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Cardiovascular system (CVS) disease is the third leading cause of mortality in systemic lupus erythematosus (SLE).1 Pathologically it is pancarditis and may affect coronary arteries.2,3 Most often, it is subclinical.2–4 Clinical manifestations, if present, are limited to pericardial and endocardial involvement.2 Clinically apparent myocarditis has been described in a few adult patients.5–9 To the best of our knowledge, no such case has been described in children. We describe two children with clinically manifest myocarditis in SLE.
A 12 year old girl, who was known to have had SLE for 2 months, presented with fever, malar rash, and photosensitivity. She had stopped taking steroids for 2 weeks. On examination, she had tachycardia with normal blood pressure. She had pallor, alopecia, oral ulcers, and malar rash. An examination of the CVS showed gallop rhythm with no murmurs. The rest of the systemic examination was unremarkable except for a tender hepatomegaly. Antinuclear factor (ANF) was strongly positive and anti-dsDNA titres were 198.2 IU/ml (normal 0–5 IU/ml). Chest radiography showed cardiomegaly with normal lung fields. “T” wave changes were seen on electrocardiography. Creatine kinase levels were 220 U/l (normal 10–80 U/l). Echocardiography showed moderate mitral regurgitation, mild tricuspid regurgitation, and global hypokinesia with a left ventricular ejection fraction (LVEF) of 56%. Pericardial effusion was minimal with no vegetations.
She recovered dramatically when given pulse methylprednisolone and decongestive treatment. Later, treatment was started with oral prednisolone at a dose of 2 mg/kg/day. Now 4 years later, she is asymptomatic with an LVEF of 72% on echocardiography.
The second patient, an 11 year old girl who was known to have had 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 shown to have generalised oedema, malar rash, and oral ulcers. She had tachycardia with normal blood pressure. Examination of the CVS showed a soft systolic murmur at the apex with normal heart sounds. She had hepatosplenomegaly. The rest of the systemic examination was unremarkable. Investigations showed normocytic, normochromic anaemia (haemoglobin 54 g/l) and deranged renal functions (blood urea nitrogen 26.5 mmol/l, serum creatinine 220 µmol/l). There was gross albuminuria, with urine microscopy showing red blood cells and casts. Chest radiography showed cardiomegaly with normal lung fields. Echocardiography showed moderate mitral regurgitation, trivial tricuspid regurgitation, and global hypokinesia with an LVEF of 18%. No pericardial effusion or vegetations were present. ANF was strongly positive and anti-dsDNA titres were 1531 IU/ml.
Treatment was started with pulse methylprednisolone. However, she developed cardiogenic shock on day 6 of admission. Pulse cyclophosphamide was started together with supportive treatment. She developed multiorgan dysfunction due to shock and died after 8 days in hospital. A necropsy showed diffuse proliferative glomerulonephritis, serositis, and myocarditis. Myocarditis was evident from the presence of focal collection of inflammatory cells with damage to adjacent myofibres, fibrinoid necrosis, and interstitial oedema. Fibrinous pericarditis was also seen, but the coronary arteries and valves were normal.
Myocardial dysfunction in SLE is multifactorial; important contributors are immune injury, coronary vasculitis, valvulopathy, hypertension, and adverse effects of drugs.3 Endomyocardial biopsy remains the preferred method for the diagnosis of myocarditis, but carries a high risk of mortality in sick children2 and was not carried out in our patients. In such a case, the presence of global hypokinesia with a low LVEF on echocardiography is a strong pointer towards the diagnosis.2 The diagnosis is even more definite in the second child as it was proved at necropsy.3 Lupus myocarditis has been associated with skeletal myositis5 and anti-Ro and anti-La antibodies.4
Immunosuppressant drugs with decongestive agents remain the preferred treatment.2,3 Most patients respond dramatically. The second patient died despite immunosuppressive treatment and supportive care, probably because of delays in diagnosis and initiation of treatment. Finally, it is important to remember that acute myocarditis can be a fatal complication of SLE. Early diagnosis and prompt treatment may be very rewarding.