J Cardiol 2007 May; 49（5）: 273 – 276

Systemic Lupus Erythematosus
Initially Manifesting as Acute
Pericarditis Complicating WithCardiac Tamponade : A Case
Report＊Tetsuichiro

SHIMIZU, MD

Mitsunobu

MURATA, MD

Hidenori

TOMIZAWA, MD

Takeshi

MITSUHASHI, MD

Takaaki

KATSUKI, MD

Kazuyuki

SHIMADA, MD, FJCC

Abstract ─────────────────────────────────────────────────────────────────────────────────────────────────────────────────────────────────────────────────────────────────────
A 23-year-old woman was admitted with progressive shortness of breath. Echocardiography showed alarge volume of pericardial effusion, which indicated cardiac tamponade. Yellowish and puriform fluidwith increased white blood cell count（neutrophil dominant）
was aspirated, but antibiotics were ineffective.
Further examination revealed the presence of positive anti ds-DNA antibody, anti SS-A antibody and anti
Sm antibody, resulting in a diagnosis of systemic lupus erythematosus. Her condition was smoothlyimproved by predonisolone administration. Cardiac tamponade is a rare initial manifestation of systemic
lupus erythematosus.
─────────────────────────────────────────────────────────────────────────────────────────────────────────────────────J Cardiol 2007 May; 49（5）: 273−276

Key Words■ Cardiac tamponade■ Complications（systemic lupus erythematosus）■ Pericarditis（neutrophil dominant pericardial effusion, steroids reactive）

INTRODUCTION

CASE REPORT

Serositis including pericarditis is a common
manifestation of systemic lupus erythematosus（SLE）, and pericarditis is the most common cardiac manifestation of this condition.1）Cardiac tamponade can occur during any stage of SLE pericarditis, but rarely appears as the initial manifestation of serositis.2）Moreover, cardiac tamponade isextremely rare as the initial manifestation of SLE.3）
This report describes a case of SLE initially manifesting as cardiac tamponade.

A 23-year-old Japanese woman with a history ofhyperthyroidosis and gestosis was admitted to Jichi
Medical School Hospital with a 1-month history offever and cough. Her body temperature hadincreased to 38.5 °C 2 days prior to admission, andwas resistant to several antibiotics. Chest radiography showed purse string-like cardiac appearance,
and ultrasonography revealed remarkable pericardial effusion（Figs. 1, 2）.
The patient had no history of diseases associatedwith cardiac tamponade, such as aneurysm,
myocardial infarction, amyloidosis, trauma, or──────────────────────────────────────────────
＊自治医科大学附属病院 循環器内科 : 〒 329−0431 栃木県下野市薬師寺 3311−1 ; （現）茅ヶ崎市立病院
外科 : 〒 253−0042
神奈川県茅ヶ崎本村 5−15−1＊of Surgery, Chigasaki Municipal
Cardiac Department of Internal Medicine, Jichi Medical School Hospital, Tochigi ; （present）Division
Hospital, Kanagawa
Address for correspondence : SHIMIZU T, MD, Division of Surgery, Chigasaki Municipal Hospital, Honson 5−15−1, Chigasaki,Kanagawa 253−0042 ; E-mail : shimitetsu@jichi.ac.jp
Manuscript received November 29, 2006 ; revised February 6, 2007 ; accepted February 6, 2007273

274

Shimizu, Murata, Tomizawa et alFig. 1 Chest radiograph showing purse string-like cardiac appearance

renal failure.4）She also had no symptoms of SLE
prior to admission, such as photosensitivity, arthritis, skin rash and neuropathy. Her height was 162
cm, weight 48 kg, blood pressure 80/60 mmHg,
pulse rate 122 beats/min, body temperature 37.2 °C,and her thyroid gland was not palpable. No edema
was found in the arms and legs.
Electrocardiography revealed sinus tachycardia
and ST elevation in all leads except for aⅤR .
Laboratory tests upon admission showed whiteblood cell count 8,500/μl, hemoglobin 12.9 g/dl,
platelet count 329,000/μl, serum creatinine
0.84 mg/dl, serum total protein 8.3 mg/dl, albumin
3.1 mg/dl, ALT 67 U/l, AST 114 U/l, LDH 334 U/l,
CPK 49 U/l, CRP 6.22 mg/dl, Na 126 mmol/l, K

