NDT Advance Access originally published online on March 29, 2007
Nephrology Dialysis Transplantation 2007 22(6):1786; doi:10.1093/ndt/gfm071
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When can we really diagnose idiopathic retroperitoneal fibrosis?
Email: wwolyniec{at}wp.plSir,
In a recent paper Moroni et al. [1] have reviewed the long-term outcomes of patients with idiopathic retroperitoneal fibrosis (RF). In Poland, we recently analysed a similar group of 15 patients in whom the diagnosis of RF was established in the same period of time (1990–2006, as in Moroni's study) in five centres. In our opinion, their published report deserves several comments.
- The increasing incidence of RF. Moroni et al. found three patients with RF (15% of their entire 15-year cohort) in the last year of their study. In our group of patients, the diagnosis of RF in 9 of 15 patients was made in the last 5 years. The incidence of RF in Poland and Italy is rising, probably due to better diagnosis and the increasing age of the general population [and the higher incidence of aortic aneurysm (AA) that would go along with the latter].
- Diagnosing idiopathic RF. In Moroni's paper, only one of the studied patients is diagnosed as having a secondary RF associated with gastric cancer. The rest are considered to have idiopathic RF—of whom two are classified as having an idiopathic RF associated with AA. In our opinion it would be better to describe those two as patients with perianeurysmal retroperitoneal fibrosis (PRF) [2]. In our study, 4 of 15 patients had AA, and therefore were classified as cases of PRF. Furthermore, in our cohort five patients with so-called idiopathic RF had autoimmune thyroiditis (4 of 17 in Moroni's group)—a rather high rate of coincidence. The remaining six patients in our group had hypertension, two of them also had long histories of diabetes mellitus (DM) and one had rheumatoid arthritis. We can speculate that the RF in these patients was caused by atherogenic changes in their aortae, despite the fact that the diameters of the aortas were not increased. It has previously been suggested that some cardiovascular risk factors are very common in patients with RF [3]. In our study one-third of the patients had aortic aneurysm, one-third autoimmune thyroiditis and one-third high cardiovascular risk factors (Table 1).
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This rising incidence of RF, if real and persistent, will present a new challenge for nephrologists. It is difficult to distinguish which case of RF is primary (idiopathic) and which secondary. A better understanding of the disease will of course help in the future to properly classify these cases. In Table 1 we present data on our 15 patients. We have divided them into three groups and compare them with two patients with inflammatory abdominal aortic aneurysm (IAAA), which is a condition similar to PRF (both are classified as chronic periaortitis). It appears that these four groups differ in age, gender and severity of kidney involvement.
Conflict of interest statement. None declared.
1Department of Nephrology,
Transplantology and
Internal Medicine
Medical University of Gdansk
ul Debinki 7
80-211 Gdansk, Poland.
2Department of Endocrinology
Medical University of Gdansk
Gdansk, Poland.
3Department of Nephrology
Medical University of Wroclaw
Wroclaw, Poland.
4Division of Nephrology
Regional Specialist Hospital No 4
Bytom, Poland.
5Nephrology Outpatient Clinic
Medical University of Warsaw
Warsaw, Poland.
References
- Moroni G, Gallelli B, Banfi G, Sandri S, Messa P, Ponticelli C. Long-term outcome of idiopathic fibrosis treated with surgical and/or medical approaches. Nephrol Dial Transplant (2006) 21:2485–2490.
[Abstract/Free Full Text] - Vaglio A, Salvarani C, Buzio C. Retroperitoneal fibrosis. Lancet (2006) 367:241–251.[CrossRef][Web of Science][Medline]
- Tallarigo C, D'Amico A, Porcaro AB, et al. Our experience in the treatment of retroperitoneal fibrosis. Arch Ital Urol Androl (2000) 72:51–58.[Medline]
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