S3102 GI Bleed Leading to Diagnosis of CREST Syndrome

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Introduction: Systemic sclerosis is a persistent autoimmune disease typically known for skin tightening around the hands and face with many variants such as CREST syndrome, a variant of systemic sclerosis that presents with calcinosis, Raynaud’s phenomenon, impaired esophageal motility, sclerodactyly, and telangiectasia. While it is typical for patients with CREST syndrome to experience cutaneous telangiectasias on the face and chest, telangiectasias are not commonly observed in the gastrointestinal tract. Herein, we describe a patient with a gastrointestinal bleed who exhibited typical signs of CREST with cecal telangiectasia. Case Description/Methods: A 61-year-old man patient presents to the hospital with recurrent, profuse dark stools. Past medical history is significant for compensated alcoholic cirrhosis, bilateral hand pain on cold exposure, and recurrent black, tarry stools requiring multiple admissions. Past surgical history is notable for a colonoscopy showing a cecal arteriovenous malformation that was ablated with Argon plasma coagulation that was complicated by cecal perforation requiring ileocecectomy with ileocolic anastomosis 4 years before presentation. Admission physical exam was remarkable for puffy fingers and calcinosis cutis without nail fold abnormality. Following adequate resuscitation, subsequent upper and lower endoscopy was performed, revealing the presence of two sessile adenomatous polyps that were removed. While investigating the causes of cirrhosis, Lab work was positive for an antinuclear antibody with a titer of 1:640 in a centromere pattern. Anti-smooth muscle antibody, ceruloplasmin, and alpha-1 antitrypsin were unremarkable (Table 1). Immune workup revealed an anticentromere antibody above the upper limit of quantification and negative anti-Scl-70 and Anti-RNA polymerase III (Figure 1). Discussion: Despite right hemicolectomy, the patient exhibits Raynaud's phenomenon, calcinosis cutis, puffy fingers, cecal telangiectasia, and recurring dark, tarry stools. Based on the 2013 ACR/EULAR criteria for systemic sclerosis, our patient's score of 13 points confirms his definite diagnosis of systemic sclerosis. Although limited systemic sclerosis commonly affects the GI tract, bleeding is rarely described as the presenting symptom.Figure 1.: Findings. Table 1. - Results Parameter Results Normal range Antinuclear antibody 1:640 (Centromere pattern) < 40 Anti-smooth muscle antibody < 20.0 < 20.0 Anticentromere antibody (U) >8.0 < 1.0 Anti-Scl-70 (U) < 0.2 < 1.0 Anti-RNA polymerase III < 10.0 < 20.0 Anti-Th/To Ab Negative Negative Ceruloplasmin (mg/dL) 38 18-36 alpha-1 antitrypsin (mg/dL) 219 83-199

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