A 34-year-old woman comes to the emergency department because of sharp chest pain that radiates to the left shoulder. The pain increases with inspiration and is partially relieved by sitting up and leaning forward. Review of her outpatient medical records shows that she was seen for a facial rash 6 months ago. She is also being evaluated for proteinuria that was identified during her last clinic appointment. Which of the following is the most likely cause of this patient's chest pain?
Manifestations of systemic lupus erythematosus | |
Clinical |
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Laboratory |
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dsDNA = double-stranded DNA. | |
This patient's chest pain is characteristic of acute pericarditis, which along with her facial rash and proteinuria is suggestive of underlying systemic lupus erythematosus (SLE). SLE is a chronic autoimmune disease predominantly affecting women age 20-40 that causes constitutional and multisystemic symptoms. Serosal inflammation is common in SLE and most often manifests as pleuritis or pericarditis.
Pericarditis presents with severe and constant middle or left chest pain that may radiate to the neck and shoulders (particularly the trapezius ridge). The pain increases on inspiration (pleuritic) and is relieved by sitting up and leaning forward (postural). Auscultation of the chest reveals a scratchy sound called a pericardial friction rub that is best heard when the patient is leaning forward or lying prone. Additional cardiovascular manifestations in SLE include pericardial effusion, verrucous (Libman-Sacks) endocarditis, and increased risk of coronary artery disease.
(Choice A) Aortic dissection occurs in patients with long-standing hypertension and those with Marfan and Ehlers-Danlos syndromes (the 2 latter conditions cause cystic medial necrosis of the aortic wall). It presents with abrupt-onset, severe, tearing chest pain that radiates to the back.
(Choice B) Cardiac tamponade is caused by accumulation of fluid in the pericardial space that prevents the heart from filling properly in diastole. It presents with dyspnea and tachypnea. Physical examination shows distended neck veins, hypotension, diminished heart sounds, and pulsus paradoxus (drop in systolic blood pressure >10 mm Hg on inspiration).
(Choice C) Intimal hyperplasia of the pulmonary arteries is characteristic of both primary and secondary pulmonary hypertension. It presents with dyspnea, malaise, and findings associated with right ventricular failure (eg, jugular venous distention, pedal edema, hepatomegaly). Severe symptomatic pulmonary hypertension is a rare complication of SLE.
(Choice D) Non-bacterial endocarditis (verrucous or Libman-Sacks endocarditis) refers to small, wart-like fibrinous lesions and generalized thickening that can affect the heart valves of patients with SLE. These are typically asymptomatic but may lead to valvular insufficiency or embolism.
Educational objective:
Pericarditis is the most common cardiovascular manifestation associated with systemic lupus erythematosus. It presents with sharp pleuritic chest pain that is relieved by sitting up and leaning forward.