
Yoriyuki Murata
Yoshikazu Yamaji
Kenji Sakamoto
Maki Asami-Noyama
Nobutaka Edakuni
Tsunahiko Hirano
Tomoyuki Kakugawa
Tomoyuki Murakami
Tamiko Takemura
Kazuto Matsunaga
author_sort Yuichi Ohteru
collection DOAJ
description Patients with granulomatosis with polyangiitis (GPA), formerly known as Wegener's granulomatosis, sometimes exhibit no clinical features. Here, we describe a case of antineutrophil cytoplasmic antibody (ANCA)-negative GPA presenting with only lung granuloma. A 55-year-old woman with a right upper lung mass underwent lobectomy for suspected lung cancer; however, only granuloma was detected, and the etiology was not identified. Serum ANCA results were negative. Four years postoperatively, another pulmonary nodule appeared in the left lung's apex. The kidneys and sinuses were not impaired, but re-examination of the resected specimen revealed necrotizing vasculitis and granulomas around the vessels. Thus, the patient was diagnosed with GPA localized to the lungs. Although this was a non-life-threatening disease, the patient was administered oral prednisolone (PSL) and intravenous cyclophosphamide (IVCY) to prevent fatal complications of GPA as she was non-elderly and had no comorbidities, leading to a decrease in the mass size. Detailed re-examination by expert pulmonary pathologists could aid in GPA diagnosis when clinical features are absent, as in our case. In patients with granulomas of unknown etiology, a careful multidisciplinary approach is pivotal in the diagnosis. If patients tolerate adverse effects, a PSL and IVCY combination may prevent fatal outcomes, even in patients with non-life-threatening disease.
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spelling doaj.art-b5eb3775cac344b9ab99fcdd25eba0722022-12-21T23:40:40ZengElsevierRespiratory Medicine Case Reports2213-00712022-01-0136101600Antineutrophil cytoplasmic antibody-negative granulomatosis with polyangiitis localized to the lungsYuichi Ohteru0Kazuki Hamada1Keiji Oishi2Junki Suizu3Misa Harada4Keita Murakawa5Ayumi Chikumoto6Kazuki Matsuda7Sho Uehara8Shuichiro Ohata9Yoriyuki Murata10Yoshikazu Yamaji11Kenji Sakamoto12Maki Asami-Noyama13Nobutaka Edakuni14Tsunahiko Hirano15Tomoyuki Kakugawa16Tomoyuki Murakami17Tamiko Takemura18Kazuto Matsunaga19Department of Respiratory Medicine and Infectious Disease, Graduate School of Medicine, Yamaguchi University, JapanDepartment of Respiratory Medicine and Infectious Disease, Graduate School of Medicine, Yamaguchi University, Japan; Corresponding author. Department of Respiratory Medicine and Infectious Disease, Graduate School of Medicine, Yamaguchi University, 1-1-1 Minami-kogushi, Ube, Yamaguchi, 755-8505, Japan.Department of Medicine and Clinical Science, Graduate School of Medicine, Yamaguchi University, JapanDepartment of Respiratory Medicine and Infectious Disease, Graduate School of Medicine, Yamaguchi University, JapanDepartment of Respiratory Medicine and Infectious Disease, Graduate School of Medicine, Yamaguchi University, JapanDepartment of Respiratory Medicine and Infectious Disease, Graduate School of Medicine, Yamaguchi University, JapanDepartment of Respiratory Medicine and Infectious Disease, Graduate School of Medicine, Yamaguchi University, JapanDepartment of Respiratory Medicine and Infectious Disease, Graduate School of Medicine, Yamaguchi University, JapanDepartment of Respiratory Medicine and Infectious Disease, Graduate School of Medicine, Yamaguchi University, JapanDepartment of Respiratory Medicine and Infectious Disease, Graduate School of Medicine, Yamaguchi University, JapanDepartment of Medicine and Clinical Science, Graduate School of Medicine, Yamaguchi University, JapanDepartment of Respiratory Medicine and Infectious Disease, Graduate School of Medicine, Yamaguchi University, JapanDepartment of Respiratory Medicine and Infectious Disease, Graduate School of Medicine, Yamaguchi