5.0 mmol/l, Cl 95 mmol/l, prothrombin time
14.7 sec, international normalized ratio 1.35, activated partial thromboplastin time 42.3 sec, C376 mg/dl, C4 9 mg/dl, CH50 26.9 U/ml. Echocardiography revealed a large volume of pericardial effusion, with ejection fraction of 60%, and dilatation
with poor collapse of the inferior vena cava.
Valvular function was normal.
On admission, she exhibited progressive shortness of breath and a markedly distended neck vein.
Immediate pericardiocentesis aspirated 225 ml ofyellowish and puriform fluid with opacity. The
opacity solidified and precipitated at the bottom ofa test tube（ Fig. 3）. Pericardial fluid analysis
revealed neutrophil dominant white blood cellcount 22,100/μl（neutrophil 81.5%, monocyte
14.0%, lymphocyte 4.5%）, characteristic of a bacterial infection. Imipenem/cilastatin sodium wasprescribed. On the following day, echocardiography
showed almost the same volume of pericardial effusion as the previous day. No bacteria were observedin the fluid.
Three days after admission, antinuclear antibody（ANA）
and rheumatoid arthritis particle agglutination test were positive, indicating the possibility ofconnective tissue disease, which was confirmed by
the presence of anti double stranded（ ds）-DNA
antibody, anti SS-A antibody and anti Sm antibody.
Therefore, the cause of the pericarditis was identified as SLE. Prednisolone 30 mg/day was administered and her pericarditis improved soon.
The diagnosis of SLE was established on thepresence of serositis（pericarditis）, leukopenia anddetection of ANA, anti ds-DNA antibody, anti SSA antibody and anti Sm antibody matching 4 of theFig. 2 Cardiac ultrasound images showing pericardial effusion
Left : Short-axis view. Right : Long-axis view.

J Cardiol 2007 May; 49（5）: 273 – 276

Neutrophil Dominant Pericardial Effusion

Fig. 3 Photograph of the product of pericardiocentesis
Puriform deposit was observed at the bottom ofthe yellowish fluid. Neutrophil dominant whiteblood cell increase seemed to indicate bacterialinfection.

11 SLE American Rheumatology Association（ARA）criteria for SLE. Follow-up echocardiography revealed a small amount of pericardial effusion. One month after admission, the patient wasdischarged.
DISCUSSION
This report describes a case of SLE which initially manifested as cardiac tamponade. The cellular
content of the fluids sampled from SLE patients is
usually yellowish, exudative and rarely bloody.5）
Most patients exhibit a preponderance of neutrophils with some fluids containing up to 96% neutrophils. 6） In our patient, the pericardial fluid
showed a white blood cell count of 22,100/μl（neutrophil 81.5%, monocyte 14.0%, lymphocyte4.5%）that is consistent with SLE. However, thefluid was puriform with opacity, suggesting bacterial infection, although the patient s history showedno events associated with bacterial pericarditis suchas trauma, operation, or compromised host.
Nevertheless, treatment by antibiotics was initiallychosen. However, a culture of the pericardial fluidrevealed no bacterial infection.
The differential diagnosis includes other conditions such as viral infection, tuberculosis, maligJ Cardiol 2007 May; 49（5）: 273 – 276

