FEATURE

Complications of Systemic 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 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, , 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 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 one study, 59% involved, or in the knee when the femoral of septic arthritis cases in patients with condyle or tibial plateau is involved. Plain SLE were due to Salmonella species; there- radiographs may reveal joint-space nar- fore, treatment for septic arthritis in this rowing and other evidence of degenerative population should include ceftriaxone in

10 EMERGENCY MEDICINE I JANUARY 2018 www.emed-journal.com addition to vancomycin for typical organ- workup should be tailored to symptoms, ...due to the isms, such as Staphylococcus and Strepto- and may include basic metabolic panel, coccus species.3 liver function tests, complete blood count, variety of and urinalysis.4 Cutaneous Manifestations Digital Gangrene. Patients with SLE may neurological Common Complications also develop digital gangrene related to se- Malar Rash. Eighty percent to 90% of pa- vere Raynaud phenomenon, vasculitis, or emergencies... tients with SLE have dermatological in- thromboembolism. Pharmacological treat- volvement,1 the most common finding of ment with vasodilators such as sildenafil, the threshold which is the malar or butterfly facial rash, endothelin receptor antagonists, or intra- which appears as raised erythema over the venous prostacyclins may be needed.5 To to obtain bridge of the nose and cheeks while spar- save the involved digit, vascular surgery ing the nasolabial folds (Figure 2). services should be consulted urgently.6 imaging on Discoid Lupus. Chronic discoid lupus ap- pears as a scarring rash often found on the Renal Complications SLE patients face, ears, and scalp. These patients may Common Complications also exhibit a photosensitive rash, which Chronic Kidney Disease. Chronic kidney dis- with any new consists of an erythematous eruption if ease (CKD) is common among SLE patients, acute, or annular scaly lesions if subacute. especially among those with a history of neurological Oral and Nasal Ulcerations. Common mu- lupus nephritis.7 Patients with CKD may cous membrane findings include oral or have persistently elevated serum creati- complaints nasal ulcers, which are typically painless. nine, chronic hypertension, and/or chron- Worsening of any of these skin findings ic peripheral edema. Patients presenting should be low. may be associated with disease flare. Sec- with new development of hypertension, ondary bacterial infection of lupus rashes peripheral edema, hematuria, or polyuria or ulcerations is uncommon, although cel- should be screened for lupus nephritis lulitis should be considered when a rash is with urinalysis and serum creatinine. El- unilateral, not in a sun-exposed area, or is evated creatinine or new or worsening pro- otherwise different from the patient’s typi- teinuria or hematuria should prompt con- cal lupus rash. Sun avoidance and topical sultation with nephrology services. corticosteroids are the mainstays of treat- ment of dermatological disease in SLE. Emergent Complications Lupus Nephritis. About 50% of SLE patients Emergent Complications will develop lupus nephritis during the . Patients with SLE are course of their lives,1 which may present susceptible to vasculitis. Although iso- as nephrotic disease with significant pro- lated cutaneous vasculitis is not typically teinuria, peripheral edema, and low serum an emergent condition, it may portend albumin, or as nephritic disease, with in- systemic vasculitis. Any palpable creased serum creatinine and hematuria. or other evidence of cutaneous vasculitis Acute kidney injury in SLE patients should should prompt a careful review of systems generally prompt admission for workup of and basic laboratory workup for systemic reversible causes and evaluation for lupus vasculitis, which can involve the kidneys, nephritis, which often includes renal bi- lungs, central or peripheral nervous sys- opsy.8 tem, or gastrointestinal tract. Symptoms of systemic vasculitis may Neuropsychiatric Complications include fevers, chills, chest pain, cough, Common Complications hemoptysis, abdominal pain, and changes Neuropsychiatric lupus is a broad category in color or amount of urine. Laboratory that includes 19 manifestations of SLE in

www.emed-journal.com JANUARY 2018 I EMERGENCY MEDICINE 11 SYSTEMIC LUPUS ERYTHEMATOSUS IN THE ED

