Theodore I. Benzer
Patients with multisystem autoimmune disorders may present for initial medical evaluation with a bewildering array of symptoms or complaints. Many of the tests needed to diagnose these “rheumatic disorders” cannot be performed during an emergency department (ED) visit. However, the emergency physician must recognize that an autoimmune disorder may be responsible for the patient’s clinical picture and must decide which patients require admission and which can safely undergo an outpatient evaluation. Likewise, when a patient with known multisystem autoimmune disease presents to the ED with new symptoms, the physician must determine whether they represent an exacerbation of known disease, a manifestation of an unrelated disorder, or a complication of therapy.
Evolving therapies and variations in individual response to treatments make rheumatologic consultation appropriate in many cases. Although the ultimate cause of the autoimmune disorders remains a mystery, it is increasingly clear that specific autoantibodies directed against cellular components are markers for, and probable causes of, many of the manifestations. Positive tests for these antibodies not only correlate with current symptoms but are also predictive of the patient’s course. The use of these specific disease markers can improve accuracy in diagnosis and can guide therapy directed at specific pathologic entities.
Despite the fact that definitive therapies for most autoimmune disorders are lacking, there is often much that can be done to alleviate symptoms and prevent progression of tissue damage. Every pharmacologic treatment has a potential for toxicity that must be balanced against the potential benefit it may provide to the patient.
SYSTEMIC LUPUS ERYTHEMATOSUS
Systemic lupus erythematosus (SLE) is the prototypical autoimmune disease. Its prevalence is 1 in 2,000 with a 10:1 predominance of women over men and a predilection for nonwhites. The prevalence is 1 in 750 for black women between the ages of 20 and 64 (1).
The cause of SLE remains unknown; hormonal, metabolic, genetic, environmental, and infectious factors have all been proposed as etiologic agents. The pathophysiology of SLE involves the production of autoantibodies initiated by an unknown stimulus. These antibodies react with cell constituents and initiate an inflammatory response that results in the activation of the complement pathway and the elaboration of chemotactic factors and other inflammatory mediators. Antibody-dependent cytotoxic leukocytes are also activated.
Antinuclear antibodies (ANAs) are almost invariably detected in patients with SLE. Although ANAs are found in low titers in many other autoimmune and nonrheumatologic disorders, high titers are more specific for SLE. High titers of antibodies to double-stranded DNA are seen only in patients with SLE (2). Antibodies to the Smith antigen are also specific for SLE, as well as being associated with more severe symptoms. During active SLE, complement levels are depressed and may be helpful in following the course of disease (1).
CLINICAL PRESENTATION
SLE can affect any tissue or organ and has been said to have replaced syphilis as “the great imitator” of other diseases (1). Patients often make multiple visits to physicians before SLE is initially recognized. Some of the more common manifestations are listed in Table 178.1.
TABLE 178.1
Systemic Lupus Erythematosus: Characteristic Features and Suggestive Signs and Symptoms
The most typical initial complaints are fatigue and arthralgias, but fever, anemia, anorexia, and a malar rash are other common presenting symptoms. The arthralgias are commonly fleeting and migratory, typically appearing in the proximal interphalangeal and metacarpophalangeal joints, the wrists, and the knees, but they may also be due to frank synovial inflammation.
The rash of SLE varies from a slight blush to a well-demarcated and somewhat edematous maculopapular erythematous eruption covering both cheeks and the bridge of the nose. It tends to spare the nasolabial folds but can cause scarring and atrophy of skin structures. The rash is characteristically exacerbated by sun exposure and may often cause a patient to seek medical attention before any other symptoms of SLE have developed.
Although the kidneys are commonly affected in SLE, nephrotic syndrome and peripheral edema are rarely seen before other manifestations of disease have led to the diagnosis of SLE (3). Patients with SLE frequently present with acute, potentially life-threatening problems. Complaints of chest pain, abdominal pain, and neurologic dysfunction can indicate an acute flare of disease or a serious complication of therapy. Patients with acute chest pain, with or without fever, may have pleuritis, pericarditis, or pneumonitis. Abdominal pain may indicate an acute flare of polyserositis.
Neurologic problems of virtually any type may be seen in SLE. Most common are central disorders ranging from mood disorders to frank psychosis. Seizures are also common, especially in younger patients. SLE should be in the differential diagnosis of new-onset seizures, especially in young women.
