FEATURE Complications of Systemic Lupus Erythematosus in the Emergency Department Joanna L. Marco, MD; Christine L. Chhakchhuak, MD Understanding and differentiating common from emergent complications of systemic lupus erythematous assists the clinician in recognizing and making appropriate treatment decisions in this complex patient population. ystemic lupus erythematosus (SLE) is a chronic autoimmune disease characterized by the chronic ac- S tivation of the immune system, leading to the formation of autoantibod- ies and multi-organ damage. The preva- lence of SLE in the United States is 20 to 150 per 100,000 persons.1 Ninety percent of patients with SLE are women, and the condition is more common and often more severe among patients of black African or of Asian descent. The most common symptoms of SLE are constitutional (fever or weight loss), mus- culoskeletal (arthralgias or myalgias), and dermatological (malar rash, photosensitive rash, discoid lupus, or oral ulcers). Howev- er, since SLE is known to affect nearly every organ system, clinical manifestations may differ markedly among patients (Table). For patients with known SLE who pres- ent to the ED, it can be a challenge to iden- tify whether their symptoms are due to a Dr Marco is a resident, department of internal medicine, University of Cincinnati, Ohio. Dr Chhakchhuak is an assistant professor of medicine, department of internal medicine, division of immunology, allergy, and rheumatology, University of Cincinnati, Ohio. Authors’ Disclosure Statement: The authors report no actual or potential conflict of interest in relation to this article. DOI: 10.12788/emed.2018.0075 6 EMERGENCY MEDICINE I JANUARY 2018 www.emed-journal.com Table. Systemic Lupus Erythematous-Associated Conditions by Chief Complainta Chief SLE-Associated Conditions Complaint (Emergent conditions bolded) Suggested Workup Clinical Pearls Fever Active SLE Chest X-ray Double-stranded DNA and C3 and C4 complements Pericarditis Urinalysis can suggest general SLE Infection (consider opportunistic infections Consider blood cultures flare, but results may not if immunocompromised) be available during ED visit. Other infectious workup based Arterial or venous thromboembolism on symptoms Acute lupus pneumonitis Imaging if concern for thromboembolic disease Diffuse alveolar hemorrhage Joint pain Active SLE Plain radiography A single inflamed, painful joint is less likely to be Avascular necrosis (especially when hips or Non-urgent MRI if avascular caused by active SLE knees are involved) necrosis is suspected and should prompt Septic arthritis Arthrocentesis if septic arthritis consideration of septic is suspected arthritis. Cover for Salmonella and typical gram-positive organisms with ceftriaxone and vancomycin. Rash Malar rash Evaluate for systemic vasculitis if cutaneous vasculitis is Photosensitive rash present Discoid lupus Cutaneous vasculitis Headache Primary headache disorder Consider imaging or LP if CT is not sensitive for headache is new or different dural sinus thrombosis. CNS infection from previous headaches, or Choose MRI when this is Dural sinus thrombosis if there are other concerning suspected. signs or symptoms CNS vasculitis Altered Lupus psychosis Chest X-ray, urinalysis, and mental blood cultures status Stroke Consider LP Sepsis Consider head CT or MRI CNS infection CNS vasculitis Continued on page 8 www.emed-journal.com JANUARY 2018 I EMERGENCY MEDICINE 7 SYSTEMIC LUPUS ERYTHEMATOSUS IN THE ED Table. Systemic Lupus Erythematous-Associated Conditions by Chief Complainta (continued) Chief SLE-Associated Conditions Complaint (Emergent conditions bolded) Suggested Workup Clinical Pearls Seizure Primary seizure disorder CT or MRI if no known history of seizure disorder Stroke LP if meningitis is suspected CNS infection CNS vasculitis Focal SLE mononeuritis CT or MRI if symptoms are neurological acute deficit Stroke LP if meningitis is suspected CNS infection (including epidural abscess) CNS vasculitis Dural sinus thrombosis Transverse myelitis Dyspnea or Pleuritis Chest X-ray cough Interstitial lung disease Echocardiogram Pulmonary hypertension Complete blood count Shrinking lung syndrome Consider chest CT Pulmonary embolism Consider cardiac biomarkers Pulmonary infection, including opportunistic infection Acute lupus pneumonitis Diffuse alveolar hemorrhage Libman-Sacks endocarditis with valvular dysfunction Acute coronary syndrome Autoimmune hemolytic anemia Continued on page 9 minor lupus flare that can be managed as an General Acute-Care Management outpatient, a presentation of urgent or emer- While a patient’s presentation could be gent conditions caused by SLE, or a condi- secondary to a lupus-related complica- tion unrelated to lupus. This article reviews tion, consideration