Case Presentation: This is a case of a 22 year old female with history of systemic lupus erythematosus (SLE) complicated by lupus nephritis class III/IV, immune thrombocytopenia, coombs positive anemia, discoid lupus & seizures, who presented to the ED with a 2 week history of feeling “unwell” and a 1 week history of epigastric pain radiating to the back associated with nausea, vomiting and decreased appetite. Non-compliant with her medications. Review of systems significant for oral and nasal ulcers, dry mouth with difficulty swallowing, dyspnea at rest and with exertion, foamy urine, worsening alopecia and rash under chin, diffuse myalgias and arthralgias with prolonged morning stiffness. Other review of systems was negative. Vitals revealed sinus tachycardia, which resolved after a 1L bolus of normal saline, other vitals unremarkable. Significant examination findings included dry mucous membranes, several shallow mouth and nasal ulcers, worsening alopecia with discoid lesions along hairline and under her chin, epigastric tenderness without guarding or rebound. Remainder of exam was unremarkable. Labs significant for pancytopenia, positive coombs test, mild hypokalemia, elevated lipase >2000, mildly elevated transaminases, and low C3. Spot urine protein 100g/dL, without blood or casts. EBV and CMV serologies negative. Calcium and triglycerides normal. CRP and ESR moderately elevated. Right upper quadrant ultrasound negative for gallstones. CT chest abdomen and pelvis showed inflammation of the pancreas without any other significant findings. She was started on high dose methylprednisolone with gradual improvement. Her hospital course remained overall unremarkable and she was discharged home on prednisone, rituximab and hydroxychloroquine with plans to taper steroids in the coming weeks.

Discussion: Lupus associated pancreatitis is a rare manifestation of pancreatitis with an annual incidence of 04-1.1/1000 lupus patients. Usually these patients have signs of active lupus or flare at the time of pancreatitis (about 84% of the time).The other common causes of pancreatitis such as gallstones, alcohol use, metabolic derangements, and drugs should be ruled out in patients presenting with pancreatitis with active SLE, as lupus pancreatitis is a diagnosis of exclusion. Interestingly, among a review of 77 cases of lupus pancreatitis, skin involvement was the most common SLE manifestation as was the case with our patient, joint and renal involvement were the next common manifestation. The etiology of lupus-associated pancreatitis is currently unknown. Multiple mechanisms have been proposed including autoimmune events, vasculitis related events, APLS-related thrombosis, or noninflammatory vasculopathy, but none have been consistently shown to have causation.For patients with lupus associated pancreatitis the treatment includes high dose steroids in addition to the normal supportive measures such as fluid resuscitation, pain control and bowel rest. It is important to note that drugs such as steroids can be associated with acute pancreatitis and there have been cases of such. In our patient she had not been on any medication, including steroids for several months, ruling out steroid induced pancreatitis for her presentation.

Conclusions: Although uncommon, lupus associated pancreatitis should be considered in any SLE patient presenting with abdominal pain and elevated lipase especially in the setting of active SLE manifestations. Other common causes should be first ruled out.