Background: Massive generalized lymphadenopathy (MGL) as an initial manifestation of systemic lupus erythematosus (SLE) is rare. The Case: A previously healthy 18-year-old man presented with MGL for 1 month. Subsequently, he developed fever, hypertension, fluid overload, and left abdominal pain. He had hemoglobin 89 g/L, serum creatinine 168 umol/L, serum albumin 29 g/L, prolonged activated partial thromboplastin time, heavy proteinuria, and hematuria. Fused PET/CT scanning showed hypermetabolic peripheral and internal MGL with splenic and right kidney infarctions. Autoimmune tests showed very low serum complements 3 and 4 with high titers of ANA 1/640 (N: 1/40) and anti-dsDNA 666 IU/ml (N: < 10). Serological tests for tuberculosis, syphilis, and Epstein-Barr virus were negative. Tests for hypercoagulable states were negative except for lupus anticoagulant and anti-beta2 glycoprotein I antibodies. Lymph node biopsy showed partial effacement of nodal architecture by areas of necrosis surrounded by extensive T-lymphocytic infiltration that lacked vasculitis, caseation, and granulomas. Moreover, his kidney biopsy showed class IV lupus nephritis. Initially, he was treated with intravenous Solumedrol followed by Prednisone as well as Mycophenolate mofetil for a total of 3 months. Subsequently, he received Rituximab infusion to be on a yearly basis. For his coagulopathy, he received Apixaban. He improved within 1 month. By 1 year of follow-up, he remained clinically stable and with normal laboratory as well as radiological testing. In Conclusion: SLE can present with MGL.
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