Adult-onset Still′s disease with myocarditis and hemophagocytic lymphohistiocytosis: Rare manifestation with fatal outcome
Adult-onset Still′s disease (AOSD) is a rare inflammatory disorder of unknown etiology characterized by fever, evanescent pink salmon rash, arthritis, and multiorgan involvement. Here, we report an unusual manifestation of AOSD in a 40-year-old male who presented to our hospital with pyrexia of unkn...
| Main Authors: | , , , |
|---|---|
| Format: | Article |
| Language: | English |
| Published: |
Wolters Kluwer Medknow Publications
2016-01-01
|
| Series: | Indian Journal of Pathology and Microbiology |
| Subjects: | |
| Online Access: | http://www.ijpmonline.org/article.asp?issn=0377-4929;year=2016;volume=59;issue=1;spage=84;epage=86;aulast=Gupta |
| _version_ | 1818253076368195584 |
|---|---|
| author | Devika Gupta Rajat Jagani Satish Mendonca Khushi Ram Rathi |
| author_facet | Devika Gupta Rajat Jagani Satish Mendonca Khushi Ram Rathi |
| author_sort | Devika Gupta |
| collection | DOAJ |
| description | Adult-onset Still′s disease (AOSD) is a rare inflammatory disorder of unknown etiology characterized by fever, evanescent pink salmon rash, arthritis, and multiorgan involvement. Here, we report an unusual manifestation of AOSD in a 40-year-old male who presented to our hospital with pyrexia of unknown origin and rash of 3 weeks duration. All his serological investigations and imaging studies were unremarkable. He was fulfilling clinical and laboratory criteria as per Yamaguchi for AOSD and was managed for the same. Our patient did not respond well to the treatment, had a downhill course, and succumbed to his illness. Autopsy confirmed myocarditis and florid bone marrow reactive hemophagocytosis as the cause of his death. |
| first_indexed | 2024-12-12T16:34:19Z |
| format | Article |
| id | doaj.art-2c3cabe5ea084dc69eb17d5cdafc5cd3 |
| institution | Directory Open Access Journal |
| issn | 0377-4929 |
| language | English |
| last_indexed | 2024-12-12T16:34:19Z |
| publishDate | 2016-01-01 |
| publisher | Wolters Kluwer Medknow Publications |
| record_format | Article |
| series | Indian Journal of Pathology and Microbiology |
| spelling | doaj.art-2c3cabe5ea084dc69eb17d5cdafc5cd32022-12-22T00:18:41ZengWolters Kluwer Medknow PublicationsIndian Journal of Pathology and Microbiology0377-49292016-01-01591848610.4103/0377-4929.174825Adult-onset Still′s disease with myocarditis and hemophagocytic lymphohistiocytosis: Rare manifestation with fatal outcomeDevika GuptaRajat JaganiSatish MendoncaKhushi Ram RathiAdult-onset Still′s disease (AOSD) is a rare inflammatory disorder of unknown etiology characterized by fever, evanescent pink salmon rash, arthritis, and multiorgan involvement. Here, we report an unusual manifestation of AOSD in a 40-year-old male who presented to our hospital with pyrexia of unknown origin and rash of 3 weeks duration. All his serological investigations and imaging studies were unremarkable. He was fulfilling clinical and laboratory criteria as per Yamaguchi for AOSD and was managed for the same. Our patient did not respond well to the treatment, had a downhill course, and succumbed to his illness. Autopsy confirmed myocarditis and florid bone marrow reactive hemophagocytosis as the cause of his death.http://www.ijpmonline.org/article.asp?issn=0377-4929;year=2016;volume=59;issue=1;spage=84;epage=86;aulast=GuptaAdult-onset Still′s diseasehemophagocytic lymphohistiocytosismyocarditis |
| spellingShingle | Devika Gupta Rajat Jagani Satish Mendonca Khushi Ram Rathi Adult-onset Still′s disease with myocarditis and hemophagocytic lymphohistiocytosis: Rare manifestation with fatal outcome Indian Journal of Pathology and Microbiology Adult-onset Still′s disease hemophagocytic lymphohistiocytosis myocarditis |
| title | Adult-onset Still′s disease with myocarditis and hemophagocytic lymphohistiocytosis: Rare manifestation with fatal outcome |
| title_full | Adult-onset Still′s disease with myocarditis and hemophagocytic lymphohistiocytosis: Rare manifestation with fatal outcome |
| title_fullStr | Adult-onset Still′s disease with myocarditis and hemophagocytic lymphohistiocytosis: Rare manifestation with fatal outcome |
| title_full_unstemmed | Adult-onset Still′s disease with myocarditis and hemophagocytic lymphohistiocytosis: Rare manifestation with fatal outcome |
| title_short | Adult-onset Still′s disease with myocarditis and hemophagocytic lymphohistiocytosis: Rare manifestation with fatal outcome |
| title_sort | adult onset still s disease with myocarditis and hemophagocytic lymphohistiocytosis rare manifestation with fatal outcome |
| topic | Adult-onset Still′s disease hemophagocytic lymphohistiocytosis myocarditis |
| url | http://www.ijpmonline.org/article.asp?issn=0377-4929;year=2016;volume=59;issue=1;spage=84;epage=86;aulast=Gupta |
| work_keys_str_mv | AT devikagupta adultonsetstillsdiseasewithmyocarditisandhemophagocyticlymphohistiocytosisraremanifestationwithfataloutcome AT rajatjagani adultonsetstillsdiseasewithmyocarditisandhemophagocyticlymphohistiocytosisraremanifestationwithfataloutcome AT satishmendonca adultonsetstillsdiseasewithmyocarditisandhemophagocyticlymphohistiocytosisraremanifestationwithfataloutcome AT khushiramrathi adultonsetstillsdiseasewithmyocarditisandhemophagocyticlymphohistiocytosisraremanifestationwithfataloutcome |