Visceral Muscle Dysmotility Syndrome With Esophageal Aperistalsis (VMDS Tetrad): A Rare Initial Manifestation of Systemic Lupus Erythematosus.

Visceral muscle dysmotility syndrome (VMDS) is a rare manifestation of systemic lupus erythematosus. VMDS with esophageal aperistalsis (VMDS tetrad) as the initial presentation of systemic lupus erythematosus has not been previously reported. We describe a case of 35-year-old woman with recurrent intestinal pseudo-obstruction associated with pyeloureterectasis, nonobstructive biliary dilatation, and esophageal aperistalsis, constituting a VMDS tetrad. The diagnosis was established based on clinical features, radiologic findings, and serologic evaluation. The patient responded promptly to immunosuppressive therapy, with complete clinical and radiologic resolution, highlighting the importance of early recognition and timely treatment.