ArticleJournal of Korean medical science2013
Extensive thrombosis in a patient with familial Mediterranean fever, despite hyperimmunoglobulin D state in serum. [corrected].
Article in Journal of Korean medical science, 2013. The graph could read no effect estimate from its abstract, so it casts no vote on the map. An erratum has been issued. Cited by 7 papers.
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Who cites it
7 citing papers in PubMed, 8 citations in OpenAlex.
- The clinical significance of the Mediterranean fever gene MEFV variants in Castleman disease.Communications medicine · 2026Article
- Identification of pathogenicJournal of rheumatic diseases · 2025Article
- Severe FMF Presentation with Rare Association of MEFV Variants (p.Pro369Ser/p.Glu148Gln).European journal of case reports in internal medicine · 2019Article
- A novel single variant in the MEFV gene causing Mediterranean fever and Behçet's disease: a case report.Journal of medical case reports · 2018Article
- Familial Mediterranean fever presenting as fever of unknown origin in Korea.Korean journal of pediatrics · 2016Article
- Familial Mediterranean Fever With Complete Symptomatic Remission During Pregnancy.Intestinal research · 2015Article
- Autoinflammatory diseases: a possible cause of thrombosis?Thrombosis journal · 2015Article
Corrections and comments
- Erratum issued
Authors and funding
7 authors at 2 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Familial Mediterranean fever (FMF) is an autosomal recessive disease characterized by recurrent episodes of fever accompanied by peritonitis, pleuritis, arthritis, or erysipelas-like erythema. It is known to occur mainly among Mediterranean and Middle Eastern populations such as non-Ashkenazi Jews, Arabs, Turks, and Armenians. FMF is not familiar to clinicians beyond this area and diagnosing FMF can be challenging. We report a 22-yr old boy who presented with fever, arthalgia and abdominal pain. He had a history of recurrent episodes of fever associated with arthalgia which would subside spontaneously or by antipyretics. Autosomal recessive periodic fever syndromes were suspected. Immunoglobulin D (IgD) level in the serum was elevated and DNA analysis showed complex mutations (p.Glu148Gln, p.Pro369Ser, p.Arg408Gln) in the MEFV gene. 3D angio computed tomography showed total thrombosis of splenic vein with partial thrombosis of proximal superior mesenteric vein, main portal vein and intrahepatic both portal vein. This is a case of FMF associated with multiple venous thrombosis and elevated IgD level. When thrombosis is associated with elevated IgD, FMF should be suspected. This is the first adult case reported in Korea.
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