Approximately the central and peripheral nervous sys- Successful thrombolysis has been report- tems.9 Conditions range from depression ed in SLE patients presenting with stroke, half of SLE or chronic headaches to seizures or psy- but it remains controversial due to risk of chosis. hemorrhagic conversion if CNS vasculitis, patients who Mood and Anxiety Disorders. Anxiety and rather than embolism, is the cause.13 Prop- depression have been observed in up to er imaging and consultation with a neurol- present to the 75% of SLE patients.1 Mood and anxiety ogist familiar with the disease is critical for disorders are likely influenced by the psy- early treatment decisions. ED with acute chosocial elements of this chronic disease, Seizures. Fifteen percent to 35% of SLE as well as by direct effects of SLE on the patients may develop seizures. These may abdominal brain.1 be focal or generalized, but generalized Peripheral Neuropathy. Approximately tonic-clonic seizures tend to be more com- pain are found 10% of SLE patients have a peripheral mon in SLE patients.2 Workup and man- neuropathy, which generally presents as agement of seizures in SLE patients is the to have either a mononeuritis (either single or multi- same as in the general population. plex), rather than the stocking-glove dis- Sinus Thrombosis. Dural sinus thrombosis mesenteric tribution seen in other systemic causes of often presents as a new-onset headache, neuropathy.10 sometimes with focal neurological deficits. vasculitis or Headache. Headache disorders may also The diagnosis of dural sinus thrombosis can develop in SLE patients, and tend to have be challenging, as CT imaging studies may pancreatitis... similar patterns to primary headache dis- be falsely negative. There should be a low orders in the general population. In most threshold for obtaining MRI/magnetic reso- cases, treatment for headache in SLE pa- nance angiography (MRA) in SLE patients tients is similar to that of the general popu- presenting with a new-onset headache.14 lation.11 However, if a patient presents with CNS Vasculitis. Patients with SLE are also concerning findings, such as focal neuro- susceptible to CNS vasculitis, which can logical deficit, meningismus, or fever, or manifest as seizures, psychosis, cognitive if the headache is new-onset or different decline, altered mental status, or coma. from previous headaches, further investi- Magnetic resonance imaging/MRA stud- gation should be considered, including a ies may suggest the diagnosis, but if this head computed tomography (CT) scan and is equivocal, angiography or even brain lumbar puncture (LP). biopsy may be needed to make the diagno- sis. Unless the patient’s symptoms are very Emergent Complications mild (eg, mild cognitive decline), she or he In general, due to the variety of neuro- should be admitted for diagnostic workup logical emergencies that may present with and consideration of aggressive immuno- SLE, and the subtlety with which true suppressive therapy.2 emergencies may present in this popula- Transverse Myelitis and Spinal Artery Throm- tion, the threshold to obtain imaging on bosis. Acute loss of lower limb sensation SLE patients with any new neurological or motor function in SLE patients may be complaints should be low. caused by transverse myelitis or spinal ar- Cerebrovascular Accidents. Patients with tery thrombosis. Epidural abscess should SLE are susceptible to cerebrovascular ac- also be considered, especially if the patient cidents (CVAs), typically from occlusive is immunocompromised.2 or embolic causes. Etiologies may include Infection. A CNS infection should be primary central nervous system (CNS) vas- considered in any SLE patient presenting culitis, embolic disease from antiphospho- with new neurological complaints. Fever lipid syndrome (APS), or embolic disease or meningismus, especially in conjunc- from a Libman-Sacks endocarditis.12 tion with headache or focal neurological

12 EMERGENCY MEDICINE I JANUARY 2018 www.emed-journal.com deficits, should prompt an LP and consid- eration for imaging. Immunocompromised patients are at increased risk for common organisms as well as atypical organisms, such as fungus or mycobacteria.15

