LAUSR

A 10-year-old girl was brought to the hospital for evaluation of upper abdominal pain and vomiting for 6 days. On physical examination, her vital signs were: axillary temperature 37.0 C, respiratory rate 19 breaths/min, heart rate 87 beats/min and blood pressure 95/70 mmHg. No rash was observed. Abdominal examination demonstrated rebound tenderness in all quadrants. There was no history of trauma, medication usage, chronic illness or alcohol intake. No family history of systemic disease or immunoglobulin A vasculitis (IgAV) was noted. Laboratory data indicated a white blood cell count of (7.72 × 10/L (normal: 3.50–9.50 × 10/L)) with normal differentiation, haemoglobin 151 g/L (normal: 115–150 g/L) and platelet count (278 × 10/L (normal: 125–350 × 10/L)). Other blood chemistry findings were C-reactive protein 0.50 mg/L (normal: 0–10.0 mg/L) and procalcitonin 0.04 ng/mL (normal: 0–0.50 ng/mL). Liver enzymes, renal function, electrolytes, myocardial enzymes, blood lipids, complement 3 (C3), C4 and erythrocyte sedimentation rate were normal. Coagulation profile, antinuclear antibody and antineutrophil cytoplasmic antibodies were also within normal limits. Helicobacter pylori antibodies, the IgM and IgG for Epstein–Barr virus and TORCH infection were negative. Human immunodeficiency virus, hepatitis C virus and hepatitis B surface antigen tests were also not reactive. Her urine tests showed no proteinuria or hematuria, and a 24-h urine protein output was quantified at 0.1 g/day (normal 0–0.15 g/day). A routine stool test showed weak positive faecal occult blood but no erythrocytes. The patient had no bleeding disorder or coagulopathy. The ultrasonography of her liver, gall-bladder, pancreas and spleen was normal. The concentration of amylase in her urine was 716 μ/L (Fig. 1), while serum pancreatic enzymes level were normal. The abdominal computed tomography (CT) showed oedema of the pancreatic head consistent with acute pancreatitis (AP) (Fig. 2a). The patient was responsive to symptomatic treatment such as fasting, parenteral nutrition, proton pump inhibitors, anti-secretory agents (octreotide) and antibiotics (amoxicillin and clavulanate potassium). However, 3 days later, the patient complained of severe abdominal pain, along with haematemesis and haematochezia. Gastroscopy showed gastric purpura, diffuse mucosal oedema, erythema, petechiae and multiple irregular ulcers in her duodenal bulb and the second part of the duodenum, while the mucosa inbetween were normal (Fig. 3a,b). Biopsy from the gastroscopy demonstrated non-specific inflammation cell infiltration, and no granuloma was found (Fig. 3c). Repeated serum amylase and lipase level were 184 μ/L and 927 μ/L, respectively (Fig. 1). Repeated CT of the abdomen showed increased oedema of the pancreas and gall-bladder (Fig. 2b). The patient was diagnosed with IgAV presenting as AP. The patient started treatment with pulse methylprednisolone for 3 days (5–10 mg/kg/day) plus intravenous immunoglobulins (IVIg) (400 mg/kg/day) for 4 days, followed by oral hydrocortisone. Her clinical status quickly improved, and amylase and lipase values returned to normal within 1 week (Fig. 1). On the 10th day after the onset of her initial symptoms, she developed an extensive purpuric rash over both lower limbs. Urinalysis was within normal limits without the presence of proteinuria or hematuria at 3-month follow-up. There was no relapse noted. The study protocol was approved by the Research Ethics Commission of the First Affiliated Hospital of Anhui Medical University. The permission letter that allowed access to the medical records for this study was obtained from the hospital management office.