NECROTIZING LYMPHADENITIS ASSOCIATED WITH SYSTEMIC LUPUS-ERYTHEMATOSUS

Objective: Systemic lupus erythematosus (SLE) may have protean manifes tations, including necrotizing lymphadenitis. After describing an illu strative case, we discuss the incidence, clinical features, and pathol ogic findings of SLE-associated necrotizing lymphadenitis. Methods: A case of SLE associated with necrotizing lymphadenitis is reported. The patient's clinical presentation, course, and response to therapy is d etailed. The literature on lupus lymphadenitis is reviewed. Results: A young man who presented with a febrile illness characterized by multi focal necrotizing lymphadenitis is described. Glomerulonephritis, meni ngoencephalitis, pericarditis, and hemolytic anemia evolved. The diagn osis of SLE was based on the clinical features, positive antinuclear a ntibody (ANA), and characteristic renal biopsy. High dose corticostero ids and cyclophosphamide induced a complete remission. In recent serie s from the literature the prevalence of lymphadenopathy was 12% to 59% of patients with SLE. The most common nodal groups involved were cerv ical (43%), mesenteric (21%), axillary (18%), and inguinal (17%). Lymp h node pathology was characterized by paracortical foci of necrosis an d infiltration by histiocytes, lymphocytes, plasma cells, and immunobl asts. The hematoxylin body, an amorphic aggregate of basophilic materi al, was pathognomonic of lupus lymphadenitis. The necrotizing lymphade nitis of SLE is pathologically similar to Kikuchi-Fujumoto disease (KF D), a distinctive, self-limited form of necrotizing lymphadenitis. The pathologic and clinical literature support a close link between SLE a nd KFD. Conclusions: SLE can be complicated by necrotizing lymphadenit is, with distinctive pathologic features. Lupus lymphadenitis and KFD share some common clinical and pathologic features, supporting a relat ionship between the disorders. Copyright (C) 1996 by W.B. Saunders Com pany