Background: Acute myocardial dysfunction is an uncommon but potentially fatal complication in systemic lupus erythematosus (SLE). We describe the outcome in a small series of Asian Indian patients and examine associated factors.
Methods: SLE patients who fulfilled the 2012 SLICC criteria and developed new-onset myocardial dysfunction were included in this retrospective case series. Acute myocardial dysfunction was defined as global hypokinesia and left ventricular ejection fraction (LVEF)<50% on echocardiography (with or without symptoms) in patients with SLE. Survival was assessed using Kaplan-Meier survival analysis and Cox regression.
Results: This study included 37 patients with mean age 28.2 ± 11.2 years and median (range) LVEF of 35% (18-48%) at presentation. A majority had active disease, with SLEDAI-2k ≥ 5 in 26 (of 28). All patients received oral corticosteroids and a majority received additional immunosuppression, including pulse methylprednisolone in 28 and cyclophosphamide in 27. Nine patients died during hospitalisation (25%), a majority due to infections. Death was significantly associated with elevated procalcitonin at presentation (p = 0.05), elevated white cell count (p = 0.02) and low complement C3 (p = 0.03). In those who survived, long-term outcomes were good, with complete myocardial recovery in 14 (64%). A higher ejection fraction at presentation was associated with complete recovery.
Conclusions: In this small series of patients of SLE with acute myocardial dysfunction, we report a significant in-hospital mortality due to infections. Many of the patients who died had elevated procalcitonin at presentation. A diligent search for infection seems prudent in lupus patients who present with acute myocardial dysfunction. Key Points • In patients of SLE with acute myocardial dysfunction who were treated with immunosuppression, there was significant short-term mortality due to infections. • This mortality was associated with elevated procalcitonin at baseline and may suggest some of them had pre-existing hidden sepsis. • A prudent search for infections in these patients before immunosuppression may help to decrease short-term mortality.
Keywords: Myocardial dysfunction; Myocardial recovery; SLE; Survival.