S3501 Severe Pancreatitis in Systemic Lupus Erythematosus: A Diagnostic and Therapeutic Challenge

Introduction: Acute pancreatitis (AP), including lupus-associated pancreatitis (LAP), occurring during lupus flares, is uncommon but may progress to severe necrotizing pancreatitis (NP). The etiologies of AP in lupus patients are complex and often overlap. Case Description/Methods: A 27-year-old woman with systemic lupus erythematosus (SLE), previously lost to follow-up, presented with fever, anorexia, diffuse myalgia, and right upper quadrant pain. Laboratory findings revealed leukocytosis (16.7 × 10⁹/L), anemia (8.4 g/dL), elevated creatinine (2.28 mg/dL), and lipase (400 U/L). Autoimmune markers confirmed active SLE with ANA (1:320 titer), anti-dsDNA, anti-Smith, and anti-RNP positivity, and low complement levels (C3, 30 mg/dL; C4, <8 mg/dL). Computed tomography showed necrotizing pneumonia and an enlarged, edematous pancreas with decreased enhancement, consistent with acute pancreatitis. The initial treatment included high-dose intravenous methylprednisolone (1000 mg), hydroxychloroquine (200 mg daily), and empiric antibiotics for pneumonia. Magnetic resonance cholangiopancreatography (MRCP) excluded obstruction and revealed a large necrotic peripancreatic fluid collection. The patient’s condition deteriorated in the intensive care unit, requiring mechanical ventilation and norepinephrine for hemodynamic support. Negative cultures led to the discontinuation of empiric antibiotics. Despite steroids and mycophenolate mofetil, the persistent symptoms prompted therapeutic plasma exchange (TPE). Following 5 sessions of TPE, her clinical status improved, with resolution of respiratory failure, stabilization of renal function, and discharge to a rehabilitation facility. Follow-up imaging revealed a small pseudocyst without signs of AP. Discussion: AP in SLE presents a diagnostic dilemma, especially when features of lupus flare and AP overlap. In this case, no definitive etiology could be confirmed, but the severity of the illness necessitated prompt intervention. Owing to the patient’s rapid clinical decline, we initiated high-dose corticosteroids, hydroxychloroquine, and empiric antibiotics without delay. Despite standard immunosuppressive therapy, her condition deteriorated, prompting escalation to TPE. The patient’s marked clinical improvement after TPE suggests a potential benefit in refractory cases, although its precise role requires further investigation. This case underscores the importance of early empirical treatment in critically ill patients with suspected LAP, even in the absence of diagnostic certainty.