IgG4‐related retroperitoneal fibrosis: a newly characterized disease

Abstract

Retroperitoneal fibrosis (RPF) is a rare disease characterized by chronic, nonspecific inflammatory and sclerotic or fibrotic tissue in the periaortic or periiliac retroperitoneum that encases adjacent structures. There will be a series of clinical manifestations once the proliferated fibrous tissues encase the abdominal aorta, iliac arteries and urinary duct. RPF is generally divided into two types: idiopathic retroperitoneal fibrosis (IRPF) without identified pathogenesis, making up about two-thirds of cases, and secondary retroperitoneal fibrosis. Recent studies on Immunoglobulin G4-related disease (IgG4-RD) reveal that abundant infiltration of IgG4 positive plasma cells is found in biopsies on the mass of RPF of some IRPF patients, which is identified as one spectrum of IgG4-RD and is named IgG4-related RPF. IgG4-related RPF is often misdiagnosed as retroperitoneal visceral malignancy and is treated with surgery. In addition, because of its good response to glucocorticoid, early detection and treatment is important. We review the definition, epidemiology, clinical features, diagnostic criteria, treatment and prognosis of IgG4-related RPF in this article to raise awareness of this newly characterized disease.

Current approach to diagnosis and management of retroperitoneal fibrosis

“…Recently, idiopathic RPF has been considered to be a spectrum of IgG4RD, a concept that has emerged during the past decade. [4][5][6][7][8] Because of the good responsiveness to medical therapy, ureteral obstruction is usually managed conservatively with ureteral stenting or PNS. In contrast, ureterolysis, an aggressive surgical treatment, is carried out to relieve ureteral obstruction and salvage renal function in selected patients, but the optimal indication for it is controversial.…”

Retroperitoneal Fibrosis and its Differential Diagnoses: The Role of Radiological Imaging

“…Darüber hinaus werden bei bis zu 60 % der RPF-Patienten erhöhte IgG4-Spiegel im Serum festgestellt; der Anteil der IgG4-assoziierten RPF an den sonst als idiopathisch bzw. primär RPF klassifizierten Patienten könnte also höher sein als bisher angenommen [5,16]. Die häufigste Form der IgG4-assoziierten Erkrankungen ist die Typ-1-Autoimmunpankreatitis.…”

The Clinicopathologic Spectrum of IgG4-Related Disease

“…IgG4-related retroperitoneal fibrosis (IgG4-RRPF) is characterized by chronic inflammatory and infiltration of fibrotic tissue in the periaortic or periiliac retroperitoneum that encases adjacent structures ( 56 ). Different locations of involvement result in varying clinical manifestations.…”

“…Kidney and urinary tract damage causes hydronephrosis, backache, and acute renal failure. Sclerosing mesenteritis can result from compression on the inferior vena cava, which may cause clinical symptoms of abdominal pain, diarrhea, intestinal obstruction, and ischemia ( 56 ). IgG4-related periaortitis and periarteritis are the most common lesions affecting various medium- to large-sized arteries, including the abdominal aorta, iliac artery, renal artery, and mesenteric artery ( 57 ).…”