Cargando…

Anti-glomerular basement membrane disease associated with thin basement membrane nephropathy: A case report

RATIONALE: Simultaneous occurrence of anti-glomerular basement membrane (anti-GBM) disease and thin basement membrane nephropathy (TBMN), both of which invade the type IV collagen subunits, is very rare. Here, we present the case of a 20-year-old male patient diagnosed with both anti-GBM disease and...

Descripción completa

Detalles Bibliográficos
Autores principales: Jung, Chi Young, Lee, Sun-Jae, Kim, Min-Kyung, Ahn, Dong Jik, Lee, In Hee
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Lippincott Williams & Wilkins 2021
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8137055/
https://www.ncbi.nlm.nih.gov/pubmed/34011133
http://dx.doi.org/10.1097/MD.0000000000026095
_version_ 1783695549789110272
author Jung, Chi Young
Lee, Sun-Jae
Kim, Min-Kyung
Ahn, Dong Jik
Lee, In Hee
author_facet Jung, Chi Young
Lee, Sun-Jae
Kim, Min-Kyung
Ahn, Dong Jik
Lee, In Hee
author_sort Jung, Chi Young
collection PubMed
description RATIONALE: Simultaneous occurrence of anti-glomerular basement membrane (anti-GBM) disease and thin basement membrane nephropathy (TBMN), both of which invade the type IV collagen subunits, is very rare. Here, we present the case of a 20-year-old male patient diagnosed with both anti-GBM disease and TBMN upon presenting dyspnea and hemoptysis. PATIENT CONCERNS: No laboratory abnormalities, except arterial hypoxemia (PaO(2)75.4 mmHg) and microscopic hematuria, were present. Chest computed tomography revealed bilateral infiltrations in the lower lung fields; thus, administration of empirical antibiotics was initiated. Gross hemoptysis persisted nonetheless, and bronchoscopy revealed diffuse pulmonary hemorrhage with no endobronchial lesions. Broncho-alveolar lavage excluded bacterial pneumonia, tuberculosis, and fungal infection. DIAGNOSIS: Enzyme-linked immunosorbent assay of his serum was positive for anti-GBM antibody (95.1 U/mL). Human leukocyte antigen (HLA) test was positive for both HLA-DR15/-DR04. Other than diffuse thinning of the GBM (average thickness, 220 nm), index kidney biopsy did not demonstrate any specific abnormalities such as crescent formation. INTERVENTIONS: Methylprednisolone was administered intravenously for 7 consecutive days (500 mg/day), followed by the daily dose of oral prednisolone (80 mg). Cyclophosphamide was also orally administered every day for 3 months (250 mg/day). Following 6 sessions of plasmapheresis, the anti-GBM antibody in serum became negative. OUTCOMES: There was no clinical evidence suggesting recurrence of pulmonary hemorrhage or azotemia during hospitalization and 12-month follow-up period. Twelve months after hospital discharge, oral prednisolone was discontinued. LESSONS: The patients with concurrent anti-GBM disease and TBMN will have a favorable prognosis after proper therapy. However, further research is needed to elucidate the pathogenesis and long-term outcome of the comorbidity of these 2 diseases.
format Online
Article
Text
id pubmed-8137055
institution National Center for Biotechnology Information
language English
publishDate 2021
publisher Lippincott Williams & Wilkins
record_format MEDLINE/PubMed
