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Medical Science Monitor Basic Research


Late-Onset Systemic Lupus Erythematosus Associated with Autoimmune Hemolytic Anemia and Sixth Cranial Nerve Palsy

Challenging differential diagnosis

Kaku Kuroda, Masaki Itagane, Mitsuyo Kinjo

USA Department of Family Medicine, State University of New York (SUNY) Upstate Medical University, Syracuse, NY, USA

Am J Case Rep 2021; 22:e932959

DOI: 10.12659/AJCR.932959

Available online: 2021-08-12

Published: 2021-09-14


BACKGROUND: Patients with late-onset systemic lupus erythematosus (SLE) do not present with typical SLE symptoms or serology, and this can lead to a major delay in diagnosis. We report a complex case of an older woman who developed autoimmune hemolytic anemia and sixth cranial nerve palsy that posed considerable challenges in diagnosing late-onset SLE.
CASE REPORT: A 78-year-old Japanese woman presented with polyarthritis associated with generalized fatigue for 2 months, who later developed diplopia. Physical examination revealed conjunctival pallor, polyarthritis, and subsequent development of sixth cranial nerve palsy. Laboratory data revealed a decreased white blood cell count; macrocytic anemia; elevated levels of lactate dehydrogenase, indirect bilirubin, and erythrocyte sedimentation rate; hypocomplementemia; positive Coombs test; antinuclear antibodies (ANAs, 1: 40); and positive anti-double-strand DNA antibodies. Lymphoma, cerebral venous sinus thrombosis, and varicella-zoster virus infection were unlikely based on head computed tomography, brain magnetic resonance imaging, and cerebrospinal fluid analysis. She was diagnosed with late-onset SLE associated with autoimmune hemolytic anemia and sixth cranial nerve palsy. The patient was successfully treated with prednisone and hydroxychloroquine.
CONCLUSIONS: The difficulty in diagnosing late-onset SLE with atypical presentations and uncommon complications must be recognized. SLE cannot be excluded based on a low titer of ANA in a particular subgroup such as the elderly, and the prozone effect should be considered responsible for low ANA titers. In this case, late-onset SLE was diagnosed by considering multisystem pathologies despite low ANA titers.

Keywords: Abducens Nerve Diseases, Anemia, Hemolytic, Autoimmune, Lupus Erythematosus, Systemic