Clinicopathologic Features of IgG4-Related Kidney Disease
Introduction: IgG4-related disease (IgG4-RD) is a systemic immune-mediated disease that can involve nearly any organ. IgG4-RD can affect the kidney in different disease patterns, collectively referred to as IgG4-related kidney disease (IgG4-RKD). Methods: We conducted a tissue-based cohort study wit...
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| Language: | English |
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Elsevier
2024-08-01
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| Series: | Kidney International Reports |
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| Online Access: | http://www.sciencedirect.com/science/article/pii/S2468024924017194 |
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| author | Alessia Buglioni Sarah M. Jenkins Samih H. Nasr Pingchuan Zhang Ian W. Gibson Mariam P. Alexander Loren P. Herrera Hernandez Mary E. Fidler Naoki Takahashi Marie C. Hogan Lynn D. Cornell |
| author_facet | Alessia Buglioni Sarah M. Jenkins Samih H. Nasr Pingchuan Zhang Ian W. Gibson Mariam P. Alexander Loren P. Herrera Hernandez Mary E. Fidler Naoki Takahashi Marie C. Hogan Lynn D. Cornell |
| author_sort | Alessia Buglioni |
| collection | DOAJ |
| description | Introduction: IgG4-related disease (IgG4-RD) is a systemic immune-mediated disease that can involve nearly any organ. IgG4-RD can affect the kidney in different disease patterns, collectively referred to as IgG4-related kidney disease (IgG4-RKD). Methods: We conducted a tissue-based cohort study with clinicopathological correlation in 125 patients with IgG4-RKD. Results: The mean age at biopsy (n = 120) or nephrectomy (n = 5) was 63 years; 80% were male. One hundred eighteen patients (94%) had IgG4-related tubulointerstitial nephritis (IgG4-TIN); 20 patients (16%) had IgG4-related membranous glomerulonephritis (IgG4-MGN; 13 with concurrent IgG4-TIN). The primary clinical indication for biopsy/nephrectomy was acute or chronic renal failure in 78%, proteinuria in 17%, and mass lesion(s) in 15% (with overlap in primary indication). Fifty-two percent patients (41/79) had abnormal radiographic findings, including masses in 30% (24/79). All patients with IgG4-MGN had proteinuria. Extrarenal involvement by IgG4-RD was present in 79%. Median serum creatinine at presentation was 2.5 mg/dl (range 0.7–12). Serum IgG and/or IgG4 was increased in 91% (53/58); hypocomplementemia was present in 56% (43/77). Light microscopy showed plasma cell–rich interstitial nephritis in all cases of IgG4-TIN. Ninety-two percent of patients showed increased IgG4+ plasma cells. Seven percent showed an acute interstitial nephritis (AIN) pattern, and 5% showed non-necrotizing arteritis. Tubular basement membrane immune deposits were present in 83% of IgG4-TIN. Treatment information was available for 71 patients; 62 were treated with immunosuppression. Of those with elevated creatinine, 72% (41/57) showed a treatment response. Conclusion: This largest tissue-based series more clearly defines the disease phenotype of IgG4-RKD. |
| format | Article |
| id | doaj-art-6a0a15a7144746febab6016aa40eef33 |
| institution | OA Journals |
| issn | 2468-0249 |
| language | English |
| publishDate | 2024-08-01 |
| publisher | Elsevier |
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| series | Kidney International Reports |
| spelling | doaj-art-6a0a15a7144746febab6016aa40eef332025-08-20T02:05:57ZengElsevierKidney International Reports2468-02492024-08-01982462247310.1016/j.ekir.2024.05.011Clinicopathologic Features of IgG4-Related Kidney DiseaseAlessia Buglioni0Sarah M. Jenkins1Samih H. Nasr2Pingchuan Zhang3Ian W. Gibson4Mariam P. Alexander5Loren P. Herrera Hernandez6Mary E. Fidler7Naoki Takahashi8Marie C. Hogan9Lynn D. Cornell10Division of Anatomic Pathology, Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, Minnesota, USADivision of Clinical Trials and Biostatistics, Mayo Clinic, Rochester, Minnesota, USADivision of Anatomic Pathology, Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, Minnesota, USADivision