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Systemic lupus erythematosus (SLE) is a multisystem autoimmune disorder characterized by the production of antibodies to components of the cell nucleus. It occurs predominantly in women of reproductive age. SLE has a diverse array of clinical manifestations and, consequently, is often difficult to diagnose. The American College of Rheumatology has designated 11 diagnostic criteria for SLE, as follows[1]:
To establish the diagnosis of SLE, at least four of these criteria must be present, either serially or simultaneously.
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Laboratory features of SLE include both hematologic abnormalities and immunologic findings indicative of autoimmunity.[2] Hematologic abnormalities that are commonly manifested include the following:
The presence of the following autoimmune antibodies indicates autoimmunity:
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With regard to clinical manifestations of SLE, every system in the body can be affected, including the following[2]:
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A 43-year-old woman presents with a history of a skin rash on her face, scalp, chest, back, arms, and legs (shown). She denies experiencing joint pain, swelling, or stiffness. She has a history of leukopenia, mucosal ulcers, photosensitivity, dry mouth, and numbness in her hands. She was recently admitted to the hospital for a low white blood cell (WBC) count.
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The patient's laboratory results are as follows:
In view of the clinical presentation and the laboratory findings, which of the following is the most likely diagnosis?
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Answer: A. SLE.
Because more than four of the 11 diagnostic criteria are satisfied (positive ANA, mucosal ulcers, photosensitivity, discoid rash, and leukopenia), the diagnosis of SLE is established. This rash has characteristics of discoid lupus.[3] Lesions of discoid lupus are most often present on the face, neck, and scalp but also occur on the ears and, infrequently, on the upper torso. They tend to expand slowly with active inflammation at the periphery and then heal, leaving depressed central scars, atrophy, telangiectasias, and hyperpigmentation or hypopigmentation. Vitiligo is an autoimmune disorder in which the melanocytes are destroyed, causing loss of pigmentation of the skin.[4] Discoid plaques of lupus erythematosus may also appear markedly devoid of pigmentation, just as in vitiligo, but atrophy is usually evident. HIV infection can present with skin rash and leukopenia and thus must be ruled out in all such cases.
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A 30-year-old African American woman with a history of migraines and a family history of rheumatoid arthritis presents to the clinic after a few months of recurring nausea and vomiting with headaches and neck pain. After a recent onset of seizures, magnetic resonance imaging (MRI) of the brain was done and revealed bilateral asymmetric FLAIR hyperintensities in the cerebellum and cerebral hemispheres. Many of the lesions were in the gray-white matter boundary zone, and probable vascular meningeal enhancements were noted. Electroencephalography (EEG) showed results consistent with mild encephalopathy. On physical examination, the patient's temperature is 99°F, her blood pressure (BP) is 150/77 mm Hg, and she has a hyperpigmented itchy rash on her back and the extensor surface of her forearms bilaterally.
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Workup includes a complete blood count (CBC), a comprehensive metabolic panel (CMP), urinalysis, lumbar puncture, immunologic assays, and complement measurement.
In view of the clinical presentation and the laboratory test results displayed in the slide, which of the following is most likely to be the cause of the patient's seizures?
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Answer: C. Lupus cerebritis.
The patient most likely has lupus cerebritis. Her BP is not high enough to cause hypertension-related seizures or headaches. Aseptic meningitis is unlikely because the patient was afebrile, had a normal CBC with differential, and had normal cerebrospinal fluid (CSF) findings. PRES is thought to be related to altered integrity of the blood-brain barrier in the setting of hypertension, eclampsia, renal failure, or immunosuppression.[5] The resultant vasogenic edema is usually bilateral and symmetrical and can occur in any lobe. MRI findings resolve with control of BP. Further investigation of this patient by means of kidney biopsy reveals focal proliferative glomerulonephritis consistent with lupus. She is treated with cyclophosphamide.
Images courtesy of Radiopaedia.org (left) and Medscape (right).
A 45-year-old woman presents with a skin rash on the face, chest, and arms. She complains of dry eyes and dry mouth, and she reports feeling tired and experiencing generalized aches and pain in muscles and joints. She has lost 19 lb in 2 months. On physical examination, the patient is found to have tiny acneiform rashes on her lower cheeks, back, and chest, for which she has been taking minocycline. A few months before this visit, she developed two enlarged lymph nodes in her neck.
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Answer: B. SLE.
Four of the 11 specified criteria are fulfilled; thus, this patient has SLE. The skin rash is consistent with malar rash in that it is not crossing the nasolabial folds. Features of minocycline-induced lupus include arthralgia, arthritis, fever, and rash; other clinical features that have been described are morning stiffness, myalgia, pneumonitis, and cutaneous vasculitis. However, the autoantibody pattern differs from those of most other forms of drug-induced lupus. Positive results for ANA (including anti-dsDNA), perinuclear antineutrophil cytoplasmic antibody (P-ANCA), and antibodies to histone are uncommon. Most cases of minocycline-induced lupus resolve within a relatively short time after discontinuance of the drug.[2,6] The distribution of the rash is important in helping to distinguish the rash of SLE from other facial rashes. Rosacea can cross the nasolabial folds; it can occur on the forehead, chin, and cheeks; and it is associated with central facial flushing. Dermatomyositis rash typically occurs on the eyelids as heliotrope rash; on the metacarpophalangeal and interphalangeal joints as Gottron papules; and on the back and chest as the shawl sign or the V sign.
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A 38-year-old white woman with a history of lupus since 2005 and a past history of lupus nephritis and hypertension was admitted a few days previously for worsening shortness of breath, orthopnea, paroxysmal nocturnal dyspnea, and lower-extremity edema. She has a history of a congenital solitary kidney. The patient denies experiencing fever, cough, chest pain, abdominal pain, or dysuria. She complains of urticarial skin rash on her arms and legs, pain in her joints (especially in the right knee), and generalized aches in her legs. She is taking oral prednisone at a dosage of 40 mg/day.
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Electrocardiography (ECG) shows a normal sinus rhythm with left ventricular hypertrophy, inferolateral ST-T changes, and a prolonged QT interval. Echocardiography reveals severe global hypokinesis, an ejection fraction of 20-25%, left ventricular systolic dysfunction, grade 2 moderate diastolic dysfunction, and normal right ventricle size and filling pressures. No pericardial effusion or valvular vegetations are apparent. The patient undergoes left-heart catheterization and is found to have mild coronary artery disease.
In view of the clinical presentation, the laboratory test results, and the findings from diagnostic imaging and ECG, which of the following could be a cause of the patient's nonischemic dilated cardiomyopathy?
Images courtesy of Wikimedia Commons (top) and Medscape (lower right, lower middle), Blausen.com staff. "Blausen gallery 2014". Wikiversity Journal of Medicine. DOI:10.15347/wjm/2014.010. ISSN 20018762 (lower left).
Answer: A. Lupus myocarditis.
The signs and symptoms are similar to those of myocarditis, and they can progress to ventricular dysfunction, dilated cardiomyopathy, and heart failure.[7] There are no typical findings on ECG, and cardiac enzymes may be normal. Myocarditis, even when mild, must be treated immediately with high-dose steroids; in the most severe cases, it is necessary to administer intravenous (IV) pulse corticosteroids, followed by high oral doses. The addition of an immunosuppressant (eg, azathioprine, cyclophosphamide, or IV immunoglobulin [IVIG]) may be helpful in the treatment of myocarditis. Anabolic androgenic steroids are a more common cause of dilated cardiomyopathy than prednisone is. Prednisone can lead to heart failure by causing arrhythmias and fluid retention.
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