We report the case of a 27 year old African-American man who presented with 6 months of generalized lymphadenopathy and nothing in his history or examination to suggest systemic lupus erythematosus. He was thought to have lymphoma, syphilis or tuberculosis and an extensive work up was done. Laboratory investigation finally revealed leukopenia (4.0), proteinuria (1.87grams), ANA (640 speckled), anti-dsDNA (640) , anticardiolipin IgG and IgM, anti-Smith, Coombs, anti-Ro, anti-La, CK (531U/L), aldolase (8.5 U/L), high erythrocyte sedimentation rate (130) and low complement (C3 15mg/dl and C4 3mg/dl). A kidney biopsy showed diffuse proliferative glomerulonephritis, ISN class IV. Generalized lymphadenopathy as the first and only manifestation for 6 months made the diagnosis of SLE challenging. Generalized diffuse lymphadenopathy has been associated with SLE, but is much less frequent now than in the past. The differential diagnosis of lymphadenopathy relevant to rheumatologists, includes Kikuchi histiocytic necrotizing lympadenitis, Castleman disease, syphilis, tuberculosis, sarcoidosis and lymphoma.
We report the case of a 25-year-old Iraqi woman who had multiple hospitalizations at an outside hospital for abdominal pain, nausea, and diarrhea without any evidence of systemic lupus erythematosus. Laboratory investigations finally showed a positive antinuclear antibody (1280), positive anti-dsDNA, anti-β2 glycoprotein I, low complement, positive Coombs tests, and leukopenia. A kidney biopsy showed ISN class II lupus nephritis. An ileal biopsy and angiogram were unremarkable. A computed tomography showed marked and dramatic bowel edema involving the small and large bowel (“target sign”), dilatation of intestinal segments, engorgement of mesenteric vessels (“comb sign”), and increased attenuation of mesenteric fat. These cardinal signs on computed tomography scan led to the correct diagnosis of lupus enteritis. Treatment was commenced with high-dose corticosteroids followed by mycophenolate mofetil, hydroxychloroquine, and then oral cyclophosphamide, but failed. The patient was eventually treated with the Euro-Lupus intravenous cyclophosphamide regimen, which resulted in significant clinical and radiological resolution.
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