Acute Pericarditis Caused by Systemic Lupus Erythematosus Case File
Eugene C. Toy, MD, Gabriel M. Aisenberg, MD
Case 10
A 27-year-old woman presents to the emergency center complaining of
retrosternal chest pain for the past 2 days. The pain is constant, unrelated to
exertion, worsened by deep breaths, and relieved by sitting up and leaning
forward. She denies any shortness of breath, nausea, or diaphoresis.
On examination, her temperature is 99.4 °F, heart rate is 104 beats per minute
(bpm), and blood pressure is 118/72 mm Hg. She is sitting forward on the stretcher
taking shallow breaths. Her conjunctivae are clear, and her oral mucosa is pink and
notable for two aphthous ulcers. Her neck veins are not distended; her chest is
clear to auscultation and is mildly tender to palpation. Her heart rhythm is regular,
with a harsh scratchy sound over the apex heard during systole and diastole. Her
abdominal examination is unremarkable, and her extremities show warmth and
swelling of the proximal interphalangeal (PIP) joints of both hands.
Laboratory studies are significant for a white blood cell count of 2100 cells/mm3 ,
hemoglobin concentration 10.4 g/dL with mean corpuscular volume 94 fL, and
platelet count 78,000/mm3 . Her blood urea nitrogen and creatinine levels are
normal. Urinalysis shows 10 to 20 white blood cells and 5 to 10 red blood cells per
high-powered field. A urine drug screen is negative.
Chest x-ray is read as normal, with a normal cardiac silhouette and no pulmonary
infiltrates or effusions. The electrocardiogram (ECG) is shown in Figure 10–1.
Figure 10–1. Electrocardiogram. (Reproduced with permission, from Stead LG, Stead SM, Kaufman MS.
First Aid for the Medicine Clerkship, 2nd ed. 2006. Copyright © McGraw Hill LLC. All rights reserved.)
Summary: A 27-year-old woman presents with
- Nonexertional pleuritic chest pain relieved by sitting forward
- Pericardial friction rub, finger arthritis, and aphthous ulcers on examination
- Changes in the ECG (classically diffuse ST elevations and PR depressions) consistent with acute pericarditis
- No radiographic evidence of a large pericardial effusion
- No clinical signs of cardiac tamponade (water bottle sign, quiet heart sounds, jugular venous distension, etc)
- Pancytopenia, pyuria, and microhematuria
Most likely diagnosis: Acute pericarditis due to systemic lupus erythematosus (SLE).
Best next step: Echocardiogram to assess for effusion and tamponade.
ANALYSIS
Objectives
- Recognize the clinical and ECG features of pericarditis and be able to recognize a pericardial friction rub. (EPA 1, 2, 3)
- List the causes of pericarditis and its treatment. (EPA 4, 12)
- List the diagnostic criteria for SLE. (EPA 3, 12)
- Describe the major complications of SLE and its treatment. (EPA 12)
Considerations
In patients with chest pain, one of the primary diagnostic considerations is always myocardial ischemia or myocardial infarction (MI). This is particularly true when the ECG is abnormal with changes that may represent myocardial injury, such as ST-segment elevation. However, other conditions may produce ST-segment elevation, such as acute pericarditis. ECG findings can help distinguish between these two diagnoses. Pericarditis may have diffuse ST elevations and PR depressions, whereas STEMI (ST-segment elevation myocardial infarction) typically will demonstrate ST elevation consistent with an anatomic area of infarction (eg, anterolateral, inferior, posterior, or lateral). The pleuritic nature of the pain and the relief by leaning forward are clues to the diagnosis. Of the multiple possible etiologies, the case discloses some features suggestive of SLE.
APPROACH TO:
Acute Pericarditis and Systemic Lupus Erythematosus
DEFINITIONS
ACUTE PERICARDITIS: An inflammation of the pericardial sac surrounding the heart.
PERICARDIAL FRICTION RUB: Harsh, high-pitched, scratchy sound with variable intensity, usually best heard at the left sternal border by auscultation.
CLINICAL APPROACH TO ACUTE PERICARDITIS
Pathophysiology
Acute pericarditis can result from a multitude of disease processes, but the most common causes are listed in Table 10–1.
Clinical Presentation
There is a wide spectrum of clinical presentations for acute pericarditis, ranging from subclinical inflammation, to the classic presentation of acute pericarditis with chest pain, to chronic inflammation, which may persist for weeks to months. Most patients with acute pericarditis seek medical attention because of chest pain. The classic description is a sudden onset of retrosternal chest pain, which worsens on inspiration and with recumbence and often radiates to the trapezius ridge; the pain is improved by sitting and leaning forward. Other clinical features vary according to the cause of the pericarditis, but most patients are thought to have viral infection and often present with low-grade fever, malaise, or upper respiratory illness symptoms.