The lupus anticoagulant, also called the anticardiolipin antibody or antiphospholipid antibody, is an antibody that produces an artifactually prolonged partial thromboplastin time in vitro but a “paradoxical” procoagulant effect in vivo. Although antiphospholipid antibodies are associated with SLE, they may occur as a part of a distinct pathologic entity referred to as the antiphospholipid syndrome (4,5). Both conditions can lead to deep venous thrombi, pulmonary emboli, stroke, chronic ulcers of the extremities, and thrombocytopenia (4,5). Patients with recurrent or unusual thrombosis should be evaluated for antiphospholipid autoantibody and SLE (6). Recent studies have shown that patients with the antiphospholipid syndrome are at increased risk for cardiovascular disease.
Patients with known SLE may present to the ED with either exacerbations of their disease or complications of therapy.
DIFFERENTIAL DIAGNOSIS AND ED EVALUATION
Given the variety of possible presentations, the differential diagnosis of SLE is exceedingly broad. A thorough history and physical examination are required to identify multisystem involvement and recurrent symptoms. The differentiation of SLE from the other autoimmune syndromes may require repeated rheumatologic evaluation and specific autoantibody testing.
Tan et al. (7) have published a list of 11 criteria diagnostic for SLE (Table 178.2). The diagnosis is established if 4 or more of these findings are present serially or simultaneously. A medication history must be elicited to rule out drug-induced lupus. Attention to detail in evaluating the patient with nonspecific symptoms may lead one to suspect the diagnosis of SLE. The diagnosis should be particularly entertained in women of childbearing age who have recurrent or vague symptoms that do not clearly point to a discrete disease entity.
TABLE 178.2
Diagnostic Criteria for Systemic Lupus Erythematosusa
Drug-induced SLE is a distinct entity that has been associated with many medications, including procainamide, hydralazine, anticonvulsants, chlorpromazine, isoniazid, methyldopa, penicillamine, quinidine, propylthiouracil, and sulfasalazine. Drug-induced SLE has a milder course than idiopathic SLE and does not cause renal or central nervous system involvement (8). Specialized ANA testing panels reveal only histone-binding ANAs rather than the multiple varieties characteristic of SLE (1).
The evaluation of patients with established SLE should focus on the presenting complaint. However, attention should also be given to signs and symptoms of the potentially life-threatening complications of SLE, such as thrombotic disease, pleural and pericardial effusions, and infections secondary to immunosuppressive therapy.
Patients presenting with new-onset monoarthritis or oligoarthritis generally require diagnostic arthrocentesis to rule out infection or crystal-induced arthritis. Fluid should be sent for Gram stain, culture, cell count and differential, and examination for crystals. The synovial fluid in autoimmune arthritis is typically inflammatory, with an elevated white cell count (20,000 to 80,000/ μ L mixed polymorphonuclear cells and lymphocytes), a negative Gram stain, and no crystals.
The specific organs affected by SLE and the severity of the disease vary widely between individuals and over time in any given individual. Not only does the variability of the disease make diagnosis difficult, but, with the exception of the characteristic rash, each of the manifestations of lupus may occur from other causes. Although a presentation of one of these conditions alone would not prompt consideration for autoimmune disease, recurrent symptoms or the sequential development of multiple conditions associated with lupus warrants referral for ANA testing.
ED MANAGEMENT
The specific presentation dictates the management indicated in the ED. Patients presenting with mild symptoms may require nothing more than screening for renal and hematologic involvement with a urinalysis and complete blood cell count. More severe symptoms are managed as in any other disease. For example, a large pleural effusion should be tapped, and anticoagulation should be started for thromboembolic disease.
ST elevations and PR depressions seen on the electrocardiogram that are associated with a pericardial friction rub indicate pericarditis. If pericarditis is suspected, a careful evaluation should be made for signs of pericardial tamponade including a bedside echocardiogram if available. Elevated neck veins, elevated pulsus paradoxus, or hypotension should prompt a formal urgent echocardiogram to rule out tamponade. Infectious complications should be considered in patients with established disease who are taking chronic immunosuppressive medications.
Patients with significant pleural or pericardial effusion, or any thrombotic complication, require hospital admission and rheumatologic consultation. Immunosuppressed patients with documented infection require admission for parenteral antibiotics.
Neuropsychiatric complications are frequent in SLE. Cerebritis, aseptic meningitis, seizures, ischemic strokes, peripheral neuropathy, and acute psychosis are all recognized manifestations of SLE. Corticosteroid treatment and other immunosuppressive agents predispose to infectious meningitis and corticosteroid-induced psychosis.
