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Retroperitoneal fibrosis (RPF) is a rare disease characterized by the proliferation of inflammatory and fibrous tissue in the retroperitoneum. These masses are commonly localized around the infrarenal part of the abdominal aorta and iliac arteries, often covering the ureters or other organs of the abdominal cavity; idiopathic diseases accounting for 70 % of cases. RPF may be associated with immunoglobulin G4 (IgG4), which accounts for two-thirds of idiopathic RPF cases. Secondary RPF may develop due to infections, malignant neoplasms, medication, retroperitoneal bleeding, or various other diseases. A clinical case of idiopathic RPF, probably associated with IgG4, was described in a patient who had undergone a symptomatic surgical treatment in an urological clinic and continued being treated by a rheumatologist, given pathogenetic therapy (methylprednisolone and mycophenolate mofetil) with a significant improvement. For rheumatologists, this pathology is of an undoubted clinical interest, since these changes are based on processes similar to those occurring in systemic diseases of the connective tissue, and the RPF is extremely rare, ranging from 0.1 to 1.3 cases per 100,000 patients per year. However, more often than not, such patients are referred to the doctors of ancillary specialties (urologists, nephrologists, surgeons, vascular surgeons). All of the abovementioned facts emphasize the importance of analyzing such a clinical case.
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