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APCU 07 The outcome of a young lady with lupus carditis
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  1. Nurul Nadiah MZ1,
  2. Nik Ahmad Hilmi NMZ1,
  3. Raja Abdul Wafy RMR1,2,
  4. Zurkurnai Y1,3,
  5. Wan Yus Haniff WI1,3,
  6. Siti Aisyah H1,3,
  7. Nor Mardhiah MY1 and
  8. Wan Syamimee WG1
  1. 1Cardiology Unit, Department of Internal Medicine, Hospital Pakar Universiti Sains Malaysia
  2. 2Department of Medicine, Faculty of Medicine and Health Sciences, Universiti Putra Malaysia
  3. 3School of Medical Sciences, Universiti Sains Malaysia, Malaysia

Abstract

Introduction Lupus carditis is a serious manifestation of systemic lupus erythematosus (SLE) affecting the heart. It is a significant contributor to morbidity and mortality in SLE patients caused by immune complex deposition in heart tissues leading to inflammation. Antinuclear antibodies (ANA) and anti-double stranded DNA (anti-dsDNA) antibodies, are commonly implicated. We report a case of lupus carditis in a young lady who improved following intravenous immunoglobulin (IVIG) on top of standard therapy.

Case Presentation A 38-year-old lady was admitted with one month history of fever, arthralgia, myalgia, and 5kg of unintentional weight loss. Initial investigations revealed pancytopenia and connective tissue disease (CTD) screening was negative. Her bone marrow aspiration and trephine biopsy suggested reactive marrow with dysplastic changes. She developed multiple episodes of altered sensorium and seizure, but brain imaging revealed no focal lesions or haemorrhages. Repeated CTD screening was positive for ANA (1:640 speckled pattern), anti-dsDNA, anti-ribosomal P protein, and anti-AMA M2. Later, she experienced cardiac arrest due to pulseless ventricular tachycardia, requiring resuscitation and defibrillation. Post-resuscitation echocardiography showed global hypokinesia with left ventricular ejection fraction (EF) of 25% and raised in ProBNP >9000. Thus, diagnosis of SLE involving multiple organs, including lupus carditis, cerebritis, musculoskeletal and haematological involvement was made. She was started high dose IVIG for 5 days together with intravenous cyclophosphamide for six cycles and tapering doses prednisolone. A follow-up echocardiography after six cycles of cyclophosphamide showed a significant improvement in EF from 25% to 60%, along with concentric remodelling.

Discussion Early and aggressive treatment is crucial in managing severe SLE with multiorgan involvement. Lupus carditis is rare but potentially fatal complication, affecting up to 10% of SLE patients. There were few case reports of patients with lupus carditis treated with mycophenolate mofetil and high dose corticosteroid, of which 50% showed significant improvement. However, there were limited case reports in the literature where IVIG was used with cyclophosphamide and prednisolone to treat lupus carditis. The gold standard for diagnosis of lupus carditis is endomyocardial biopsy, but not routinely used due to its potential complications and non-specific. This case illustrated the novel use of IVIG with cyclophosphamide and prednisolone, significantly improving patient outcomes.

Conclusion Recognizing lupus carditis is vital so that appropriate therapy can be initiated. Early treatment with IVIG, in addition to standard immunosuppressive therapy, may be an alternative treatment in severe cases of lupus carditis, as demonstrated in this case.

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