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CASE REPORT

Spontaneous coronary artery thrombosis in the setting of active

lupus mesenteric vasculitis
YNV Reddy1, V Sundaram2, M Tam2 and SA Parikh2
1
Division of Cardiovascular Diseases, Mayo Clinic, Rochester, MN, USA; and 2University Hospitals Case Medical Center,
Harrington Heart & Vascular Institute Division of Cardiovascular Medicine

A 33-year-old male with systemic lupus erythematosus (SLE) presented with acute abdominal
pain and was found to have lupus mesenteric vasculitis on imaging and during exploratory
laparotomy. Post laparotomy he continued to have persistent nausea and dyspepsia and an
electrocardiogram showed evidence of an inferior ST elevation myocardial infarction
(STEMI). Emergency cardiac catheterization showed evidence of thrombotic right coronary
artery occlusion. His coronaries were otherwise normal with no evidence of underlying cor-
onary artery disease. Extensive workup with trans-esophageal echo, serologies for antipho-
spholipid antibody syndrome (APS) and bubble study was negative. This effectively ruled out
LibmanSacks endocarditis, APS-induced arterial thrombus and paradoxical emboli as poten-
tial causes of his STEMI. By exclusion of other causes, the etiology of his STEMI was felt to
be secondary to in-situ coronary artery thrombosis in the setting of active SLE. To the best of
our knowledge, this is the first report of a patient with SLE presenting with both lupus
mesenteric vasculitis and in-situ coronary arterial thrombosis in the absence of
APS. Lupus (2015) 0, 14.

Key words: Cardiovascular disease; thrombosis; systemic lupus erythematosus

Case report leukocytosis of 13 200 cells/uL with neutrophilia,

an ESR of 58 mm/h, CRP 1.92 mg/dL, and
A 33-year-old male with known systemic lupus ery- normal lactate, amylase, lipase and renal function
thematosus (SLE) and Kikuchi disease presented panel. Lupus antibody serologies (anti-double
with a four-day history of nausea, abdominal stranded DNA 17.0 IU/mL, anti-smith >8.0 AI,
pain and bilious vomiting. He had just returned anti-SSA 0.3 AI, anti-SSB <0.2 AI) and comple-
from vacation, where he had had signicant sun ment levels (C3 29 mg/dL, C4 4 mg/dL) were con-
exposure with some resultant photosensitivity and sistent with active disease.
a new rash on his face. He also complained of a Due to his severe pain and signicantly dilated
week-long history of arthralgias, predominantly in loops of bowel on abdominal x-ray, he underwent
his hand joints, pharyngitis and conjunctivitis. He abdominal computerized tomography (CT) with
attempted to control his symptoms at home but contrast. This was highly suspicious for lupus mes-
presented to the hospital due to severe unrelenting enteric vasculitis with signicant bowel wall
abdominal pain with vomiting following ingestion thickening, edema and possible early pneumatosis
of even water. His initial physical examination was of bowel wall with new onset ascites (Figure 1). He
signicant for an erythematous malar rash, dif- had dilated loops of jejunum and proximal ileum
fusely tender abdomen without peritoneal signs with no clear transition point, and evidence of col-
and hypoactive bowel sounds. His vitals were lapsed distal ileum and colon. There was no evi-
otherwise stable. Initial labs were signicant for a dence of thrombus in the superior mesenteric
artery or vein. He had a history of appendectomy
and it was decided to perform an exploratory lapar-
Correspondence to: Dr. Yogesh NV Reddy, Department of otomy to exclude mechanical intestinal obstruction
Cardiology, Mayo Clinic, 200 1st Street SW, Rochester, MN 55905,
USA.
from adhesions and evaluate the viability of the
Email: Reddy.yogesh@mayo.edu bowel, given the concern for mesenteric vasculitis
Received 17 November 2014; accepted 7 January 2015 and gut ischemia.
! The Author(s), 2015. Reprints and permissions: http://www.sagepub.co.uk/journalsPermissions.nav 10.1177/0961203315570167

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Spontaneous coronary artery thrombosis in the setting of active lupus mesenteric vasculitis
YNV Reddy et al.
2

Figure 1 (a) CT showing mesenteric vasculitis with dilated edematous bowel loops (transverse view) (b): CT abdomen with dilated
bowel loops (sagittal view) (c): CT angiogram showing absence of arterial thrombosis in celiac, superior and inferior mesenteric
arteries.

