AB0518\u2005- JUVENILE SYSTEMIC LUPUS ERYTHEMATOSUS RELATED PANCREATITIS: AN UNCOMMON MANIFESTATION OF A COMMON DISEASE

Abstract

Background: Pancreatitis is a rare but potentially life-threatening complication of juvenile systemic lupus erythematosus (jSLE). Objectives: We report 3 children with SLE who presented with acute pancreatitis. Methods: We have reviewed the clinical records of 140 children with SLE between period of 1993-2018. Three of them present with acute pancreatitis. Results: Case 1 -12-year-girl presented with fever of 1 month and alopecia. Examination revealed pedal oedema, periorbital puffiness, generalised lymphadenopathy, large joint arthritis and mild hepatomegaly.Investigations were consistent with lupus.Renal biopsy revealed Class 3 lupus nephritis and initiated on intravenous methylprednisolone.Two days after beginning her medication,she developed severe epigastric pain and vomiting which did not respond to antacids and analgesic.Serum amylase and lipase were elevated.Clinical possibilities included steroid induced pancreatitis and lupus pancreatitis.Intravenous methylprednisolone was continued following which she showed a dramatic improvement.Case 2-A-6-year-old presented with pain abdomen and vomiting. Physical examination showed epigastric tenderness.Investigations showed elevated amylase levels.Computerised tomography(CT) abdomen revealed acute necrotising pancreatitis.A ultrasound abdomen revealed a pancreatic pseudocyst.He had a second episode of acute pancreatitis along with anasarca after 3 months.In follow-up,he presented with anasarca. Investigation were consistent with lupus.Following the initiation of steroids,he improved and there has been no recurrence of pancreatitis over the next 4 years.Case 3 - 9-year-girl presented with generalised rash and alopecia for 5 months.She also had pain abdomen for last 2 months.Investigations showed elevated amylase and ultrasound abdomen revealed acute pancreatitis. She had undergone a laparotomy elsewhere.Examination showed generalised pigmented rash, periorbital edema, alopecia, periorbital puffiness, hard palate ulcer and surgical scar on the abdomen.Urinalysis showed nephrotic range proteinuria.Serum amylase levels were elevated. Ultrasound abdomen revealed a pancreatic pseudocyst.Further investigations were suggestive of lupus.Workup for APLA revealed positive lupus anticoagulant. She was initiated on oral prednisolone and was given pulses of intravenous cyclophosphamide.There has been no recurrence of pancreatitis over 12-years follow-up. Investigation Case 1 Case 2 Case 3 Haemoglobin (g/L) 92g/L 96 g/L 80 g/L White cell countsLymphocyte count 7.7 X 106/L2.15x 106/L 7.8 X 106/L1.9 X 106/L 7.5 X 106/L1.95X106/L Platelets 130×109/L 150 ×109/L 420 x 109/L Urine routineUrine protein (mg/m2/hour) 10-12 RBC, 3 + albumin- Few RBC, 3 + albumin82 mg/m2/hr No RBC, 3+ albumin40 mg/m2/hr C3 (Normal 50-150 mg/dL)C4 (Normal 20-50 mg/dL) 23.4mg/dl2.98mg/dl 129 mg/dL37mg/dL 34 mg/dl10 mg/dl ANA 4+ diffuse 3 + 3+ diffuse Anti dsDNA (N: <60 IU/mL) 890 <60 123 Antiphospholipid antibodies: a) Lupus anticoagulant b) Anticardiolipin antibody (IgG and IgM) c) Anti ß2 Glycoprotein -1 antibody (IgG & IgM) NegativeNegativeNegative NegativeNegativeNegative PositiveNegative negative Skin biopsy Not done Positivity of lupus band test with high positive IgG, IgA, IgM and C3 in dermal vessels Lupus band test -positive Renal biopsy Class 3 lupus nephritis IgG, Ig A, Ig M positivity in the mesangium as well as capillary loops and C3 in small sized blood vessels Class 4 lupus nephritis Serum amylase (< 100 U/L) 238 U/L 400 U/L 290 U/L Serum lipase (< 60 U/L) 231U/L Not done Not done Conclusion: Pancreatitis can at times, be the presentation of childhood lupus and requires prompt and aggressive management. Disclosure of Interests: None declared