Clinical Manifestations and Long-term Outcomes of IgG4-Related Kidney and Retroperitoneal Involvement in a United Kingdom IgG4-Related Disease Cohort.

IgG4-related disease membranous nephropathy retroperitoneal fibrosis tubulointerstitial nephritis

Journal

Kidney international reports
ISSN: 2468-0249
Titre abrégé: Kidney Int Rep
Pays: United States
ID NLM: 101684752

Informations de publication

Date de publication:
Jan 2019
Historique:
received: 29 06 2018
revised: 15 08 2018
accepted: 27 08 2018
entrez: 1 1 2019
pubmed: 1 1 2019
medline: 1 1 2019
Statut: epublish

Résumé

IgG4-related disease (IgG4-RD) is a relapsing multisystem fibro-inflammatory disease, which may involve the kidney (IgG4-related kidney disease [IgG4-RKD]) and retroperitoneum (IgG4-related retroperitoneal fibrosis [IgG4-RPF]). The aim of this study was to describe IgG4-RKD and IgG4-RPF in the United Kingdom. We conducted a retrospective observational study of patients with IgG4-RKD and IgG4-RPF in a multicenter IgG4-RD cohort. Data were collected through review of medical records. We describe clinical parameters at baseline, histological and radiological findings, treatment, and patient outcomes. Of 154 patients with IgG4-RD, 14 (9.1%) had IgG4-RKD, 10 (6.5%) had IgG4-RPF, and 4 (2.6%) had both. Patients were aged 58.2 ± 14.2 years, and 26 (92.9%) were male. Creatinine at presentation was worse in those with intrinsic renal disease (229 μmol/l vs. 110 μmol/l; IgG4-RKD and IgG4-RPF represent major organ manifestations of IgG4-RD, and should be identified early with prompt treatment to prevent progression to ESRD.

Identifiants

pubmed: 30596168
doi: 10.1016/j.ekir.2018.08.011
pii: S2468-0249(18)30196-7
pmc: PMC6308386
doi:

Types de publication

Journal Article

Langues

eng

Pagination

48-58

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Auteurs

Rhys D R Evans (RDR)

University College London Centre for Nephrology, Royal Free Hospital, London, UK.

Tamsin Cargill (T)

Translational Gastroenterology Unit, John Radcliffe Hospital, and Nuffield Department of Medicine, University of Oxford, Oxford, UK.

George Goodchild (G)

Gastroenterology Department, University College London Hospital NHS Trust, London, UK.

Ben Oliveira (B)

University College London Centre for Nephrology, Royal Free Hospital, London, UK.

Manuel Rodriguez-Justo (M)

Histopathology Department, University College London Hospital NHS Trust, London, UK.

Ruth Pepper (R)

University College London Centre for Nephrology, Royal Free Hospital, London, UK.

John Connolly (J)

University College London Centre for Nephrology, Royal Free Hospital, London, UK.

Alan Salama (A)

University College London Centre for Nephrology, Royal Free Hospital, London, UK.

George Webster (G)

Gastroenterology Department, University College London Hospital NHS Trust, London, UK.

Eleanor Barnes (E)

Translational Gastroenterology Unit, John Radcliffe Hospital, and Nuffield Department of Medicine, University of Oxford, Oxford, UK.

Emma L Culver (EL)

Translational Gastroenterology Unit, John Radcliffe Hospital, and Nuffield Department of Medicine, University of Oxford, Oxford, UK.

Classifications MeSH