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Internal Medicine

Training Level

Resident PGY 3


Henry Ford Macomb


A 26 year old African American female presented with the chief complaint of persistent fever and a single painful axillary lymph node for two weeks. This is a unique case of systemic lupus erythematous (SLE) presenting with painful lymphadenitis. Moreover, the result of her lymph node biopsy was also unusual, showing histiocytic necrotizing lymphadenitis (HNL) presenting in SLE. She presented with a fever of 103 degrees Fahrenheit and a single painful yet mobile axillary lymph node. She had no rash, joint tenderness, or hepatosplenomegaly on physical exam. Initial lab work showed absolute neutropenia and lymphopenia as well as normocytic anemia. There was initial concern for a possible infectious etiology due to the painful lymphadenopathy, thus broad spectrum antibiotics were initiated. Despite three days of antibiotics, the patient continued to have low grade fevers. HIV, CMV, and EBV testing was negative. Concern for malignancy led to an excisional lymph node biopsy. Additional lab testing that was pending prior to lymph node biopsy subsequently came back positive for ANA (antinuclear antibody) with titer >1:1280 in homogeneous pattern, anti-ds DNA antibody, and anti-Smith antibody, thus pointing towards SLE. She was subsequently started on high dose steroids with improvement of symptoms and her fevers resolved. Histiocytic necrotizing lymphadenitis (HNL) is a histologic feature most commonly associated with Kikuchi-Fujimoto Disease; there are few case reports that have shown an association between HNL and SLE [2, 3]. Given the strongly positive ANA, anti-ds DNA and anti-Smith antibodies, Kikuchi-Fujimoto Disease was deemed less likely and more consistent with SLE. Due to the variability of presentations and the vast differential that Lupus can encompass, Lupus can often be a difficult diagnosis to achieve. Four or more out of 11 criteria must be met of the American College of Rheumatology (ACR) criteria for classifying as possible systemic lupus diagnosis [1]. However none of these criteria include lymphadenopathy. In this case, the patient only met 3 criteria. Lymphadenopathy is seen in 12-59% of patient with SLE, however is often generalized and painless [4,5,6], thus making her presentation so rare. Due to the solitary lymph node that was painful, this initially pointed towards a possible infectious etiology. Also, Lupus can often be mistaken for malignancy; malignancy must be ruled out as patients with SLE are at an increased risk of developing non-Hodgkin lymphoma, Hodgkin lymphoma, and leukemia [1,7]. Therefore, even in patients who do not meet ACR criteria, SLE should still be investigated, if suspected, as patients may be presenting early in the disease course or in an atypical presentation.

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A Rare Presentation of Systemic Lupus Erythematous with Lymphadenitis