Pulmonary Arterial Hypertension in Adult-Onset Still’s Disease: Rapid Response to Anakinra
Adult-onset Still’s disease (AOSD) is a rare inflammatory condition characterized by spiking quotidian fever, rash, chronic arthralgia, leukocytosis, and occasional pulmonary involvement such as pleural effusion and transient pulmonary infiltrates. Pulmonary arterial hypertension (PAH) is a rare pul...
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| Format: | Article |
| Language: | English |
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Wiley
2012-01-01
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| Series: | Case Reports in Rheumatology |
| Online Access: | http://dx.doi.org/10.1155/2012/537613 |
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| author | Marc Campos Elena Schiopu |
| author_facet | Marc Campos Elena Schiopu |
| author_sort | Marc Campos |
| collection | DOAJ |
| description | Adult-onset Still’s disease (AOSD) is a rare inflammatory condition characterized by spiking quotidian fever, rash, chronic arthralgia, leukocytosis, and occasional pulmonary involvement such as pleural effusion and transient pulmonary infiltrates. Pulmonary arterial hypertension (PAH) is a rare pulmonary complication of AOSD, and we are aware of only 5 cases reported in the literature. We report the case of a 27-year-old woman of Middle Eastern descent, with a 7-year history of AOSD, who developed severe pulmonary arterial hypertension (PAH). After unsuccessful exposure to various immunosuppressive regimens, shortly following the initiation of anakinra, an interleukin-1 (IL-1) receptor antagonist, her disease became quiescent and the PAH resolved. With this case report, we hope to show that anakinra, either by virtue of controlling the overall inflammation in AOSD, or by direct effect on the pulmonary microangiopathy, can improve severe PAH. |
| format | Article |
| id | doaj-art-388ceb1ef03045c3be088499f9d0b6dd |
| institution | Kabale University |
| issn | 2090-6889 2090-6897 |
| language | English |
| publishDate | 2012-01-01 |
| publisher | Wiley |
| record_format | Article |
| series | Case Reports in Rheumatology |
| spelling | doaj-art-388ceb1ef03045c3be088499f9d0b6dd2025-02-03T05:54:16ZengWileyCase Reports in Rheumatology2090-68892090-68972012-01-01201210.1155/2012/537613537613Pulmonary Arterial Hypertension in Adult-Onset Still’s Disease: Rapid Response to AnakinraMarc Campos0Elena Schiopu1Division of Rheumatology, University of Michigan, P.O. Box 481, Ann Arbor, MI 48109, USADivision of Rheumatology, University of Michigan, P.O. Box 481, Ann Arbor, MI 48109, USAAdult-onset Still’s disease (AOSD) is a rare inflammatory condition characterized by spiking quotidian fever, rash, chronic arthralgia, leukocytosis, and occasional pulmonary involvement such as pleural effusion and transient pulmonary infiltrates. Pulmonary arterial hypertension (PAH) is a rare pulmonary complication of AOSD, and we are aware of only 5 cases reported in the literature. We report the case of a 27-year-old woman of Middle Eastern descent, with a 7-year history of AOSD, who developed severe pulmonary arterial hypertension (PAH). After unsuccessful exposure to various immunosuppressive regimens, shortly following the initiation of anakinra, an interleukin-1 (IL-1) receptor antagonist, her disease became quiescent and the PAH resolved. With this case report, we hope to show that anakinra, either by virtue of controlling the overall inflammation in AOSD, or by direct effect on the pulmonary microangiopathy, can improve severe PAH.http://dx.doi.org/10.1155/2012/537613 |
| spellingShingle | Marc Campos Elena Schiopu Pulmonary Arterial Hypertension in Adult-Onset Still’s Disease: Rapid Response to Anakinra Case Reports in Rheumatology |
| title | Pulmonary Arterial Hypertension in Adult-Onset Still’s Disease: Rapid Response to Anakinra |
| title_full | Pulmonary Arterial Hypertension in Adult-Onset Still’s Disease: Rapid Response to Anakinra |
| title_fullStr | Pulmonary Arterial Hypertension in Adult-Onset Still’s Disease: Rapid Response to Anakinra |
| title_full_unstemmed | Pulmonary Arterial Hypertension in Adult-Onset Still’s Disease: Rapid Response to Anakinra |
| title_short | Pulmonary Arterial Hypertension in Adult-Onset Still’s Disease: Rapid Response to Anakinra |
| title_sort | pulmonary arterial hypertension in adult onset still s disease rapid response to anakinra |
| url | http://dx.doi.org/10.1155/2012/537613 |
| work_keys_str_mv | AT marccampos pulmonaryarterialhypertensioninadultonsetstillsdiseaserapidresponsetoanakinra AT elenaschiopu pulmonaryarterialhypertensioninadultonsetstillsdiseaserapidresponsetoanakinra |