Kikuchi-Fujimoto Disease Preceding Overlap Syndrome of Sjögren’s Syndrome and Systemic Lupus Erythematosus: Literature Review Based on a Case Report
Kikuchi-Fujimoto disease (KFD) is a benign, self-limiting histiocytic necrotizing lymphadenitis systemic disorder with unknown etiology. KFD has been known for half a century, but difficulties in distinguishing it remain. Its diagnostic significance is related to the increasing prevalence of KFD wi...
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Interna Publishing
2024-07-01
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| Series: | Acta Medica Indonesiana |
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| Online Access: | http://www.actamedindones.org/index.php/ijim/article/view/2371 |
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| author | Cindy Cindy Suryo Anggoro Kusumo Wibowo Anna Ariane Rudy Hidayat |
| author_facet | Cindy Cindy Suryo Anggoro Kusumo Wibowo Anna Ariane Rudy Hidayat |
| author_sort | Cindy Cindy |
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Kikuchi-Fujimoto disease (KFD) is a benign, self-limiting histiocytic necrotizing lymphadenitis systemic disorder with unknown etiology. KFD has been known for half a century, but difficulties in distinguishing it remain. Its diagnostic significance is related to the increasing prevalence of KFD with autoimmune diseases in various timeframes. Systemic lupus erythematosus (SLE) is the most prevalent autoimmune connective tissue disease (AICTD) appearing alongside KFD. An 18-year-old female presented with acute muscle weakness, shortness of breath, fever, and significant weight loss for 5 months before admission. Pain and morning joint stiffness had been felt for 9 months. One year ago, she lumped her right neck and was diagnosed with KFD from the excision biopsy and immunohistochemical staining (CD68). Creatine-kinase enzymes and C-Reactive protein were elevated with a high anti-Ku and anti-Jo-1 negative level. There was a low level of complements, high anti-nuclear antibody titer, with positive anti-SS-A. Sialometry and Schirmer test showed reduced salivary and lacrimal gland production. We diagnosed this patient as having an overlap syndrome preceded by KFD. The AICTD involved was Sjögren’s syndrome and SLE. Although KFD is considered a self-limiting disease, its occurrence should be noticed regarding the possibility of other autoimmune conditions. KFD usually coincides with AICTD, although it could also precede or occur afterward. This case is reported to raise awareness of the overlap syndrome preceded by KFD.
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| format | Article |
| id | doaj-art-91b250959e8b42f1b6490f394b97d81c |
| institution | Matheson Library |
| issn | 0125-9326 2338-2732 |
| language | English |
| publishDate | 2024-07-01 |
| publisher | Interna Publishing |
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| series | Acta Medica Indonesiana |
| spelling | doaj-art-91b250959e8b42f1b6490f394b97d81c2025-07-14T07:00:22ZengInterna PublishingActa Medica Indonesiana0125-93262338-27322024-07-01562Kikuchi-Fujimoto Disease Preceding Overlap Syndrome of Sjögren’s Syndrome and Systemic Lupus Erythematosus: Literature Review Based on a Case ReportCindy Cindy0Suryo Anggoro Kusumo Wibowo1Anna Ariane2Rudy Hidayat3Fellow of Rheumatology, Division of Rheumatology, Department of Internal Medicine, Faculty of Medicine Universitas Indonesia – Dr. Cipto Mangunkusumo Hospital, Jakarta, Indonesia.Fellow of Rheumatology, Division of Rheumatology, Department of Internal Medicine, Faculty of Medicine Universitas Indonesia – Dr. Cipto Mangunkusumo Hospital, Jakarta, Indonesia.Division of Rheumatology, Department of In ternal Medicine, Faculty of Medicine Universitas Indonesia - Cipto Mangunkusumo Hospital, Jakarta, Indonesia.Division of Rheumatology, Department of In ternal Medicine, Faculty of Medicine Universitas Indonesia - Cipto Mangunkusumo Hospital, Jakarta, Indonesia. Kikuchi-Fujimoto disease (KFD) is a benign, self-limiting histiocytic necrotizing lymphadenitis systemic disorder with unknown etiology. KFD has been known for half a century, but difficulties in distinguishing it remain. Its diagnostic significance is related to the increasing prevalence of KFD with autoimmune diseases in various timeframes. Systemic lupus erythematosus (SLE) is the most prevalent autoimmune connective tissue disease (AICTD) appearing alongside KFD. An 18-year-old female presented with acute muscle weakness, shortness of breath, fever, and significant weight loss for 5 months before admission. Pain and morning joint stiffness had been felt for 9 months. One year ago, she lumped her right neck and was diagnosed with KFD from the excision biopsy and immunohistochemical staining (CD68). Creatine-kinase enzymes and C-Reactive protein were elevated with a high anti-Ku and anti-Jo-1 negative level. There was a low level of complements, high anti-nuclear antibody titer, with positive anti-SS-A. Sialometry and Schirmer test showed reduced salivary and lacrimal gland production. We diagnosed this patient as having an overlap syndrome preceded by KFD. The AICTD involved was Sjögren’s syndrome and SLE. Although KFD is considered a self-limiting disease, its occurrence should be noticed regarding the possibility of other autoimmune conditions. KFD usually coincides with AICTD, although it could also precede or occur afterward. This case is reported to raise awareness of the overlap syndrome preceded by KFD. http://www.actamedindones.org/index.php/ijim/article/view/2371Kikuchi-Fujimoto diseaseOverlap SyndromeSjögren’s SyndromeSystemic Lupus ErythematosusInflammatory Myositis |
| spellingShingle | Cindy Cindy Suryo Anggoro Kusumo Wibowo Anna Ariane Rudy Hidayat Kikuchi-Fujimoto Disease Preceding Overlap Syndrome of Sjögren’s Syndrome and Systemic Lupus Erythematosus: Literature Review Based on a Case Report Acta Medica Indonesiana Kikuchi-Fujimoto disease Overlap Syndrome Sjögren’s Syndrome Systemic Lupus Erythematosus Inflammatory Myositis |
| title | Kikuchi-Fujimoto Disease Preceding Overlap Syndrome of Sjögren’s Syndrome and Systemic Lupus Erythematosus: Literature Review Based on a Case Report |
| title_full | Kikuchi-Fujimoto Disease Preceding Overlap Syndrome of Sjögren’s Syndrome and Systemic Lupus Erythematosus: Literature Review Based on a Case Report |
| title_fullStr | Kikuchi-Fujimoto Disease Preceding Overlap Syndrome of Sjögren’s Syndrome and Systemic Lupus Erythematosus: Literature Review Based on a Case Report |
| title_full_unstemmed | Kikuchi-Fujimoto Disease Preceding Overlap Syndrome of Sjögren’s Syndrome and Systemic Lupus Erythematosus: Literature Review Based on a Case Report |
| title_short | Kikuchi-Fujimoto Disease Preceding Overlap Syndrome of Sjögren’s Syndrome and Systemic Lupus Erythematosus: Literature Review Based on a Case Report |
| title_sort | kikuchi fujimoto disease preceding overlap syndrome of sjogren s syndrome and systemic lupus erythematosus literature review based on a case report |
| topic | Kikuchi-Fujimoto disease Overlap Syndrome Sjögren’s Syndrome Systemic Lupus Erythematosus Inflammatory Myositis |
| url | http://www.actamedindones.org/index.php/ijim/article/view/2371 |
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