Background Myocarditis in pediatric lupus is uncommon but carry a significant morbidity and mortality.
Methods We reviewed our cohort of children with lupus registered between January 1993 to November 2018. Of the 140 lupus patients that were diagnosed during this time, 4 had myocarditis.
Results Patient 1 was an 11-year-old girl who presented with fever, rash and generalized body swelling for 3 months. Physical examinations revealed heart rate 148/min, respiratory rate 30/min, and blood pressure (BP) 100/80 mmHg. She had pallor, malar rash, hepatomegaly and gallop rhythm. Investigations are detailed in the table. She received intravenous immunoglobulin and initiated on pulse methylprednisolone and followed by pulse intravenous cyclophosphamide. She was discharged on oral prednisolone and subsequently tapered. Patient 2 was a 9-year-old girl presented with fever and rash for 3 months with lethargy for 1 day. She had pallor, malar rash, hepatomegaly. Physical examinations revealed heart rate 132/min, respiratory rate 32/min, feeble peripheral pulses and BP 100/80 mmHg. She received intravenous cyclophosphamide monthly for 6 months along with oral prednisolone in tapering dose. Patient 3 was a 12-year-old girl, who was known to have SLE for 2 months, presented with fever, malar rash, and photosensitivity. She had stopped taking steroids for 2 weeks. On examination, she had heart rate 122/min, respiratory rate 28/min and BP 100/72 mmHg. She had pallor, alopecia, oral ulcers, malar rash, gallop rhythm and tender hepatomegaly. She was given pulse methylprednisolone and furosemide therapy followed by oral prednisolone in gradually tapering dose. Patient 4 was an 11 year-old-girl who was known to have SLE for 6 months, presented with pain in the small joints, low grade fever, and cough for 1 month. She had stopped steroids on her own. On examination, she was found to have anasarca, malar rash and oral ulcers. She had heart rate 128/min, respiratory rate 32/min, BP 108/74 mmHg, soft systolic murmur at the apex with normal heart sounds and hepatosplenomegaly. She was given pulse methylprednisolone along with cyclophosphamide in view of severity of symptoms. She developed multiorgan dysfunction and died after 8 days in hospital.
Conclusions Myocarditis in lupus is uncommon but carries a significant morbidities. The presence of global hypokinesia with a low ejection fraction on two dimensional echocardiography (2DE) is a strong pointer towards the diagnosis. Immunosuppressant drugs with supportive therapy remain the preferred treatment. Early diagnosis and prompt treatment may be very rewarding.
Funding Source(s): None
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