• Jeonghun Lee, Young Joo Lee, and Youngsun Kim.
• Department of Internal Medicine, Ye Hospital, Anyang.
• Medicine (Baltimore). 2021 Jun 4; 100 (22): e26238e26238.

RationaleAcute acalculous cholecystitis (AAC) is an extremely rare manifestation of systemic lupus erythematous (SLE). In previous reports, most of the patients were already diagnosed cases of SLE upon confirmation of AAC.Patient ConcernsA 24-year-old female who initially presented with fever and acute right upper quadrant abdominal pain. She had no medical history.DiagnosesAbdominal ultrasonography and computed tomography (CT) showed gallbladder thickening with pericholecystic edema without gallstones or sludge, demonstrating acalculous cholecystitis. She revealed discoid rash on the both shin. Laboratory tests revealed pancytopenia. The titer of antinuclear antibody (ANA) was 1:1280. Anti-dsDNA antibody, anti-phospholipid antibody, anti-Sm antibody test, and proteinuria in 24 hours were positive. Both C3 and C4 were low. Echocardiography and chest CT showed pericardial effusion and pleural effusion. Using the 2019 European League Against Rheumatism/American College of Rheumatology (EULAR/ACR) classification criteria, the score was 31. We thought AAC of this case that was one of the initial manifestations of SLE.InterventionsThe patient was treated with high-dose prednisolone (1 mg/kg) and hydroxychloroquine 400 mg.OutcomesAfter 4 days of administration of high-dose corticosteroid therapy, symptoms rapidly improved. After 35 days of the treatment, her symptoms and disease activity of SLE were markedly improved.LessonsAlthough AAC being the initial manifestation of SLE is very rare, prompt diagnosis and management with corticosteroids precluded surgical intervention. Physicians need to be cognizant of AAC as a disease flare and as a rare initial manifestation of SLE.Copyright © 2021 the Author(s). Published by Wolters Kluwer Health, Inc.

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