must always be given the most common and emergent complica- to common conditions that are not related tions of SLE by organ system to assist emer- to SLE. Biomarkers such as erythrocyte gency physicians (EPs) in better diagnosing sedimentation rate, C-reactive protein, C3 and managing this complicated disease. and C4 complement, and double-stranded 8 EMERGENCY MEDICINE I JANUARY 2018 www.emed-journal.com Table. Systemic Lupus Erythematous-Associated Conditions by Chief Complainta (continued) Chief SLE-Associated Conditions Complaint (Emergent conditions bolded) Suggested Workup Clinical Pearls Chest pain Pleuritis Chest X-ray Patients with SLE have a 10-fold higher Pericarditis Echocardiogram risk of coronary artery Interstitial lung disease Cardiac biomarkers disease than the general population, so acute Pulmonary hypertension Consider chest CT coronary syndrome should be considered even in Shrinking lung syndrome Consider cardiac stress young patients without testing other risk factors. Pulmonary embolism Pulmonary infection, including opportunistic infection Acute lupus pneumonitis Diffuse alveolar hemorrhage Libman-Sacks endocarditis with valvular dysfunction Acute coronary syndrome Abdominal Pancreatitis Liver function tests pain Peptic ulcer disease Lipase Mesenteric vasculitis Lactate Abdominal CT if severe pain or no other etiology found aIn addition to SLE-associated conditions presented here, conditions that are common in the general population should always be considered. Abbreviations: CNS, central nervous system; CT, computed tomography; LP, lumbar puncture; MRI, magnetic resonance imaging; SLE, systemic lupus erythematous. DNA levels can be helpful in assessing Musculoskeletal Complications lupus disease activity and differentiating Common Complications a lupus-related complication from an un- Polyarthralgias and Polymyalgias. More than related event. Comparing these biomark- 90% of SLE patients experience polyar- ers to the patient’s baseline values can be thralgias and polymyalgias. Physical ex- informative; however, depending on the amination findings may be normal, even laboratory facilities, test results may not be when joint pain is present, which is often available during an ED visit. Lastly, infec- due to mild synovitis. In some cases, Jac- tions should be considered more strongly coud arthropathy is seen, which presents than usual in the differential diagnosis as deformities such as swan neck defor- due to the immunocompromised status of mities and ulnar deviations that are char- a substantial proportion of these patients, acteristically reducible on manipulation by virtue of their disease or the cytotoxic (Figures 1a and 1b). These deformities are medications used for treatment. not caused by direct joint damage, but by www.emed-journal.com JANUARY 2018 I EMERGENCY MEDICINE 9 SYSTEMIC LUPUS ERYTHEMATOSUS IN THE ED A B Figure 1. (A) Photo demonstrating swan neck deformities of the second, third, and fifth digits of the right hand. B( ) Normal bilateral hand X-ray suggesting Jaccoud arthropathy. Reproduced with permission from Küçükşahin, et al.28 joint disease. Magnetic resonance imag- ing (MRI) is more sensitive in diagnosing avascular necrosis, and may be indicated when clinical suspicion is high despite negative plain radiographs, although this would not typically need to be performed urgently in the ED.2 While analgesics and physical therapy may provide some pain relief to patients with avascular necrosis, Figure 2. Photo demonstrating a malar rash with sparing of the this condition generally requires nonemer- nasolabial fold in a patient with systemic lupus erythematous. gent operative intervention. Reproduced with permission from Uva et al.29 Emergent Complications chronic tenosynovitis and the resulting Septic Arthritis. When a patient with SLE pres- laxity of tendons and ligaments.1 Classi- ents with an isolated swollen joint, septic cally, plain radiographic imaging reveals arthritis should be suspected, and diagno- nonerosive joint changes. Muscle and joint sis should be confirmed by arthrocentesis. pains may worsen with disease progres- Synovial fluid samples showing a white sion or flare. blood cell count greater than 50 × 109/L Avascular Necrosis. Avascular necrosis suggest infection, which can be confirmed affects 5% to 12% of SLE patients.2 Most by gram stain and cultures. commonly, this involves the femoral head, For reasons that remain unclear, but may but it may also involve the femoral con- involve primary immune defects and the dyle or tibial plateau. Patients may present use of immunosuppressant medications, with acute or subacute onset of pain in the patients with SLE are predisposed to Sal- groin or buttocks when the femoral head is monella joint infections. In