A Rare Case of Reynolds Syndrome Revealed by Hematemesis

Reynolds syndrome (RS) is an autoimmune disorder characterized by overlapping primary biliary cholangitis (PBC) and systemic sclerosis (SSc) [3]. Diagnosing overlap syndrome remains a challenge,
During systemic sclerosis, the prevalence of liver injury is approximately 1% [6]. Few RS case reports were published. The diagnosis is usually based on clinical, immunological and histological findings, and mainly focused on dermatologic features [8]. Our aim is to make the physicians aware of this rare association and help with its diagnosis.

Case Presentation: We here report, the case of a 45-year-old woman who was diagnosed with scleroderma one year before her admission, treated by Corticotherapy, colchicine and methotrexate. She was admitted to our department for hematemesis. Her vital signs on admission were normal; her conjunctiva was pale with icteric scleras. Skin inspection revealed hyperpigmentation predominant in face, sclerosis predominantly on the back of both hands. Upper gastrointestinal endoscopy test was performed, reporting a grade III esophageal varices and congestive hypertrophic gastric mucosa. Abdominal ultrasound revealed a splenomegaly and a cirrhotic liver morphology . Immunoflorecence reported mitochondrial and centromere patterns of antinuclear-antibodies. Therefore, RS was diagnosed and ursodeoxycholic acid was started. She has had no further complications during follow-ups.

Conclusion: In summary, it should be kept in mind that PBC is commonly associated with extrahepatic autoimmune diseases; This is why we must perform hepatic function tests in these patients to detect abnormalities that can often coexist silently.

Keywords: systemic sclerosis; Primary biliary cholangitis; Overlap syndrome; Reynolds syndrome; Autoimmunity; hematemesis.