A pericardial friction rub is pathognomonic and virtually 100% specific for acute pericarditis. The sensitivity of this sign varies, however, because friction rubs may soften and return. Classically, a rub is a harsh, high-pitched, scratchy sound with variable intensity, usually best heard at the left sternal border. It can have one, two, or three components: presystolic (correlating with atrial systole), systolic, and diastolic. The large majority of rubs are triphasic (all three components) or biphasic,
having a systolic and either an early or a late diastolic component. In these cases, it usually is easy to diagnose the pericardial friction rub and acute pericarditis. When the rub is monophasic (just a systolic component), it often is difficult to distinguish a pericardial friction rub from a harsh murmur, making bedside diagnosis difficult and uncertain. In these cases, one should look for ECG evidence of pericarditis (Table 10–2) and perform serial examinations because the rub may vary with time.
The classic ECG findings in acute pericarditis include diffuse ST-segment elevation in association with PR-segment depression, as seen in this patient. The opposite findings (PR-segment elevation and ST-segment depression) are often seen in leads aVR and V1. Acute pericarditis may be confused with acute MI due to the presentation with chest pain and ST-segment elevation on ECG. This is potentially a serious problem because if a patient is treated with thrombolytics for infarction, the patient may develop pericardial hemorrhage and cardiac tamponade. Several clinical features can help to differentiate the two conditions: Acute ischemia is more likely to have a gradual onset of pain with a crescendo pattern, more likely to present with a heavy pressure or squeezing sensation (as opposed to the sharp pain of pericarditis), typically does not vary with respiration, and is relieved with nitrates (whereas the pain of pericarditis is not). In addition, several ECG features can help to make the distinction (Table 10–2). Moreover, if the ECG reveals arrhythmias or conduction abnormalities, the condition is much more likely to represent ischemia rather than pericarditis.
Treatment
Most patients with acute viral or idiopathic pericarditis have excellent prognoses. Treatment is mainly symptomatic, with aspirin or another nonsteroidal anti-inflammatory drug (NSAID), such as indomethacin, for relief of chest pain. Colchicine or corticosteroids may be used for refractory symptoms or comorbid conditions. In most patients, symptoms typically resolve within days to 2 to 3 weeks. Any form of pericarditis can cause pericardial effusion and bleeding; however, the most serious consequence would be cardiac tamponade. It is a common misconception that a pericardial friction rub cannot coexist with an effusion (both are very common in uremic pericarditis). Therefore, it is important to monitor these patients for signs of developing hemodynamic compromise resulting from cardiac tamponade.
CLINICAL APPROACH TO SYSTEMIC LUPUS ERYTHEMATOSUS
Pathophysiology
Our patient is very young and has no significant previous medical history. The presence of symmetric arthritis and laboratory findings suggest a systemic disease, such as SLE, as the cause of her pericarditis. SLE is a systemic inflammatory disease that mainly affects women. It is characterized by autoimmune multiorgan involvement, such as pericarditis, nephritis, pleuritis, arthritis, and skin disorders. To diagnose SLE, the patient must meet 4 of the 11 criteria listed in Table 10–3 (96% sensitive and 96% specific). The need of 4 out 11 criteria is imperative for the diagnosis of SLE among patients included in clinical studies; however, it is noteworthy that patients can actually be diagnosed with SLE even with fewer criteria in the right clinical context.
Our patient has serositis (pericarditis), oral ulcers, hematologic disorders (leukopenia, lymphopenia, and thrombocytopenia), arthritis, and renal involvement (hematuria)—she clearly meets the clinical criteria for SLE. Although the patient in the scenario, like most patients with SLE, sought medical attention because of the pain of arthritis or serositis, both these problems are generally manageable or self-limited. The arthritis is generally nonerosive and nondeforming, and the serositis usually resolves spontaneously without sequelae.
Complications
The major complication of SLE usually is related to renal involvement, which can cause hypertension, chronic renal failure, nephrotic syndrome, or end-stage renal disease. In the past, renal disease was the most common cause of death of SLE patients; however, currently lupus neprhritis can be treated with powerful immunosuppressants, such as high-dose corticosteroids and mycophenolate or cyclophosphamide. Other serious complications of lupus include central nervous system (CNS) disorders, which are highly variable and unpredictable and can include seizures, psychosis, stroke syndromes, and cranial neuropathies. In addition to renal failure and CNS involvement, the most common causes of death in SLE patients are infection (often related to the immunosuppression used to treat the disease) and vascular disease, for example, MI.
