Clinical manifestations and long-term outcomes of IgG4-related kidney and retroperitoneal involvement in a United Kingdom IgG4-related disease cohort
<strong>Introduction</strong> 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 d...
| Main Authors: | , , , , , , , , , , |
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| Format: | Journal article |
| Language: | English |
| Published: |
Elsevier
2018
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| _version_ | 1797078572650725376 |
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| author | Evans, R Cargill, T Goodchild, G Oliveira, B Rodriguez-Justo, M Pepper, R Connolly, J Salama, A Webster, G Barnes, E Culver, E |
| author_facet | Evans, R Cargill, T Goodchild, G Oliveira, B Rodriguez-Justo, M Pepper, R Connolly, J Salama, A Webster, G Barnes, E Culver, E |
| author_sort | Evans, R |
| collection | OXFORD |
| description | <strong>Introduction</strong> 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. <strong>Methods</strong> 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. <strong>Results</strong> 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; P = 0.0076). Serum IgG4 was elevated in the majority of patients (87.5%), and hypocomplementemia was present in half of those with IgG4-RKD. Fifteen patients underwent renal biopsy; tubulointerstitial nephritis with abundant IgG4+ plasma cells was the most common finding (n = 14; 93.3%), and 4 (26.7%) patients had membranous nephropathy. Most patients (89.3%) were treated with corticosteroids, and 4 (16.0%) with additional azathioprine as initial management. Thirteen patients (46.4%) relapsed over 60 ± 48 months of follow-up, at median 18 (12–36) months after renal/RPF diagnosis; 61.5% of relapses were in the kidney. Renal function deteriorated in 5 patients (20.8%), including 2 (8.3%) who reached end-stage renal disease (ESRD). <strong>Conclusion</strong> 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. |
| first_indexed | 2024-03-07T00:33:50Z |
| format | Journal article |
| id | oxford-uuid:80b6bfff-14ed-46eb-99d6-59c75c52bf63 |
| institution | University of Oxford |
| language | English |
| last_indexed | 2024-03-07T00:33:50Z |
| publishDate | 2018 |
| publisher | Elsevier |
| record_format | dspace |
| spelling | oxford-uuid:80b6bfff-14ed-46eb-99d6-59c75c52bf632022-03-26T21:25:20ZClinical manifestations and long-term outcomes of IgG4-related kidney and retroperitoneal involvement in a United Kingdom IgG4-related disease cohortJournal articlehttp://purl.org/coar/resource_type/c_dcae04bcuuid:80b6bfff-14ed-46eb-99d6-59c75c52bf63EnglishSymplectic Elements at OxfordElsevier2018Evans, RCargill, TGoodchild, GOliveira, BRodriguez-Justo, MPepper, RConnolly, JSalama, AWebster, GBarnes, ECulver, E<strong>Introduction</strong> 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. <strong>Methods</strong> 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. <strong>Results</strong> 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; P = 0.0076). Serum IgG4 was elevated in the majority of patients (87.5%), and hypocomplementemia was present in half of those with IgG4-RKD. Fifteen patients underwent renal biopsy; tubulointerstitial nephritis with abundant IgG4+ plasma cells was the most common finding (n = 14; 93.3%), and 4 (26.7%) patients had membranous nephropathy. Most patients (89.3%) were treated with corticosteroids, and 4 (16.0%) with additional azathioprine as initial management. Thirteen patients (46.4%) relapsed over 60 ± 48 months of follow-up, at median 18 (12–36) months after renal/RPF diagnosis; 61.5% of relapses were in the kidney. Renal function deteriorated in 5 patients (20.8%), including 2 (8.3%) who reached end-stage renal disease (ESRD). <strong>Conclusion</strong> 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. |
| spellingShingle | Evans, R Cargill, T Goodchild, G Oliveira, B Rodriguez-Justo, M Pepper, R Connolly, J Salama, A Webster, G Barnes, E Culver, E Clinical manifestations and long-term outcomes of IgG4-related kidney and retroperitoneal involvement in a United Kingdom IgG4-related disease cohort |
| title | Clinical manifestations and long-term outcomes of IgG4-related kidney and retroperitoneal involvement in a United Kingdom IgG4-related disease cohort |
| title_full | Clinical manifestations and long-term outcomes of IgG4-related kidney and retroperitoneal involvement in a United Kingdom IgG4-related disease cohort |
| title_fullStr | Clinical manifestations and long-term outcomes of IgG4-related kidney and retroperitoneal involvement in a United Kingdom IgG4-related disease cohort |
| title_full_unstemmed | Clinical manifestations and long-term outcomes of IgG4-related kidney and retroperitoneal involvement in a United Kingdom IgG4-related disease cohort |
| title_short | Clinical manifestations and long-term outcomes of IgG4-related kidney and retroperitoneal involvement in a United Kingdom IgG4-related disease cohort |
| title_sort | clinical manifestations and long term outcomes of igg4 related kidney and retroperitoneal involvement in a united kingdom igg4 related disease cohort |
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