a b s t r a c t

Background: The objective of this study was to describe the characteristics of patients presenting to the emergency department with cardiac symptoms subsequently diagnosed to have systemic lupus erythematosus (SLE).

Methods: The authors performed a review of newly diagnosed SLE patients at 2 hospitals in Tainan city between January 2010 and December 2013. Patients initially presenting with cardiac symptoms were included. Demographic data, presenting symptomatology, laboratory data, and imaging studies were obtained and analyzed.

Results: Eight cases, including 5 female and 3 male patients, were identified during the 4-year study period. The mean age was 37 (range, 15-54) years. Pericardial effusion (63%) and Mitral regurgitation (63%) were the most common Cardiac abnormalities, followed by impairment of left ventricular systolic function (25%) and tricuspid regurgitation (13%). Most patients showed signs of increased generalized inflammation and immunological activity with elevated levels of C-reactive protein (100%) and anti-dsDNA (88%) and decreased complement levels (63%). The median duration from admission to the diagnosis of SLE was 6.3 (range, 1-13) days, and all patients showed multiple-organ involvement in addition to the cardiovascular system.

Conclusions: Patients presenting to the emergency department with cardiac symptoms without a history of cardiopulmonary disease or traditional cardiovascular disease risk factors should be assessed for an underlying cause of cardiac decompensation. If the patients exhibit extraCardiac manifestations or their illnesses involve multiple-organ systems, Screening tests for autoimmune diseases such as SLE are mandatory.

(C) 2014

Introduction

systemic lupus erythematosus is a systemic autoimmune disease involving multiple-organ systems with a myriad of clinical presentations. The heart is a frequently affected organ, and the prevalence of cardiac involvement has been estimated to be up to 50% [1,2]. Although cardiovascular disease in SLE patients occurs earlier than in the general population and is the major cause of death [3], cardiac involvement is rarely the presenting manifesta- tion of SLE [4]. We present a series of previously undiagnosed SLE patients who visited the emergency department (ED) with cardiac symptoms. The aim of this study was to describe the clinical characteristics, results of imaging studies and laboratory tests, and the diagnostic clues of these patients.

Methods

This study was approved by the institutional review board of Chi-Mei Medical Center. We reviewed newly diagnosed SLE

* Corresponding author. Emergency Department, Chi-Mei Medical Center, 901 Chung-Hwa Road, Yung Kang, Tainan 710, Taiwan. Tel.: +886 6 2812811×57196; fax: +886 6 2816161.

E-mail address: 890502@mail.chimei.org.tw (K.-T. Chen).

patients admitted to Chi-Mei Medical Center and Chi-Mei Hospital, Liouying, from January 2010 to December 2013 and identified patients who presented to the ED with cardiac symptoms. All of the patients fulfilled the 1997 revised American College of Rheumatology criteria of SLE [5]. Patients with preexisting cardiopulmonary disease were excluded. The demographic data, clinical presentations, imaging studies, laboratory results, and the hospital course of these patients were collected for analysis. We recorded the chest radiograph, electrocardiograph, blood count, creatinine, and troponin I data from the first day of ED admission. The remaining laboratory and imaging examinations were per- formed at varioUS times during hospitalization. The results of the imaging studies were based on official Radiology reports. Systemic lupus erythematosus disease activity was quantified using the SLE disease activity index (SLEDAI).

Results

Demographics and clinical features

Eight patients, including 5 female and 3 male patients, were identified during the 4-year period. The mean age was 37 (range, 15- 54) years. The most common symptoms were dyspnea, cough, fever,

http://dx.doi.org/10.1016/j.ajem.2014.06.036 0735-6757/(C) 2014

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and chest pain, each of which was present in more than 50% of the cases. In addition, abdominal/flank pain, skin rash, arthralgia, and decreased urine output were discovered at the initial ED visit (Fig.). A few of these patients had traditional atherosclerotic risk factors:

3 were male (38%), 3 had hypertension (38%), 2 were habitual

smokers (25%), and 3 were older than 45 years (38%).

Laboratory tests

The inflammatory markers of most patients exhibited elevations of C-reactive protein (CRP; 100%) and anti-ds DNA (88%) and low complement levels (63%). Renal abnormalities included hematuria (43%), pyuria (43%), proteinuria (57%), and elevation of creatinine (29%). Among the 5 patients whose Albumin level was measured, all cases showed hypoalbuminemia. Anemia (63%) and thrombocytopenia (50%) were discovered in more than half of the patients, whereas none of these cases exhibited leukopenia. Sixty-three percent of the patients showed elevations of troponin I. Nevertheless, high Brain natriuretic peptide and anticardiolipin antibody titers were found only in 25% and 14% of the patients, respectively. The results of the laboratory tests are tabulated in Table 1.

Electrocardiography and imaging studies

The most common electrographic abnormalities were sinus tachycardia (88%) and nonspecific ST/T-wave changes (25%). Pericar- dial effusion and mitral valve regurgitation were discovered in 63% of the patients. In addition, impairment of the left ventricular function and Tricuspid valve regurgitation were exhibited in 25% and 13% of all cases, respectively. Cardiomegaly, pulmonary edema, and Pleural effusions were frequently found on chest radiographs (88%, 63%, and 38%, respectively). Five patients underwent Computed tomographic scans of the chest, and the common abnormalities were pericardial effusion, pleural effusions, and pulmonary edema (60%, 60%, and 20%, respectively). Table 2 presents the findings of the electrocardiography and imaging studies.