CASE CORRELATION
- See also Case 3 (Acute Coronary Syndrome), Case 5 (Aortic Dissection, Marfan Syndrome), and Case 20 (Peptic Ulcer Disease).
COMPREHENSION QUESTIONS
10.1 A 68-year-old man with a history of end-stage renal disease is admitted to the hospital for chest pain. On examination, a pericardial friction rub is noted. His ECG shows diffuse ST-segment elevation. Which of the following is the best definitive treatment?
A. Nonsteroidal anti-inflammatory drugs
B. Dialysis
C. Steroids
D. Sodium polystyrene sulfonate (Kayexalate)
10.2 The patient described in Question 10.1 is hospitalized, but there is a delay in initiating treatment. You are called to the bedside because he has become hypotensive with a systolic blood pressure of 85/68 mm Hg, a heart rate of 122 bpm, and pulsus paradoxus. A repeat ECG is unchanged from admission. Which of the following is the most appropriate immediate intervention?
A. Draw blood cultures and initiate broad-spectrum antibiotics for suspected sepsis.
B. Give intravenous furosemide for fluid overload.
C. Perform echocardiographic-guided pericardiocentesis.
D. Perform percutaneous coronary intervention for acute MI.
10.3 A 25-year-old woman complains of pain in her PIP and metacarpophalangeal joints and reports a recent positive antinuclear antibody (ANA) laboratory test. Which of the following clinical features would not be consistent with a diagnosis of SLE?
A. Pleural effusion
B. Malar rash
C. Sclerodactyly
D. Urinary sediment with red blood cell casts
ANSWERS
10.1 B. Uremic pericarditis is considered a medical emergency and an indication for urgent dialysis. While NSAIDs (answer A) can help with pain, they do not attack the pathophysiologic underlying process. Steroids (answer C) do not offer benefit in this setting. Resins like sodium polystyrene sulfonate (answer D) are beneficial in the treatment of hyperkalemia.
10.2 C. The clinical picture suggests the patient has developed pericardial tamponade, which may be life threatening and often requires urgent pericardiocentesis. The question stem does not suggest sepsis or an MI, making answers A and D incorrect. Furosemide (answer B) can worsen the picture by decreasing the intravascular compartment further and is therefore contraindicated.
10.3 C. Sclerodactyly, which is thickened and tight skin of the fingers and toes, is a classic feature of patients with scleroderma (who may also have a positive ANA test but will likely have either anticentromere or antitopoisomerase antibodies), but it is not seen in SLE. Malar rash (answer B), serositis, and glomerulonephritis are typical of SLE but are not seen in scleroderma. The other answer choices such as pleural effusion (answer A) and red blood cell casts (answer D) would suggest lupus nephritis are often found in SLE.
CLINICAL PEARLS
▶ Acute pericarditis is characterized by pleuritic chest pain, a pericardial friction rub, and ECG findings of diffuse ST-segment elevation and PR-segment depression.
▶ Pericardial friction rub does not exclude a pericardial effusion; patients with acute pericarditis should be monitored for development of effusion and tamponade.
▶ Treatment of pericarditis is directed at the underlying cause; for example, uremic pericarditis requires urgent dialysis. For viral or inflammatory causes, treatment is NSAIDs or corticosteroids for refractory cases.
▶ Systemic lupus erythematosus can be diagnosed if a patient has four of the following features: malar rash, discoid rash, photosensitivity, oral ulcers, arthritis, serositis, renal disease, neurologic manifestations, hematologic cytopenias, immunologic abnormalities (eg, false-positive Venereal Disease Research Laboratory [VDRL] test), or positive ANA.
▶ The major morbidity and mortality of SLE result from renal disease, CNS involvement, or infection.
REFERENCES
Braunwald E. Pericardial disease. In: Jameson JL, Fauci AS, Kasper DL, et al., eds. Harrison’s Principles of Internal Medicine. 20th ed. New York, NY: McGraw Hill; 2018:1971-1978.
Hahn BH. Systemic lupus erythematosus. In: Jameson JL, Fauci AS, Kasper DL, et al., eds. Harrison’s Principles of Internal Medicine. 20th ed. New York, NY: McGraw Hill; 2018:2724-2735.
Lange RA, Hillis LD. Acute pericarditis. N Engl J Med. 2004;351:2195-2202.
Spodick DH. Acute pericarditis: current concepts and practice. JAMA. 2003;289:1150-1153.
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