Dataset Information

Sub internal limiting membrane hemorrhage followed by bilateral optic disc hemorrhage in Kikuchi-Fujimoto disease: a case report.

ABSTRACT:

Background

Kikuchi-Fujimoto disease (KFD) is a necrotizing lymphadenitis, and presents fever of unknown origin and cervical lymphadenopathy. Ocular complications are unusual in KFD. Here we report a case of sub internal limiting membrane (ILM) hemorrhage followed by bilateral optic disc hemorrhage in KFD.

Case presentation

A 16-year-old Japanese man perceived a sudden decrease of right vision 3 days after onset of fever with unknown origin and left cervical lymphadenopathy. At presentation, visual acuity (VA) of right eye was 0.05 in decimal chart (1.30: converted to logarithm of minimum angle of resolution: logMAR). Fundus photograph showed extensive sub-ILM hemorrhage in right eye, and optic disc hemorrhages in both eyes. Fluorescein angiography presented hypo- and hyperfluorescences in optic disc of right eye, and hyperfluorescence in the disc of left eye. To make a definitive diagnosis, cervical lymph node biopsy was performed, and KFD was diagnosed pathologically. Thereafter, fever, headache and the cervical lymphadenopathy disappeared spontaneously. The sub-ILM hemorrhage was drained into the vitreous cavity by neodymium:yttrium-aluminum-garnet laser (Nd: YAG) hyaloidotomy. VA recovered to 1.5 (- 0.18: logMAR VA) in right eye.

Conclusion

Sub-ILM hemorrhage and optic disc hemorrhage are a KFD-related ocular complication.

SUBMITTER: Sato T

PROVIDER: S-EPMC8499551 | biostudies-literature |

REPOSITORIES: biostudies-literature

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Project description:Background Kikuchi-Fujimoto disease (KFD) is typically a benign, self-limiting inflammatory disease. However, some patients may have a prolonged or recurrent disease course, or present with life-threatening complications such as macrophage activation syndrome (MAS). In this study, we aimed to describe the incidence and clinical features of MAS in KFD and to access potential laboratory markers for the diagnosis of KFD-associated MAS. Methods Patients with KFD were retrospectively enrolled from January 2015 to November 2021 at Shenzhen Children’s Hospital. Clinical data were collected from inpatient or outpatient medical records. Data collected included clinical manifestations, laboratory and imaging findings, treatment, and clinical outcomes. Data were analyzed using GraphPad Prism 8.0 statistical software (GraphPad Software Inc., La Jolla, CA, USA). A receiver operating characteristic (ROC) curve analysis was further performed to access the potential predictors for the KFD-MAS diagnosis. Results Of 58 patients with a histological diagnosis of KFD, 15 (25.9%) patients had MAS. Compared to patients without MAS, patients with KFD-MAS presented with a higher proportion of skin rash (26.7%, p = 0.01), glucocorticoid treatment (80%, p = 0.003), and disease recurrence (33.3%, p = 0.04). KFD-MAS patients had lower absolute peripheral white blood cell (WBC, p = 0.02), platelet (p = 0.002), serum albumin levels (p = 0.01), and lymphocyte count (p < 0.0001), and higher lactate dehydrogenase (LDH) levels (p < 0.0001). ROC curve analysis showed that the cutoff values of absolute lymphocyte count, an absolute platelet count, serum albumin level, and serum LDH level for KFD-MAS diagnosis were < 1235/μL, < 171 × 106/μL, < 35.6 g/L, and > 679 IU/mL, respectively. Conclusions The presence of KFD-MAS in children may be more common than previously expected, especially in those with skin rash. KFD-MAS may be associated with a higher recurrence rate. An extremely elevated serum LDH level and moderate to severe lymphopenia may be useful diagnostic markers for MAS in KFD. Trial registration Not applicable; this was a retrospective study. Supplementary Information The online version contains supplementary material available at 10.1186/s12969-023-00788-w.