Article, Rheumatology

Central cyanosis due to severe pulmonary hypertension combined with pericarditis as the initial manifestation of systemic lupus erythematosus

Case Report

Central cyanosis due to severe pulmonary hypertension combined with pericarditis as the initial manifestation of systemic lupus erythematosus


systemic lupus erythematosus is a multisystemic autoimmune disorder. The initial manifestation complicating any organ system either singly or in combination is protean. Herein we report a 26-year-old female patient with fresh SLE whose initial manifestation was central cyanosis caused by severe pulmonary hypertension and acute pericarditis. The symptoms were relieved dramatically after treatment with steroid and bosentan. Accurate and Timely diagnosis in SLE-associated pulmonary hypertension may be life saving.

systemic lupus erythematosus is a multisystemic autoimmune disorder. Articular, cutaneous, and renal systems are the most common areas of involvement [1]. Nonspecific symptoms, such as fatigue, malaise, anorexia, and fever, are also common presenting symptoms. Initial manifestation with central cyanosis caused by severe pulmonary hypertension (PH) and cardiac involvement is rare. We describe a fresh case of SLE whose initial presenting feature of SLE was central cyanosis due to severe PH combined with acute pericarditis.

A 26-year-old woman presented to our emergency depart- ment (ED) with progressive shortness of breath, Dyspnea on exertion, and fever for 1 day. Her medical history was unremarkable. She visited a clinic 1 day before, and common cold was told. On ED arrival, the vital signs were blood pressure of 115/57 mm Hg, heart rate of 126 beats per minute, respiratory rate of 32 breaths per minute, body temperature of

38.78C (101.78F), and oxygen saturation as measured by pulse oximetry was 80% on room air. On physical examination, she had lips cyanosis, pale conjunctiva, and cold distal extremities. Rapid heart beats with pericardial friction rub and engorged jugular vein were also noted. The breath sound was clear bilaterally. The chest x-ray revealed enlarged cardiac silhouette and pulmonary trunk engorgement without obvious pneumo- nia patches. The 12-lead Electrocardiography showed sinus tachycardia with Right axis deviation, poor R-wave progression, and electrical alternans. She received an oxygen mask with a flow rate of 8 L/min and continuous 3-lead ECG

monitoring. Under this condition, the arterial blood gas analysis showed a pH of 7.48, Pco2 of 29.8 mm Hg, Po2 of

197.5 mm Hg, and HCO of 22.4 mmol/L. The laboratory tests showed a white blood count of 10200/lL, hemoglobin level of


8.7 g/dL, platelet count of 282000/lL, and C-reactive protein level of 16.9 mg/dL. The cardiac enzyme was within normal level. The echocardiogram revealed severe PH (estimated pulmonary artery pressure of 93 mm Hg), tricuspid regurgi- tation, and dilated right atrium, right ventricle, and pulmonary

trunk. There was also moderate pericardial effusion without chamber compression. After initial stabilization, she was admitted to the intensive care unit under the impression of acute pericarditis combined with severe PH. During hospital- ization, ventilation-perfusion scan showed no V-Q mismatch. Autoimmune profiles revealed hypocomplementemia with C3 of 41 mg/dL (normal, 79-152 mg/dL), C4 of 10 mg/dL (normal, 16-38 mg/dL), antinuclear antibody titer of more than 1:5120, positivity for anti-double-stranded DNA antibody, and positivity for anti-smith antibody. She fulfilled the revised criteria of American Rheumatism Association for the diagnosis of SLE including serositis, hematologic disorder, immunologic disorder, and antinuclear antibody [2]. She received methyl- prednisolone and bosentan (a dual endothelin receptor antagonist) treatments. The symptoms were relieved dramat- ically, and she was discharged 12 days later.

Pulmonary hypertension is an uncommon pulmonary manifestation of SLE, occurring in 0.5% to 14% of patients with SLE [3-7], although it has well-known association with other connective tissue disease, especially scleroderma and overlap syndrome [8]. Goupille et al [9] found that PH is often diagnosed after a well-established SLE, with a mean delay of 4.9 years. The initial clinical presentation of PH is usually mild or nonspecific. Pulmonary hypertension can also occur secondary to the pulmonary embolism, hypoxia, respiratory failure, or heart failure. These would make the Diagnosis difficult. Symptomatic PH includes progressively worsening dyspnea, fatigue, palpitation, and dry cough [10]. Loud secondary heart sound, ascites, increased jugular venous pressure, and parasternal heave have been men- tioned. There is up to 60% of patients having Raynaud phenomenon [10]. However, most cases are diagnosed until PH is symptomatic or moderate to severe. Early detection is rare. It is also unusual for combined severe PH and acute pericarditis to be the initial manifestation of SLE.

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248.e2 Case Report

The differential diagnoses for fever, dyspnea, and central cyanosis are many, for example, pneumonia, myocarditis, pulmonary embolism, Infective endocarditis with septic emboli to lung parenchyma, severe sepsis, and septic shock. In the ED setting, the diagnostic clue may be the combined positive physical findings (engorged jugular vein, desatura- tion, pale conjunctiva, etc), 12-lead ECG, and chest x-ray implying multiorgan involvement such as right ventricular strain, possible pulmonary hypertension, and pericardial effusion. Bedside-focused assessment of pericardial space and bilateral pleural space may help diagnosis. In the young female patient group, SLE should be considered, although there is no history of connective tissue disease.

In general, SLE-associated PH often has devastating prognosis. Chung et al [11] reported 1- and 5-year survival rates of 50.5% and 16.8%, respectively. Moreover, PH is the third leading cause of death in Korea [12]. Accurate and timely diagnosis may be life saving. The treatment including the use of immunosuppressants, steroids, vasodilators, prostaglandin I2 analogues, bosentan, and anticoagulants have been reported [7,13,14].

In conclusion, because the clinical presentation of SLE is protean, timely diagnosis and treatment for such patients in the ED remains as a challenging task, which mainly depends on the vigilance of emergency physicians.

Ruei-Fang Wang MD Tzu-Yao Hung MD Emergency Department

Shin-Kong Wu Ho-Su Memorial Hospital

Taipei 111, Taiwan

Chee-Fah Chong MD, MS Tzong-Luen Wang MD, PhD Chien-Chih Chen MD, MS Emergency Department

Shin-Kong Wu Ho-Su Memorial Hospital

Taipei 111, Taiwan School of Medicine

Fu Jen Catholic University Taipei 242, Taiwan

E-mail address: [email protected] doi:10.1016/j.ajem.2007.04.007


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