The acute presentation of an SLE patient with a neuropsychiatric complaint is always a challenge. The diagnosis of lupus flare should be made only after infectious etiologies have been ruled out. Patients presenting with severe disease are often treated with “stress” doses of corticosteroids (hydrocortisone, 100 mg intravenously, or methylprednisolone, 125 mg intravenously) and often receive empiric broad-spectrum antibiotic coverage after samples of blood, urine, sputum, and possibly cerebral spinal fluid are obtained for culture.
Patients with established disease who present with a flare of symptoms may require the initiation of corticosteroids or an increase in the dose. When more than 5 to 10 mg of prednisone a day is required, many rheumatologists prescribe combination therapy in an effort to limit adverse effects. Medications commonly used include nonsteroidal anti-inflammatory drugs (NSAIDs), antimalarial agents, cyclophosphamide, and mycophenolate mofetil. More recently B-cell inhibitors, including rituximab and belimumab, have been found useful in treatment of SLE patients resistant to more traditional therapy. Because every therapeutic agent has adverse effects, the risks of medication must be weighed against the morbidity of the clinical presentation. The aggressiveness and urgency of treatment depend on the presence of major organ involvement. Changes in a patient’s usual medication regimen are best carried out in conjunction with the patient’s rheumatologist.
DISPOSITION
Patients with suspected or newly diagnosed SLE may be managed as outpatients, if the manifestations are mild and a life-threatening pathologic process is absent. An ANA panel should be ordered. Rheumatologic consultation can be obtained by telephone concerning any further studies, initiation of corticosteroid therapy, and appropriate follow-up. Newly diagnosed patients with SLE should be educated about the importance of adequate sleep and avoidance of ultraviolet light. Ibuprofen and estrogens may exacerbate disease and should be avoided (3). Hypertension should be controlled vigorously because it appears to have a synergistic effect in exacerbating renal disease. In drug-induced lupus, after the medication is discontinued, symptoms usually resolve within days to weeks (8,9).
Common Pitfalls
• Failure to recognize vague, atypical complaints as a presentation of SLE or other autoimmune disease.
• Failure to include SLE in the differential diagnosis of patients with pericarditis, pleuritis, renal dysfunction, neurologic symptoms, or recurrent venous thrombosis.
• Failure to differentiate disease exacerbation from an adverse effect of therapy.
• Failure to suspect infection in an immunosuppressed patient.
RHEUMATOID ARTHRITIS
Rheumatoid arthritis (RA) is a chronic autoimmune disorder characterized by widespread synovial inflammation and, often, progressive destruction of joints. It affects women three times as often as men. The prevalence increases with age and is approximately 1%.
The pathophysiology of RA involves the production of immunoglobulins against native IgG; these immunoglobulins are referred to as rheumatoid factor. Helper T-cells are also activated and produce lymphokines that promote cellular proliferation (10). Recently, researchers have identified autoantibodies to the citrullinated protein filaggrin that seem to be sensitive and specific for RA. Citrullinated proteins are exclusively produced in the synovial tissue. These autoantibodies to citrullinated proteins may be the initial target of the autoimmune response in RA (11). Activation of the inflammatory response leads to the elaboration of vasoactive substances, chemotactic factors, and complement in synovial tissues. Mononuclear cells infiltrate the subsynovial stroma, and polymorphonuclear cells appear in the synovial fluid. A “pannus” of inflamed, thickened, redundant synovium invades and replaces cartilage and periarticular bone and tendon. Polymorphonuclear cells in the synovial fluid release lysozymes that break down hyaluronic acid polymers in the synovial fluid and articular cartilage (1). Although the symptoms and signs are primarily related to the joints, RA is a multisystem autoimmune disease that frequently involves the heart, lungs, and blood.
CLINICAL PRESENTATION
RA typically presents initially as constitutional symptoms such as low-grade fever, weight loss, fatigue, and lymphadenopathy. Joint stiffness in the morning or after periods of inactivity may also be a complaint.
Articular symptoms initially involve any number or size of joints. Either arthralgia (pain) or arthritis (inflammation) may be present in an unpredictable pattern. Later, in established disease, the typical symmetric pattern of arthritis develops.
The most commonly affected joints are the metacarpophalangeal and proximal interphalangeal joints, wrists, knees, and upper spine. Although some cases of RA remain mild or even resolve, the typical course is one of unpredictable exacerbations and remissions with progressive deformity and disability.
