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Symptoms and clinical features <p>Patients suffering from Kikuchi–Fujimoto disease (KFD; histiocytic necrotizing lymphadenitis) develop fatigue, lymphadenopathy, night sweats, fever or myalgia and can even develop cutaneous manifestations such as erythema, exanthema or involvement of liver and bone marrow. In very rare cases it can also affect the central nervous system. The most important differential diagnoses are lymphoma and systemic lupus erythematosus.</p> Pathogenesis <p>The origin of the disease remains unclear, but hypotheses indicate infectious diseases as possible triggers of the autoimmune inflammatory condition. Other hypotheses suggest a&#xa0;role of T&#xa0;cells and plasmacytoid dendritic cells that induce a&#xa0;proinflammatory response of T&#xa0;cells, ultimately leading to autoimmune systemic disease. Yet further studies are necessary to reveal the cause, which appears to be a&#xa0;special challenge, as KFD is regarded an orphan disease.</p> Diagnosis <p>Patients may develop elevated C‑reactive protein, elevated erythrocyte sedimentation rate, and neutropenia. The laboratory findings are very unspecific and the gold standard for diagnosis is still histopathologic analysis of an affected lymph node.</p> Treatment <p>KFD is considered a&#xa0;self-limiting disease, but patients can be treated with nonsteroidal antirheumatic drugs, hydroxychloroquine, or glucocorticoids. Only a&#xa0;small percentage of patients experience recurrence. All patients, even after remission, should be observed over time to screen possible development of other autoimmune disease such as systemic lupus erythematosus.</p>

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Kikuchi-Fujimoto-Erkrankung (histiozytär-nekrotisierende Lymphadenopathie): eine seltene Systemerkrankung

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  • 发表日期：

    2025年6月24日

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  • Elisabeth Simader

摘要

Symptoms and clinical features

Patients suffering from Kikuchi–Fujimoto disease (KFD; histiocytic necrotizing lymphadenitis) develop fatigue, lymphadenopathy, night sweats, fever or myalgia and can even develop cutaneous manifestations such as erythema, exanthema or involvement of liver and bone marrow. In very rare cases it can also affect the central nervous system. The most important differential diagnoses are lymphoma and systemic lupus erythematosus.

Pathogenesis

The origin of the disease remains unclear, but hypotheses indicate infectious diseases as possible triggers of the autoimmune inflammatory condition. Other hypotheses suggest a role of T cells and plasmacytoid dendritic cells that induce a proinflammatory response of T cells, ultimately leading to autoimmune systemic disease. Yet further studies are necessary to reveal the cause, which appears to be a special challenge, as KFD is regarded an orphan disease.

Diagnosis

Patients may develop elevated C‑reactive protein, elevated erythrocyte sedimentation rate, and neutropenia. The laboratory findings are very unspecific and the gold standard for diagnosis is still histopathologic analysis of an affected lymph node.

Treatment

KFD is considered a self-limiting disease, but patients can be treated with nonsteroidal antirheumatic drugs, hydroxychloroquine, or glucocorticoids. Only a small percentage of patients experience recurrence. All patients, even after remission, should be observed over time to screen possible development of other autoimmune disease such as systemic lupus erythematosus.