Pulmonary Complications Common Complications Pleuritis. Many patients with SLE develop pleuritis, with or without effusion. This may be treated with nonsteroidal anti- inflammatory drugs, or corticosteroids if symptoms are more severe. Pleuritis is the most common respiratory complication of SLE, but due to the number of serious car- diopulmonary complications associated with SLE, pleuritis should be a diagnosis of exclusion. Interstitial Lung Disease. Interstitial lung disease may be caused by SLE or may be medication-induced. This commonly pres- ents as subacute or chronic dyspnea and/ Figure 3. Computed tomography image of the chest revealing anterior ground-glass or cough. Patient workup may be done on opacities in a patient with acute lupus pneumonitis. an outpatient basis with high resolution Reproduced with permission from Nishant Gupta, MD. chest CT and pulmonary function testing. Pulmonary Hypertension. Patients with SLE may develop pulmonary hypertension, ei- ate clinical picture. Patients with APS are ther directly due to SLE or from chronic at particularly high risk for thromboembolic thromboembolic disease. In general, pul- disease. However, even SLE patients with- monary hypertension is managed as an out this APS are known to be at an increased outpatient, but may require emergent inpa- risk of developing thromboembolism com- tient treatment if the condition is rapidly pared to the general public.17 Pulmonary progressive or associated with right heart embolism in SLE patients should be diag- failure. nosed and treated in the usual manner. Shrinking Lung Syndrome. This condition Pneumonia. Immunosuppressed patients may cause subacute or chronic dyspnea and are susceptible to opportunistic pulmo- pleuritic chest pain. Shrinking lung syn- nary infections as well as typical commu- drome is caused by diaphragmatic dysfunc- nity pathogens. Fungal or mycobacterial tion rather than from a primary disease of infections may be suspected with a more the lungs, and it is characterized by a restric- subacute onset of symptoms. tive pattern on pulmonary function testing Acute Lupus Pneumonitis. This serious and an elevated hemidiaphragm. Shrinking condition may present with severe pneu- lung syndrome typically responds well to monia-like signs and symptoms, including immunosuppressive therapy.16 fever, cough, dyspnea, hypoxia, and infil- trates on chest radiograph (Figure 3). Emergent Conditions Acute lupus pneumonitis is caused by Pulmonary Embolism. A pulmonary embolism disease flare, and not by infection, although should be strongly considered in any pa- it may not be possible to distinguish it from tient with SLE presenting with the appropri pneumonia in the ED setting. The mortality

www.emed-journal.com JANUARY 2018 I EMERGENCY MEDICINE 13 SYSTEMIC LUPUS ERYTHEMATOSUS IN THE ED

rate of acute lupus pneumonitis is as high However, among patients with symptomat- as 50%, and survivors often progress to ic pericardial effusions, tamponade can be chronic interstitial pneumonitis.1 present in 21%.19 Corticosteroid therapy is Diffuse Alveolar Hemorrhage. A rare com- often required to treat SLE-associated peri- plication with a mortality rate of 50% to carditis, but colchicine is being explored 90%, SLE patients who develop diffuse as a possible steroid-sparing agent in this alveolar hemorrhage may present with fe- patient population.20,21 ver, cough, dyspnea, and hypoxia.18 The Valvular Abnormalities. Approximately 60% condition may be suggested by infiltrates of SLE patients have valvular abnormali- on chest radiograph, a drop in hemoglo- ties detectable by echocardiography. The bin representing bleeding into the lungs, most common abnormalities in one study and/or hemoptysis. However, the absence were valvular thickening or regurgita- of hemoptysis does not rule out diffuse al- tion.22 Many of these abnormalities oc- veolar hemorrhage, so clinical suspicion curred in asymptomatic patients and never should remain high, even in the absence of progressed to clinical disease in a 5-year this symptom. follow-up. However, patients with any Because emergent pulmonary conditions valvular abnormality were more likely to often present with similar symptoms, most develop complications, including stroke, patients with acute or new-onset symp- peripheral embolism, infective endocardi- toms will require admission for diagnostic tis, need for valve replacement, congestive workup (likely to include chest CT scan heart failure, or death.22 and/or bronchoscopy with bronchoalveo- lar lavage), as well as for close monitoring Emergent Complications and initiation of treatment. If hypoxia or Acute Coronary Syndrome. Even in relatively respiratory distress is severe, or if diffuse young patients, acute coronary syndrome alveolar hemorrhage is suspected, admis- (ACS) should be considered in SLE pa- sion to the intensive care unit (ICU) should tients presenting with chest pain, as this be considered. We suggest that antibiotics patient population has a 10-fold higher be started in the ED when pneumonia is risk of developing coronary artery disease part of the differential diagnosis. As in (CAD) than the general population, and the general population, coverage should SLE patients with CAD often lack tradi- be chosen based on the patient’s risk fac- tional risk factors, such as advanced age, tors for antibiotic-resistant organisms. Ini- family history, or metabolic syndrome.1 tiation of corticosteroid therapy or other A high clinical suspicion should be changes in immune therapy can be de- maintained even in patients who would layed until the EP consults with rheuma- traditionally be considered low-risk. The tology and/or pulmonology services. EP should have a low-threshold for ECG, cardiac biomarker testing, and stress test- Cardiac Complications ing for SLE patients presenting with chest Common Complications pain. The treatment of ACS in SLE patients Pericarditis. Pericarditis with or without is the same as in the general population. pericardial effusion is very common in Libman-Sacks Endocarditis. A sterile, fibrin- SLE patients and is usually related to lu- ous valvular vegetation, Libman-Sacks en- pus itself, rather than an infectious etiol- docarditis is unique to patients with SLE. ogy. Patients may present with substernal, When present, patients usually develop a positional chest pain, tachycardia, and subacute or chronic onset of dyspnea or diffuse ST-segment elevation on electro- chest pain. However, patients may become cardiogram. Most effusions are small, as- acutely ill if they develop severe valvular ymptomatic, and discovered incidentally. regurgitation. Additionally, the valve dam-