University, JapanDepartment of Respiratory Medicine and Infectious Disease, Graduate School of Medicine, Yamaguchi University, JapanDepartment of Respiratory Medicine and Infectious Disease, Graduate School of Medicine, Yamaguchi University, JapanDepartment of Respiratory Medicine and Infectious Disease, Graduate School of Medicine, Yamaguchi University, JapanDepartment of Pulmonary and Gerontology, Graduate School of Medicine, Yamaguchi University, JapanDepartment of Pathology, National Hospital Organization Kanmon Medical Center, JapanDepartment of Pathology, Kanagawa Cardiovascular and Respiratory Center, JapanDepartment of Respiratory Medicine and Infectious Disease, Graduate School of Medicine, Yamaguchi University, JapanPatients with granulomatosis with polyangiitis (GPA), formerly known as Wegener's granulomatosis, sometimes exhibit no clinical features. Here, we describe a case of antineutrophil cytoplasmic antibody (ANCA)-negative GPA presenting with only lung granuloma. A 55-year-old woman with a right upper lung mass underwent lobectomy for suspected lung cancer; however, only granuloma was detected, and the etiology was not identified. Serum ANCA results were negative. Four years postoperatively, another pulmonary nodule appeared in the left lung's apex. The kidneys and sinuses were not impaired, but re-examination of the resected specimen revealed necrotizing vasculitis and granulomas around the vessels. Thus, the patient was diagnosed with GPA localized to the lungs. Although this was a non-life-threatening disease, the patient was administered oral prednisolone (PSL) and intravenous cyclophosphamide (IVCY) to prevent fatal complications of GPA as she was non-elderly and had no comorbidities, leading to a decrease in the mass size. Detailed re-examination by expert pulmonary pathologists could aid in GPA diagnosis when clinical features are absent, as in our case. In patients with granulomas of unknown etiology, a careful multidisciplinary approach is pivotal in the diagnosis. If patients tolerate adverse effects, a PSL and IVCY combination may prevent fatal outcomes, even in patients with non-life-threatening disease.http://www.sciencedirect.com/science/article/pii/S2213007122000223Localized granulomatosis with polyangiitisAntineutrophil cytoplasmic antibodyNon-life-threatening diseaseGranuloma of unknown cause
spellingShingle Yuichi Ohteru
Kazuki Hamada
Keiji Oishi
Junki Suizu
Misa Harada
Keita Murakawa
Ayumi Chikumoto
Kazuki Matsuda
Sho Uehara
Shuichiro Ohata
Yoriyuki Murata
Yoshikazu Yamaji
Kenji Sakamoto
Maki Asami-Noyama
Nobutaka Edakuni
Tsunahiko Hirano
Tomoyuki Kakugawa
Tomoyuki Murakami
Tamiko Takemura
Kazuto Matsunaga
Antineutrophil cytoplasmic antibody-negative granulomatosis with polyangiitis localized to the lungs
Respiratory Medicine Case Reports
Localized granulomatosis with polyangiitis
Antineutrophil cytoplasmic antibody
Non-life-threatening disease
Granuloma of unknown cause
title Antineutrophil cytoplasmic antibody-negative granulomatosis with polyangiitis localized to the lungs
title_full Antineutrophil cytoplasmic antibody-negative granulomatosis with polyangiitis localized to the lungs
title_fullStr Antineutrophil cytoplasmic antibody-negative granulomatosis with polyangiitis localized to the lungs
title_full_unstemmed Antineutrophil cytoplasmic antibody-negative granulomatosis with polyangiitis localized to the lungs
title_short Antineutrophil cytoplasmic antibody-negative granulomatosis with polyangiitis localized to the lungs
title_sort antineutrophil cytoplasmic antibody negative granulomatosis with polyangiitis localized to the lungs
topic Localized granulomatosis with polyangiitis
Antineutrophil cytoplasmic antibody
Non-life-threatening disease
Granuloma of unknown cause
url http://www.sciencedirect.com/science/article/pii/S2213007122000223
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