A Rare Case of Hereditary Pancreatitis Unveiling Systemic Lupus Erythematosus in a Young Female Patient.

Systemic lupus erythematosus (SLE) is a chronic autoimmune disease with varied clinical presentations, sometimes occurring in atypical presentations that cause a delay in diagnosis. Pancreatitis, though uncommon, may be an initial presentation of SLE and needs to be carefully evaluated to rule out other causes. We present the case of a 19-year-old woman who presented with vomiting and abdominal pain and was later diagnosed with hereditary pancreatitis following the detection of a heterozygous SPINK1 gene mutation. Additional evaluation showed systemic manifestations such as non-scarring alopecia, painful oral ulcers, pancytopenia, lupus hepatitis, and a highly positive autoimmune profile, in keeping with a new diagnosis of SLE. This case highlights the diagnostic challenge at the interface of genetic and autoimmune pancreatitis and reinforces the need for a thorough clinical, immunologic, and genetic evaluation in young patients with unexplained abdominal complaints.