of Anatomic Pathology, Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, Minnesota, USADepartment of Pathology, University of Manitoba College of Medicine, Winnipeg, Manitoba, CanadaDivision of Anatomic Pathology, Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, Minnesota, USADivision of Anatomic Pathology, Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, Minnesota, USADivision of Anatomic Pathology, Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, Minnesota, USADivision of Abdominal Imaging, Department of Radiology, Mayo Clinic, Rochester, Minnesota, USADivision of Nephrology and Hypertension, Department of Medicine, Mayo Clinic, Minnesota, USADivision of Anatomic Pathology, Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, Minnesota, USA; Correspondence: Lynn D. Cornell, Department of Laboratory Medicine and Pathology, Mayo Clinic, 200 First Street SW, Rochester, Minnesota 55905, USA.Introduction: IgG4-related disease (IgG4-RD) is a systemic immune-mediated disease that can involve nearly any organ. IgG4-RD can affect the kidney in different disease patterns, collectively referred to as IgG4-related kidney disease (IgG4-RKD). Methods: We conducted a tissue-based cohort study with clinicopathological correlation in 125 patients with IgG4-RKD. Results: The mean age at biopsy (n = 120) or nephrectomy (n = 5) was 63 years; 80% were male. One hundred eighteen patients (94%) had IgG4-related tubulointerstitial nephritis (IgG4-TIN); 20 patients (16%) had IgG4-related membranous glomerulonephritis (IgG4-MGN; 13 with concurrent IgG4-TIN). The primary clinical indication for biopsy/nephrectomy was acute or chronic renal failure in 78%, proteinuria in 17%, and mass lesion(s) in 15% (with overlap in primary indication). Fifty-two percent patients (41/79) had abnormal radiographic findings, including masses in 30% (24/79). All patients with IgG4-MGN had proteinuria. Extrarenal involvement by IgG4-RD was present in 79%. Median serum creatinine at presentation was 2.5 mg/dl (range 0.7–12). Serum IgG and/or IgG4 was increased in 91% (53/58); hypocomplementemia was present in 56% (43/77). Light microscopy showed plasma cell–rich interstitial nephritis in all cases of IgG4-TIN. Ninety-two percent of patients showed increased IgG4+ plasma cells. Seven percent showed an acute interstitial nephritis (AIN) pattern, and 5% showed non-necrotizing arteritis. Tubular basement membrane immune deposits were present in 83% of IgG4-TIN. Treatment information was available for 71 patients; 62 were treated with immunosuppression. Of those with elevated creatinine, 72% (41/57) showed a treatment response. Conclusion: This largest tissue-based series more clearly defines the disease phenotype of IgG4-RKD.http://www.sciencedirect.com/science/article/pii/S2468024924017194autoimmuneIgG4-related diseaseinterstitial nephritismembranous nephropathytubulointerstitial nephritis |
| spellingShingle | Alessia Buglioni Sarah M. Jenkins Samih H. Nasr Pingchuan Zhang Ian W. Gibson Mariam P. Alexander Loren P. Herrera Hernandez Mary E. Fidler Naoki Takahashi Marie C. Hogan Lynn D. Cornell Clinicopathologic Features of IgG4-Related Kidney Disease Kidney International Reports autoimmune IgG4-related disease interstitial nephritis membranous nephropathy tubulointerstitial nephritis |
| title | Clinicopathologic Features of IgG4-Related Kidney Disease |
| title_full | Clinicopathologic Features of IgG4-Related Kidney Disease |
| title_fullStr | Clinicopathologic Features of IgG4-Related Kidney Disease |
| title_full_unstemmed | Clinicopathologic Features of IgG4-Related Kidney Disease |
| title_short | Clinicopathologic Features of IgG4-Related Kidney Disease |
| title_sort | clinicopathologic features of igg4 related kidney disease |
| topic | autoimmune IgG4-related disease interstitial nephritis membranous nephropathy tubulointerstitial nephritis |
| url | http://www.sciencedirect.com/science/article/pii/S2468024924017194 |
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