Werner Syndrome Revealed by a Severe Metabolic Acute Pancreatitis: A Unique Clinical Observation
Achraf El Kabli *
Department of Internal Medicine, Ibno Rochd University Hospital, Casablanca, Morocco.
Safaa Mourabit
Department of Internal Medicine, Ibno Rochd University Hospital, Casablanca, Morocco.
Safaa Mhaber
Department of Internal Medicine, Ibno Rochd University Hospital, Casablanca, Morocco.
Barakat Laila
Department of Internal Medicine, Ibno Rochd University Hospital, Casablanca, Morocco.
Khadija Echchilali
Department of Internal Medicine, Ibno Rochd University Hospital, Casablanca, Morocco.
Mina Moudatir
Department of Internal Medicine, Ibno Rochd University Hospital, Casablanca, Morocco.
Hassan El Kabli
Department of Internal Medicine, Ibno Rochd University Hospital, Casablanca, Morocco.
*Author to whom correspondence should be addressed.
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.
Keywords: Werner syndrome, adult progeria, hypertriglyceridemia, acute pancreatitis, aging syndromes, case report