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IgG4-Related Disease Manifesting as Interstitial Nephritis Accompanied by Hypophysitis
Patient: Male, 85 Final Diagnosis: IgG4-related disease Symptoms: Renal failure Medication: — Clinical Procedure: — Specialty: Nephrology OBJECTIVE: Rare disease BACKGROUND: IgG4-related disease is a systemic disease with marked infiltration of IgG4-positive plasma cells into affected organs and ele...
Autores principales: | , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
International Scientific Literature, Inc.
2017
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5459220/ https://www.ncbi.nlm.nih.gov/pubmed/28552902 http://dx.doi.org/10.12659/AJCR.902187 |
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author | Matsuda, Ken Saito, Ayako Takeuchi, Yoichi Fukami, Hirotaka Sato, Hiroyuki Nagasawa, Tasuku |
author_facet | Matsuda, Ken Saito, Ayako Takeuchi, Yoichi Fukami, Hirotaka Sato, Hiroyuki Nagasawa, Tasuku |
author_sort | Matsuda, Ken |
collection | PubMed |
description | Patient: Male, 85 Final Diagnosis: IgG4-related disease Symptoms: Renal failure Medication: — Clinical Procedure: — Specialty: Nephrology OBJECTIVE: Rare disease BACKGROUND: IgG4-related disease is a systemic disease with marked infiltration of IgG4-positive plasma cells into affected organs and elevated serum IgG4. On clinical examination, swelling, nodules, and hypertrophic lesions might appear simultaneously or metachronously in different organs. CASE REPORT: An 85-year-old man with sudden-onset polydipsia and polyuria insipidus was transported to our hospital because of hypothermia and general malaise. Laboratory tests revealed renal failure and central diabetes insipidus. According to his serum IgG4 level, the patient was diagnosed with possible IgG4-related kidney disease accompanied by IgG4-related hypophysitis. Abdominal contrast-enhanced computed tomography, hypophysis magnetic resonance imaging, and histological examination of the kidney were performed. Glucocorticoid therapy was administered and his renal function improved gradually. However, his central diabetes insipidus did not improve. CONCLUSIONS: Glucocorticoid therapy showed different therapeutic effects on the kidney and posterior lobe of the hypophysis. It is possible that glucocorticoid therapy needs to be supported by other immunomodulatory therapies to have an effect on all affected organs. |
format | Online Article Text |
id | pubmed-5459220 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2017 |
publisher | International Scientific Literature, Inc. |
record_format | MEDLINE/PubMed |
spelling | pubmed-54592202017-06-13 IgG4-Related Disease Manifesting as Interstitial Nephritis Accompanied by Hypophysitis Matsuda, Ken Saito, Ayako Takeuchi, Yoichi Fukami, Hirotaka Sato, Hiroyuki Nagasawa, Tasuku Am J Case Rep Articles Patient: Male, 85 Final Diagnosis: IgG4-related disease Symptoms: Renal failure Medication: — Clinical Procedure: — Specialty: Nephrology OBJECTIVE: Rare disease BACKGROUND: IgG4-related disease is a systemic disease with marked infiltration of IgG4-positive plasma cells into affected organs and elevated serum IgG4. On clinical examination, swelling, nodules, and hypertrophic lesions might appear simultaneously or metachronously in different organs. CASE REPORT: An 85-year-old man with sudden-onset polydipsia and polyuria insipidus was transported to our hospital because of hypothermia and general malaise. Laboratory tests revealed renal failure and central diabetes insipidus. According to his serum IgG4 level, the patient was diagnosed with possible IgG4-related kidney disease accompanied by IgG4-related hypophysitis. Abdominal contrast-enhanced computed tomography, hypophysis magnetic resonance imaging, and histological examination of the kidney were performed. Glucocorticoid therapy was administered and his renal function improved gradually. However, his central diabetes insipidus did not improve. CONCLUSIONS: Glucocorticoid therapy showed different therapeutic effects on the kidney and posterior lobe of the hypophysis. It is possible that glucocorticoid therapy needs to be supported by other immunomodulatory therapies to have an effect on all affected organs. International Scientific Literature, Inc. 2017-05-29 /pmc/articles/PMC5459220/ /pubmed/28552902 http://dx.doi.org/10.12659/AJCR.902187 Text en © Am J Case Rep, 2017 This work is licensed under Creative Common Attribution-NonCommercial-NoDerivatives 4.0 International (CC BY-NC-ND 4.0 (https://creativecommons.org/licenses/by-nc-nd/4.0/) ) |
spellingShingle | Articles Matsuda, Ken Saito, Ayako Takeuchi, Yoichi Fukami, Hirotaka Sato, Hiroyuki Nagasawa, Tasuku IgG4-Related Disease Manifesting as Interstitial Nephritis Accompanied by Hypophysitis |
title | IgG4-Related Disease Manifesting as Interstitial Nephritis Accompanied by Hypophysitis |
title_full | IgG4-Related Disease Manifesting as Interstitial Nephritis Accompanied by Hypophysitis |
title_fullStr | IgG4-Related Disease Manifesting as Interstitial Nephritis Accompanied by Hypophysitis |
title_full_unstemmed | IgG4-Related Disease Manifesting as Interstitial Nephritis Accompanied by Hypophysitis |
title_short | IgG4-Related Disease Manifesting as Interstitial Nephritis Accompanied by Hypophysitis |
title_sort | igg4-related disease manifesting as interstitial nephritis accompanied by hypophysitis |
topic | Articles |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5459220/ https://www.ncbi.nlm.nih.gov/pubmed/28552902 http://dx.doi.org/10.12659/AJCR.902187 |
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