E.Z. FATYKHOVA¹, S.P. YAKUPOVA¹, T.B. SIBGATULLIN², I.L. SERDYUK³

¹Kazan State Medical University, Kazan

²Kazan Federal University, Medical-sanitary Unit

³Republican Clinical Hospital of the MH of RT, Kazan

 Contact:

Fatykhova E.Z. ― resident doctor–rheumatologist of the Department of Hospital Therapy

Address: 49 Butlerov Str., Kazan, Russian Federation, 420012, tel. (843) 237-36-18, e-mail: elzafatykhova@mail.ru

Retroperitoneal fibrosis (RPF) is a rare fibro-inflammatory disease that develops around the abdominal aorta and the iliac arteries, and spreads into the adjacent retroperitoneum, where it frequently causes ureteral obstruction and renal failure. There are primary (idiopathic) and secondary retroperitoneal fibroses (RPF). In the development of idiopathic RPF the leading role belongs to autoimmune mechanisms. Idiopathic fibrosis may be associated with an IgG4 subclass of immunoglobulins. IgG4 should be determined not only in serum, but also in tissues. The pathogenesis of the disease is multifactorial, leading to non-specificity and variety of symptoms. The most informative method for the diagnosis and evaluation of the dynamics of treatment is multispiral CT (MSCT) with contrast agent and MRI, RPF which complement each other. There is the lack of standards for the management of patients with RPF and the lack of clear criteria of remission due to the low prevalence of the disease in the world. Retroperitoneal fibrosis has a high percentage (72%) of relapses, thus patients should be followed-up lifelong.

Key words: retroperitoneal fibrosis, fibrosis, Ormond’s disease, IgG4, vasculitis, immunosuppressive therapy.

 (For citation: Fatykhova E.Z., Yakupova S.P., Sibgatullin T.B., Serdyuk I.L. Retroperitoneal fibrosis ― diagnostic features, possibilities of therapy. Practical medicine. 2019 . Vol. 17, № 6 (part 1), P. 23-25)

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