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Retroperitoneal Fibrosis: Case Series of 20 Patients

Received: 29 March 2017     Accepted: 12 April 2017     Published: 13 June 2017
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Abstract

Retroperitoneal fibrosis (RPF) is a rare disease. The objective of this paper is to study the prevalence, clinical, biological and radiological aspects of the RPFand to specify its therapeutic modalities. This study provides descriptive evidence for a series of 20 patients with RPF. We conducted a retrospective study of cases of RPF diagnosed in the Urology and Internal Medicine departments of the Ibn Sina Military Hospital in Marrakech over a five-year period from July 2010 to December 2015. The positive diagnosis of RPF was retained either on the basis of a histological analysis from a biopsy fragment or, in the absence of histological evidence, by the detection on a scan of an infiltrate or "sleeve" Regular and homogeneous tissue density, perivascular topography, surrounding the abdominal aorta, more or less extended to the iliac vessels and able to take the contrast after injection. They were 15 men and 5 women with an average age of 53 years with extremes of 41 to 74 years. Almost all patients had mainly lumbar and abdominal pain. An inflammatory syndrome existed in all cases and renal insufficiency in 15 cases. Radiological investigations showed unilateral or bilateral hydronephrosis in 16 cases. RPF was idiopathic in 14 patients. Secondary forms were present in 6 patients (three atheromatous diseases, one gastric tumor, one case associated with Riedel's thyroiditis and one case of systemic fibrosis associating RPF, Riedel's thyroiditis and liver fibrosis). The treatment consisted of corticosteroids in 19 cases, surgery in 17 cases associated with corticosteroid therapy or even immunosuppressant in the case of steroid-dependence. Two deaths were observed in this series; one of these two patients had developed cervix carcinoma with pleural and peritoneal metastases after 5 years of immunosuppressive therapy. The second died due to the complications of his gastric neoplasm. The general characteristics of patients included are similar to the other series of the literature. The frequency of secondary forms seems to be underestimated, hence the value of a rigorous etiological investigation. The therapeutic strategy and the use of the cortisone saving treatments should be specified by randomized therapeutic trials. The prognosis is generally good; however, the possibility of recurrences, the incidence of which is greater during the first five years, requires prolonged clinical, biological and radiological monitoring.

Published in Science Journal of Public Health (Volume 5, Issue 4)
DOI 10.11648/j.sjph.20170504.13
Page(s) 294-300
Creative Commons

This is an Open Access article, distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution and reproduction in any medium or format, provided the original work is properly cited.

Copyright

Copyright © The Author(s), 2017. Published by Science Publishing Group

Keywords

Retroperitoneal Fibrosis, Atheroma, Neoplasm, Vasculitis, Riedel's Thyroiditis, Corticosteroids

References
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  • APA Style

    Kaddouri Said, Ghoundale Omar, Chahbi Zakaria, Essadi Ismail, Qacif Hassan, et al. (2017). Retroperitoneal Fibrosis: Case Series of 20 Patients. Science Journal of Public Health, 5(4), 294-300. https://doi.org/10.11648/j.sjph.20170504.13

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    ACS Style

    Kaddouri Said; Ghoundale Omar; Chahbi Zakaria; Essadi Ismail; Qacif Hassan, et al. Retroperitoneal Fibrosis: Case Series of 20 Patients. Sci. J. Public Health 2017, 5(4), 294-300. doi: 10.11648/j.sjph.20170504.13

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    AMA Style

    Kaddouri Said, Ghoundale Omar, Chahbi Zakaria, Essadi Ismail, Qacif Hassan, et al. Retroperitoneal Fibrosis: Case Series of 20 Patients. Sci J Public Health. 2017;5(4):294-300. doi: 10.11648/j.sjph.20170504.13

