A 16-year-old African American woman presented to an outside hospital with a 3-week history of fatigue, intermittent fevers, nausea, and vomiting and swelling on the right side of the neck a week prior to presentation. She was treated with intravenous fluids and antibiotics but continued to have fever up to 101.7°F, and the swelling in the neck had enlarged. Review of systems was significant for decreased appetite, a 6-lb weight loss, night sweats, and headaches. She denied any rash, oral ulcers, visual changes, chest pain, dyspnea, dysurea, or diarrhea. Her past medical history and family history were noncontributory except for a distant relative in her father's family having lupus.On physical exam, the vital signs were significant for temperature of 101.4°F, blood pressure of 131/86, and heart rate of 96. A swollen (3 × 4 cm 2 ), tender right anterior cervical lymph node, in addition to several smaller lymph nodes, were seen in the same region. Her joints showed no signs of swelling or arthritis. Laboratory analysis revealed leukopenia with a white blood cell count of 3.5 × 10 3 /µL (3.7-11.7), a hemoglobin level of 8.3 g/dL (10.2-14.0), a hematocrit of 25% (31%-42%), and platelets count of 124 × 10 3 /µL (135-450). Her creatinine level was 1.3 mg/dL (0.1-1.4); serum lactate dehydrogenase (LDH) 267 mg/dL, and uric acid 5.7 mg/dL. Erythrocyte sedimentation rate and C-reactive protein were elevated at 135 mm/h and 13 mg/dL, respectively. Urine analysis showed 15 to 19 red blood cells/hpf, and 1+ protein.Blood and urine cultures were negative. Epstein-Barr virus and cytomegalovirus titers were normal. HIV and hepatitis panel were negative. Purified protein derivative (PPD) skin test was negative. The chest radiograph and CT scan of the abdomen were normal. Ophthalmological consult showed no intraocular inflammatory changes.Because malignancy was still a strong possibility, an excisional biopsy of the right cervical lymph node was done, which showed necrotizing lymphadenitis with stellate microabscesses harboring prominent neutrophilic infiltrates surrounded by fibrin and an ill-defined histiocytic rim, outside of which were lymphocytes (Figure 1). Lymph node acid fast bacillus (AFB) stain, bacterial cultures, and fungal cultures were negative. Based on the biopsy results, atypical Kikuchi-Fujimoto's disease (KFD) alone versus KFD associated with an underlying autoimmune disease was suspected. Further testing showed positive antinuclear antibodies (ANA) with a titer of 1:2560, positive antibodies to ds-DNA, Smith, RNP, and SSA antibodies. The SSB, SCL-70, and Jo-1 antibodies were negative. Serum was high, at IgG 2460 mg/dL; C3 and C4 levels were low, at 17.9 and 3.3 mg/dL, respectively. ACE levels were normal. Renal biopsy was performed to evaluate the hematuria and showed class IV-S-namely, diffuse segmental proliferative lupus nephritis.Based on the clinical features and laboratory findings, our patient fulfilled 4 of the 11 necessary American College of Rheumatology criteria for the diagnosis of systemic lupus e...

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