====== Retroperitoneal fibrosis ======

//Retroperitoneal fibrosis (RPF)// - also known as //Ormond's disease// - is a rare progressive disease which features fibrosis (fibrous thickening) of the tissues of the lower body cavity.

It was first noted in 1905, and was subsequently 're-discovered' by Dr. J.K. Ormond in 1948. For many years it was notoriously difficult to diagnose, but now shows up clearly on MRI scans. It can sometimes be alleviated with steroid-type drugs, or by surgery.

It's cause is unknown.

>[The] idiopathic disease was thought to result from a local inflammatory reaction to antigens in the atherosclerotic plaques of the abdominal aorta, but clinicolaboratory findings—namely, the presence of constitutional symptoms and the high concentrations of acute-phase reactants—and the frequent association of the disease with autoimmune diseases that involve other organs suggest that it might be a manifestation of a systemic autoimmune or inflammatory disease."
>\\ Source : [[https://www.thelancet.com/journals/lancet/article/PIIS0140-6736(06)68035-5/abstract|The Lancet, vol. 367, issue 9506, pp. 241-251]]{{:oa_padlock_red.png?16}}<sup>[ paywalled ]</sup>

//Note :// One drug used to help treat the disorder -// tamoxifen// - appears to work by an as yet unknown mechanism.