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13 Management of paediatric lupus
  1. Joan Calzada-Hernández
  1. Hospital Sant Joan de Déu, Barcelona, Catalonia, Spain


Case 1: Newborn with cutaneous lesions A 23-day-old newborn referred by his primary care pediatrician for skin lesions on the face, scalp, and upper trunk, in the form of erythematous annular plaques with hyperkeratosis, somewhat scaly. No other clinical manifestations were found. There was no history of fever and the baby was feeding well. No recent vaccination.

The mother had one spontaneous abortion in the past. The recent pregnancy was uneventful, and the baby was born from a spontaneous vaginal delivery at week 37. The birth weight was 2.6 kg and the Apgar score was normal.

The laboratory findings showed normal white blood cell count, no evidence of inflammation and hepatic and renal function. ANA 1/640; anti-Ro and anti-La antibodies were positive.

In relation to this clinical picture, indicate what your approach would be at this moment.

Learning Objectives

  • Describe the clinical features of neonatal lupus

  • Describe the diagnostic and therapeutic approach of neonatal lupus

  • Discuss the prenatal study of pregnant women with lupus

Case 2: Child with skin lesions related to cold exposure A 5-year-old boy presented with no significant medical history or known drug allergies. He was born at 39 weeks after an uncomplicated pregnancy to unrelated parents, birth weight 3.1 kg. There was no evidence of TORCH or other infections and he has been correctly vaccinated to date. His parents report that every winter, since he was 1 year old, he presents painful violaceous lesions on his hands and feet, but there are no lesions during summer. On examination, he presented chilblain lesions on his hands and feet.

Laboratory test findings were unremarkable, including liver and renal function and acute phase reactants. Rheumatic factor, ANA, anti-dsDNA antibodies, antiphospholipid serology were negative. Normal complement C3/C4. There was no evidence for hypergammaglobulinemia, cold agglutinins, viral or bacterial infection.

The father tells us that he suffers similar episodes and we see scars with tissue loss in his earlobes.

What additional examinations would you consider next?

Learning Objectives

  • Discus approach to the main causes of monogenic lupus

  • Describe suggestions for genetic studies on suspicion of monogenic lupus

Case 3. Diffuse alveolar hemorrhage in an adolescent girl with lupus A 16-year-old female presents with asthenia, anorexia and low-grade fever in the last 2 weeks. She is otherwise asymptomatic with no other medical history of interest.

Laboratory test findings show lymphocytes 800/mm3; platelets 113.000/mm3, Hb 10.5 g/dl, creatinine 1.04 mg/dl, hypoalbuminemia 26 g/l, low C3 and C4 fractions (160 mg/l; < 29 mg/l); ESR 34 mm, CRP 4 mg/l. ANA 1/640, anti-dsDNA Ab >600 UI/ml. Urine: hematuria >100/hpf, pyuria 10/hpf, UPCR 4.74 mg/mg. Lupus anticoagulant negative, IgG/IgM β2-GPI negative, IgG/IgM anti-cardiolipin negative.

Kidney biopsy shows class IV glomerulonephritis -ISN classification-. She received pulsed methylprednisolone plus oral prednisone and mycophenolate mofetil 2 g/day.

One week later, she suddenly deteriorated with acute severe respiratory distress, severe hypoxemia, unstable shock, and hemoptysis. Chest X-ray showed bilateral lung infiltrate suggestive of diffuse alveolar hemorrhage. She was transferred to paediatric intensive care and mechanical ventilation, including high-frequency ventilation, was required. Flexible bronchoscopy confirmed diffuse alveolar hemorrhage. She was treated with pulses of methylprednisolone, intravenous cyclophosphamide, and plasmapheresis.

Learning Objectives

  • Explain pulmonary manifestations in lupus

  • Discuss therapeutic approach to diffuse alveolar hemorrhage

  • Describe prognosis of this life-threatening complication of SLE

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