Retroperitoneal fibrosis reviewed

In an article by O’Brien et al published in BJUI (2016) the authors review the current state of knowledge on RPF.

They identify 7 types:-

1) IgG4 related

2) Systemic autoimmune eg SLE, Wegemer’s, APS, PMR/GCA

3) degenerative aorto-iliac arterial disease (either aneurysmal or non-dilated (may also be IgG4 positive)

4) idiopathic peri-aortitis

5) drug related

6) paraneoplastic

7) radiation induced

The authors suggest that initial tests should include FBC, CrP, renal profile, ESR and screen for SLE, Wegemer’s, APS, PMR/GCA. CT is normal imaging although PET CT may be a useful investigational marker of inflammation. Biopsy should be attempted

The aim of treatment is preserve renal function, and make stent free. Prednisone with 20md bd is used if there is evidence of inflammation. Azothiprine or methotrexate are useful if steroids are successful but relapse occurs after reducing. Rituximab can control where steroids fail. Ureterolysis should be used early.