Urological manifestations of the disease related to immunoglobulin G4

Abstract

Immunoglobulin G4 related disease (IgG4-RD) is a fibro-inflammatory disease of unknown etiology, characterized by lesions in the form of tumors, elevated serum IgG4 levels, plasma cells with significant IgG4 infiltration, accompanied by phlebitis obliterans and fibrosis. This disease usually has multiorgan disease, including pancreas, biliary tract, salivary glands, peri orbital tissues, kidneys, lungs, lymph nodes and retro peritoneum. IgG4-RD mainly affects men with a predominance of age by young adults until old age. The clinical manifestations of IgG4-RD, depend mainly on the organs affected and the response to steroids. His forecast is not yet clear. Within the affected urogenital organs can be observed kidney, retroperitoneum, ureter, bladder, urachus, testis/epididymis, paratesticular region, prostate and urethra.

Human epididymis protein 4 (HE4) is a novel biomarker for fibrosis in IgG4-related disease and can predict poor prognosis

“…HE4 is mainly secreted by activated or damaged epithelial cells and fibroblasts,14 22 and according to the Human Protein Atlas, commonly affected organs (pancreas, lacrimal gland, salivary gland, bile duct, retroperitoneum tissue, etc) in IgG4-RD25–27 either do not express HE4 or have low levels under normal conditions. Although reproductive organs including cervix and epididymis could also be affected in patients with IgG4-RD, the incidence was extremely low, with only a few case reports 28 29. Thus, the disease environment in IgG4-RD might lead to the activation and upregulation of HE4 in the epithelium or fibroblasts of the affected organs, which would lead to its secretion and accumulation in the tissues, or its release into peripheral circulation.…”

Immunoglobulin G4–related disease of the paratestis: Can we avoid radical treatment?

“…IgG4-RD is often a multi-organ disease affecting the pancreas, biliary tract, salivary glands, lungs, lymph nodes, peri-orbital tissues and urinary tract organs. 1 The paratesticular region is not considered a primary lesion site for IgG4-RD, with only 11 previously reported cases. [2][3][4][5][6][7][8] Literature reporting the epidemiology of IgG4-RD is very limited.…”

“…17 IgG4-related retroperitoneal fibrosis can be diagnosed on core biopsy, and cytology of paratesticular fluid has previously been diagnostic of IgG4-RD. 1,8 Percutaneous biopsies for scrotal lesions remain a controversial entity due to the theoretical risk of tumour seeding from scrotal violation, but this technique would facilitate testis-sparing management of this condition. The evidence that scrotal violation negatively impacts prognosis for testicular neoplasms is limited, and a large meta-analysis comparing scrotal versus inguinal approaches found no difference in prognosis or overall survival rate.…”

“…Numerous urological manifestations have been reported, including within the kidney, ureter, retroperitoneum, bladder, prostate, urethra and scrotum. 1 IgG4-RD is immune mediated and characterised by fibrosis, pseudotumours, plasma cells with significant IgG4 infiltrations and potentially elevated serum IgG4 levels. Paratesticular IgG4 pseudotumours have rarely been reported, with only 11 cases identified in the literature.…”

Renal Manifestations of IgG4‐Related Disease: A Concise Review

“…More distally, ureteral IgG4-RD can manifest as inflammatory pseudotumours and segmental ureteritis [ 88 , 89 ]. In the bladder, transmural bladder wall thickening and tumour-like masses have been documented [ 90 – 95 ]. Lymphoplasmacytic infiltration of the prostate gland may present as prostatitis and enlargement, as well as periprostatic tumour-like masses [ 96 ].…”