Localized bilateral perirenal fibrosis, a rare cause of idiopathic retroperitoneal fibrosis
<p class="MsoNormal" style="line-height: 200%;"><strong style="mso-bidi-font-weight: normal;"><span style="font-size: 10.0pt; line-height: 200%; font-family: Calibri; mso-ansi-language: EN-GB;" lang="EN-GB">Background</span>&l...
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| Format: | Article |
| Language: | English |
| Published: |
Slovenian Medical Association
2014-08-01
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| Series: | Zdravniški Vestnik |
| Subjects: | |
| Online Access: | http://vestnik.szd.si/index.php/ZdravVest/article/view/108 |
| _version_ | 1828304774695485440 |
|---|---|
| author | Maja Kveder Radoslav Kveder |
| author_facet | Maja Kveder Radoslav Kveder |
| author_sort | Maja Kveder |
| collection | DOAJ |
| description | <p class="MsoNormal" style="line-height: 200%;"><strong style="mso-bidi-font-weight: normal;"><span style="font-size: 10.0pt; line-height: 200%; font-family: Calibri; mso-ansi-language: EN-GB;" lang="EN-GB">Background</span></strong><span style="font-size: 10.0pt; line-height: 200%; font-family: Calibri; mso-ansi-language: EN-GB;" lang="EN-GB">: Idiopathic retroperitoneal fibrosis is an infrequent process of unknown aetiology characterised by fibrous tissue proliferation in the retroperitoneum. Even less frequent is a localized form of this disease by a proliferation of fibrous tissue around single or both kidneys.</span></p><p class="MsoNormal" style="line-height: 200%;"><strong style="mso-bidi-font-weight: normal;"><span style="font-size: 10.0pt; line-height: 200%; font-family: Calibri; mso-ansi-language: EN-GB;" lang="EN-GB">Case report</span></strong><span style="font-size: 10.0pt; line-height: 200%; font-family: Calibri; mso-ansi-language: EN-GB;" lang="EN-GB">: We describe a case of 46-year old man in whom medical management was started for accidentally discovered arterial hypertension, which turned out to be difficult to control.<span style="mso-spacerun: yes;"> </span>During diagnostic work-up of hypertension, an abdominal ultrasound was obtained a year later demonstrating slight bilateral caliectasis without obvious visible cause for it. Laboratory exams have shown significantly impaired renal function, normocytic anaemia, slightly higher sedimentation rate, increased CRP and normal urinalysis. Nephrologist has decided for hospitalisation during which magnetic resonance imaging was performed<span style="mso-spacerun: yes;"> </span>showing a few mm wide tissue coats surrounding both kidneys with fluid lying between the coat and kidney capsule. A biopsy of perirenal mass has confirmed a dense cellular lesion consisted of interweaved fascicles of spindle-shaped cells. After exclusion of tumours and other causes, a diagnosis of retroperitoneal fibrosis was confirmed. Clinical picture and laboratory data corresponded to idiopathic form of this disease. A treatment with tamoxifen was started after patient refused treatment with methylprednisolone. During tamoxifen monotherapy, there was gradual significant improvement of general symptoms, notable decline in inflammation markers, improvement of anaemia, normalisation of kidney function, and normalisation of blood pressure. </span></p><p class="MsoNormal" style="line-height: 200%;"><strong style="mso-bidi-font-weight: normal;"><span style="font-size: 10.0pt; line-height: 200%; font-family: Calibri; mso-ansi-language: EN-GB;" lang="EN-GB">Conclusion</span></strong><span style="font-size: 10.0pt; line-height: 200%; font-family: Calibri; mso-ansi-language: EN-GB;" lang="EN-GB">: Retroperitoneal fibrosis is still an obscure and multifaceted disease. A proper selection of diagnostic methods is the key to correct and fast diagnosis as well as good grounding for proper treatment.</span></p><p class="MsoNormal" style="line-height: 200%;"><span style="font-size: 10.0pt; line-height: 200%; font-family: Calibri; mso-ansi-language: EN-GB;" lang="EN-GB"><br /></span></p> |
| first_indexed | 2024-04-13T14:17:19Z |
| format | Article |
| id | doaj.art-3b5ee828001846569a8dd07cacd8e62c |
| institution | Directory Open Access Journal |
| issn | 1318-0347 1581-0224 |
| language | English |
| last_indexed | 2024-04-13T14:17:19Z |
| publishDate | 2014-08-01 |
| publisher | Slovenian Medical Association |
| record_format | Article |
| series | Zdravniški Vestnik |
