長崎大学医学部小児科 冨永典男5 text pages, 1 page of reference, 2 tables, 1 figure, and 1 page of figure legends.2
IntroductionSystemic lupus erythematosus (SLE) is a multisystemic disease, and can involve the digestive system. Although the occurrence of pancreatitis in SLE has long been recognized 1 , it is a rare but often fatal complication especially in childhood 2 .Plasmapheresis has been recommended in extremely ill patients with autoimmune disorders who have not responded to conventional therapies 3 . It is expected to diminish circulating immune complexes, antibodies and active complement components as well as proinflammatory cytokines.Here we present a girl with SLE who developed acute pancreatitis shortly after presentation. Plasmapheresis, followed by intravenous pulse therapy with methyl prednisolone and cyclophosphamide, has brought complete recovery from this fatal complication.
Case reportA 12-year-old girl was referred to us because annual medical examinations at school pointed out proteinuria and hematuria. She also complained of malaise and had Raynaud's phenomenon, but otherwise she had stable vital signs and normal physical findings. Laboratory studies on admission showed elevated antinuclear antibody (ANA) titers of 1: 640 with a speckled pattern using Hep2 cells, and elevated anti-single-stranded DNA and anti-double-stranded DNA antibodies (Table I).Antibodies to Sm-antigen, RNP-antigen and La/SSB-antigen were also detectable.Further laboratory analyses showed elevated IgG level (1950 mg/dl) and decreased C4 level (Table II). C-reactive protein was repeatedly negative and serum amylase level was slightly elevated. Urinalysis showed proteinurea (2.5 g/day) and hematuria. 24-h endogenous creatinine clearance revealed normal result (101ml/min). After admission, her white blood cell count decreased to below 4000/μl. Renal biopsy were compatible with membranous lupus nephritis (Class V-B by WHO classification). On the 10 th hospital day, she started treatment with prednisolone (1 mg/kg daily), azathioprine (1 mg/kg daily), dipyridamole, and warfarin. Within a week after treatment, malaise, proteinuria and hematuria were disappeared but there were no significant changes of laboratory parameters indicative for SLE activity, and hyperamylasemia yet existed.After 2 weeks of clinically stable period upon treatment, she developed high-grade fever and butterfly rash associated with serological disease activity. Because CRP value was 3 markedly increased and she was highly immunosuppressed, she was suspected to acquire invasive bacterial infections, and received intravenous immunoglobulin therapy.But the patient's condition deteriorated rapidly and profoundly with cardiovascular collapse and severe upper abdominal pain. Hyperamylasemia became exacerbated and other blood chemistry parameters also elevated (Table II). Abdominal computed tomography (CT) revealed diffusely dilated pancreas, mild ascites, and intact gallbladder and bile duct ( Fig. 1 A, B). Diagnosed as pancreatogenic shock associated with acute exacerbat...
