A 31‐year‐old woman with Raynaud's syndrome presented to the ED with a 2‐day history of a severe headache and blurry vision lasting 2 days. Review of systems was notable for photosensitivity, malar rash, and arthralgias. Physical examination revealed a partial CN III palsy, axillary lymphadenopathy, and multiple oral ulcers. After admission to the hospital, MRI with contrast and venography of the brain demonstrated findings consistent with thrombosis of the left sigmoid sinus and resultant venous infarction in the left temporal lobe. On the second day of hospitalization, she developed severe substernal chest pain, and EKG showed transient ST elevations in leads II, III, and aVF. Laboratory data revealed troponin I elevation to 33.7 ng/mL (reference range: 0.00‐0.079 ng/mL) and CKMB elevation to 329 U/L (reference range: <16 U/L). Rheumatologic workup demonstrated an ANA titer of 1:1280 in a speckled pattern, SS‐B titer > 6 (reference range: <1), ESR elevation to 70 mm/hour, and markedly decreased levels of C3 and C4. A hypercoagulable workup demonstrated decreased protein S levels, normal protein C levels, negative factor V Leiden, and negative anticardiolipin antibodies. Transthoracic echocardiography showed a normal ejection fraction with no wall motion abnormalities. Coronary angiography the following day revealed normal coronary arteries.
The findings in this patient led to the initial diagnosis of systemic lupus erythematosus (SLE). The concurrence of variant angina and central venous thrombosis in this patient with SLE is an unusual clinical presentation that has not previously been described. Variant angina (also known as Prinzmetal's angina) is a rare phenomenon characterized by a spontaneous episode of angina associated with ST‐segment elevations on EKG. Variant angina is thought to involve coronary artery vasospasm that results in a transient marked reduction in the lumen of an epicardial coronary artery resulting in myocardial ischemia or infarction depending on the time course. The mechanism of coronary artery vasospasm has not been completely elucidated; possibilities include changes in autonomic neural control, changes in contractile function, and the presence of vasoactive mediators in contact with dysfunctional endothelium. This case suggests that inflammatory and vasoactive mediators play a multisystemic role in thrombosis and endothelial function and may have led to the concurrence of these events.
This case supports the suggestion that coronary artery vasospasm may result from inflammatory mediators that play a role in thrombosis as well as endothelial function.
M. Markowitz, none.
To cite this abstract:Markowitz M, Flansbaum B, Dibner R. A Beguiling Concurrence of Central Venous Thrombosis and Variant Angina in a Newly Diagnosed Case of SLE. Abstract published at Hospital Medicine 2008, April 3-5, San Diego, Calif. Abstract 159. Journal of Hospital Medicine. 2008; 3 (suppl 1). https://www.shmabstracts.com/abstract/a-beguiling-concurrence-of-central-venous-thrombosis-and-variant-angina-in-a-newly-diagnosed-case-of-sle/. Accessed April 1, 2020.