275

nant tumor, endocrine disease, amyloidosis, andconnective tissue disease. Detection of ANA, anti
ds-DNA antibody, and anti SS-A antibody on theeighth day of admission confirmed the diagnosis ofSLE.
Of the ARA criteria for diagnosis of SLE, ANA
titer is very sensitive but is non-specific for thediagnosis of lupus serositis. The SLE latex agglutination slide test and cytologic LE cell examination
are complementary tools that can aid in the differential diagnosis, but are not included in the ARA
criteria for SLE.7）In the present case, the lupus
erythematosus cell test was negative in both peripheral blood and pericardial effusion, although ANA
was positive in both. Unfortunately, no SLE latex
agglutination slide test was performed.
Immunological analysis of pericardial fluid andperipheral blood from SLE patients showed lymphocytic populations and cytokine concentration
pattern.8）
Cardiac tamponade is a conspicuously unusual
event as an initial manifestation of SLE. The rangeof clinical manifestations and the outcome of pericardial tamponade in 395 patients with SLE included tamponade in 10 patients（2.5%）, but tamponade
was the initial manifestation of SLE in only 4patients（1%）.3）In one patient, the tamponade wasfatal, and 2 other patients had recurrent effusionand pericardial thickening.
Other cases of SLE presented with cardiac tamponade as the initial manifestation.6,9,10）The diagnosis was based on the presence of numerous lupus
erythematosus cells in the pericardial effusion. Asimilar case was diagnosed by the detection ofexcessive ANA, and confirmed by the presence of
ds-DNA antibody in serum, and low complementlevels in the blood. 11）The diagnoses were confirmed by appropriate consideration of the manifestations of connective tissue diseases.
Pericarditis associated with SLE is very responsive to prednisolone.12）If the patient is severely ill,
immunoglobulin therapy is occationally added.13）
Despite the patient s discomfort resulting from cardiac tamponade, prednisolone can be expected tosmoothly improve dyspnea or shortness of breath
due to pericardial effusion. If cardiac tamponade is
the first manifestation, the presence of SLE remains
unknown. Consequently, pericardiocentesis or pericardial window placement is indicated to improve
the patient s condition.
Generally, prednisolone improves serositis in276

Shimizu, Murata, Tomizawa et al

most patients, and recurrence is rare. One case ofrecurrence of a moderate sized effusion required anincrease in the prednisolone dosage 2 months after
pericardiocentesis among 10 patients treated in thismanner.3）In addition, a pericardial window wasindicated in 4 of 10 patients.
In summary, cardiac tamponade due to SLE pericarditis is rarely identified immediately, primarily
要because of the low incidence as the initial manifestation. In addition, cardiac effusion associated with
rapid sideration usually indicates bacterial infection（neutrophil dominant）. The present case reportsuggests that careful evaluation of serous effusion
is necessary to determine the most effective courseof treatment.

約急性心膜炎で発症し心タンポナーデをきたした全身性エリテマトーデスの 1 例
清水徹一郎

村田光延

富澤英紀

三橋勝木孝明

島田和幸武司症例は 23 歳，女性．2004 年 5 月半ばより持続する感冒様症状が 6 月某日に増悪し，呼吸困難感を伴ったため当院外来を受診した．胸部 X 線写真上にきんちゃく様心陰影，心エコー図上で全周性の心 水の貯留が認められたため，精査加療の目的で当科に入院した．第 1 病日，心タンポナーデに進展したため，心 穿刺を施行した．心 液は膿性黄色混濁で細胞数 22,100/μl，好中球優位（81.5%）であったため，細菌性心外膜炎を疑いカルバペネム系抗生物質の投与を開始した．しかし，翌々日に心 液はほぼ穿刺前の状態に戻っていた．この間 37−38 ℃の発熱を繰り返していた．第 8病日，抗核抗体 5,120 倍（speckled pattern），血清抗 ds-DNA 抗体 27.1 IU/ml で，漿膜炎（心膜炎）とリンパ球減少（450/μl）を伴うことから，全身性エリテマトーデスによる急性心膜炎と診断した．ステロイド 30 mg/day 内服開始，心 液は速やかに消失した．全身性エリテマトーデスの急性心膜炎は初発症状となることはまれで，心タンポナーデに進展することはさらに極めてまれである．また，詳しい心 液所見の報告例が少ないことから，文献的考察も加えて報告した．

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