14 EMERGENCY MEDICINE I JANUARY 2018 www.emed-journal.com age from Libman-Sacks endocarditis can sant therapy (high-dose corticosteroids, predispose patients to developing infec- cyclophosphamide, and/or plasma ex- tive endocarditis.20 change), and admission to the ICU.24

Hematological Complications Gastrointestinal Complications Common Complications Common Complications Patients with SLE commonly have mild-to- Intestinal Pseudo-obstruction. Dysphagia relat- moderate leukopenia (especially lympho- ed to esophageal dysmotility is present in penia), anemia, and thrombocytopenia. up to 13% of SLE patients.25 Intestinal pseu- This may be related to the disease process do-obstruction may be seen in SLE patients, or may be secondary to prescribed medica- and is characterized by symptoms of intes- tions. A comparison to recent baseline lab- tinal obstruction caused by decreased intes- oratory studies should be sought if there is tinal motility, rather than from mechanical suspicion for new or worsening cytopenia. obstruction. Presenting symptoms may be Antiphospholipid Syndrome. Nearly 40% of acute or chronic, and include nausea, vom- SLE patients also have APS, which is defined iting, and abdominal distension. Abdomi- by a clinical history of thrombosis in con- nal CT studies will show dilated bowel junction with one of the antiphospholipid loops without evidence of mechanical ob- antibodies (anticardiolipin, anti-beta-2-gly- struction. Manometry reveals widespread coprotein, lupus anticoagulant). Antiphos- hypomotility. Intestinal pseudo-obstruction pholipid syndrome causes both venous and typically responds well to corticosteroids arterial thrombosis and may be associated and other immunosuppressant therapies.26 with recurrent miscarriage. Acute throm- botic events should be treated with heparin Emergent Conditions or enoxaparin and transitioned to warfarin. Acute Abdominal Pain. Approximately half of The new generation of direct oral anticoag- SLE patients who present to the ED with ulants have not been well studied in APS, acute abdominal pain are found to have though, multiple small case series suggest either mesenteric vasculitis or pancreati- a higher thrombotic risk with these drugs tis, both of which are thought to be related than with warfarin.23 Patients who have re- to SLE disease activity.27 Other causes of current venous thromboembolism, or who acute abdominal pain that are common in have any arterial thromboembolism should the general population remain common be on lifelong anticoagulation therapy.2 in SLE patients, including gallbladder disease, gastroenteritis, appendicitis, and Emergent Complications peptic ulcer disease. Thrombocytopenia. Severe thrombocytope- Mesenteric Vasculitis. Also known as lupus nia or hemolytic anemia can be life-threat- enteritis, mesenteric vasculitis is a unique ening, and often requires inpatient ad- cause of acute abdominal pain in SLE pa- mission for immunosuppressive therapy, tients. The condition presents with acute, monitoring, and supportive care. diffuse abdominal pain and may be associ- Catastrophic Antiphospholipid Syndrome. ated with nausea and vomiting, diarrhea, This condition should be suspected in pa- or hematochezia. Abdominal CT findings tients with SLE who present with multiple suggestive of diffuse enteritis support sites of thrombosis or new multi-organ the diagnosis. Medical management with damage. Catastrophic APS (CAPS) may pulse-dose corticosteroids and supportive occur in SLE patients who have no prior care is generally sufficient, but if bowel ne- history of APS. Since the mortality rate crosis or intestinal perforation is present or for CAPS approaches 50%, these patients suspected, surgical consultation should be require anticoagulation, immunosuppres- obtained immediately.15

