Abstract
Systemic lupus erythematosus (SLE) is a chronic systemic autoimmune disease characterized
by an almost 10:1 female predominance, the presence of deleterious nuclear autoantibodies,
a tendency for flare, and striking protean manifestations. Early diagnosis is associated
with less damage accrual, lower costs, and improved quality of life due to timely
treatment. However, early disease may not uncommonly show nonspecific presentation,
a single classification criterion, or an unusual organ involvement contributing to
frequent, often substantial diagnostic delays. We reviewed the literature (1982-2022)
to accumulate and classify all reports of rare, atypical, and unusual presentations.
These can involve almost every organ and system, and thus, present to physicians in
every discipline and setting. Increasing physicians’ awareness of the potential of
occult SLE to appear in varied, diverse, and unexpected presentations, may encourage
the inclusion of SLE in the differential. Informed history and examination focusing
on systemic and joint symptoms and mucocutaneous involvement, and basic tests (focusing
on leukopenia, thrombocytopenia, and proteinuria; followed by antinuclear antibodies
and complement levels) will correctly diagnose most patients on presentation or within
the following months and enable timely treatment.
Keywords
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Article Info
Publication History
Published online: June 04, 2022
Publication stage
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Funding: None applicable.
Conflicts of Interest: The author declares he has no conflict of interest.
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