Abstract
Background/Objectives Lupus Myocarditis (LM) is one of the rare, but life-threatening manifestations of SLE. The objective was to describe the clinical, laboratory and echocardiographic findings, management, and outcome of 10 Lupus Nephritis (LN) patients presented with LM.
Patients and Methods A retrospective analysis of data of patients presented to our tertiary care center with LN and new onset myocardial involvement from June 2017 till May 2019, was done. All patients fulfilled the SLICC criteria for the diagnosis of SLE. LM was diagnosed by a combination of new-onset cardiac symptoms and echocardiography showing myocardial involvement in the form of global/segmental hypokinesia and reduced left ventricular ejection fraction (LVEF).
Results Regarding baseline characteristics, there were 10 patients with 8 females, with a mean age of 22.3 (15–31) years and mean duration of SLE diagnosis of 3.8 (0–12) years. 2 patients had a history of hypertension, while none of them had a previous cardiac history or DM.
Regarding the onset of LM, in 6 patients it coincided with a diagnosis of LN, in the others, LN preceded LM with a 2-year mean interval.
Regarding clinical presentation, 4 patients presented with cardiogenic shock, while 5 had symptoms of acute heart failure and 1 had repeated syncope.
Regarding laboratory and renal biopsy data, mean creatinine was 2.6 (0.3–9.6) mg/dl, Protein: Creatinine ratio was 3.6 (1–14) mg/mg. 5 patients had a renal biopsy, 1 had class III LN, 4 had class IV LN, 2 of them progressed to end-stage renal disease (ESRD) and were on maintenance hemodialysis (MHD) at time of presentation with myocarditis.
Baseline Echocardiography showed mean LVEF of 26.3 (15–40)%, LV end-diastolic dimension (LVEDD) of 56.3 (46–62) mm, and LV end-systolic dimensions (LVESD) of 46.1 (30–55) mm.
Regarding management, all patients received pulse steroids, 7 were treated with IV cyclophosphamide, 3 started mycophenolate mofetil, and 1 patient underwent 3 sessions of immunoadsorption.
Follow-up echocardiography after 3 months showed a mean LVEF of 42.8 (15–66)%, LVEDD of 52 (42–62) mm, and LVESD of 38.9 (22–55) mm.
Regarding outcomes, 4 patients had a complete recovery and normalization of LVEF, all of them were females, 3 presented with cardiogenic shock and 1 with acute heart failure, with prompt initiation of immunosuppressive therapy. 2 patients became asymptomatic but achieved partial LVEF improvement. Mean duration to LVEF improvement in all patients was 2.7 (1–4) weeks. 4 patients failed to respond to treatment, three of them died within 3-months of LM diagnosis, including the 2 males and those on MHD, all 3 had biopsy-proven class IV LN.
Conclusion It is crucial to recognize LM in the setting of acute heart failure & cardiogenic shock in SLE patients, as early diagnosis and prompt treatment with pulse steroids and other immunosuppressive drugs may achieve complete remission of myocarditis. Male gender with class IV LN biopsy proven or on MHD may be useful as predictors of poor prognosis.
