Ormond’s Syndrome: Etiology, Pathogenesis, Diagnosis, and Treatment (A Guide for the Practicing Physician)

Ormond’s syndrome is a rare and little-known disease characterized by nonspecific inflammation in the retroperitoneal adipose tissue, leading to the formation of dense fibrous tissue.

The aim is to highlight the etiology, pathogenesis, diagnosis, and treatment of Ormond’s syndrome. The causes of this pathology continue to be actively discussed in the scientific literature. It has been established that etiological factors provoke an immune response, which leads to damage to the peritoneum, and then to fibrosis. The main pathogenetic trigger is the increased expression of the IgG4 complex by plasma cells. In addition, the disease may be genetic or secondary.

Its ability to mimic other diseases and its prolonged latent course make diagnosis difficult. It is essential to recognize the disease in time and select optimal treatment, because delayed diagnosis invariably leads to irreversible sclerotic changes in the retroperitoneal tissue with compression of tubular structures.

Given the difficulties of primary diagnosis and a number of other unresolved issues, it is necessary to further study and develop standards for the management of patients with Ormond's syndrome.

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