Gastric Variceal Hemorrhage in a Patient With Sclerodactyly: An Unexpected Cause of Hematemesis in Limited Cutaneous Systemic Sclerosis

Microvascular injury, immunological dysregulation, and increasing collagen fibrosis are the hallmarks of systemic sclerosis (SSc), an inflammatory connective tissue disease.Its limited cutaneous subset, historically termed calcinosis, Raynaud phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasia syndrome, is characterized by the presence of anticentromere antibodies.Gastrointestinal involvement is almost universal, and when overt bleeding occurs, it is usually attributed to gastric antral vascular ectasia (GAVE) or mucosal telangiectasia.This article describes a 66-year-old woman with limited cutaneous SSc who presented with acute upper gastrointestinal bleeding due to actively bleeding gastric varices associated with portal hypertension, chronic liver disease, and extensive portal-vein thrombosis.Her physical examination revealed sclerodactyly, salt-and-pepper pigmentation, restricted mouth opening, and moderate splenomegaly, along with Raynaud phenomenon.Antinuclear antibody was positive at a titer of 1:100 in a centromere (AC-3) pattern, with anticentromere protein B reactivity, and a skin biopsy was performed.A diagnosis of SSc was made on the basis of clinical, serological, and skin biopsy findings.Urgent esophagogastroduodenoscopy demonstrated small esophageal varices and large gastric varices that were actively bleeding and were managed with 1 mL of cyanoacrylate glue.Triple-phase computed tomography showed a cirrhotic liver, partially occlusive portal vein thrombosis, moderate splenomegaly, and a large gastrorenal shunt.Even though the etiology of liver disease was unknown, a raised serum immunoglobulin G level supported coexisting autoimmune liver disease.Secondary prophylaxis was achieved by retrograde transvenous obliteration of the gastrorenal shunt with plug assistance employing an Amplatzer vascular plug (Abbott Medical, Plymouth, MN).The patient remained free of rebleeding with a stable hemoglobin level at two and six weeks.This case illustrates that hematemesis in SSc must not be assumed to be a scleroderma-specific mucosal lesion such as GAVE or mucosal telangiectasia, that coexisting autoimmune liver disease and portal hypertension should be actively excluded, and that endovascular shunt obliteration is an effective secondary-prophylaxis strategy.