| spelling | doaj.art-3b5ee828001846569a8dd07cacd8e62c2022-12-22T02:43:35ZengSlovenian Medical AssociationZdravniški Vestnik1318-03471581-02242014-08-01837-8921Localized bilateral perirenal fibrosis, a rare cause of idiopathic retroperitoneal fibrosisMaja Kveder0Radoslav Kveder1Department of nephrology, Division of Internal medicine, University clinical center LjubljanaDepartment of nephrology, Division of Internal medicine University clinical center Ljubljana<p class="MsoNormal" style="line-height: 200%;"><strong style="mso-bidi-font-weight: normal;"><span style="font-size: 10.0pt; line-height: 200%; font-family: Calibri; mso-ansi-language: EN-GB;" lang="EN-GB">Background</span></strong><span style="font-size: 10.0pt; line-height: 200%; font-family: Calibri; mso-ansi-language: EN-GB;" lang="EN-GB">: Idiopathic retroperitoneal fibrosis is an infrequent process of unknown aetiology characterised by fibrous tissue proliferation in the retroperitoneum. Even less frequent is a localized form of this disease by a proliferation of fibrous tissue around single or both kidneys.</span></p><p class="MsoNormal" style="line-height: 200%;"><strong style="mso-bidi-font-weight: normal;"><span style="font-size: 10.0pt; line-height: 200%; font-family: Calibri; mso-ansi-language: EN-GB;" lang="EN-GB">Case report</span></strong><span style="font-size: 10.0pt; line-height: 200%; font-family: Calibri; mso-ansi-language: EN-GB;" lang="EN-GB">: We describe a case of 46-year old man in whom medical management was started for accidentally discovered arterial hypertension, which turned out to be difficult to control.<span style="mso-spacerun: yes;"> </span>During diagnostic work-up of hypertension, an abdominal ultrasound was obtained a year later demonstrating slight bilateral caliectasis without obvious visible cause for it. Laboratory exams have shown significantly impaired renal function, normocytic anaemia, slightly higher sedimentation rate, increased CRP and normal urinalysis. Nephrologist has decided for hospitalisation during which magnetic resonance imaging was performed<span style="mso-spacerun: yes;"> </span>showing a few mm wide tissue coats surrounding both kidneys with fluid lying between the coat and kidney capsule. A biopsy of perirenal mass has confirmed a dense cellular lesion consisted of interweaved fascicles of spindle-shaped cells. After exclusion of tumours and other causes, a diagnosis of retroperitoneal fibrosis was confirmed. Clinical picture and laboratory data corresponded to idiopathic form of this disease. A treatment with tamoxifen was started after patient refused treatment with methylprednisolone. During tamoxifen monotherapy, there was gradual significant improvement of general symptoms, notable decline in inflammation markers, improvement of anaemia, normalisation of kidney function, and normalisation of blood pressure. </span></p><p class="MsoNormal" style="line-height: 200%;"><strong style="mso-bidi-font-weight: normal;"><span style="font-size: 10.0pt; line-height: 200%; font-family: Calibri; mso-ansi-language: EN-GB;" lang="EN-GB">Conclusion</span></strong><span style="font-size: 10.0pt; line-height: 200%; font-family: Calibri; mso-ansi-language: EN-GB;" lang="EN-GB">: Retroperitoneal fibrosis is still an obscure and multifaceted disease. A proper selection of diagnostic methods is the key to correct and fast diagnosis as well as good grounding for proper treatment.</span></p><p class="MsoNormal" style="line-height: 200%;"><span style="font-size: 10.0pt; line-height: 200%; font-family: Calibri; mso-ansi-language: EN-GB;" lang="EN-GB"><br /></span></p>http://vestnik.szd.si/index.php/ZdravVest/article/view/108ultrasonography, magnetic resonance imaging, kidney biopsy, tamoxifen, outcome |
| spellingShingle | Maja Kveder Radoslav Kveder Localized bilateral perirenal fibrosis, a rare cause of idiopathic retroperitoneal fibrosis Zdravniški Vestnik ultrasonography, magnetic resonance imaging, kidney biopsy, tamoxifen, outcome |
| title | Localized bilateral perirenal fibrosis, a rare cause of idiopathic retroperitoneal fibrosis |
| title_full | Localized bilateral perirenal fibrosis, a rare cause of idiopathic retroperitoneal fibrosis |
| title_fullStr | Localized bilateral perirenal fibrosis, a rare cause of idiopathic retroperitoneal fibrosis |
| title_full_unstemmed | Localized bilateral perirenal fibrosis, a rare cause of idiopathic retroperitoneal fibrosis |
| title_short | Localized bilateral perirenal fibrosis, a rare cause of idiopathic retroperitoneal fibrosis |
| title_sort | localized bilateral perirenal fibrosis a rare cause of idiopathic retroperitoneal fibrosis |
| topic | ultrasonography, magnetic resonance imaging, kidney biopsy, tamoxifen, outcome |
| url | http://vestnik.szd.si/index.php/ZdravVest/article/view/108 |
| work_keys_str_mv | AT majakveder localizedbilateralperirenalfibrosisararecauseofidiopathicretroperitonealfibrosis AT radoslavkveder localizedbilateralperirenalfibrosisararecauseofidiopathicretroperitonealfibrosis |