As tissue damage progresses, typical ulnar deviation of the carpometacarpal joints and valgus deformity of the knees develop. Baker cysts may develop in the popliteal fossa. Of prime importance for the emergency physician is the development of instability of the atlantoaxial joint, with laxity and even rupture of the transverse ligament. The result is an unstable cervical spine, which may lead to spinal cord compression following even apparently trivial injury (6).
RA is a systemic disease. Over time, subcutaneous nodules over the elbows, occiput, and sacrum, thought to be vasculitic in origin, develop in 20% to 25% of patients. Pericarditis, pleuritis, pulmonary fibrosis, scleritis, Sjögren syndrome, nerve entrapment, and vasculitis may also occur. Felty syndrome, consisting of splenomegaly, anemia, thrombocytopenia, and granulocytopenia, sometimes complicates RA.
DIFFERENTIAL DIAGNOSIS AND ED EVALUATION
RA is rarely misdiagnosed when it reaches the stage of symmetric polyarthritis in characteristic joints. At earlier stages, or with milder involvement, the clinical picture may be suggestive of inflammatory or infectious joint disease or other autoimmune diseases. When joint manifestations become apparent, infectious and crystalline synovitis must be considered.
In practice, most patients presenting to the ED with oligoarthropathy should have joint aspiration to rule out infection or crystal-induced arthritis. Although RA or another autoimmune disorder may be suspected, the definitive diagnosis is made at follow-up, when further data, such as final culture results, rheumatoid factor tests, and response to anti-inflammatory therapy, are available.
In 2010, the American College of Rheumatology and the European League against Rheumatism developed new diagnostic criteria that focus on early diagnosis (Table 178.3) (12). In practice, most such patients presenting to the ED must have other significant disease ruled out (e.g., infectious arthritis, SLE). Although there may be a suspicion of RA, the definitive diagnosis is usually made on further evaluation at follow-up.
TABLE 178.3
ACR/EULAR Criteria for the Diagnosis of Rheumatoid Arthritis
Patients with known RA who present with an exacerbation of joint inflammation require evaluation for possible secondary infections or crystalline arthropathy. Others may present with new systemic symptoms. Although the problem may be related to RA, this relationship should not be assumed to be the case until other serious causes have been excluded. Evaluation based on presentation should be pursued as in any patient without RA. Special consideration should be given to complications of therapy; for example, pulmonary interstitial disease may be a result of either RA or treatment.
If a diagnosis of RA is considered, a rheumatoid factor titer should be ordered. If other autoimmune disorders are considered possible, other autoantibody titers, complement levels, and an erythrocyte sedimentation rate (ESR) may be indicated. Monoarticular or oligoarticular arthritis should cause one to consider crystalline and infectious arthritis. Joint aspiration is indicated, as well as oropharyngeal and genital cultures for Neisseria gonorrhoeae.
Few laboratory test results are abnormal in RA, and the results tend to be nonspecific. The ESR is elevated and parallels disease activity. There may be a mild normocytic anemia. A test for mixed cryoglobulins, reflecting large amounts of circulating immune complexes, may be positive, but complement levels are usually normal. Arthrocentesis of involved joints typically yields sterile fluid with hallmarks of inflammation such as decreased mucin clot formation and 20,000 to 80,000 white blood cells per microliter, of which 50% to 70% are polymorphonuclear cells (13). The classic laboratory finding of a positive serum rheumatoid factor is found in 80% of patients, but a positive rheumatoid factor can also be found in 1% to 5% of persons without RA (e.g., patients with other rheumatic disorders, leprosy, tuberculosis, liver disease, or bacterial endocarditis).
ED MANAGEMENT AND DISPOSITION
NSAIDs are the mainstay of chronic treatment of RA. Patients experiencing a flare of known disease often require therapy in addition to NSAIDs. These therapies include heat modalities, antimalarial agents, sulfasalazine, methotrexate, and surgical replacement of joints. Recently, several biologic therapies have become available for RA that interfere with the action of tumor necrosis factor, B-cells, T-cells, and interleukin-6. These drugs have been shown to improve symptoms and slow progression of disease in those patients who have had an inadequate response to traditional therapies (14,15).
Corticosteroids are usually reserved for the treatment of extra-articular manifestations. Each of the therapeutic agents has associated adverse effects. Sulfasalazine may cause leucopenia. The antineoplastic agents can cause myelosuppression and a host of other adverse effects. Biologic therapy has been associated with infections and may be associated with increased incidence of demyelinating disease, lymphoma, and worsening symptoms of congestive heart failure. These antineoplastic and biologic agents should be initiated only after discussion with a rheumatologist who will be observing the patient.
