Safety of SGLT-2 inhibitors in patients with glycogen storage disease type Ib and their efficacy in treating disease-associated digestive symptoms and disorders.

BACKGROUND/

objectivesEmploying rigorous epidemiological approaches, we performed a systematic evaluation of Sodium Glucose Cotransporter-2 (SGLT-2) inhibitors for managing digestive system disorders and symptoms associated with Glycogen Storage Disease Type Ib (GSD Ib).

methodsElectronic searches were conducted in three databases, with the latest update on 27 October 2025 (PROSPERO reference: CRD420251021881). Two reviewers independently screened records by title and abstract for full-text review. Eligible studies meeting the predefined criteria were assessed for quality using the Joanna Briggs Institute (JBI) Critical Appraisal Checklist for observational studies and the Methodological Index for Non-Randomized Studies (MINORS) for clinical trials, followed by data extraction.

resultsMeta-analysis or descriptive synthesis was performed for six common GSD Ib-related digestive symptoms. Multiple studies reported improvement in abnormal gastrointestinal patterns following SGLT-2 inhibitor therapy. Meta-analysis further demonstrated symptomatic relief in moderate-to-severe IBD, recurrent oral mucosal lesions, and anemia, with pooled remission rates (95% CI) under the two models being: 81.1% (68.4%-89.5%) and 85.3% (63.6%-99.2%) for IBD; 81.5% (73.6%-87.4%) and 83.5% (75.7%-90.3%) for oral lesions; and 60.5% (52.6%-68.0%) and 63.8% (48.0%-78.3%) for anemia. Regarding safety, hypoglycemia was the most frequently reported adverse event, followed by urinary tract and genital infections.

conclusionsThe efficacy analysis demonstrated beneficial effects of SGLT-2 inhibitors on six GSD Ib-related digestive disorders and its extra-digestive manifestations, showing potential for improving abnormal gastrointestinal patterns, moderate-to-severe inflammatory bowel disease, recurrent oral mucosal lesions, and anemia.