www.emed-journal.com JANUARY 2018 I EMERGENCY MEDICINE 15 SYSTEMIC LUPUS ERYTHEMATOSUS IN THE ED

Conclusion 15. Arntfield RT, Hicks CM. Systemic Lupus Erythema- tosus and the Vasculitides. In: Marx JA, ed. Rosen’s Complications of SLE are diverse and may Emergency Medicine. 8th ed. Philadelphia, PA: be difficult to diagnose. Understanding the Elsevier Saunders; 2014. 16. Borrell H, Narváez J, Alegree JJ, et al. Shrinking common and emergent complications of lung syndrome in systemic lupus erythematosus: SLE will help the EP to recognize severe a case series and review of the literature. Medi- cine (Baltimore). 2016;95(33):e4626. doi:10.1097/ illness and make appropriate treatment de- MD.0000000000004626. cisions in this complex patient population. 17. Aviña-Zubieta JA, Vostretsova K, De Vera MA, et al. The risk of pulmonary embolism and deep venous thrombosis in systemic lupus erythematosus: a References general population-based study. Semin Arthritis 1. Dall’Era M, Wofsy D. Clinical Features of Systemic Rheum. 2015;45(2):195-201. doi:10.1016/j.semar- Lupus Erythematosus. In: Firestein GS et al, eds. Kel- thrit.2015.05.008. ley and Firestein’s Textbook of Rheumatology. 10th 18. Martínez-Martínez MU, Abud-Mendoza C. Predictors ed. Philadelphia, PA: Elsevier; 2017. of mortality in diffuse alveolar haemorrhage associ- 2. Dvorkina O, Ginzler EM. Clinical features of sys- ated with systemic lupus erythematosus. Lupus. temic lupus erythematosus. In: Hochberg MC, ed. 2011;20(6):568-574. doi:10.1177/0961203310392430. Rheumatology. 6th ed. Philadelphia, PA: Elsevier; 19. Rosenbaum E, Krebs E, Cohen M, Tiliakos A, 2015. Derk CT. The spectrum of clinical manifestations, outcome and treatment of pericardial tamponade 3. Huang JL, Hung JJ, Wu KC, Lee WI, Chan CK, in patients with systemic lupus erythematosus: a Ou LS. Septic arthritis in patients with systemic retrospective study and literature review. Lupus. lupus erythematosus: salmonella and nonsal- 2009;18(7):608-612. doi:10.1177/0961203308100659. monella infections compared. Semin Arthritis 20. Miner JJ, Kim AH. Cardiac manifestations of sys- Rheumatol. 2006;36(1):61-67. doi:10.1016/j.semar- temic lupus erythematosus. Rheum Dis Clin North thrit.2006.04.003 4. Barile-Fabris L, Hernández-Cabrera MF, Barragan- Am. 2014;40(1):51-60. doi:10.1016/j.rdc.2013.10.003. Garfias JA. Vasculitis in systemic lupus erythe- 21. Morel N, Bonjour M, Le Guern V, et al. Colchi- matosus. Curr Rheumatol Rep. 2014;16(9):440. cine: a simple and effective treatment for peri- doi:10.1007/s11926-014-0440-9. carditis in systemic lupus erythematosus? A 5. Campion EW, Wigley FM, Flavahan NA. Raynaud’s report of 10 cases. Lupus. 2015;24(14):1479-1485. phenomenon. N Engl J Med. 2016;375(6):556-565. doi:10.1177/0961203315593169. doi:10.1056/NEJMra1507638. 22. Roldan CA, Shively BK, Crawford MH. An echocar- 6. Bouaziz JD, Barete S, Le Pelletier F, et al. Cutaneous diographic study of valvular heart disease associated lesions of the digits in systemic lupus erythemato- with systemic lupus erythematosus. N Engl J Med. sus: 50 cases. Lupus. 2007;16(3):163-167. 1996;335(19):1424-1430. 7. Pokroy-Shapira E, Gelernter I, Molad Y. Evolution 23. Dufrost V, Risse J, et al. Direct oral anticoagulants of chronic kidney disease in patients with systemic use in antiphospholipid syndrome: are these drugs lupus erythematosus over a long-period follow-up: a an effective and safe alternative to warfarin? A sys- single-center inception cohort study. Clin Rheumatol. tematic review of the literature. Curr Rheumatol Rep. 2014;33(5):649-657. doi:10.1007/s10067-014-2527-0. 2016;18(12):74. doi:10.1007/s11926-016-0623-7.