Appropriate disposition depends on the severity of the presenting symptoms. Outpatient treatment is appropriate in individuals who do not appear toxic, including those with a new diagnosis of RA. These patients should be started on a standard dose of an NSAID and have rheumatologic follow-up arranged. Patients who appear ill require hospital admission for more intensive evaluation to rule out infectious disorders and rheumatologic consultation. Hospitalization may be required for social reasons or when disability prevents adequate functioning at home.
Common Pitfalls
• Prescribing corticosteroids as a first-line therapy for RA.
• Failure to recognize nonspecific complaints as a manifestation of autoimmune disease.
• Failure to consider possible C1–C2 instability when intubating an RA patient.
SCLERODERMA
Scleroderma (or systemic sclerosis) is an autoimmune disorder characterized by abnormal collagen production that results in pathologic fibrosis of the skin and internal organs. It occurs equally in all races and geographic areas, but it is four times more common in women than in men. In most cases, the onset is during the third decade of life (16). The course is typically progressive over decades, but it may be fulminant and fatal. Collagen accumulation and endothelial cell proliferation in the vascular intima lead to severe narrowing of the arterial lumen.
Scleroderma can be classified according to its predominant manifestations. The CREST syndrome consists of subcutaneous calcinosis, Raynaud phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasia and is associated with a specific antibody (17,18). “Diffuse scleroderma” denotes the full systemic disease. A third subset is mixed connective-tissue disease or undifferentiated connective-tissue disorder, which combines features of SLE, scleroderma, and polymyositis. Clinical criteria have been developed for the diagnosis of systemic sclerosis (Table 178.4).
TABLE 178.4
Clinical Criteria for the Diagnosis of Systemic Sclerosis
CLINICAL PRESENTATION
Patients with scleroderma most commonly present to the ED because of painful fingers due to Raynaud phenomenon, dyspnea due to restrictive lung disease, or dysphagia due to esophageal dysfunction. Malignant hypertension due to scleroderma renal disease is a true emergency.
Raynaud phenomenon is present in virtually all cases of scleroderma and is the initial manifestation in 70% of cases. Skin involvement is usually first manifested as edema of the fingers. As the disease progresses, the skin becomes shiny and taut with loss of the normal skin folds. Joints become immobilized from tight encasement in thickened skin and from contractures of muscles, tendons, and palmar fascia.
DIFFERENTIAL DIAGNOSIS AND ED EVALUATION
Established scleroderma causes a characteristic taut, smooth appearance of the hands and face. This sign should be apparent to the physician, although the patient may not have noticed the gradual changes.
Early disease may be difficult to recognize, however. The most common early symptom is painful fingers caused by Raynaud phenomenon, but this symptom is a nonspecific feature of many autoimmune disorders that may also be caused by occlusive arterial disease, repetitive trauma to the fingers, cryoglobulinemia, neurogenic lesions, or vasospastic drugs. Raynaud phenomenon unassociated with scleroderma is extremely common in women. Specific autoantibody testing may distinguish early cases of scleroderma from other causes of Raynaud phenomenon. Radiography of the hand may also be helpful. Subcutaneous calcinosis and resorption of the tufts of the distal phalanges are pathognomonic findings. More than 90% of patients with scleroderma have a positive ANA test.
An awareness of the systemic manifestations of disease is important in the evaluation of the patient with known scleroderma. Systemic collagen deposition may result in cardiac conduction abnormalities or cardiomyopathy, restrictive lung disease and pulmonary hypertension, gastrointestinal dysmotility and malabsorption, and renal arteriolar necrosis leading to malignant hypertension. Recognition of these manifestations in patients with advanced illness is critical.
ED MANAGEMENT
The majority of patients with Raynaud syndrome do not have symptoms requiring treatment in an ED. Patients with persistent vasospasm may respond to simple interventions, such as warming of the hands or oral administration of nifedipine. Only rarely are more invasive maneuvers, such as interarterial or local phenoxybenzamine or prazosin, required.
Systemic manifestations may require evaluation for dysfunction caused by scarring in many organ systems. Intestinal hypomotility may be treated with metoclopramide. Esophageal reflux should be treated with antireflux maneuvers and medications as necessary. Patients with increased blood pressure, renal failure, and internal organ involvement are frequently treated with angiotensin-converting enzyme inhibitors.
