Title: Diagnose systemic lupus erythematosus with pancreatitis and pancytopenia as initial presentation

Biography:

Bilawal Ahmed is Hematology/Oncology fellow from University of Missouri-Columbia. His research interests include inflammatory breast cancer and axillary lymphadenopathy.

Abstract

Systemic lupus erythematosus (SLE) is a multisystem disease, that may affect multiple organ systems in the body. As a rare initial presentation of SLE, acute pancreatitis presents as generalized flare-ups in most cases of patients previously diagnosed with SLE. Here we report a rare case of acute pancreatitis and pancytopenia as the initial presentation of systematic lupus erythematosus.

15 year old female patient presented with chief complaints of abdominal pain, nausea, vomiting and non bloody diarrhea. The onset of abdominal pain started 4 days prior to admission, localizing to the right upper quadrant on day 2. Associated symptoms consisted of nonbloody diarrhea and nonbloody/nonbilious vomit for 4 days. There was no contributory past medical history or past surgical history. Patient denies any drug allergies. On general physical examination, a fine lacy rash was noted on all four extremities. Her vitals were T 37.9, HR 104, RR 18, BP 99/58. No guarding/rigidity and no masses or organomegaly were appreciated. CBC revealed profound pancytopenia with a platelet count of 115 (150-400 x10³/uL), hemoglobin 8.5 (12-15 g/dL) and white blood cell 1.5 (4.5-13 x10³/uL). Lipase was 489 u/L and Lactate Dehydrogenase was 2392 u/L. Other laboratories, including comprehensive metabolic panel, lipid panel, C-reactive protein, and pregnancy test, were all within normal limits. Patient was admitted with diagnosis of pancreatitis with pancytopenia and supportive treatment with investigative studies was initiated. Bone marrow biopsy was unequivocal. Infectious Disease was consulted. New laboratory test results indicated positive IgG and IgM antibodies to Brucella, Rickettsia, Ebstein Barr Virus and Parvovirus, complicating the diagnosis. Patient started on doxycycline empirically. Rheumatology consulted most likely etiology to be SLE and await Lupus Panel Results. Erythrocyte sedimentation rate was 74 mm/hr, positive antinuclear antibody (ANA) 1/640, positive anti doublestranded DNA Antibody 1/320, positive anti Smith antibody, negative rheumatoid factor, C3 <40mg/dL, Lipase 2784 u/L, fecal occult bkood test positive. Following lab results and clinical course, the diagnosis of Systemic Lupus Erythematosus was made on day 4 of admission. Steroids were initiated, and the patient’s status was observed closely. Lipase continued to rise to 8,136 u/L which prompted an MRCP. MRCP showed extensive changes of pancreatitis with upper abdominal fluid, no organized collection or dilation of ducts. Also noted was extensive wall edema of the gallbladder with no filling defect. Lipase began trending down on day 2 of steroids. CBC corrected to within normal limits. Patient reported feeling better and continued improving.

This case aims to facilitate diagnosticians in their evaluation of patients presenting with rare manifestations of SLE. SLE should be considered in cases of pancreatitis when common etiologies have been ruled out and symptoms are not residing despite appropriate treatment. In regards to hematologic abnormalities, SLE can cause various cytopenias as an initial presentation but pancytopenia has been reported fewer times in the literature.