8. Almaani S, Meara A, Rovin BH. Update on lupus 24. Cervera R, Rodríguez-Pintó I; G Espinosa on behalf nephritis. Clin J Am Soc Nephrol. 2017;12(5):825- of the Task Force on Catastrophic Antiphospholipid 835. doi:10.2215/CJN.05780616. Syndrome. Catastrophic antiphospholipid syndrome: 9. The American College of Rheumatology nomencla- task force report summary. Lupus. 2014;23(12):1283- ture and case definitions for neuropsychiatric lupus 1285. doi:10.1177/0961203314540764. syndromes. Arthritis Rheumatol. 1999;42(4):599-608. 25. Sultan SM, Ioannou Y, Isenberg DA. A review of 10. Oomatia A, Fang H, Petri M, et al. Peripheral neu- gastrointestinal manifestations of systemic lupus ropathies in systemic lupus erythematosus: clinical erythematosus. Rheumatology (Oxford). 1999;38(10): features, disease associations, and immunologic char- 917-932. acteristics evaluated over a twenty-five year study 26. Xu N, Zhao J, Liu J, et al. Clinical analysis of 61 sys- period. Arthritis Rheumatol. 2014;66(4):1000-1009. temic lupus erythematosus patients with intestinal 11. Mitsikostas DD, Sfikakis PP, Goadsby PJ. A meta- pseudo-obstruction and/or ureterohydronephrosis: analysis for headache in systemic lupus erythemato- a retrospective observational study. Medicine (Balti- sus: the evidence and the myth. Brain. 2004;127(pt more). 2015;94(4):e419. 5):1200-1209. 27. Vergara-Fernandez O, Zeron-Medina J, Mendez- 12. Timlin H, Petri M. Transient ischemic at- Probst C, et al. Acute abdominal pain in patients tack and stroke in systemic lupus erythe- with systemic lupus erythematosus. J Gastrointest matosus. Lupus. 2013;22(12):1251-1258. Surg. 2009;13(7):1351-1357. doi:10.1007/s11605-009- doi:10.1177/0961203313497416. 0897-4. 13. Majdak MR, Vuletić V. Thrombolysis for acute stroke 28. Küçükşahin O, Düzgün N, Okoh AK, Kulahçio- in patient with systemic lupus erythematosus: a case glu E. Response to rituximab in a case of lupus report. J Neurol Sci. 2016;(361):7-8. doi:10.1016/j. associated digital ischemia. Case Rep Rheumatol. jns.2015.12.014. 2014;2014:763608. doi:10.1155/2014/763608. 14. Chen WL, Chang SH, Chen JH, Wu YL. Isolated 29. Uva L, Miguel D, Pinheiro C, Freitas JP, Gomes MM, headache as the sole manifestation of dural sinus Filipe P. Cutaneous manifestations of systemic lupus thrombosis: a case report with literature review. Am J erythematosus. Autoimmune Dis. 2012;2012:834291. Emerg Med. 2007;25(2):218-219. doi:10.1155/2012/834291.

16 EMERGENCY MEDICINE I JANUARY 2018 www.emed-journal.com