Enfermedad renal relacionada con la inmunoglobulina g4 / Inmunoglobulin g4-related kidney disease

RESUMEN La enfermedad relacionada con la inmunoglobulina G4 (IgG4) es un proceso fibroinflamatorio relacionado con la inmumomediación. En las últimas décadas, esta enfermedad ha sido reconocida como un trastorno sistémico que engloba afecciones individuales de órganos, antes no relacionadas y conocidas como entidades independientes. La afectación renal de la enfermedad relacionada con la IgG4 puede ser tanto sincrónica como metacrónica al compromiso de otro órgano como el que se da en la pancreatitis autoinmune, la colangitis esclerosante, la fibrosis retroperitoneal o la linfadenopatía relacionadas con IgG4. En esta revisión presentamos las manifestaciones más frecuentes de la afectación renal por la enfermedad relacionada con IgG4, destacando el papel que tienen las pruebas de imagen. El tratamiento tanto de la enfermedad relacionada con IgG4 en general como de la afectación renal en particular son los glucocorticoides. Es importante conocer esta enfermedad, sospecharla y realizar un diagnóstico precoz y preciso.

ABSTRACT Immunoglobulin G4-related disease (IgG4-RD) is an immunomediated fibroinflammatory process. In the last few decades, this disease has been recognized as a systemic disorder encompassing individual involvement of organs, previously unrelated and known as independent entities. Renal involvement in IgG4-RD may be both synchronous and metachronous to other organ compromise, such as that seen in autoimmune pancreatitis, sclerosing cholangitis, retroperitoneal fibrosis, or IgG4-related lymphadenopathy. In this review we present the most frequent manifestations of renal involvement due to IgG4-RD, highlighting the role of imaging tests. The treatment of both IgG4-RD in general and renal involvement in particular are glucocorticoids. It is important to know about this disease, be suspicious about it and make an early and accurate diagnosis.

Computed Tomography Imaging Features of IgG4-related Nephropathy / 中国医学科学院学报

Objective To investigate the computed tomography(CT)imaging features of IgG4-related kidney disease(IgG4-RKD).Methods The clinicopathological and imaging data of 36 IgG4-RKD patients(including 26 cases of renal parenchyma,10 cases of renal pelvis,24 cases of double kidney or multiple lesions,and 12 cases of single focus)were retrospectively analyzed.Results IgG4-RKD had specific clinicopathological and imaging features.Although the kidney or renal pelvis was involved,there were no common clinical manifestations of malignant tumors such as hydronephrosis and hematuria.The boundary was clear and complete,and it had typical characteristics of continuous progressive enhancement.The peak value was mostly in the solid and excretory phase,and there were no imaging manifestations such as liquefaction,necrosis,cystic degeneration,and calcification.Even if the renal pelvis was obviously involved and the focus was large,there was no invasion of the peripheral renal parenchyma,the shape of the renal pelvis still existed,the wall was smooth,and there was no enlarged peripheral lymph nodes.Conclusions CT images of IgG4-RKD are similar to those of renal malignant tumors.Based on clinicopathological features,CT findings,and blood IgG4-related tests,a definite diagnosis can be made and unnecessary operation can be avoided.

Perirenal capsule involvement in IgG4-related chronic interstitial nephritis: a case report and literature review / 中华肾脏病杂志

Objective@#To explore the clinicopathological features and the renal biopsy process of a case of IgG4-related chronic interstitial nephritis with perirenal capsule involved and review associated literature to improve the clinician's understanding for this disease and to perform a better renal biopsy.@*Methods@#The onset, diagnosis and treatment course of the disease were described and associated literature were reviewed to summary the clinicopathologic features and key points in renal biopsy.@*Results@#The data of the patient showed that the urine specific gravity was 1.011, with urine protein ± and urine sugar 3+. The concentration of hemoglobin was 53 g/L, serum creatinine was 1665 μmol/L, and IgG4 was 9.39 g/L. Computed tomography showed that both kidneys enlarged slightly with decreased density and low density shadow around the kidneys. On contrast-enhanced scan, irregular low-density enhancement areas were found in both kidneys, and the edge of the boundary was not clear. For the first renal biopsy, no renal parenchyma was found except mainly hyaline collagen fibrils. At the second time, 3 pieces of tissues were obtained, which showed chronic interstitial glomerulonephritis. The IgG4 positive plasma cells were about 60/HPF and the IgG4+/IgG+cells ratio was more than 40%. The diagnosis of IgG4-related chronic interstitial glomerulonephritis was confirmed. After corticosteroid treatment, the serum creatinine decreased to 502 μmol/L after the patient got rid of dialysis.@*Conclusions@#There are various manifestations of renal damage caused by IgG4-related disease. It is necessary to pay attention to the involvement of the perirenal capsule, and to balance the risk of bleeding and poor sampling in renal biopsy.

IgG4-related kidney disease / 国际儿科学杂志

IgG4-related disease is a newly recognized systematic inflammatory and fibrotic disease which affects one or more organs.This disease mainly occurs in the middle-aged and old males.Currently only a few cases were reported in children.Renal involvement was referred to as IgG4-related kidney disease,including IgG4-related tubulointerstitiai nephrisis,membraneous nephropathy and renal pyelitis.The clinical features,laboratory tests,pathological findings and treatment progresses have been summarized in this review.

A Case of IgG4-Related Pancreas and Kidney Disease Mimicking a Renal Pelvic Malignancy / 대한내과학회지

IgG4-related sclerosing disease is a disease entity that has recently attracted attention, manifesting as a multiorgan disease characterized by high serum IgG4 levels, extensive IgG4-positive plasma cells and lymphocyte infiltration of the affected organs, with the pancreas (autoimmune pancreatitis) and kidney as representative targets. In cases of renal involvement, parenchymal lesions are predominant, such as renal cortical lesions or diffuse renal enlargement. However, mass-like lesions involving the renal pelvis are very rare, and mass forming or pelvic involvement types should be distinguished from lymphomas, metastatic cancers and other genitourinary malignancies to avoid unnecessary surgery. Herein, we report a case of IgG4-related sclerosing disease involving the kidney as an unusual involvement pattern presenting as a mass-like lesion with pelvic and perirenal involvement.