At surgery he was found to have inamed,

dilated and erythematous bowel and mesentery
with no frank necrosis. There was no transition
point in his bowel and no evidence of obstruction
or adhesions. Biopsies were not taken due to the
friable appearance of his gut. About 1 L of ascitic
uid was drained during surgery. Postoperatively,
he continued to complain of nausea and abdominal
pain. His telemetry was noted to have ST segment
changes and an electrocardiogram (ECG) was
obtained which showed 23 mm convex ST segment
elevations and small Q waves in the inferolateral
leads with no PR segment depression. He under-
went emergent cardiac catheterization which
showed an occluded distal right coronary artery
(RCA) with thrombus in the posterolateral
branch of the right coronary artery (Figure 2).
This was treated with thrombectomy, balloon
angioplasty and distal nitroprusside infusion. The
remainder of his coronary arteries appeared normal Figure 2 Coronary angiogram showing thrombus in the pos-
terolateral branch of the right coronary artery.
with no angiographic evidence of underlying coron-
ary artery disease. His initial troponin was 10.4 ng/
ml and it peaked at 68.9 ng/ml before down
trending.
Patients with SLE have accelerated atherosclerosis
by virtue of their chronic systemic inammatory
milieu, which worsens inammation and thereby
Discussion progression within atherosclerotic plaques.1,2 Our
patient however, presented with a ST-segment ele-
The most common cause of a STEMI, even in SLE, vation myocardial infarction (STEMI) in the
is rupture of an underlying atherosclerotic plaque. absence of coronary artery disease. The initial
Lupus

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Spontaneous coronary artery thrombosis in the setting of active lupus mesenteric vasculitis
YNV Reddy et al.
3

dierential for his STEMI with active SLE in the for a thrombotic origin of MI, we opted for antith-
absence of coronary disease included (1) Libman rombin therapy in the form of heparin and single
Sacks endocarditis with embolization to the coron- antiplatelet therapy with aspirin. We could also not
ary artery, (2) in-situ arterial thrombosis from APS, denitely exclude the controversial entity of sero-
(3) paradoxical embolus from a patent foramen negative APS given his spontaneous coronary
ovale (PFO) or pulmonary shunt, or (4) coronary arterial thrombosis and deep vein thrombosis,
vasculitis with thrombus formation in resultant pushing us further towards recommending long-
aneurysm.3,4 His bubble study was negative ruling term anticoagulation.7 Our case was further com-
out a PFO or pulmonary shunt. His antiphospho- plicated by his abdominal surgery and mesenteric
lipid antibodies (aPLs) and dilute Russell viper vasculitis, greatly increasing his bleeding risk.
venom test (anti-cardiolipin IgA 3.6 U/mL, IgG Fortunately, he tolerated anticoagulation well and
11.7 U/mL, IgM 4.5 U/mL; anti-beta-2-glycopro- was transitioned to aspirin and coumadin. He was
tein IgA<9 GPI unit, IgG<9 GPI unit, IgM<9 also treated with a beta-blocker and an angiotensin
GPI unit; dilute Russells viper venom time 50.7 s) converting enzyme inhibitor. We elected not to
were all negative ruling out APS. Trans-esophageal start him on statin therapy as he had no coronary
echocardiogram showed no evidence of vegetation artery disease or dyslipidemia. He was eventually
on the mitral or aortic valve, and no left atrial converted from coumadin to rivaroxaban as an
thrombus. After extensive work up it was felt outpatient and is being maintained on long-term
that, by exclusion, the most likely etiology of anticoagulation with excellent functional recovery.
his RCA thrombus was in-situ primary arterial At the time of writing, almost a year post STEMI,
thrombosis related to active lupus. A CT angio- he currently has no angina or dyspnea and can run
gram of his abdomen showed no evidence of vas- 10 miles at a time without diculty.
culitis of his superior or inferior mesenteric arteries.
His course was also complicated by diagnosis of
a deep venous thrombosis in his right leg with
no clinical concern for heparin-induced thrombo- Conclusion
cytopenia. He was treated with aspirin and
heparin bridged to coumadin for his thrombotic In summary, we present a rare case of a thrombotic
STEMI, and he completed a course of pulse- STEMI in the setting of underlying SLE and active
dose methylprednisolone and cyclophosphamide mesenteric vasculitis. Primary arterial thrombosis
for his lupus enteritis with signicant improvement in the setting of active SLE without APS is excep-
in his abdominal pain and bowel function. His tionally rare and we were able to nd only one
post-infarct echocardiogram showed an ejection
further case report of a 12-year-old female with
fraction of 55% with basal and inferolateral
SLE-induced coronary artery thrombosis with no
hypokinesis.
other apparent etiology.8 To the best of our know-
Primary arterial thrombosis is very uncommon
ledge this is the rst report of an adult patient with
in SLE in the absence of APS. Korkmaz et al. have
summarized the available literature on myocardial SLE presenting with both lupus mesenteric vascu-
infarction (MI) in patients with SLE; among litis and in-situ coronary arterial thrombosis in the
16 patients with no coronary artery disease, 93% absence of APS.
of patients had APS and the remainder had coron- Our patients abdominal pain was initially
ary vasculitis with aneurysm formation or emboli related to his mesenteric vasculitis, but some of
from LibmanSacks endocarditis.5 Our patients his refractory nausea and abdominal pain was
presentation is thus highly unusual, and may rep- probably related to the inferior wall myocardial
resent the fact that SLE by itself (without APS) can infarction. Right coronary artery ischemia is often
cause a hypercoagulable state during ares, rarely confused with upper gastrointestinal complaints
predisposing to arterial thrombosis. Heparin- due to the frequent occurrence of nausea and dys-
induced thrombocytopenia with thrombosis must pepsia, and the proximity of the lower chest and
always be considered in the dierential diagnosis upper abdomen. This case illustrates how a single
of patients with arterial thrombosis but our patient unifying diagnosis does not always apply to every
did not have a decline in his platelet count during patient. Persistent and refractory symptoms should
his hospitalization. lead one to question the initial diagnosis and search
There is very little evidence to guide the treat- for potential alternative etiologies of symptoms and
ment of SLE-related acute coronary syndrome in signs. In this case, identication of the alternative
the absence of coronary disease.6 Given the concern etiology of his symptoms was life-saving.
Lupus

