Original Investigation
Pathogenesis and Treatment of Kidney Disease
Treatment of Idiopathic Retroperitoneal Fibrosis With Colchicine and Steroids: A Case Series

https://doi.org/10.1053/j.ajkd.2008.09.025Get rights and content

Background

Idiopathic retroperitoneal fibrosis (IRPF) is an uncommon, but increasingly recognized, disease. Autoimmune mechanisms probably participate in its pathogenesis. Patients have been treated with high-dose steroids and sometimes immunosuppressive drugs, resulting in toxicity risk during prolonged treatments; thus, it would be wise to look for safer drugs. Colchicine has antifibrotic, anti-inflammatory, and immunosuppressive effects. Hence, it is a potential therapeutic resource.

Study Design

Case series.

Setting & Participants

7 consecutives patients with IRPF diagnosed since 1993 at a navy hospital were prescribed colchicine plus prednisone or deflazacort. After reaching a clinical response, the steroid dose was tapered, maintaining daily colchicine therapy.

Outcomes & Measurements

Symptomatic improvement was defined as time to relief of symptoms; clinical improvement, time to normalization of inflammatory parameters with symptomatic improvement; radiological changes, percentage of decrease in retroperitoneal mass; recurrence, as the need to increase steroid dose or add immunosuppressors because of reactivation of inflammation; and treatment failure, as persistence of signs of active inflammation and/or decrease in retroperitoneal mass less than 50% and/or deterioration in kidney function at the last visit.

Results

At month 3 of treatment, all patients had symptomatic improvement and 67% had clinical improvement. Between 8 and 12 months, 67% of patients had a decrease in retroperitoneal mass greater than 50%. No recurrence or treatment failure was observed during follow-up (mean, 72.5 months). At the last visit, all patients had a significant decrease in retroperitoneal mass (mean, 79.8% ± 19.7%). One patient died of septic shock on day 21 of treatment.

Limitations

This series did not have a control group.

Conclusions

Colchicine in association with a low induction dose of steroids (<0.5 mg/kg/d) produced remission rates in patients with IRPF similar to those for regimens using greater steroid doses alone or in combination with immunosuppressive drugs. In addition, colchicine may have contributed to recurrence prevention.

Section snippets

Methods

All patients with a diagnosis of IRPF at discharge in the 14 years from October 1993 to August 2005 and treated by 1 of the authors were included. Pathological diagnosis was obtained in all cases. Six men and 1 woman were enrolled, and age varied from 35 to 83 years (median, 64 years). One patient had another fibrotic disease (Dupuytren disease), and no patient's history was consistent with the diagnosis of secondary or drug-related retroperitoneal fibrosis. Relevant information between the

Results

Comorbid conditions, symptoms, signs and laboratory determinations at the time of diagnosis of IRPF are listed in Table 1.

CT scans showed hydronephrosis (71%); calcified aortic atheromatosis (71%); dense tissue surrounding the abdominal aorta (86%), iliac arteries (100%), renal arteries (57%), and inferior cava vein (43%); and a partially thrombosed aortic aneurism (29%; Table 2).

IRFP was clinically suspected in 4 patients, and in 3 patients, the diagnosis became evident during a laparotomy

Discussion

IRPF, also called Ormond disease, has been known since 1905 after its first description by Albarran.3 Currently, IRPF is considered a variation of “chronic periaortitis,” an entity that includes abdominal aortic inflammatory aneurysm and perianeurysmal fibrosis.1, 24 The pathogenesis of the disease is unknown. Given the frequent association with advanced atherosclerosis, 1 theory suggested that a local inflammatory reaction may develop by means of B- and T-lymphocyte activation by antigens from

Acknowledgements

The authors thank Manuel Kukuljan, MD, PhD, and Leopoldo Ardiles, MD, for assistance in the revision of the manuscript.

Support: None.

Financial Disclosure: None.

References (41)

  • W. Grotz et al.

    Treatment of retroperitoneal fibrosis by mycophenolate mofetil and corticosteroids

    Lancet

    (1998)
  • D. Kershenobich et al.

    Treatment of cirrhosis with colchicineA double-blind randomized trial

    Gastroenterology

    (1979)
  • M. Rojkind et al.

    Effect of colchicine on collagen, albumin and transferrin synthesis by cirrhotic rat liver slices

    Biochim Biophys Acta

    (1975)
  • E. Van Bommel et al.

    Long-term renal and patient outcome in idiopathic retroperitoneal fibrosis treated with prednisone

    Am J Kidney Dis

    (2007)
  • A. Vaglio et al.

    Retroperitoneal fibrosis

    Lancet

    (2006)
  • E. Van Bommel

    Retroperitoneal fibrosis

    Neth J Med

    (2002)
  • J. Albarran

    Retention renale para periureterite: Liberation externe de l'uretere

    Assoc Fr Urol

    (1905)
  • P. Greco et al.

    Idiopathic retroperitoneal fibrosis

    G Ital Nefrol

    (2004)
  • L. Baker et al.

    Idiopathic retroperitoneal fibrosisA retrospective analysis of 60 cases

    Br J Urol

    (1987)
  • T. Kamisawa et al.

    IgG4-related sclerosing disease

    World J Gastroenterol

    (2008)
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    Originally published online as doi:10.1053/j.ajkd.2008.09.025 on January 5, 2009.

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