When Lupus Strikes Twice: Pancreatitis and Nephritis in an Overlap Syndrome - A Rare Multisystem Flare

In patients with systemic lupus erythematosus (SLE), an unusual yet potentially serious complication is acute pancreatitis. When patients have other underlying disorders such as Sjögren's syndrome and dermatomyositis (collectively referred to as "overlap syndromes"), the signs and symptoms of acute pancreatitis can vary from patient to patient. Therefore, when evaluating a patient for acute pancreatitis in the context of one or more overlap syndromes, it may be difficult to get a definitive diagnosis due to similar clinical presentations and hence, determining when to initiate treatment may not be straightforward. The earlier a diagnosis is made and appropriate immunosuppressive treatment initiated, the more likely there will be favorable outcomes. We present a case of a young female recently diagnosed with SLE, overlapping with Sjögren's syndrome and dermatomyositis (overlap syndrome), who presented to the Emergency Department with an acute onset of severe epigastric pain radiating to her back, vomiting, and fever. On clinical examination, she had marked epigastric tenderness, rash on her skin, and oral ulcers. Her laboratory evaluation showed anemia, leukopenia, thrombocytopenia, high serum amylase and lipase levels, hypocalcemia and hypoalbuminemia, transaminitis, and urinary findings with significant proteinuria. Her immunological evaluation showed low complement levels (hypocomplementemia), multiple autoantibodies are positive including: antidouble-stranded deoxyribonucleic acid, SSA, SSB, nucleosomes, histones, and Mi-2 indicative of active lupus disease with overlap features. Ultrasound and computerized tomography (CT) scan of her abdomen demonstrated; acute interstitial edematous pancreatitis with fluid collections around the pancreas, moderate ascites, and pleural effusions with a modified CT severity index of 6 out of 10. Her clinical course was complicated by suspected lupus nephritis and the presence of papilledema and seizure episodes with neuroimaging findings consistent with either; posterior reversible encephalopathy syndrome or vasculitic infarct. This patient was treated with bowel rest, vigorous intravenous fluids, correction of electrolyte-disturbance, antibiotics, albumin, and analgesic therapy, with high-dose intravenous methylprednisolone pulses, followed by cyclophosphamide with MESNA. Antiepileptic and antiplatelet therapy were also started to deal with neurological involvement. With this multidisciplinary approach, there was improvement in the biochemistry, with improvement in pancreatic enzymes, hepatic parameters, renal function and global symptoms. She was discharged on oral corticosteroid, hydroxychloroquine, antiepileptic therapy and supportive medications, with nephrology follow-up and renal biopsy guided immunosuppression. This is an example of an acute pancreatitis complication of active SLE in an overlap syndrome with multisystem involvement, including involvement of the kidneys and the central nervous system. More generally, this interesting case highlights the need to consider an autoimmune etiology, in particularly young patients presenting with atypical unexplained pancreatitis. Immunosuppressive therapy is life-saving in these individuals, leading to good clinical courses when combined with supportive pancreatitis therapy.