Pericardial Effusion in Systemic Sarcoidosis: A Rare Manifestation of Cardiac Sarcoid.

We present a 65-year-old African American woman who was found to have pericardial effusion secondary to cardiac sarcoid. Pericardial effusion is a rare manifestation of cardiac sarcoid. All cases of sustemic sarcoid should be evaluated for cardiac involvement which can be difficult to detect.

INTRODUCTION

Sarcoidosis is a multisystemic disorder characterized by the presence of noncaseating granulomas on biopsy.[1] Cardiac sarcoidosis (CS) was first described by Jonathan Hutchinson in 1869.[2] Several environmental and genetic factors have shown association with it, but the etiology remains unclear. Clinically significant CS comprises 2%–7% of systemic sarcoidosis.[34] It is the second leading cause of death in patients with sarcoidosis in the United States.[5] In Japan, where sarcoidosis is more prevalent, it is the most common cause of death in these patients.[6]

CASE PRESENTATION

We present a 65-year-old African-American woman, with a medical history of hypertension, diabetes, and morbid obesity, admitted with progressive dyspnea on exertion and intermittent substernal pain without exacerbating or alleviating factors. Review of systems was pertinent for diffuse abdominal pain, left-sided temporal headache, generalized fatigue, hyporexia, and unintentional 10-pound weight loss. Vital signs were stable. Physical examination was significant for mild epigastric and right upper quadrant abdominal tenderness and trace bilateral pitting pedal edema. Laboratory data revealed normocytic normochromic anemia (10 g/dl [12.0–15.6 g/dl]), transaminitis (alanine aminotransferase 123 U/L [13–61 U/L] and aspartate aminotransferase 115 U/L [5–40 U/L]) with elevated alkaline phosphatase (484 U/L [38–126 U/L]), and elevated angiotensin-converting enzyme (103 U/L [14–82 U/L]).

Contrast-enhanced computerized tomography (CT) of the chest, abdomen, and pelvis showed mediastinal, hilar, porta hepatis and retroperitoneal lymphadenopathy and a pericardial effusion of 12 mm thickness. Electrocardiography (ECG) showed normal sinus rhythm without any acute abnormalities. Echocardiography (echo) [Figure 1a] showed a small-to-moderate effusion mostly adjacent to the right atrium, without tamponade physiology. Contrast-enhanced magnetic resonance imaging of the brain showed four small abnormal dural-based enhancing lesions in the vertex without mass effect or adjacent edema. CT-guided core biopsy of the right retrocaval lymph node showed fibroconnective tissue with granulomatous formation and focal areas of incipient necrosis, establishing the diagnosis of sarcoidosis [Figure 2]. Prednisone therapy was initiated at a dose of 40 mg daily and subsequently was tapered down to 10 mg daily. Follow-up echo showed near-resolution of the pericardial effusion [Figure 1b]. Dural lesions responded to steroids with decrease in size on follow-up imaging.

Figure 1

(a) Left ventricular ejection fraction 55%. Wall thickness moderately increased. No regional wall motion abnormalities. Pericardium: A small-to-moderate pericardial effusion with most of the pericardial fluid noted to be adjacent to the right atrium. There is some right atrial indentation, but no right ventricular indentation or mitral valve inflow respiratory variation to suggest cardiac tamponade. (b) Left ventricular wall thickness mildly increased. Doppler parameters consistent with abnormal left ventricular relaxation (grade 1 diastolic dysfunction). Pericardium: Trivial pericardial effusion identified

Figure 2

Right retrocaval lymph node biopsy showing granulomatous formation and giant cells with minute areas of necrosis

The patient maintained close outpatient follow-up. Liver biopsy was performed for persistent transaminitis and showed evidence of sarcoidosis with scattered noncaseating granulomas and focal necrosis [Figure 3]. A year later, the patient was started on infliximab.

Figure 3

Liver biopsy showing two well-circumscribed nodules characterized by abundant histiocytes and no necrosis

DISCUSSION

CS has a wide array of manifestations, from asymptomatic involvement to sudden cardiac death. The most common symptoms based on a retrospective study of 41 cases are dyspnea, palpitations, and syncope.[7] Five major sites of cardiac involvement have been identified, namely interventricular septum, left ventricular free wall, right ventricular free wall, atria, and finally, the pericardium.[8] Pericardial disease constitutes 2%–12% of patients with CS.[9] Its spectrum comprises pericardial effusion, cardiac tamponade, acute pericarditis, relapsing pericarditis, and constrictive chronic pericarditis.[910]

Given the burden of the disease, all patients diagnosed with sarcoidosis should be screened for cardiac involvement. History, physical examination, baseline ECG, and transthoracic echo are part of initial screening.[11] Echo may show pericardial disease in up to 20% of these patients, but the impact of additional testing in an asymptomatic population is unknown.[8]

Widely accepted guidelines for diagnosis of CS are awaited. Although endomyocardial biopsy confirms diagnosis, treatment should not be delayed in those with a high index of suspicion. In addition to standard therapy and assessment for cardiac device placement, patients also require immunosuppressive therapy of which steroids are the first line. Corticosteroid-sparing agents are used if symptoms remain refractory to steroid treatment, if side effects are not tolerated, or if maintenance dose of prednisone is greater than 10 mg/day.[12]

Other therapeutic approaches depend on hemodynamic instability and symptoms. Pericardiocentesis is indicated for cardiac tamponade. Pericardial window is indicated for recurrent pericardial effusion in spite of immunosuppressive medications.

Prognosis of CS is worse than other forms of systemic sarcoidosis, even with treatment. This may be related to an increased incidence of malignant arrhythmias and sudden cardiac death.[13] Independent risk factors for mortality include New York Heart Association classification, left ventricular end-diastolic diameter, and sustained ventricular tachycardia.[14] Implementation of appropriate therapy and cardiac device placement is expected to positively impact prognosis.

Our case has one limitation. The diagnosis of CS was based on symptoms, echographic findings, and clinical course, but an endomyocardial biopsy was not performed. According to the World Association of Sarcoidosis and other Granulomatous Disorders organ assessment instrument, an organ is involved even in the absence of biopsy if two conditions are accomplished: sarcoidosis has been demonstrated histologically in other organs and alternative causes have been reasonably excluded.[15] In this case, sarcoidosis of the lung and liver was proven by biopsy. Echo resolution of pericardial effusion was achieved after initiating therapy, reinforcing established diagnosis.

CONCLUSION

Screening for CS should be pursued in patients with sarcoidosis as its presence impacts prognosis. Manifestations vary depending on the location and extent of disease. The pericardium is the least frequently involved site in CS. Since the advent of sophisticated cardiac imaging, endomyocardial biopsy may not be essential. Treatment is often multidisciplinary and consists of immunosuppressive therapy and invasive procedures, such as pericardiocentesis or pericardial window, based on overall clinical assessment.

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Conflicts of interest

There are no conflicts of interest.