Kikuchi-Fujimoto Disease Complicated by Rheumatoid Arthritis, Type 1 Diabetes Mellitus, and Hypothyroidism

Division

Gulf Coast

Hospital

HCA Houston Healthcare Kingwood

Document Type

Case Report

Publication Date

1-7-2022

Keywords

autoimmune hypothyroidism, neutropenic fever, kikuchi-fujimoto disease, lymphadenopathy, rheumatoid arthritis, lymphoma, lymphoproliferative disorders

Disciplines

Immune System Diseases | Internal Medicine

Abstract

Kikuchi-Fujimoto disease (KFD) is a rare, benign, self-limited syndrome characterized by tender lymphadenopathy and low-grade fever. It may also present with rash, arthritis, fatigue, and splenomegaly. Data on the disease is limited, and its etiology remains largely unknown.

Here, we present the case of a 30-year-old female with a medical history of rheumatoid arthritis (RA), previously treated with etanercept, type 1 diabetes mellitus (DM-1), and Hashimoto’s hypothyroidism; she was brought in to an emergency department (ED) in Houston after a generalized tonic-clonic seizure and loss of consciousness. She was hypoglycemic, which was thought to have caused her DM-1 and seizure. CT scan of her chest showed multiple enlarged lymph nodes throughout the neck, superior mediastinum, and axilla, along with interstitial edema and bilateral pleural effusions. She was treated with dextrose drip and regained her consciousness. However, she had persistent pancytopenia, low-grade fever, and tender axillary lymphadenopathy. Infectious workup for tuberculosis (TB), human immunodeficiency virus (HIV), herpes simplex virus (HSV), Epstein-Barr virus (EBV), and parvovirus B-19 were negative. Her bone marrow biopsy revealed iron-deficiency anemia, while excisional axillary lymph node biopsy showed extensive necrosis consistent with KFD. She was treated with supportive care. Her neutrophilic fever resolved, and she was discharged home after 48-hours of remaining afebrile. Six months after her hospitalization, the patient remained well, and her complete blood count showed no abnormalities.

Due to the non-specific clinical features and laboratory findings of KFD, it is commonly misdiagnosed as infectious, autoimmune, or malignant lymphadenitis, leading to excessive diagnostic tests and unnecessary treatments. Physicians need to be cognizant of KFD and consider it in young patients presenting with tender lymphadenopathy, low-grade fevers, and leukopenia. To our best knowledge, this is the first reported case of a patient with concurrent RA, Hashimoto’s hypothyroidism, and KFD. This report elucidates the autoimmune nature of KFD and its association with other autoimmune diseases.

Publisher or Conference

Cureus

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