Necrotizing lymphadenitis associated with systemic lupus erythematosus

Abstract

Objective: Systemic lupus erythematosus (SLE) may have protean manifestations, including necrotizing lymphadenitis. After describing an illustrative case, we discuss the incidence, clinical features, and pathologic 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 detailed. The literature on lupus lymphadenitis is reviewed. Results: A young man who presented with a febrile illness characterized by multifocal necrotizing lymphadenitis is described. Glomerulonephritis, meningo-encephalitis, pericarditis, and hemolytic anemia evolved. The diagnosis of SLE was based on the clinical features, positive antinuclear antibody (ANA), and characteristic renal biopsy. High dose corticosteroids and cyclophosphamide induced a complete remission. In recent series from the literature the prevalence of lymphadenopathy was 12% to 59% of patients with SLE. The most common nodal groups involved were cervical (43%), mesenteric (21 %), axillary (18%), and inguinal (17%). Lymph node pathology was characterized by paracortical foci of necrosis and infiltration by histiocytes, lymphocytes, plasma cells, and immunoblasts. The hematoxylin body, an amorphic aggregate of basophilic material, was pathognomonic of lupus lymphadenitis. The necrotizing lymphadenitis of SLE is pathologically similar to Kikuchi-Fujumoto disease (KFD), a distinctive, self-limited form of necrotizing lymphadenitis. The pathologic and clinical literature support a close link between SLE and KFD. Conclusions: SLE can be complicated by necrotizing lymphadenitis, with distinctive pathologic features. Lupus lymphadenitis and KFD share some common clinical and pathologic features, supporting a relationship between the disorders.