spelling pubmed-81370552021-05-25 Anti-glomerular basement membrane disease associated with thin basement membrane nephropathy: A case report Jung, Chi Young Lee, Sun-Jae Kim, Min-Kyung Ahn, Dong Jik Lee, In Hee Medicine (Baltimore) 6700 RATIONALE: Simultaneous occurrence of anti-glomerular basement membrane (anti-GBM) disease and thin basement membrane nephropathy (TBMN), both of which invade the type IV collagen subunits, is very rare. Here, we present the case of a 20-year-old male patient diagnosed with both anti-GBM disease and TBMN upon presenting dyspnea and hemoptysis. PATIENT CONCERNS: No laboratory abnormalities, except arterial hypoxemia (PaO(2)75.4 mmHg) and microscopic hematuria, were present. Chest computed tomography revealed bilateral infiltrations in the lower lung fields; thus, administration of empirical antibiotics was initiated. Gross hemoptysis persisted nonetheless, and bronchoscopy revealed diffuse pulmonary hemorrhage with no endobronchial lesions. Broncho-alveolar lavage excluded bacterial pneumonia, tuberculosis, and fungal infection. DIAGNOSIS: Enzyme-linked immunosorbent assay of his serum was positive for anti-GBM antibody (95.1 U/mL). Human leukocyte antigen (HLA) test was positive for both HLA-DR15/-DR04. Other than diffuse thinning of the GBM (average thickness, 220 nm), index kidney biopsy did not demonstrate any specific abnormalities such as crescent formation. INTERVENTIONS: Methylprednisolone was administered intravenously for 7 consecutive days (500 mg/day), followed by the daily dose of oral prednisolone (80 mg). Cyclophosphamide was also orally administered every day for 3 months (250 mg/day). Following 6 sessions of plasmapheresis, the anti-GBM antibody in serum became negative. OUTCOMES: There was no clinical evidence suggesting recurrence of pulmonary hemorrhage or azotemia during hospitalization and 12-month follow-up period. Twelve months after hospital discharge, oral prednisolone was discontinued. LESSONS: The patients with concurrent anti-GBM disease and TBMN will have a favorable prognosis after proper therapy. However, further research is needed to elucidate the pathogenesis and long-term outcome of the comorbidity of these 2 diseases. Lippincott Williams & Wilkins 2021-05-21 /pmc/articles/PMC8137055/ /pubmed/34011133 http://dx.doi.org/10.1097/MD.0000000000026095 Text en Copyright © 2021 the Author(s). Published by Wolters Kluwer Health, Inc. https://creativecommons.org/licenses/by/4.0/This is an open access article distributed under the Creative Commons Attribution License 4.0 (CCBY), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. http://creativecommons.org/licenses/by/4.0 (https://creativecommons.org/licenses/by/4.0/)
spellingShingle 6700
Jung, Chi Young
Lee, Sun-Jae
Kim, Min-Kyung
Ahn, Dong Jik
Lee, In Hee
Anti-glomerular basement membrane disease associated with thin basement membrane nephropathy: A case report
title Anti-glomerular basement membrane disease associated with thin basement membrane nephropathy: A case report
title_full Anti-glomerular basement membrane disease associated with thin basement membrane nephropathy: A case report
title_fullStr Anti-glomerular basement membrane disease associated with thin basement membrane nephropathy: A case report
title_full_unstemmed Anti-glomerular basement membrane disease associated with thin basement membrane nephropathy: A case report
title_short Anti-glomerular basement membrane disease associated with thin basement membrane nephropathy: A case report
title_sort anti-glomerular basement membrane disease associated with thin basement membrane nephropathy: a case report
topic 6700
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8137055/
https://www.ncbi.nlm.nih.gov/pubmed/34011133
http://dx.doi.org/10.1097/MD.0000000000026095
work_keys_str_mv AT jungchiyoung antiglomerularbasementmembranediseaseassociatedwiththinbasementmembranenephropathyacasereport
AT leesunjae antiglomerularbasementmembranediseaseassociatedwiththinbasementmembranenephropathyacasereport
AT kimminkyung antiglomerularbasementmembranediseaseassociatedwiththinbasementmembranenephropathyacasereport
AT ahndongjik antiglomerularbasementmembranediseaseassociatedwiththinbasementmembranenephropathyacasereport
AT leeinhee antiglomerularbasementmembranediseaseassociatedwiththinbasementmembranenephropathyacasereport