Ormond's disease - the current state of knowledge about diagnosis and treatment
Introduction: Retroperitoneal fibrosis (Ormond's disease; RPF) is a rare condition characterized by chronic inflammation and resulting progressive fibrosis of the retroperitoneal space. Due to its low prevalence and uncharacteristic symptoms, it is often overlooked in the diagnostic process, r...
| Main Authors: | , , , , , , , , , |
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| Format: | Article |
| Language: | English |
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Kazimierz Wielki University
2022-09-01
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| Series: | Journal of Education, Health and Sport |
| Subjects: | |
| Online Access: | https://apcz.umk.pl/JEHS/article/view/39793 |
| _version_ | 1811211157371879424 |
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| author | Sara Dankiewicz Sebastian Bróż Przemysław Żelazny Joanna Filipczak Aleksandra Swora Joanna Borowik Oliwer Sygacz Wojciech Brodowski Piotr Pawłowski Katarzyna Basta-Arciszewska |
| author_facet | Sara Dankiewicz Sebastian Bróż Przemysław Żelazny Joanna Filipczak Aleksandra Swora Joanna Borowik Oliwer Sygacz Wojciech Brodowski Piotr Pawłowski Katarzyna Basta-Arciszewska |
| author_sort | Sara Dankiewicz |
| collection | DOAJ |
| description |
Introduction: Retroperitoneal fibrosis (Ormond's disease; RPF) is a rare condition characterized by chronic inflammation and resulting progressive fibrosis of the retroperitoneal space. Due to its low prevalence and uncharacteristic symptoms, it is often overlooked in the diagnostic process, resulting in a late-stage diagnosis when complications are already present and treatment options are limited.
The aim of the study: The purpose of our study was to present, based on the available literature, the latest reports on the diagnosis and treatment of Ormond disease.
State of knowledge: The diagnosis of RPF is based on an anamnesis and imaging. Sometimes a surgical biopsy of the fibrous tissue is necessary. The pathogenesis remains partly unclear. There is no characteristic laboratory marker for early diagnosis of the disease. It is important to distinguish the primary form from the secondary form, in which the trigger factors are known. In therapy, steroid therapy is still the standard. Attempts are being made to treat with other immunosuppressants and biologic drugs with good results, which speaks to the autoimmune basis of the disease. In some cases, surgical treatment and removal of fibrous tissue compressing abdominal structures is essential.
Summary: Although the state of knowledge about retroperitoneal fibrosis has been greatly expanded in recent years, many questions remain to be clarified, especially about its pathogenesis and possible early clinical clues to facilitate diagnosis.
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| first_indexed | 2024-04-12T05:08:00Z |
| format | Article |
| id | doaj.art-2db7ce992ff74d1db49765260d3eecae |
| institution | Directory Open Access Journal |
| issn | 2391-8306 |
| language | English |
| last_indexed | 2024-04-12T05:08:00Z |
| publishDate | 2022-09-01 |
| publisher | Kazimierz Wielki University |
| record_format | Article |
| series | Journal of Education, Health and Sport |
| spelling | doaj.art-2db7ce992ff74d1db49765260d3eecae2022-12-22T03:46:49ZengKazimierz Wielki UniversityJournal of Education, Health and Sport2391-83062022-09-0112910.12775/JEHS.2022.12.09.071Ormond's disease - the current state of knowledge about diagnosis and treatmentSara Dankiewicz0Sebastian Bróż1Przemysław Żelazny2Joanna Filipczak3Aleksandra Swora4Joanna Borowik5Oliwer Sygacz6Wojciech Brodowski7Piotr Pawłowski8Katarzyna Basta-Arciszewska9Wojewódzki Szpital Specjalistyczny Nr 1 imienia Fryderyka Chopina w RzeszowieWojewódzki Szpital Specjalistyczny Nr 1 imienia Fryderyka Chopina w Rzeszowie1 Wojskowy Szpital Kliniczny z Polikliniką SPZOZ w LublinieAbsolwent Uniwersytetu Medycznego w LublinieSamodzielny Publiczny Szpital Kliniczny nr 1 w LublinieSamodzielny Publiczny Szpital Kliniczny nr 1 w LublinieSamodzielny Publiczny Szpital Kliniczny nr 4 w LublinieSamodzielny Publiczny Zakład Opieki Zdrowotnej MSWiA w LublinieSamodzielny Publiczny Zakład Opieki Zdrowotnej MSWiA w LublinieWojewódzki Szpital Specjalistyczny im. Stefana Kardynała Wyszyńskiego Samodzielny Publiczny Zakład Opieki Zdrowotnej w Lublinie | Introduction: Retroperitoneal fibrosis (Ormond's disease; RPF) is a rare condition characterized by chronic inflammation and resulting progressive fibrosis of the retroperitoneal space. Due to its low prevalence and uncharacteristic symptoms, it is often overlooked in the diagnostic process, resulting in a late-stage diagnosis when complications are already present and treatment options are limited. The aim of the study: The purpose of our study was to present, based on the available literature, the latest reports on the diagnosis and treatment of Ormond disease. State of knowledge: The diagnosis of RPF is based on an anamnesis and imaging. Sometimes a surgical biopsy of the fibrous tissue is necessary. The pathogenesis remains partly unclear. There is no characteristic laboratory marker for early diagnosis of the disease. It is important to distinguish the primary form from the secondary form, in which the trigger factors are known. In therapy, steroid therapy is still the standard. Attempts are being made to treat with other immunosuppressants and biologic drugs with good results, which speaks to the autoimmune basis of the disease. In some cases, surgical treatment and removal of fibrous tissue compressing abdominal structures is essential. Summary: Although the state of knowledge about retroperitoneal fibrosis has been greatly expanded in recent years, many questions remain to be clarified, especially about its pathogenesis and possible early clinical clues to facilitate diagnosis. https://apcz.umk.pl/JEHS/article/view/39793retroperitoneal fibrosisOrmond's diseaseglucocorticosteroid therapyimmunosuppressive treatmentIgG4-related disease |
| spellingShingle | Sara Dankiewicz Sebastian Bróż Przemysław Żelazny Joanna Filipczak Aleksandra Swora Joanna Borowik Oliwer Sygacz Wojciech Brodowski Piotr Pawłowski Katarzyna Basta-Arciszewska Ormond's disease - the current state of knowledge about diagnosis and treatment Journal of Education, Health and Sport retroperitoneal fibrosis Ormond's disease glucocorticosteroid therapy immunosuppressive treatment IgG4-related disease |
| title | Ormond's disease - the current state of knowledge about diagnosis and treatment |
| title_full | Ormond's disease - the current state of knowledge about diagnosis and treatment |
| title_fullStr | Ormond's disease - the current state of knowledge about diagnosis and treatment |
| title_full_unstemmed | Ormond's disease - the current state of knowledge about diagnosis and treatment |
| title_short | Ormond's disease - the current state of knowledge about diagnosis and treatment |
| title_sort | ormond s disease the current state of knowledge about diagnosis and treatment |
| topic | retroperitoneal fibrosis Ormond's disease glucocorticosteroid therapy immunosuppressive treatment IgG4-related disease |
| url | https://apcz.umk.pl/JEHS/article/view/39793 |
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