Löfgren syndrome, characteristics of Japanese cases: a case and a review of the literature.
Hirokazu TaguchiShuji SumitomoHideki OkaShigeo HaraKoichiro OhmuraPublished in: Modern rheumatology case reports (2024)
Löfgren syndrome (LS) is a sarcoidosis subtype characterized by an acute disease course, bilateral hilar lymphadenopathy, erythema nodosum, and ankle arthritis. LS in Caucasians appears to be self-limiting; however, our patients required glucocorticoid (GC) treatment. Here, we present a case of LS and review the literature to identify the characteristics of the Japanese patients with LS. Sixty-six-year-old woman was referred to our hospital; she initially presented with an acute onset of low-grade fever and ankle arthritis, followed by erythema nodosum. Skin biopsy revealed a non-caseating granuloma, and chest computed tomography scan displayed bilateral hilar lymphadenopathy; she was diagnosed with LS. Her arthralgia ameliorated spontaneously, but erythema persisted, necessitating GC treatment. Literature review revealed that the Japanese LS patients showed more fever, were more frequently treated with GC and more patients seemed to relapse, which may be explained by the absence of human leukocyte antigen-DR isotype 3, a good prognostic allele in Caucasians. Japanese LS may cause severe symptoms after development because of the differences in human leukocyte antigen from foreign countries. For early diagnosis, it is important to evaluate erythema nodosum and bilateral hilar lymphadenopathy in patients with polyarthritis involving ankle arthralgia.
Keyphrases
- fine needle aspiration
- end stage renal disease
- computed tomography
- low grade
- newly diagnosed
- case report
- ejection fraction
- chronic kidney disease
- systematic review
- prognostic factors
- endothelial cells
- liver failure
- healthcare
- mass spectrometry
- single cell
- high grade
- intensive care unit
- patient reported outcomes
- image quality
- early onset
- peripheral blood
- smoking cessation
- combination therapy
- soft tissue
- simultaneous determination
- sleep quality