Bilateral Renal Colic as an Initial Presentation of Erdheim-Chester Disease
Erdheim-Chester disease (ECD) is a rare non-Langerhans cells histiocytosis characterized by multiorgan involvement, with renal-ECD documented in over one-third of patients. Renal disease is generally asymptomatic, rarely causing hydronephrosis and kidney impairment. In addition, the diverse clinical...
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| Format: | Article |
| Language: | English |
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Wiley
2019-01-01
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| Series: | Case Reports in Urology |
| Online Access: | http://dx.doi.org/10.1155/2019/4670376 |
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| author | Julien Sarkis Fady Haddad Sarah Nasr Elie Hanna Ahmad Mroueh Elie Nemr |
| author_facet | Julien Sarkis Fady Haddad Sarah Nasr Elie Hanna Ahmad Mroueh Elie Nemr |
| author_sort | Julien Sarkis |
| collection | DOAJ |
| description | Erdheim-Chester disease (ECD) is a rare non-Langerhans cells histiocytosis characterized by multiorgan involvement, with renal-ECD documented in over one-third of patients. Renal disease is generally asymptomatic, rarely causing hydronephrosis and kidney impairment. In addition, the diverse clinical picture of Erdheim-Chester disease arises slowly with sequential manifestations. We present a rare case of a 75-year-old woman on long-term treatment for panhypopituitarism and steroid therapy for vasculitis, presenting to the emergency department with bilateral renal colic and acute kidney injury. Abdominopelvic CT scan revealed renal infiltration with signs of retroperitoneal fibrosis and hydronephrosis. Kidney CT-guided biopsy and 18-fluorodeoxyglucose (FDG) positron emission tomography whole body scan as well as the history of hypopituitarism and vasculitis confirmed the diagnosis of Erdheim-Chester disease. Proper therapy with interferon-α was started. This case describes the multifaced manifestation of this disease and the difficulty to establish the diagnosis, as well as the pivotal role that a urologist can play in its management. |
| format | Article |
| id | doaj-art-a5a6adce400448188ceb3af7e7ed9a3c |
| institution | Kabale University |
| issn | 2090-696X 2090-6978 |
| language | English |
| publishDate | 2019-01-01 |
| publisher | Wiley |
| record_format | Article |
| series | Case Reports in Urology |
| spelling | doaj-art-a5a6adce400448188ceb3af7e7ed9a3c2025-02-03T01:11:49ZengWileyCase Reports in Urology2090-696X2090-69782019-01-01201910.1155/2019/46703764670376Bilateral Renal Colic as an Initial Presentation of Erdheim-Chester DiseaseJulien Sarkis0Fady Haddad1Sarah Nasr2Elie Hanna3Ahmad Mroueh4Elie Nemr5Department of Urology, Hôtel-Dieu de France, Beirut, LebanonDepartment of Internal Medicine, Hôtel-Dieu de France, Beirut, LebanonDepartment of Pathology, Hôtel-Dieu de France, Beirut, LebanonDepartment of Urology, Hôtel-Dieu de France, Beirut, LebanonDepartment of Nephrology, Hôtel-Dieu de France, Beirut, LebanonDepartment of Urology, Hôtel-Dieu de France, Beirut, LebanonErdheim-Chester disease (ECD) is a rare non-Langerhans cells histiocytosis characterized by multiorgan involvement, with renal-ECD documented in over one-third of patients. Renal disease is generally asymptomatic, rarely causing hydronephrosis and kidney impairment. In addition, the diverse clinical picture of Erdheim-Chester disease arises slowly with sequential manifestations. We present a rare case of a 75-year-old woman on long-term treatment for panhypopituitarism and steroid therapy for vasculitis, presenting to the emergency department with bilateral renal colic and acute kidney injury. Abdominopelvic CT scan revealed renal infiltration with signs of retroperitoneal fibrosis and hydronephrosis. Kidney CT-guided biopsy and 18-fluorodeoxyglucose (FDG) positron emission tomography whole body scan as well as the history of hypopituitarism and vasculitis confirmed the diagnosis of Erdheim-Chester disease. Proper therapy with interferon-α was started. This case describes the multifaced manifestation of this disease and the difficulty to establish the diagnosis, as well as the pivotal role that a urologist can play in its management.http://dx.doi.org/10.1155/2019/4670376 |
| spellingShingle | Julien Sarkis Fady Haddad Sarah Nasr Elie Hanna Ahmad Mroueh Elie Nemr Bilateral Renal Colic as an Initial Presentation of Erdheim-Chester Disease Case Reports in Urology |
| title | Bilateral Renal Colic as an Initial Presentation of Erdheim-Chester Disease |
| title_full | Bilateral Renal Colic as an Initial Presentation of Erdheim-Chester Disease |
| title_fullStr | Bilateral Renal Colic as an Initial Presentation of Erdheim-Chester Disease |
| title_full_unstemmed | Bilateral Renal Colic as an Initial Presentation of Erdheim-Chester Disease |
| title_short | Bilateral Renal Colic as an Initial Presentation of Erdheim-Chester Disease |
| title_sort | bilateral renal colic as an initial presentation of erdheim chester disease |
| url | http://dx.doi.org/10.1155/2019/4670376 |
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