Rare Manifestation of a Rare Disease, Acute Liver Failure in Adult Onset Still’s Disease: Dramatic Response to Methylprednisolone Pulse Therapy—A Case Report and Review
Adult onset Still’s disease (AOSD) is a rare systemic inflammatory disorder of unknown etiology. It is characterized by daily fevers, arthralgias or arthritis, typical skin rash, and leukocytosis. Hepatic involvement is frequently observed in the course of AOSD with mildly elevated transaminases and...
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Wiley
2014-01-01
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| Series: | Case Reports in Medicine |
| Online Access: | http://dx.doi.org/10.1155/2014/375035 |
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| author | Nalini Valluru Venkata S. Tammana Michael Windham Eyasu Mekonen Rehana Begum Andrew Sanderson |
| author_facet | Nalini Valluru Venkata S. Tammana Michael Windham Eyasu Mekonen Rehana Begum Andrew Sanderson |
| author_sort | Nalini Valluru |
| collection | DOAJ |
| description | Adult onset Still’s disease (AOSD) is a rare systemic inflammatory disorder of unknown etiology. It is characterized by daily fevers, arthralgias or arthritis, typical skin rash, and leukocytosis. Hepatic involvement is frequently observed in the course of AOSD with mildly elevated transaminases and/or hepatomegaly. Fulminant hepatic failure, occasionally requiring urgent liver transplantation, is a rare manifestation of AOSD. Here, we present a case of 22-year-old woman with no significant medical history who initially came with fever, arthralgias, myalgias, generalized weakness, and sore throat. Laboratory data showed mildly elevated transaminases and markedly elevated ferritin levels. She was diagnosed with AOSD based on Yamaguchi diagnostic criteria and was started on prednisone. Three months later, while she was on tapering dose of steroid, she presented with fever, abdominal pain, jaundice, and markedly elevated transaminases. Extensive workup excluded all potential causes of liver failure. She was diagnosed with AOSD associated acute liver failure (ALF). Intravenous (IV) methylprednisolone pulse therapy was started, with dramatic improvement in liver function. Our case demonstrated that ALF can present as a complication of AOSD and IV mega dose pulse methylprednisolone therapy can be employed as a first-line treatment in AOSD associated ALF with favorable outcome. |
| format | Article |
| id | doaj-art-b8d19f1799fa45ea8f2ff89400ba07c2 |
| institution | Kabale University |
| issn | 1687-9627 1687-9635 |
| language | English |
| publishDate | 2014-01-01 |
| publisher | Wiley |
| record_format | Article |
| series | Case Reports in Medicine |
| spelling | doaj-art-b8d19f1799fa45ea8f2ff89400ba07c22025-02-03T01:30:55ZengWileyCase Reports in Medicine1687-96271687-96352014-01-01201410.1155/2014/375035375035Rare Manifestation of a Rare Disease, Acute Liver Failure in Adult Onset Still’s Disease: Dramatic Response to Methylprednisolone Pulse Therapy—A Case Report and ReviewNalini Valluru0Venkata S. Tammana1Michael Windham2Eyasu Mekonen3Rehana Begum4Andrew Sanderson5Department of Internal Medicine, Division of Gastroenterology and Hepatology, Virginia Commonwealth University, West Hospital, 14th Floor, 1200 East Broad Street, P.O. Box 980341, Richmond, VA 23298, USADepartment of Internal Medicine, Division of Gastroenterology and Hepatology, Howard University Hospital, 2041 Georgia Avenue NW, Washington, DC 20060, USADepartment of Internal Medicine, Division of Gastroenterology and Hepatology, Howard University Hospital, 2041 Georgia Avenue NW, Washington, DC 20060, USADepartment of Internal Medicine, Division of Gastroenterology and Hepatology, Howard University Hospital, 2041 Georgia Avenue NW, Washington, DC 20060, USADepartment of Internal Medicine, Division of Gastroenterology and Hepatology, Howard University Hospital, 2041 Georgia Avenue NW, Washington, DC 20060, USADepartment of Internal Medicine, Division of Gastroenterology and Hepatology, Howard University Hospital, 2041 Georgia Avenue NW, Washington, DC 20060, USAAdult onset Still’s disease (AOSD) is a rare systemic inflammatory disorder of unknown etiology. It is characterized by daily fevers, arthralgias or arthritis, typical skin rash, and leukocytosis. Hepatic involvement is frequently observed in the course of AOSD with mildly elevated transaminases and/or hepatomegaly. Fulminant hepatic failure, occasionally requiring urgent liver transplantation, is a rare manifestation of AOSD. Here, we present a case of 22-year-old woman with no significant medical history who initially came with fever, arthralgias, myalgias, generalized weakness, and sore throat. Laboratory data showed mildly elevated transaminases and markedly elevated ferritin levels. She was diagnosed with AOSD based on Yamaguchi diagnostic criteria and was started on prednisone. Three months later, while she was on tapering dose of steroid, she presented with fever, abdominal pain, jaundice, and markedly elevated transaminases. Extensive workup excluded all potential causes of liver failure. She was diagnosed with AOSD associated acute liver failure (ALF). Intravenous (IV) methylprednisolone pulse therapy was started, with dramatic improvement in liver function. Our case demonstrated that ALF can present as a complication of AOSD and IV mega dose pulse methylprednisolone therapy can be employed as a first-line treatment in AOSD associated ALF with favorable outcome.http://dx.doi.org/10.1155/2014/375035 |
| spellingShingle | Nalini Valluru Venkata S. Tammana Michael Windham Eyasu Mekonen Rehana Begum Andrew Sanderson Rare Manifestation of a Rare Disease, Acute Liver Failure in Adult Onset Still’s Disease: Dramatic Response to Methylprednisolone Pulse Therapy—A Case Report and Review Case Reports in Medicine |
| title | Rare Manifestation of a Rare Disease, Acute Liver Failure in Adult Onset Still’s Disease: Dramatic Response to Methylprednisolone Pulse Therapy—A Case Report and Review |
| title_full | Rare Manifestation of a Rare Disease, Acute Liver Failure in Adult Onset Still’s Disease: Dramatic Response to Methylprednisolone Pulse Therapy—A Case Report and Review |
| title_fullStr | Rare Manifestation of a Rare Disease, Acute Liver Failure in Adult Onset Still’s Disease: Dramatic Response to Methylprednisolone Pulse Therapy—A Case Report and Review |
| title_full_unstemmed | Rare Manifestation of a Rare Disease, Acute Liver Failure in Adult Onset Still’s Disease: Dramatic Response to Methylprednisolone Pulse Therapy—A Case Report and Review |
| title_short | Rare Manifestation of a Rare Disease, Acute Liver Failure in Adult Onset Still’s Disease: Dramatic Response to Methylprednisolone Pulse Therapy—A Case Report and Review |
| title_sort | rare manifestation of a rare disease acute liver failure in adult onset still s disease dramatic response to methylprednisolone pulse therapy a case report and review |
| url | http://dx.doi.org/10.1155/2014/375035 |
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