Retroperitoneal fibrosis

Abstract

Retroperitoneal fibrosis (RPF) is a rare disease that is marked by systemic inflammation and the development of a periaortic fibroinflammatory mass. The fibroinflammatory infiltration can encase the abdominal aorta, ureters, and other abdominal organs. The clinical presentation often includes constitutional symptoms, abdominal pain, and signs of renal insufficiency or renal failure related to ureteral obstruction. Less frequently, RPF may present with vascular complications, such as venous thrombosis or claudication. The idiopathic form of RPF is most common but secondary forms have been described and are associated with malignancy and a variety of different medications. The pathophysiology is uncertain, but RPF has been linked with periaortitis and IgG4-related disease. Treatment centers on the relief of symptoms and complications associated with mass effects. Corticosteroids and other immunosuppressant therapies can improve constitutional symptoms, reduce infiltrate mass, and achieve disease remission, but a chronic relapsing course is not uncommon.

IgG4‐related retroperitoneal fibrosis: a newly characterized disease

“…It is a rare disease characterized by the development of inflammation and fibrosis in the soft tissues of the retroperitoneum and other abdominal organs. There will be a series of clinical manifestions once the fibro‐inflammatory tissue encases the abdominal aorta, iliac arteries and urinary duct . RPF is generally divided into two types: idiopathic RPF (IRPF) and secondary RPF.…”

“…RPF is generally divided into two types: idiopathic RPF (IRPF) and secondary RPF. The former, usually with no clear pathogenesis, makes up two‐thirds of RPF cases, while the latter often follows drug therapy, malignant tumors (such as lymphoma), infection and radiographic exposure …”

The Development of Rapidly Progressive Glomerulonephritis in Patient Diagnosed with Idiopathic Chronic Periaortitis

“…5 Kronik periaortit tanısı görüntüleme yöntemlerine dayanılarak konulur. 6 Distal abdominal aortada geniş-leme oluşturarak veya oluşturmadan kronik adventisyal inflamasyon ile beraber ateroskleroz ve aort duvarı mediya tabakasında kalınlaşmaya neden olan ve komşu dokulara da yayılabilen inflamatuar bir süreçtir.…”

Molecular diagnosis: Implications for ophthalmology

“…However, prominence of IgG4 plasma cells in tissue correlates with fibrosis and with a multisystem disease that often responds favorably to rituximab therapy (Deshpande et al, 2012; Khosroshahi et al, 2010). Retroperitoneal fibrosis is an example of a disease that might be IgG4 mediated in some instances (Tzou et al, 2014). IgG4-mediated disease has been reported in both the lacrimal gland (Koizumi et al, 2013) and in orbital adipose tissue (Lindfield et al, 2012).…”