Primary Sjӧgren's syndrome with renal Fanconi syndrome: Good responses to treatment with glucocorticoids.

Fanconi Syndrome Female Glucocorticoids / therapeutic use Humans Nephritis, Interstitial / drug therapy Retrospective Studies Sjogren's Syndrome
Fanconi syndrome Primary Sjӧgren's syndrome Treatment Young-onset

Journal

Seminars in arthritis and rheumatism
ISSN: 1532-866X
Titre abrégé: Semin Arthritis Rheum
Pays: United States
ID NLM: 1306053

Informations de publication

Date de publication:
12 2020
Historique:
received: 03 02 2020
revised: 19 03 2020
accepted: 23 03 2020
pubmed: 19 5 2020
medline: 30 9 2021
entrez: 19 5 2020
Statut: ppublish

Résumé

Renal Fanconi syndrome (FS) is rare in primary Sjӧgren's syndrome (pSS). We aimed to describe the clinicopathological characteristics of pSS associated FS (pSS-FS) and its responses to treatment. We reported 25 cases of pSS-FS patients and retrospectively reviewed their clinical records, kidney pathology and follow-up data. The 25 pSS-FS patients were mainly female (92.0%) and the mean age at diagnosis was 43.6±11.3 years. They showed different degrees of proximal tubular dysfunctions and eGFR decline (60.9±32.3 ml/min/1.73m In pSS-FS patients, use of glucocorticoids could improve eGFR and tubular functions. The young-onset pSS group presented with a particular pattern in immunological features and kidney involvement.

Sections du résumé

BACKGROUND
Renal Fanconi syndrome (FS) is rare in primary Sjӧgren's syndrome (pSS). We aimed to describe the clinicopathological characteristics of pSS associated FS (pSS-FS) and its responses to treatment.
METHODS
We reported 25 cases of pSS-FS patients and retrospectively reviewed their clinical records, kidney pathology and follow-up data.
RESULTS
The 25 pSS-FS patients were mainly female (92.0%) and the mean age at diagnosis was 43.6±11.3 years. They showed different degrees of proximal tubular dysfunctions and eGFR decline (60.9±32.3 ml/min/1.73m
CONCLUSIONS
In pSS-FS patients, use of glucocorticoids could improve eGFR and tubular functions. The young-onset pSS group presented with a particular pattern in immunological features and kidney involvement.

Identifiants

DOI: 10.1016/j.semarthrit.2020.03.017 PMID: 32418614
pubmed: 32418614
pii: S0049-0172(20)30093-7
doi: 10.1016/j.semarthrit.2020.03.017
pii:
doi:

Substances chimiques

Glucocorticoids 0

Types de publication

Journal Article Research Support, Non-U.S. Gov't

Langues

eng

Sous-ensembles de citation

IM

Pagination

1326-1332

Informations de copyright

Copyright © 2020 Elsevier Inc. All rights reserved.

Auteurs

Xiaoxiao Shi (X)

Department of Nephrology, Peking Union Medical College Hospital, Chinese Academy of Medical Science, No 1, Shuaifuyan, Wangfujing St, Beijing 100730, China.

Zhixin Chen (Z)

Department of Nephrology, Peking Union Medical College Hospital, Chinese Academy of Medical Science, No 1, Shuaifuyan, Wangfujing St, Beijing 100730, China.

Jing Wang (J)

Department of Pathology, Peking Union Medical College Hospital, Chinese Academy of Medical Science, Beijing, China.

Yubing Wen (Y)

Department of Nephrology, Peking Union Medical College Hospital, Chinese Academy of Medical Science, No 1, Shuaifuyan, Wangfujing St, Beijing 100730, China.

Linfeng Zou (L)

Department of Nephrology, Peking Union Medical College Hospital, Chinese Academy of Medical Science, No 1, Shuaifuyan, Wangfujing St, Beijing 100730, China.

Yunyun Fei (Y)

Department of Rheumatology and Clinical Immunology, Peking Union Medical College Hospital, Chinese Academy of Medical Science, Beijing, China.

Wei Ye (W)

Department of Nephrology, Peking Union Medical College Hospital, Chinese Academy of Medical Science, No 1, Shuaifuyan, Wangfujing St, Beijing 100730, China.

Yan Qin (Y)

Department of Nephrology, Peking Union Medical College Hospital, Chinese Academy of Medical Science, No 1, Shuaifuyan, Wangfujing St, Beijing 100730, China.

Hang Li (H)

Department of Nephrology, Peking Union Medical College Hospital, Chinese Academy of Medical Science, No 1, Shuaifuyan, Wangfujing St, Beijing 100730, China.

Mingxi Li (M)

Department of Nephrology, Peking Union Medical College Hospital, Chinese Academy of Medical Science, No 1, Shuaifuyan, Wangfujing St, Beijing 100730, China.

Xuemei Li (X)

Department of Nephrology, Peking Union Medical College Hospital, Chinese Academy of Medical Science, No 1, Shuaifuyan, Wangfujing St, Beijing 100730, China.

Fengchun Zhang (F)

Department of Rheumatology and Clinical Immunology, Peking Union Medical College Hospital, Chinese Academy of Medical Science, Beijing, China.

Xuewang Li (X)

Department of Nephrology, Peking Union Medical College Hospital, Chinese Academy of Medical Science, No 1, Shuaifuyan, Wangfujing St, Beijing 100730, China.

Limeng Chen (L)

Department of Nephrology, Peking Union Medical College Hospital, Chinese Academy of Medical Science, No 1, Shuaifuyan, Wangfujing St, Beijing 100730, China. Electronic address: chenlimeng@pumch.cn.

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Classifications MeSH

questionsmedicales.fr › Malformations et maladies congénitales, héréditaires et néonatales › Maladies génétiques congénitales › Syndrome de Fanconi › Primary Sjӧgren's syndrome with renal Fanconi...
questionsmedicales.fr › Actions chimiques et utilisations › Actions pharmacologiques › Effets physiologiques des médicaments › Hormones, substituts d'hormones et antagonistes d'hormones › Hormones › Glucocorticoïdes › Primary Sjӧgren's syndrome with renal Fanconi...
questionsmedicales.fr › Eucaryotes › Animaux › Chordés › Vertébrés › Mammifères › Eutheria › Primates › Haplorhini › Catarrhini › Hominidae › Humains › Primary Sjӧgren's syndrome with renal Fanconi...
questionsmedicales.fr › Maladies urogénitales › Maladies urologiques › Maladies du rein › Néphrite › Néphrite interstitielle › Primary Sjӧgren's syndrome with renal Fanconi...
questionsmedicales.fr › Environnement et santé publique › Santé publique › Méthodes épidémiologiques › Caractéristiques des études épidémiologiques › Études épidémiologiques › Études de cohortes › Études rétrospectives › Primary Sjӧgren's syndrome with renal Fanconi...
questionsmedicales.fr › Maladies du système immunitaire › Maladies auto-immunes › Polyarthrite rhumatoïde › Syndrome de Gougerot-Sjögren › Primary Sjӧgren's syndrome with renal Fanconi...