Atypical presentation of systemic lupus erythematosus flare with ileocecal ulceration and lymphadenopathy in a tuberculosis-endemic region

RATIONALE: Systemic lupus erythematosus can involve the gastrointestinal tract, but lupus enteritis (LE) with ileocecal ulceration and generalized lymphadenopathy as an initial presentation is exceptionally rare. In regions where tuberculosis (TB) is endemic, overlapping clinical and imaging findings can lead to diagnostic delays and inappropriate treatment. This case highlights the challenges in differentiating lupus enteritis from abdominal TB and underscores the importance of considering autoimmune etiologies in atypical gastrointestinal presentations. PATIENT CONCERNS: A 20-year-old woman presented with acute right lower quadrant abdominal pain, solitary ulceration of the ileocecal valve observed during colonoscopy, and generalized lymphadenopathy involving the abdomen, thorax, and inguinal areas. DIAGNOSES: The diagnosis of lupus enteritis was initially complicated and delayed. Nonspecific endoscopic findings, widespread lymphadenopathy, and an indeterminate result from the tuberculosis interferon-gamma release assay (TB-IGRA) raised suspicion for tuberculosis or lymphoma. Further obscuring the diagnosis, a false-negative antinuclear antibody (ANA) test was obtained using the enzyme-linked immunosorbent assay technique. However, persistent gastrointestinal symptoms, markedly elevated levels of anti-double-stranded DNA antibodies, decreased complement levels, and negative tuberculosis testing ultimately supported the diagnosis of lupus enteritis. Confirmation was achieved through an antinuclear antibody immunoblot test. INTERVENTIONS: The patient received intravenous methylprednisolone at a dose of 80 milligrams per day, followed by tapering of corticosteroids and maintenance therapy with hydroxychloroquine and azathioprine. OUTCOMES: Clinical symptoms resolved within 48 hours. Inflammatory markers normalized within 1 week. At 1-year follow-up, the patient remained in remission, and follow-up colonoscopy confirmed mucosal healing. LESSONS: This case highlights that in TB-endemic regions, clinicians should consider lupus enteritis as a differential diagnosis in patients with nonspecific gastrointestinal lesions and lymphadenopathy, even when tuberculosis is a common concern. False-negative ANA ELISA and indeterminate TB-IGRA results can delay diagnosis. Lymphadenopathy, although not included in current lupus classification criteria, may be an important clinical clue for lupus flares.