Gastrointestinal Hemorrhage in CREST Syndrome: A Case Report

TL;DR

This case report highlights gastrointestinal hemorrhage as a rare but serious complication of CREST syndrome, a form of scleroderma.

Contribution

The paper presents a rare clinical case of GI bleeding in CREST syndrome and emphasizes the need for clinician awareness.

Findings

GI hemorrhage is an uncommon but serious complication in CREST syndrome.

Endoscopic evaluation and acid suppression therapy are effective in managing GI bleeding in this condition.

Anemia due to chronic bleeding can be corrected with parenteral iron therapy.

Abstract

Systemic scleroderma is an autoimmune disorder caused by microvascular dysfunction, excessive collagen deposition, and progressive fibrosis affecting the skin and other organs. CREST syndrome, also known as limited scleroderma, is an acronym for calcinosis, Raynaud’s phenomenon, esophageal dysfunction, sclerodactyly, and telangiectasias. Among patients with systemic sclerosis (SSc), anticentromere antibodies are typically associated with the CREST syndrome variant. These antibodies are considered disease-specific and are known to correlate with the extent of disease progression. The pathogenesis of SSc is complex, with unusual presentations increasingly seen among patients. Gastrointestinal (GI) hemorrhage can be observed in CREST syndrome as an uncommon but serious complication. Endoscopic interventions are warranted in such patients to evaluate and manage bleeding. This report aims to raise awareness among physicians that CREST syndrome can present with GI hemorrhage, with skin signs such as calcinosis and sclerodactyly. In our case, H2 blockers and proton pump inhibitors were administered as part of the management plan to reduce acid-related mucosal injury and mitigate GI bleeding. Due to chronic mucosal hemorrhage, anemia was noted and corrected with parenteral iron therapy. The prognosis of CREST syndrome is relatively good when compared to the diffuse variant. Further research is needed to understand the pathophysiology of CREST syndrome, reliable biomarkers for early diagnosis, and targeted preventive treatment for this condition. Sensitizing clinicians regarding the changing disease pattern will help in prompt diagnosis.

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