Obojestranska lokalizirana perirenalna fibroza, redka oblika idiopatske retroperitonealne fibroze

Abstract

Background: Idiopathic retroperitoneal brosis is an infrequent process of unknown aetiology characterised by brous tissue proliferation in the retroperitoneum. Even less frequent is a localized form of this disease by a proliferation of brous tissue surrounding one or both kidneys.

Case report: We describe a case of a 46-year old man in whom medical management was started for accidentally discovered arterial hypertension, which turned out to be difficult to control. During diagnostic work-up of hypertension, an abdominal ultrasound was obtained a year later demonstrating mild bilateral caliectasis without an obvious visible cause for it. Laboratory exams have shown signicantly impaired renal function, normocytic anaemia, slightly higher sedimentation rate, increased CRP and normal urinalysis. Nephrologist decided for hospitalisation during which magnetic resonance imaging was performed showing a few millimetres wide tissue coats surrounding both kidneys with uid accumulation between the coat and kidney capsule. A biopsy of the perirenal mass conrmed a dense cellular lesion consisting of interweaved fascicles of spindle-shaped cells. Aer exclusion of tumours and other causes, a diagnosis of retroperitoneal brosis was conrmed. Clinical picture and laboratory data corresponded to the idiopathic form of this disease. A treatment with tamoxifen was started aer patient refused treatment with methylprednisolone. During tamoxifen monotherapy, there was gradual signicant improvement of general symptoms, notable decline in inammation markers, improvement of anaemia, normalisation of kidney function, and normalisation of blood pressure.

Conclusion: Retroperitoneal brosis is still an obscure and multifaceted disease. A proper selection of diagnostic methods is the key to correct and fast diagnosis as well as the basis for proper treatment.