Renal Involvement in IgG4-Related Disease: From Sunlight to Twilight

Capecchi, Riccardo; Giannese, Domenico; Moriconi, Diego; Bonadio, Angelo Giovanni; Pratesi, Federico; Croia, Cristina; Egidi, Maria Francesca; Puxeddu, Ilaria; Tavoni, Antonio; Migliorini, Paola

doi:10.3389/fmed.2021.635706

Cited by 17 publications

(10 citation statements)

References 63 publications

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“… 1 The location of the urinary tract obstructions and the lesions causing it vary widely. 12 In addition, renal insufficiency can be caused by renal parenchymal lesions referred to as IgG4-related kidney disease (IgG4-RKD) 13 , 14 especially in IgG4-related RPF. Such renal involvement in RPF can lead to not only acute kidney injury (AKI) but also chronic kidney disease (CKD) including renal atrophy or even end-stage renal disease (ESRD).…”

Section: Introductionmentioning

confidence: 99%

Renal Involvement in Retroperitoneal Fibrosis: Prevalence, Impact and Management Challenges

Mizushima¹,

Kawano²

2021

IJNRD

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Retroperitoneal fibrosis (RPF) is a rare disorder consisting of idiopathic and various secondary forms and characterized by chronic inflammatory infiltrates and marked fibrosis in the retroperitoneal space. In idiopathic RPF (IRPF), 35–60% of cases have been reported to be IgG4-related RPF, the retroperitoneal lesions of IgG4-related disease (IgG4-RD). IRPF can frequently lead to renal insufficiency mediated by urinary tract obstruction and hydronephrosis irrespective of being IgG4-related or not. Clinical pictures, laboratory and imaging findings, and location of the urinary tract obstruction are generally similar in IgG4-related and non-IgG4-related IRPF although multiple organ involvement and serum IgG4 elevation may be characteristic of the IgG4-related forms. Periaortic/periarterial lesions are the most frequent cause of renal insufficiency. Although the response to glucocorticoids is generally good, relapse does occur in a considerable proportion of patients, and may require an additional immunosuppressive agent and/or urological intervention in cases with multiple relapses or refractory obstructive uropathy. In general, the prognosis of patients with IRPF is good, but careful attention needs to be paid to chronic kidney disease as a major complication and rupture of the affected aorta/artery as a life-threatening one. Further studies are necessary to better understand the pathogenesis of the disease and to establish the optimal diagnostic and therapeutic strategies for it.

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Section: Introductionmentioning

confidence: 99%

Renal Involvement in Retroperitoneal Fibrosis: Prevalence, Impact and Management Challenges

Mizushima¹,

Kawano²

2021

IJNRD

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“…Nevertheless, treatment regimens that combine glucocorticoids with cyclophosphamide or RTX have shown favorable outcomes. [17,18,43] In the present case, glucocorticoid treatment was initially effective for IgG4-RD when the patient presented with clinical symptoms. After two years of follow-up, the patient exhibited nephrotic syndrome and was diagnosed with PLA2R-positive pMN, characterized by positive PLA2R antigen expression and IgG1-dominant deposition in the glomerulus.…”

Section: Discussionmentioning

confidence: 75%

IgG4-Related Disease with IgG1-Dominant Membranous Nephropathy: A Rare Case Report

Duan,

Qu,

Zhuang

et al. 2023

Integr Med Nephrol Androl

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Immunoglobulin G4-related disease (IgG4-RD) is an immune-mediated fibroinflammatory disorder affecting various organs and tissues. Kidney involvement in IgG4-RD commonly manifests as IgG4-related tubulointerstitial nephritis or IgG4-related membranous nephropathy, with the latter occurring less frequently. The identification of the phospholipase A2 receptor (PLA2R) antigen has improved our understanding of primary membranous nephropathy. Here, we present the case of a 60-year-old Chinese man initially diagnosed with IgG4-related pancreatitis, who achieved clinical remission with glucocorticoid therapy followed by low-dose glucocorticoid maintenance therapy. Two years later, the patient developed nephrotic syndrome. A kidney biopsy revealed membranous nephropathy characterized by glomerular subepithelial deposition of IgG1 and positive PLA2R staining. Rituximab (RTX) treatment resulted in complete clinical remission of nephrotic syndrome, and no recurrence was observed during the 24-month follow-up period. IgG4-RD complicated by membranous nephropathy is a rare pathological feature characterized by glomerular subepithelial IgG1 deposition and positive PLA2R antigen staining. In the present case, RTX treatment was effective in achieving clinical remission. These findings contribute to the evolving understanding of the relationship between IgG4-RD and membranous nephropathy.

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“…No fue posible efectuar la inmunohistoquímica por la escasez del material. La paciente realizó tratamiento con altas dosis de corticoides y posteriormente con rituximab, con evidente mejoría clínica en controles posteriores 1,[7][8] .…”

Section: Discussionunclassified

Pacientes con compromiso orbitario y renal. Diagnósticos diferenciales poco frecuentes

Lemos¹,

Sager²,

Ortíz³

et al. 2021

Rev. Argent. Reumatol.

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Se comunica una serie de tres casos clínicos que consultaron al servicio de Reumatología por compromiso orbitario y renal. Uno de ellos presentó pseudotumor orbitario con proteinuria en rango nefrótico; se realizó biopsia y se encontró infiltrado linfoplasmocitario denso y fibrosis estoriforme con inmunohistoquímica: 15 células IgG4+ por campo de alto poder y relación IgG/ IgG4 ≤40%, concluyendo diagnóstico de enfermedad relacionada por IgG4. El segundo y tercer caso presentaron compromiso ocular con "ojos de mapache" y lesiones amarillentas en párpados, ambos con proteinuria >500 mg/24 h, con biopsia de piel rojo Congo positiva y birrefringencia verde manzana con luz polarizada. Se discuten distintos diagnósticos diferenciales poco frecuentes a tener en cuenta en estos pacientes.

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Renal Involvement in IgG4-Related Disease: From Sunlight to Twilight

Cited by 17 publications

References 63 publications

Renal Involvement in Retroperitoneal Fibrosis: Prevalence, Impact and Management Challenges

Renal Involvement in Retroperitoneal Fibrosis: Prevalence, Impact and Management Challenges

IgG4-Related Disease with IgG1-Dominant Membranous Nephropathy: A Rare Case Report

Pacientes con compromiso orbitario y renal. Diagnósticos diferenciales poco frecuentes

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