Hospital course

The common admission impressions included heart failure (50%), pneumonia (38%), and Infective endocarditis (25%). In addition, acute coronary syndrome, malignant hypertension, myocarditis, and dengue fever were the other tentative impressions in the ED. It took an average of 6.3 days (range, 1-13 days) to establish the diagnosis of SLE. Two patients underwent drainage for pleural effusions, and 1 patient underwent cardiac catheterization, which was negative

Table 1

Laboratory tests of the involved organ systems

Laboratory tests No. (positive/ordered) % Inflammatory markers

Elevation of CRP 8/8 100

Elevation of anti-dsDNA 7/8 88

Low C3 5/8 63

Low C4 4/8 50

Renal abnormalities

Hematuria	3/7	43
Pyuria	3/7	43
Proteinuria	4/7	57
Elevated creatinine	2/7	29
Hypoalbuminemia	5/5	100
ematologic abnormalities Anemia 5/8 63
Thrombocytopenia	4/8	50
Leukopenia	0/8	0

H

Cardiac abnormalities

Elevation of troponin I 5/8 63

Elevation of BNP 1/4 25

Elevation of anticardiolipin antibody 1/7 14

for coronary artery disease. Three cases required admission to an intensive care unit with an average length of stay of 5.3 days (range, 3-7 days). The average hospital stay was 13.5 days (range, 4-19 days), and the SLEDAI of these patients was 17.3 (range, 12-28). All patients showed multiple-organ involvement. In addition to cardiac disease, the hematologic, renal, and Pulmonary systems were the most frequently involved organs.

Discussion

We describe a series of 8 patients presenting to our ED with cardiac symptoms who were later diagnosed as having SLE. There was a slight preponderance of female (63%) and younger patients (mean age, 37 years). Heart failure, coronary artery disease, and valvular heart disease are less prevalent in the young female patients. Hence, a search for an underlying cause of cardiac decompensation is mandatory in these patients [5]. In addition, these patients presented with multiple extracardiac symptoms such as fever, arthralgia, skin rash, and abdominal/flank pain. Furthermore, most of these patients exhibited various hematologic and renal abnormalities. This pattern of multiorgan system involvement suggests the possibility of autoim- mune disease.

Pericardial and pleural effusions were common findings on imaging studies. Accordingly, many of the patients were mistakenly suspected to have had heart failure. However, low BNP and high CRP

Fig. The most common symptoms of patients with SLE.

Table 2

Imaging study results of these SLE patients

Imaging studies No. (positive/ordered) % Electrocardiography

Sinus tachycardia 7/8 88

Nonspecific ST/T-wave changes 2/8 25

Echocardiography

Pericardial effusion	5/8	63
Mitral valve regurgitation	5/8	63
Impairment of left ventricular function	2/8	25
Tricuspid valve regurgitation	1/8	13
Chest radiograph
Cardiomegaly	7/8	88
Pulmonary edema	5/8	63
Pleural effusion	3/8	38
Chest CT Pericardial effusion	3/5	60
Pleural effusion	3/5	60
Pulmonary edema	1/5	20

P.-Y. Chen et al. / American Journal of Emergency Medicine 32 (2014) 1117–1119 1119

levels suggest that serositis, instead of a transudate from heart failure, was the Underlying etiology of the pericardial and pleural effusions.

The combination of fever and elevations of CRP typically prompted physicians to search for infectious sources, such as pneumonia and infective endocarditis, as initial impressions for these patients. Nevertheless, the symptoms often deteriorated despite the commence- ment of antibiotic treatment. The poor response to antibiotics suggests that systemic inflammatory diseases, instead of infectious causes, may be the real cause of the patient’s illness.

Previous studies had shown that many of the presentations of cardiac involvement, including pericarditis, myocarditis, valvular heart disease, and coronary artery disease, are associated with flares of SLE [1,3,6]. In addition, active disease in other organs usually accompanies the presence of cardiac abnormalities [1]. In our study, high levels of anti-ds DNA and CRP, as well as low levels of complement, indicate elevated generalized inflammation and immunological activity [6]. Moreover, all patients had active disease with mean SLEDAI scores of 17.3 during hospitalization. These findings are consistent with previous studies.

Early diagnosis and initiation of treatment of SLE often leads to a rapid recovery of the cardiac abnormalities. A case series from Singapore revealed that early immunosuppressive therapy in acute lupus myocarditis resulted in good outcomes and normalization of the heart functions [6]. Roldan et al [7] presented the results of long-term follow-up for SLE-related valvular disease and discovered that valvular abnormalities frequently resolved or reduced in size after treatment of SLE. Estes and Christian [8] reported that pericarditis often occurs together with pleural effusions as a part of a generalized serositis. High-dose corticosteroids alleviate pericardial effusion and may diminish the requirement for drainage [9]. In addition to accelerated atherosclerosis, cardiac ischemia due to vasculitis is also

encountered in SLE patients with active disease. These patients may also benefit from Corticosteroid therapy to avoid unnecessary invasive procedures [1].

Patients presenting to the ED with cardiac symptoms without a history of cardiopulmonary disease or traditional cardiovascular disease risk factors should be assessed for an underlying cause of cardiac decompensation. If the patients exhibit multiorgan system involvement, screening tests for autoimmune diseases such as SLE are mandatory. Early diagnosis and initiation of treatment of SLE often leads to a rapid recovery of the cardiac abnormalities.

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