Objective We aimed to investigate the clinical features of acute acalculous 
cholecystitis (AAC) in patients with systemic lupus erythematosus (SLE). Methods 
SLE patients with AAC hospitalized in the Peking Union Medical College Hospital 
(PUMCH) from January 2001 to September 2015 were retrospectively analyzed. Their 
medical records were systematically reviewed. The diagnosis of AAC was based on 
clinical manifestations and confirmed by radiologic findings including a 
distended gallbladder with thickened wall, pericholecystic fluid and absence of 
gallstones. Results Among the 8411 hospitalized SLE patients in PUMCH, 13 
(0.15%) were identified to have SLE-AAC. Eleven (84.6%) of them were female, 
with a mean age of 30.1 ± 8.6 years. AAC was the initial manifestation of SLE in 
four (30.8%) cases. Eleven (84.6%) patients complained of fever and abdominal 
pain, four (30.8%) had positive Murphy's sign and six (46.2%) had elevated liver 
enzymes. The median SLE Disease Activity Index was 8.0 (range 0-20.0) at the 
time of AAC. Other affected organs in SLE-AAC included kidney (11, 84.6%) and 
hematologic system (11, 84.6%), followed by mucocutaneous (seven, 53.8%), 
musculoskeletal (seven, 53.8%) and neuropsychiatric (two, 15.4%) systems. All 
patients received treatment of glucocorticoids and immunosuppressants but none 
underwent surgical intervention. During a median follow-up of 28 months (range, 
2-320 months), 12 cases (92.4%) responded to treatment with no relapse and one 
patient (7.6%) died of septic shock. Conclusion Our study suggests that AAC is a 
relatively uncommon and underestimated gastrointestinal involvement of SLE that 
is often associated with active disease. For patients with AAC in SLE, treatment 
with aggressive glucocorticoids could result in a good prognosis.