A 41-year-old black woman presented to an emergency department in the UK with new-onset chest pain, fevers, arthritis, and facial rash. Echocardiographic evaluation revealed a large (2 cm) pericardial effusion, urinalysis revealed 4+ proteinuria, and serology testing revealed a strongly positive antinuclear antibody. A work-up for cardiac ischemia was negative. Systemic lupus erythematosus (SLE) was diagnosed, and the patient was treated with prednisolone 60 mg daily. Over the following weeks, the patient's symptoms improved and her proteinuria gradually resolved. She was treated with azathioprine 125 mg daily and her prednisolone dose was slowly tapered.
The patient relocated to the US 2 months after her initial presentation, and initiated care at a rheumatology clinic. Her symptoms of SLE were adequately controlled at this time. Her medical history was notable for migraines, mild mitral regurgitation, and large uterine fibroids, which caused menorrhagia and iron-deficiency anemia. She was premenopausal with two living children, and she had experienced one spontaneous pregnancy loss before 10 weeks' gestation. She had no history of alcohol abuse, tobacco use, illicit drug use, or drug allergies. Her family history was remarkable for the death of her maternal grandmother from myocardial infarction at the age of 56 years. Her medications at presentation in the US included azathioprine 125 mg daily, prednisolone 30 mg daily, ranitidine 150 mg daily, iron and vitamin B complex.
The patient appeared well on physical examination, and she had a blood pressure of 132/82 mmHg and pulse rate of 68 beats/min. The remainder of her examination was unremarkable. Routine serologic evaluation revealed moderate-titer antinuclear antibodies with a coarse-speckled pattern on immunofluorescence, high-titer antibodies directed against Ro/SSA autoantigens and Smith nuclear antigens, negative tests for double-stranded DNA and antiphospholipid antibodies, and normal complement C3 and C4 levels. Her total cholesterol level was elevated ( Table 1 ). Additional laboratory evaluation data are provided in Table 1 (initial clinic visit).
Over the ensuing months, the patient's disease was relatively quiescent, and azathioprine and prednisone (converted from prednisolone in the UK) were gradually tapered and discontinued. Hydroxychloroquine 200 mg twice daily was initiated. In the setting of erratic medication compliance, the patient developed occasional arthralgias and facial rashes, which typically resolved upon resumption of therapy with hydroxychloroquine and occasional low-dose prednisone.
One year after presentation in the US, the patient entered into an observational study to evaluate risk factors associated with the development of subclinical atherosclerosis in patients with SLE. At this time the patient was asymptomatic: her blood pressure was 100/70 mmHg, her pulse rate was 74 beats/min, and her weight was 82.7 kg, with a BMI of 27.9 kg/m2 (normal BMI 18.524.9 kg/m2). Laboratory evaluations and disease scores are provided in Table 1 (first study visit). As part of the study, the patient underwent echocardiography, which revealed normal ventricular size and function, mild mitral and tricuspid regurgitation, and a thickened pericardium. The patient also underwent carotid ultrasonography, which did not reveal any discrete atherosclerotic plaque.
Over the next 4 years, the patient experienced intermittent flares of SLE characterized by arthritis, new auricular discoid lesions and leukopenia. During this period, treatment consisted of hydroxychloroquine 200 mg twice daily, aspirin 81 mg daily, occasional use of NSAIDs including celecoxib, meloxicam and ibuprofen at various dosages, and prednisone 520 mg daily. Serial laboratory evaluations over this time revealed elevated levels of double-stranded DNA antibodies, decreasing C3 and C4 levels, and an increasing erythrocyte sedimentation rate. Approximately 3 years after enrollment into the study, the patient was hospitalized with progressive shortness of breath. She was found to have a large right-sided pleural effusion and bilateral axillary lymphadenopathy on chest CT. Pleural fluid analysis revealed lymphocytosis, with negative Gram stain, cultures, and cytology. The patient required a chest tube placement and talc pleurodesis for the management of recurrent effusions. An axillary lymph-node biopsy revealed reactive changes and no evidence of malignancy. Her prednisone dose was increased to 20 mg twice daily. The patient's symptoms gradually improved and she was discharged following a 2-week hospitalization period. She was then lost to follow-up for several months, during which time she self-tapered prednisone to 5 mg daily.
In this setting, the patient returned for follow-up in the SLE atherosclerosis study, 4 years after her initial study visit. Her blood pressure was 90/60 mmHg, her pulse rate was 87 beats/min, and her weight had decreased to 72.6 kg, with a BMI of 24.5 kg/m2. Physical examination revealed pallor, painful hard palate ulcerations, alopecia, active discoid lesions of both ears, and painful synovitis of her wrists, knees, and ankles. Laboratory evaluations and disease scores at this time are provided in Table 1 (second study visit). A repeat echocardiogram demonstrated new eccentric left ventricular hypertrophy, and carotid ultrasonography revealed new non-obstructive plaque in the right carotid bulb (Figure 1).
Ultrasound image of the patient's carotid artery, which shows atherosclerotic plaque in the carotid bulb (indicated by arrow).
The patient experienced persistent, active SLE over the following months, characterized by arthralgias, worsening discoid skin lesions and alopecia. A trial of azathioprine 50 mg daily resulted in nausea and vomiting, and mycophenolate mofetil 500 mg twice daily resulted in pruritis. Oral methotrexate 12.5 mg weekly was subsequently initiated. The patient was again lost to follow-up for several months, and later returned with persistent SLE symptoms and new anasarca. A 24-hour urine collection demonstrated 2.4 g of protein; other laboratory values are provided in Table 1 (final clinic visit). The patient refused a renal biopsy, and was again prescribed mycophenolate mofetil 500 mg twice daily with prednisone 20 mg daily. She was ultimately lost to clinical follow-up despite repeated contact attempts.
Nat Clin Pract Rheumatol. 2007;3(8):473-478. © 2007 Nature Publishing Group
Written consent was obtained from this patient to participate in the Hospital for Special Surgery Autoimmune Disease Registry and Accelerated Atherosclerosis in SLE study. Both these protocols were approved by the Hospital for Special Surgery Institutional Review Board.
Cite this: Subclinical Carotid Atherosclerosis in a Patient with Systemic Lupus Erythematosus - Medscape - May 18, 2007.