Uncommon Coronary Abnormalities in a Patient with Discoid Lupus Erythematosus

Abstract

A 43-year-old male presented with shortness of breath and palpitations. Physical examination was significant for skin lichenification, an erythematous maculopapular rash with annular plaques, a fissured tongue, and digital clubbing. Electrocardiogram captured a supraventricular tachycardia and right bundle branch block. Left heart catheterization found several proximal left coronary artery branch aneurysms, a right coronary–pulmonary artery fistula, venous–luminal vessels between the right heart chambers and cardiac veins, and thebesian venous lakes. Functional cardiac capacity was reduced, with left ventricular ejection fraction of 35 to 40%, on ventriculogram. A diagnosis of discoid lupus erythematosus was established after skin biopsies revealed systemic lupus erythematosus-like features but negative anti-nuclear, anti-Smith, anti-ds-deoxyribonucleic acid, anti-Ro, and anti-La antibodies. The case reported is unique, in that while our patient had pathologic electrophysiologic changes typically seen with autoimmune disease, the patient had many anatomical cardiac anomalies without atherosclerotic coronary disease. It is uncertain whether these findings are purely incidental or are related to an underlying genetic disorder.

The patient is a 43-year-old male who presented with palpitations, preceded by 4 days of worsening dyspnea on exertion, and cough productive of yellow sputum. Past medical history includes recurrent staphylococcal cellulitis. Family history included systemic lupus erythematosus (SLE) in his brother and mother. On physical examination, the patient was found to have coarse skin consistent with lichenification, an erythematous maculopapular rash with annular plaques along the neck and shoulders, digital clubbing, and a thickened fissured tongue ( Fig. 1A , B ).

Fig. 1.

( A ) Maculopapular rash on neck, shoulders, and upper thorax consistent with discoid lupus erythematosus. ( B ) Fissured tongue.

Chest radiograph revealed a small right inferomedial consolidation. Follow-up computerized tomography of the chest without contrast demonstrated moderate diffuse emphysema. Electrocardiogram captured a supraventricular tachycardia (SVT) (heart rate of 222 beats/minute) with right bundle branch block (RBBB). The underlying RBBB can be seen in Fig. 2 after rate control was achieved.

Fig. 2.

Electrocardiogram depicting normal sinus rhythm, right bundle branch block (QRS interval–155 milliseconds), first-degree atrioventricular block (PR interval—220 milliseconds), left ventricular hypertrophy with secondary repolarization abnormality.

The patient's condition improved without any pharmacologic intervention after administration of intravenous normal saline (1L bolus). The patient's shortness of breath and palpitations resolved. Cardiology was consulted and recommended pharmacologic (dobutamine) cardiac stress testing, which revealed areas of reversible ischemia in the basal and anterolateral myocardial segments. Follow-up of left heart catheterization and angiocardiogram revealed several proximal left coronary artery branch aneurysms ( Fig. 3A ), a right coronary–pulmonary artery fistula, venous–luminal vessels between the right heart chambers and cardiac veins, and thebesian venous lakes ( Fig. 3B ). Pulmonary artery pressure was 28/16 mm Hg. Left ventricular ejection fraction was 35 to 40% on ventriculogram. No significant atherosclerotic disease was found. The patient was started on carvedilol 12.5 mg twice daily for rate control.

Fig. 3.

( A ) Left coronary angiogram with multiple aneurysms of left coronary artery branches (arrows). ( B ) Right coronary angiogram with a coronary–pulmonary artery fistula (solid black arrow), venous–luminal vessels between right heart chambers and cardiac veins (arrowheads), and thebesian venous lakes (open arrows).

Histopathology on skin biopsy reported SLE-like features including hyperkeratosis, follicular damage, and leukocytic infiltration. Immunofluorescence was not performed. Serologic studies were negative for anti-nuclear, anti-Smith, anti-ds-deoxyribonucleic acid, anti-Ro, and anti-La antibodies consistent with the diagnosis of discoid lupus erythematosus (DLE).

The patient was started on topical corticosteroids and discharged. On 6 months follow-up, the patient remained asymptomatic without recurrence of palpitations or maculopapular rash.

Discussion

Cardiac involvement is a major concern for patients suffering from autoimmune disease, traditionally atherosclerotic coronary artery disease. ¹ Pathologic electrophysiologic changes, as exhibited in the case presented, have also been observed and are well documented in patients with SLE. ^{1 2 3} The cardiac changes associated with autoimmune diseases are typically those brought on by inflammation and fibrosis throughout the myocardial layers. ² The case reported is interesting in that, while our patient had pathologic electrophysiologic changes (RBBB and SVT), no atherosclerotic coronary artery disease was identified. The patient also had several cardiac anatomical abnormalities, which are not typically associated with DLE. Aneurysms were noted throughout the left coronary artery branches, a right coronary artery to pulmonary artery fistula was found, as well as several venous–luminal vessels between the right heart chambers and cardiac veins were found. Several thebesian venous lakes were also present. It is uncertain whether these findings are purely incidental or are related to an underlying genetic disorder. Previous reports detail a correlation between autoimmune disease, specifically lupus erythematosus, and coronary artery aneurysms, ^{4 5} although the extent of anatomical anomalies in our patient does not seem to be common. Reports of myocardial infarction from coronary steal after coronary artery aneurysms and rupture have been documented, ^{6 7} and have been considered as possible etiology for the ischemic changes noted on our patient's cardiac stress testing.