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Spontaneous coronary artery thrombosis in the setting of active lupus mesenteric vasculitis
YNV Reddy et al.
4
Funding 2 Manzi S, Meilahn EN, Rairie JE, et al. Age-specific incidence rates
of myocardial infarction and angina in women with systemic lupus
erythematosus: comparison with the Framingham Study.
This research received no specic grant from any Am Epidemiol 1997; 145: 408415.
funding agency in the public, commercial or not- 3 Caracciolo EA, Marcu CB, Ghantous A, et al. Coronary vasculitis
for-prot sectors. with acute myocardial infarction in a young woman with systemic
lupus erythematosus. J Clin Rheumatol 2004; 10: 668.
4 Nijjar PS, Mountis M, Amanullah AM. Acute myocardial infarc-
tion in a patient with systemic lupus erythematosus and normal
coronary arteries. Rev Cardiovasc Med 2007; 8: 3640.
Conflict of interest statement 5 Korkmaz C, Cansu DU, Kasifoglu T. Myocardial infarction in
young patients (35 years of age) with systemic lupus erythemato-
The authors have no conicts of interest to declare. sus: a case report and clinical analysis of the literature. Lupus 2007;
16(4): 28997.
6 Patel PJ, Matthai WH Jr, Untereker WJ. Effective management of
acute coronary thrombosis in a young woman with lupus using
aggressive medical therapy. Cardiology 2011; 118: 459.
References 7 Cervera R, Conti F, Doria A, Iaccarino L, Valesini G. Does sero-
negative antiphospholipid syndrome really exist? Autoimmun Rev
1 Asanuma Y, Oeser A, Shintani AK, et al. Premature coronary- 2012; 11: 5814.
8 Doyle R, Perry JC, Brennan J, Fahey J. Acute myocardial infarction
artery atherosclerosis in systemic lupus erythematosus. N Engl J
as the presenting symptom of systemic lupus erythematosus. Pediatr
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Lupus

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