Werner Syndrome Revealed by a Severe Metabolic Acute Pancreatitis: A Unique Clinical Observation

Abstract

Werner syndrome (WS), or adult progeria, is a rare autosomal recessive disorder characterized by premature aging and multisystem involvement. Its initial presentation through a metabolic emergency remains exceptional. We report the case of a 41-year-old woman with a history of chronic dexamethasone use (16 years), insulin-dependent diabetes, bilateral femoral head osteonecrosis requiring hip arthroplasties, bilateral cataracts, glaucoma, osteoporosis, hypothyroidism, and uncontrolled hypertension. She was admitted for a third episode of acute pancreatitis. Imaging revealed stage E necrotizing pancreatitis. MRCP excluded a biliary cause. Serum triglyceride level was 112 g/L. The metabolic etiology was retained. Treatment included digestive rest, insulin glargine, analgesics, and antispasmodics, with clinical and biological improvement (TG = 5 g/L). Physical examination revealed signs suggestive of WS: short stature, bird-like facies, premature graying, diffuse alopecia, cutaneous atrophy, and hallux valgus. Although genetic testing was unavailable, clinical suspicion was supported by the presence of a similarly affected sibling. This case highlights a rare and severe metabolic manifestation of WS. It emphasizes the importance of recognizing WS in adults presenting with multisystem premature aging and atypical metabolic crises.