Case 1: Cat-girl and an unexpected renal biopsy A 10-year-old girl presents with a prolonged fever and rash, most prominent around the eyes, with annular lesions encircling the periorbital regions. She is otherwise well, with normal blood pressure, a polymorphous rash, one aphthous ulcer on her lip and mild polyarthritis of the small joints of her hands.

Investigations show leukopenia (2.22x10⁹/L), normocytic anemia (hemoglobin 9.5 g/dL), an elevated ESR (57 mm/hr) and C-reactive protein (36 mg/L), hypoalbuminemia (30 g/L), mild proteinuria (0.08 g/mmol), hematuria (29/hpf), pyuria (20/hpf), slightly elevated creatinine (88 µmol/L, giving an estimated GFR of 56 ml/min/1.73 m²). Eventually, urine culture returns positive for E. coli. She also has a positive Direct Coombs’ test, antinuclear antibodies, anti-RNP, anti-β₂-glycoprotein IgG, anti-cardiolipin IgM. Her complement 3 (80 mg/dl) is slightly low, but she has a normal complement 4 level and negative anti-double stranded DNA. Ultrasound shows a dysplastic left kidney. A renal biopsy of the right kidney reveals an unexpected result. Come and see!

Learning Objectives

• Describe the lesser-known cutaneous manifestations of lupus in children

• Describe renal involvement in lupus beyond the known classification of lupus nephritis

• Discuss the treatment of lupus nephritis

Case 2: Two girls with lupus and abdominal pain

1. A 16-year-old girl presents with fever lasting one-month, extreme lethargy, loss of weight, alopecia and a facial rash. The diagnosis of systemic lupus erythematosus (SLE) is made with additional features of pancytopenia, positive direct Coombs’ test, positive ANA and anti-double stranded DNA, hypocomplementemia. She is started on oral prednisolone 30 mg twice daily and hydroxychloroquine. One day after initiation of therapy, she returns to the Emergency Department with severe abdominal pain and vomiting.

2. An 18-year-old girl has received treatment for SLE since the age of 13 years old. She presented with cutaneous vasculitis and arthritis with cytopenia. Her current treatment included prednisolone 5 mg daily, mycophenolate mofetil 1 g twice daily and hydroxychloroquine. She has had persistent neutropenia for the last 2 years without infection and low complement 3 (75 mg/dl) without clinical features of lupus. She has had one month of abdominal bloating and abdominal pain following an acute food poisoning with another family member. While on a trip to Italy, the abdominal pain and nausea worsened and she was admitted to the Emergency Department. What could this be and what must be done?

Learning Objectives

• Describe the treatment approach to abdominal pain in a patient with lupus

• Describe the clinical features and outcomes of acute pancreatitis in childhood lupus

• Explain the clinical features and outcomes of ‘lupus gut’ in childhood lupus

• Discuss the best practice for management of acute pancreatitis in lupus and ‘lupus gut’