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  • @article{10.11648/j.sjph.20170504.13,
      author = {Kaddouri Said and Ghoundale Omar and Chahbi Zakaria and Essadi Ismail and Qacif Hassan and Zamraoui Nadir and Zyani Mohamed},
      title = {Retroperitoneal Fibrosis: Case Series of 20 Patients},
      journal = {Science Journal of Public Health},
      volume = {5},
      number = {4},
      pages = {294-300},
      doi = {10.11648/j.sjph.20170504.13},
      url = {https://doi.org/10.11648/j.sjph.20170504.13},
      eprint = {https://article.sciencepublishinggroup.com/pdf/10.11648.j.sjph.20170504.13},
      abstract = {Retroperitoneal fibrosis (RPF) is a rare disease. The objective of this paper is to study the prevalence, clinical, biological and radiological aspects of the RPFand to specify its therapeutic modalities. This study provides descriptive evidence for a series of 20 patients with RPF. We conducted a retrospective study of cases of RPF diagnosed in the Urology and Internal Medicine departments of the Ibn Sina Military Hospital in Marrakech over a five-year period from July 2010 to December 2015. The positive diagnosis of RPF was retained either on the basis of a histological analysis from a biopsy fragment or, in the absence of histological evidence, by the detection on a scan of an infiltrate or "sleeve" Regular and homogeneous tissue density, perivascular topography, surrounding the abdominal aorta, more or less extended to the iliac vessels and able to take the contrast after injection. They were 15 men and 5 women with an average age of 53 years with extremes of 41 to 74 years. Almost all patients had mainly lumbar and abdominal pain. An inflammatory syndrome existed in all cases and renal insufficiency in 15 cases. Radiological investigations showed unilateral or bilateral hydronephrosis in 16 cases. RPF was idiopathic in 14 patients. Secondary forms were present in 6 patients (three atheromatous diseases, one gastric tumor, one case associated with Riedel's thyroiditis and one case of systemic fibrosis associating RPF, Riedel's thyroiditis and liver fibrosis). The treatment consisted of corticosteroids in 19 cases, surgery in 17 cases associated with corticosteroid therapy or even immunosuppressant in the case of steroid-dependence. Two deaths were observed in this series; one of these two patients had developed cervix carcinoma with pleural and peritoneal metastases after 5 years of immunosuppressive therapy. The second died due to the complications of his gastric neoplasm. The general characteristics of patients included are similar to the other series of the literature. The frequency of secondary forms seems to be underestimated, hence the value of a rigorous etiological investigation. The therapeutic strategy and the use of the cortisone saving treatments should be specified by randomized therapeutic trials. The prognosis is generally good; however, the possibility of recurrences, the incidence of which is greater during the first five years, requires prolonged clinical, biological and radiological monitoring.},
     year = {2017}
    }
    

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  • TY  - JOUR
    T1  - Retroperitoneal Fibrosis: Case Series of 20 Patients
    AU  - Kaddouri Said
    AU  - Ghoundale Omar
    AU  - Chahbi Zakaria
    AU  - Essadi Ismail
    AU  - Qacif Hassan
    AU  - Zamraoui Nadir
    AU  - Zyani Mohamed
    Y1  - 2017/06/13
    PY  - 2017
    N1  - https://doi.org/10.11648/j.sjph.20170504.13
    DO  - 10.11648/j.sjph.20170504.13
    T2  - Science Journal of Public Health
    JF  - Science Journal of Public Health
    JO  - Science Journal of Public Health
    SP  - 294
    EP  - 300
    PB  - Science Publishing Group
    SN  - 2328-7950
    UR  - https://doi.org/10.11648/j.sjph.20170504.13
    AB  - Retroperitoneal fibrosis (RPF) is a rare disease. The objective of this paper is to study the prevalence, clinical, biological and radiological aspects of the RPFand to specify its therapeutic modalities. This study provides descriptive evidence for a series of 20 patients with RPF. We conducted a retrospective study of cases of RPF diagnosed in the Urology and Internal Medicine departments of the Ibn Sina Military Hospital in Marrakech over a five-year period from July 2010 to December 2015. The positive diagnosis of RPF was retained either on the basis of a histological analysis from a biopsy fragment or, in the absence of histological evidence, by the detection on a scan of an infiltrate or "sleeve" Regular and homogeneous tissue density, perivascular topography, surrounding the abdominal aorta, more or less extended to the iliac vessels and able to take the contrast after injection. They were 15 men and 5 women with an average age of 53 years with extremes of 41 to 74 years. Almost all patients had mainly lumbar and abdominal pain. An inflammatory syndrome existed in all cases and renal insufficiency in 15 cases. Radiological investigations showed unilateral or bilateral hydronephrosis in 16 cases. RPF was idiopathic in 14 patients. Secondary forms were present in 6 patients (three atheromatous diseases, one gastric tumor, one case associated with Riedel's thyroiditis and one case of systemic fibrosis associating RPF, Riedel's thyroiditis and liver fibrosis). The treatment consisted of corticosteroids in 19 cases, surgery in 17 cases associated with corticosteroid therapy or even immunosuppressant in the case of steroid-dependence. Two deaths were observed in this series; one of these two patients had developed cervix carcinoma with pleural and peritoneal metastases after 5 years of immunosuppressive therapy. The second died due to the complications of his gastric neoplasm. The general characteristics of patients included are similar to the other series of the literature. The frequency of secondary forms seems to be underestimated, hence the value of a rigorous etiological investigation. The therapeutic strategy and the use of the cortisone saving treatments should be specified by randomized therapeutic trials. The prognosis is generally good; however, the possibility of recurrences, the incidence of which is greater during the first five years, requires prolonged clinical, biological and radiological monitoring.
    VL  - 5
    IS  - 4
    ER  - 

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Author Information
  • Department of Internal Medicine, Ibn Sina Military Hospital, Marrakesh, Morocco

  • Department of Urology, Ibn Sina Military Hospital, Marrakesh, Morocco

  • Department of Internal Medicine, Ibn Sina Military Hospital, Marrakesh, Morocco

  • Department of Medical Oncology, Ibn Sina Military Hospital, Marrakesh, Morocco

  • Department of Internal Medicine, Ibn Sina Military Hospital, Marrakesh, Morocco

  • Department of Nephrology, Ibn Sina Military Hospital, Marrakesh, Morocco

  • Department of Internal Medicine, Ibn Sina Military Hospital, Marrakesh, Morocco

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