Discoid Lupus. Chronic discoid lupus appears as a scarring rash often found on the face, ears, and scalp. These patients may also exhibit a photosensitive rash, which consists of an erythematous eruption if acute, or annular scaly lesions if subacute.
Oral and Nasal Ulcerations. Common mucous membrane findings include oral or nasal ulcers, which are typically painless.
Worsening of any of these skin findings may be associated with disease flare. Secondary bacterial infection of lupus rashes or ulcerations is uncommon, although cellulitis should be considered when a rash is unilateral, not in a sun-exposed area, or is otherwise different from the patient’s typical lupus rash. Sun avoidance and topical corticosteroids are the mainstays of treatment of dermatological disease in SLE.
Emergent Complications
Systemic Vasculitis. Patients with SLE are susceptible to vasculitis. Although isolated cutaneous vasculitis is not typically an emergent condition, it may portend systemic vasculitis. Any palpable purpura or other evidence of cutaneous vasculitis should prompt a careful review of systems and basic laboratory workup for systemic vasculitis, which can involve the kidneys, lungs, central or peripheral nervous system, or gastrointestinal tract.
Symptoms of systemic vasculitis may include fevers, chills, chest pain, cough, hemoptysis, abdominal pain, and changes in color or amount of urine. Laboratory workup should be tailored to symptoms, and may include basic metabolic panel, liver function tests, complete blood count, and urinalysis.4
Digital Gangrene. Patients with SLE may also develop digital gangrene related to severe Raynaud phenomenon, vasculitis, or thromboembolism. Pharmacological treatment with vasodilators such as sildenafil, endothelin receptor antagonists, or intravenous prostacyclins may be needed.5 To save the involved digit, vascular surgery services should be consulted urgently.6
Renal Complications
Common Complications
Chronic Kidney Disease. Chronic kidney disease (CKD) is common among SLE patients, especially among those with a history of lupus nephritis.7 Patients with CKD may have persistently elevated serum creatinine, chronic hypertension, and/or chronic peripheral edema. Patients presenting with new development of hypertension, peripheral edema, hematuria, or polyuria should be screened for lupus nephritis with urinalysis and serum creatinine. Elevated creatinine or new or worsening proteinuria or hematuria should prompt consultation with nephrology services.
Emergent Complications
Lupus Nephritis. About 50% of SLE patients will develop lupus nephritis during the course of their lives,1 which may present as nephrotic disease with significant proteinuria, peripheral edema, and low serum albumin, or as nephritic disease, with increased serum creatinine and hematuria. Acute kidney injury in SLE patients should generally prompt admission for workup of reversible causes and evaluation for lupus nephritis, which often includes renal biopsy.8
Neuropsychiatric Complications
Common Complications
Neuropsychiatric lupus is a broad category that includes 19 manifestations of SLE in the central and peripheral nervous systems.9 Conditions range from depression or chronic headaches to seizures or psychosis.
Mood and Anxiety Disorders. Anxiety and depression have been observed in up to 75% of SLE patients.1 Mood and anxiety disorders are likely influenced by the psychosocial elements of this chronic disease, as well as by direct effects of SLE on the brain.1
Peripheral Neuropathy. Approximately 10% of SLE patients have a peripheral neuropathy, which generally presents as a mononeuritis (either single or multiplex), rather than the stocking-glove distribution seen in other systemic causes of neuropathy.10
Headache. Headache disorders may also develop in SLE patients, and tend to have similar patterns to primary headache disorders in the general population. In most cases, treatment for headache in SLE patients is similar to that of the general population.11 However, if a patient presents with concerning findings, such as focal neurological deficit, meningismus, or fever, or if the headache is new-onset or different from previous headaches, further investigation should be considered, including a head computed tomography (CT) scan and lumbar puncture (LP).
Emergent Complications
In general, due to the variety of neurological emergencies that may present with SLE, and the subtlety with which true emergencies may present in this population, the threshold to obtain imaging on SLE patients with any new neurological complaints should be low.
Cerebrovascular Accidents. Patients with SLE are susceptible to cerebrovascular accidents (CVAs), typically from occlusive or embolic causes. Etiologies may include primary central nervous system (CNS) vasculitis, embolic disease from antiphospholipid syndrome (APS), or embolic disease from a Libman-Sacks endocarditis.12
Successful thrombolysis has been reported in SLE patients presenting with stroke, but it remains controversial due to risk of hemorrhagic conversion if CNS vasculitis, rather than embolism, is the cause.13 Proper imaging and consultation with a neurologist familiar with the disease is critical for early treatment decisions.
Seizures. Fifteen percent to 35% of SLE patients may develop seizures. These may be focal or generalized, but generalized tonic-clonic seizures tend to be more common in SLE patients.2 Workup and management of seizures in SLE patients is the same as in the general population.
Sinus Thrombosis. Dural sinus thrombosis often presents as a new-onset headache, sometimes with focal neurological deficits. The diagnosis of dural sinus thrombosis can be challenging, as CT imaging studies may be falsely negative. There should be a low threshold for obtaining MRI/magnetic resonance angiography (MRA) in SLE patients presenting with a new-onset headache.14
CNS Vasculitis. Patients with SLE are also susceptible to CNS vasculitis, which can manifest as seizures, psychosis, cognitive decline, altered mental status, or coma. Magnetic resonance imaging/MRA studies may suggest the diagnosis, but if this is equivocal, angiography or even brain biopsy may be needed to make the diagnosis. Unless the patient’s symptoms are very mild (eg, mild cognitive decline), she or he should be admitted for diagnostic workup and consideration of aggressive immunosuppressive therapy.2
Transverse Myelitis and Spinal Artery Thrombosis. Acute loss of lower limb sensation or motor function in SLE patients may be caused by transverse myelitis or spinal artery thrombosis. Epidural abscess should also be considered, especially if the patient is immunocompromised.2
Infection. A CNS infection should be considered in any SLE patient presenting with new neurological complaints. Fever or meningismus, especially in conjunction with headache or focal neurological deficits, should prompt an LP and consideration for imaging. Immunocompromised patients are at increased risk for common organisms as well as atypical organisms, such as fungus or mycobacteria.15