IgG4-related Diseases - A Rare Polycystic Form of Ormond's Disease.
Currently, Ormond's disease is classified among IgG4-associated diseases. Its clinical manifestation varies and is characterized by the presence of fibrous retroperitoneal tissue that often affects the ureters or abdominal aorta and iliac arteries. We present a unique case of the polycystic form of Ormond's disease, imitating tumour in the retroperitoneal space. At the time of diagnosis, the disease was not metabolically active and did not require immunosuppressive therapy. The polycystic mass was removed surgically. There has been no exacerbation of the disease during the last 12 months.
Department of Clinical Biochemistry, Haematology and Immunology, Na Homolce Hospital, Prague, Czech Republic. miroslav.prucha@homolka.cz.
Miroslav
M
Průcha
Department of Vascular Surgery, Na Homolce Hospital, Prague, Czech Republic.
Petr
P
Czinner
Department of Pathology, Na Homolce Hospital, Prague, Czech Republic.
Petra
P
Prokopová
D006801:Humans; D007074:Immunoglobulin G; D008297:Male; D008875:Middle Aged; D012185:Retroperitoneal Fibrosis / Q000175:diagnosis* / Q000473:pathology* / Q000601:surgery; D012187:Retroperitoneal Space / Q000473:pathology; D014057:Tomography, X-Ray Computed; D013520:Urologic Surgical Procedures
D007074:Immunoglobulin G
Ormond’s disease; Polycystic form; Retroperitoneal fibrosis