INTRODUCTION: Systemic lupus erythematosus is a systemic autoimmune disease that affects numerous organ systems and can affect any part of heart. Few case reports have demonstrated a beneficial effect of intravenous immunoglobulin (IVIG) therapy in lupus myocarditis. We present a case of severe lupus myocarditis treated with IVIG under hemodynamic monitoring.
CASE PRESENTATION: A 32-year-old woman presented with a chief complaint of worsening arthralgia and myalgia over the preceding 2 months. She had presented to her primary care physician 6 months earlier with complaints arthralgia and she was found to have a positive anti-nuclear antibody (ANA). A 2-D echocardiogram showed normal ejection fraction (EF) and trace mitral regurgitation (MR). Physical examination on admission was significant for discoid rash over the bridge of her nose and a grade III/VI holosystolic murmur loudest over the apex, radiating to axilla. Laboratory results revealed pancytopenia, positive ANA and anti-DNA titers, rhabdomyolysis, liver and renal dysfunction.She was treated with pulse dose steroids, cyclophosphamide and oral hydroxychloroquin for lupus flare-up. By day ten there was marked improvement in laboratory tests. On 18th hospital day she was intubated for pulmonary edema following a blood transfusion. Physical examination at that time revealed S3 and S4 gallops, bilateral crackles and 2 + peripheral edema. A portable CXR showed pulmonary edema with bilateral pleural effusion and 2-D echocardiogram showed global hypokinesis (EF 20%), 3+ MR and a small-moderate size pericardial effusion. A Swan-Ganz catheter (SGC) inserted the same day revealed: pulmonary capillary wedge pressure (PCWP) = 27 mm Hg, cardiac output (CO) = 2.76 L/min, systemic vascular resistance (SVR) = 1894 dyne sec/cm5. Milrinone infusion with IV furosemide was started and CO increased to 3.5 L/min. The following day milrinone was switched to dobutamine and nesiritide and CO remained at 3.68 L/min. On day 20 the patient was also started on IVIG. On day 21 the hemodynamics while on same doses of dobutamine and nesiritide were: PCWP = 19 mm Hg, CO = 5.19 L/min, SVR = 1264 dyne sec/cm5. A repeat echocardiogram on day 22 also showed an increase in stroke volume and CO without any change in left ventricle dimensions (Table 1). Over the next few days nesiritide, IVIG and dobutamine were stopped and on day 26 CO was 5.34 L/min (Figure 1). On day 29 the patient became febrile and septic secondary to a urinary tract infection requiring pressor support. Her condition deteriorated further and she died of a cardiac arrest a week later.
DISCUSSIONS: To our knowledge this is the first case report of IVIG use for lupus myocarditis that showed improvement in cardiac function within 48 hours, both by SGC and echocardiogram. Myocarditis in SLE may be related to an immunological phenomenon although accelerated coronary artery disease, hypertension, anemia, valvular disease may also contribute towards systolic myocardial dysfunction.IVIG has been used to treat different clinical manifestations of SLE with an overall success rate between 33-100%. Overall, an increase in C3, C4, and total complement hemolytic activity and a fall in anti-ds DNA antibody levels can be expected with IVIG therapy.
CONCLUSION: Our case shows the modest hemodynamic response to dobutamine, milrinone and nesiritide in a patient with lupus myocarditis and cardiogenic shock. The introduction of IVIG therapy coincided with significant and sustained hemodynamic recovery. Whether this represented the natural clinical history in this patient or was directly related to the introduction of IVIG therapy cannot be proven. However the dramatic temporal association certainly suggests the beneficial role that IVIG played in our patient. Additional case series would provide important information on the utility and timing of this therapy.
DISCLOSURE: Ather Anis, None.