Case of Lupus Myocarditis

Dr Theresa Raju, Dr Suma Balan(Rheumatology), Dr C Jayakumar, Amrita Institute of Medical Sciences, Kochi
 
Seven ear old female child 1st born of a consanguineous marriage parentage LSCS presented with history of fever associated with breathlessness 3 months back. 
Evaluation at a local hospital found heart failure(moderate LV dysfunction and EF of 30%, NT pro BNP >25000). The child was treated as viral myocarditis and was treated with IVIG 2gm/kg after pre hydration along with spironolactone 3mg/kg ,furosemide 1mg /kg ,Carvedilol – 3.125mg, and Enlapril 2.5 mg for 1 week 
Following this patient developed  reddish dicolouration of urine for which the child was evaluated in a local hospital which revealed Autoimminehemolytic anemia(Hb-4.6, DCT- Positive), elevated ferritin (806). 
The child was treated with blood transfusion(2 PRBC), methylpred 100mg/day and was referred to AIMS for further management.
At admission she was afebrile, conscious. Systemic examination revealed bilateral basal crackles . Labs showed low C3 and C4, AG reversal , elevated Anti DsDNA, anemia(Hb:7.7g/dl), thrombocytopenia(105ku/ml), marginally elevated CRP, with elevated ESR(105mm/hr) and stonglypositive Lupus Anticoagulant(LAC). 
Cardiac evaluation including ECHO showed Lupus Myocarditis with LV dp/dt:1102mmhg/s, LVEDV:72.4ml, LVESV:39.5ml, LVEF:45% With this background , she was started on pulse methylprednisolone(20mg/kg ) for 3 days and changed to ora lprednisolone (2mg/kg as BD )She was also given first dose of Rituximab. Following the treatment her NTPRo BNP improved to 5737 from >25000.
Eye examination showed one tiny white lesion superior to disc in left eye otherwise normal. With the given treatment, she improved symptomatically and is being discharged with stable vitals. The child was discharged onMycophenolate Mofetil (25mg/kg/day), Tab Omnacortil(1.5mg/kg/day)OD, Tab HCQ (5mg/kg/day),Tab Aspirin (3.5mg/kg/day) OD, Tab Carvidelol(0.1mg/kg/day)OD, Tab Envas (0.1mg/kg)and was asked to review after 2 weeks for next dose of Rituximab.
DISCUSSION:
Lupus Myocarditis: It is a potentially life threateningcondition clinically found in 3-9% of SLE patients. Patient may present with complaints of dysnea, orthopnea, chest pain, pedal edema, fever, diaphoresis, nausea, vomiting, or palpitations. Myocarditis may progress to arrhythmias, conduction disturbances, dilated cardiomyopathy, and heart failure which need to be identified and treated early. The pathophysiology of myocardial dysfunction in SLE is usually multifactorial. The prime factors involved are immunological injury and ischemia. Treatment with high dose corticosteroids  has been shown to improve left ventricular function in lupus myocarditis. Other drugs used in treatment are azathioprine, cyclophosphamide, rituximab and intravenous immunoglobulins