DISPOSITION
Most patients with scleroderma do not require hospitalization, but admission may be necessary for dehydration due to gastrointestinal dysfunction or for pulmonary compromise. Patients with hypertensive emergency caused by renal crisis should be placed on nitroprusside and admitted to an intensive care unit. Patients with pulmonary artery hypertension can be treated with the endothelin receptor antagonist bosentan, which also has been shown to decrease the incidence of digital ulcerations in scleroderma (19).
Only those women with severe Raynaud phenomenon and those men without a history of trauma to the hands require periodic monitoring for the development of scleroderma. Patients should be instructed in the importance of keeping the hands warm and avoiding nicotine and caffeine. Suppressive pharmacologic therapy may be necessary if attacks are frequent.
Patients with signs and symptoms beyond Raynaud phenomenon should be referred for diagnostic autoantibody testing, occupational therapy, or orthopedics.
Common Pitfalls
• Failure to consider the systemic manifestations of scleroderma.
• Failure to recognize scleroderma as the cause of a hypertensive emergency.
GIANT-CELL ARTERITIS
Giant-cell arteritis is characterized by inflammation of branches of the carotid artery (temporal arteritis) or the aortic arch (Takayasu arteritis) and can present with emergent manifestations.
CLINICAL PRESENTATION
Temporal arteritis usually occurs in women older than 50 years. Symptoms may be nonspecific: Fever, headache, myalgias, and fatigue. Patients may have the syndrome of polymyalgia rheumatica, manifested by chronic stiffness and aching of the neck, shoulder, and hip girdle. There is frequent vestibular dysfunction and hearing loss at presentation (20). Chronic or subacute headache is often the presenting complaint.
The temporal artery may be tender and nodular (21), and there may be jaw claudication. The chief complication of temporal arteritis is monocular visual loss caused by ischemic optic neuritis. Visual loss or impairment generally occurs 3 or 4 months after the onset of initial symptoms (22).
Takayasu arteritis has a predilection for involvement of branches of the aortic arch. It is a rare disease. Patients are usually young women who present with nonspecific symptoms of fever, night sweats, fatigue, myalgias, and weakness. Signs of large vessel occlusion with decreased peripheral pulses, cerebrovascular accidents, or myocardial ischemia occur only months to years after initial presentation (23).
DIFFERENTIAL DIAGNOSIS AND ED EVALUATION
The differential diagnosis of patients with temporal arteritis is wide because of the nonspecific symptoms. Mild anemia is typical. A markedly elevated ESR in a patient older than 50 with headache and nonspecific symptoms, particularly polymyalgia rheumatica, should prompt further evaluation.
Takayasu arteritis, although rare, should be considered in any young female with signs of large vessel ischemia (e.g., cerebrovascular accident, upper extremity ischemia, cardiac ischemia).
ED MANAGEMENT AND DISPOSITION
Patients with suspected temporal arteritis rarely need admission to the hospital. Prompt diagnosis and treatment are essential to prevent visual impairment. In the ED, patients should be started on 40 to 60 mg of prednisone daily. Arrangements should be made for definite follow-up and semi-urgent temporal artery biopsy. Response to corticosteroid treatment is usually dramatic, with relief of systemic symptoms and headaches within days. Treatment is generally continued for 1 to 2 years.
The diagnosis of Takayasu arteritis is not usually made in the ED. The diagnosis should be entertained in young women who need admission for large vessel occlusive disease. Angiography of the affected vessels shows narrowing or occlusion of large vessels with well-developed collateral circulation (24). Recent advances in computed tomography (CT) and magnetic resonance angiography (MRA) make these modalities important in evaluating the great vessels in patients (25). No treatment has been proven effective, but corticosteroids, cyclophosphamide and Tocilizumab an interleukin-6 inhibitor have been used.
Common Pitfalls
• Failure to consider temporal arteritis in an elderly patient who presents with headache or shoulder and neck stiffness.
• Failure to consider Takayasu arteritis in a young woman with signs of large vessel occlusion.
CRITICAL INTERVENTIONS
• Perform a diagnostic arthrocentesis to rule out infection or crystal-induced arthritis in patients presenting with new-onset monoarthritis or oligoarthritis.
• Treat cervical spine trauma in the RA patient carefully because of the development of instability of the atlantoaxial joint.
• Initiate corticosteroid therapy in the ED in the patient suspected of having temporal arteritis.
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