Discussion:
To come up with a final diagnosis of our patient, we summarized our patient’s disease course as followed;
A 36-year-old male patient was admitted to our emergency department with pleuritic chest pain, dyspnea, intermittent fever, and a CT scan carried out few days before, demonstrating severe pericardial effusion. The first suspected differential diagnosis was acute pericarditis. According to the 2015 European society of cardiology (ESC) guidelines for diagnosis and management of pericardial diseases, our patient’s high fever (i.e., 39.5°C) and large pericardial effusion are considered major predicting factors of a poor prognosis [2]. Therefore, patient admission and search for the pericarditis etiology were mandatory [2]. Our patient was admitted and administration of NSAIDs was started.
With clinical signs and symptoms (i.e., characteristic chest pain, and pericardial rub) suggestive for pericarditis, the diagnosis was confirmed by the ECG (i.e., generalized low voltages), TTE (i.e., fibrinous circumferential pericardial effusion, and thickened pericardium), and laboratory findings (i.e., elevated CRP, and ESR). In doppler investigations, constrictive patterns of pericarditis were observed. Since our patient’s disease manifestations had started 4 weeks earlier, the diagnosis of acute fibrinous pericarditis was confirmed after the completion of the mentioned investigations. Even though our patient could not be categorized as a case of cardiac tamponade, hemodynamic alteration was present [2]. These hemodynamic changes are mainly because of pericardial thickness and the huge amount of fibrin strands in the pericardial effusion [8]. We monitored him closely, for the sake of any further hemodynamic alterations, started our search for the etiology, and decided to postpone the pericardiocentesis till the results of our primary investigations were ready [8].
The diagnosis of constrictive pericarditis was confirmed by TTE investigations which in a sum up demonstrated the following: 1) thickened and hyperechoic pericardial layers; 2) massive circumferential pericardial effusion; 3) ventricular septal motion toward left ventricle during inspiration (septal bounce) detected in M-mode; 4) dilated IVC without respiratory collapse; 5) more than 50% respiratory variation of the mitral peak E velocity (with more than 25% variation suggestive for constrictive pattern); 6) more than 50% respiratory variation of the tricuspid peak E velocity (with more than 40% variation suggestive for constrictive pattern); 7) reverse pattern for early diastolic peak velocity (e’) of lateral and septal mitral annulus known as annulus reversus (i.e., e’ lateral > e’ septal).
There are specific forms of constrictive pericarditis described in the literature making it difficult to classify our patient. Given the signs of elevated JVP, severe pericardial effusion, and constrictive features in an acute course which completely resolved after 6 weeks of receiving anti-inflammatory medications, we assumed transient constrictive and effusive-constrictive forms to be the major differential diagnoses [2, 9]. The transient form was ruled out with regards to our patient’s large pericardial effusion containing fibrin strands. Effusive-constrictive pericarditis is defined as constrictive pericarditis with tamponade presentation. Even though our patient had elevated JVP, his hemodynamic status was not consistent with a tamponade presentation. Therefore, by not fitting into any of these two specific forms, we prefer to present our case as a merely constrictive pericarditis. The most important and tricky differential diagnosis of constrictive pericarditis is restrictive cardiomyopathy [10]. This was ruled out for our patient by the TTE findings (e.g., presence of respiratory variations of mitral valve inflow) [9].
According to the 2015 ESC guidelines for diagnosis and management of pericardial diseases, there are two main categories for the etiology of pericardial syndromes: infectious and non-infectious. In the infectious category, viral infections and TB are the most common causes. We ruled out TB infection by tuberculin skin test, and viral causes were not probable as there was no history of any symptoms that triggered our clinical suspicion. For non-infectious causes, autoimmune diseases and malignancies secondary to metastasis are the main focus. We searched for any abnormal presentations or lesions in the chest CT scan and abdominal sonography consistent with laboratory findings for a neoplastic diagnosis, but none was found. Therefore, we continued our etiology investigation by executing autoimmune-specific laboratory tests [2].
After one week of treatment with NSAIDs, neither his signs and symptoms, nor his TTE results showed any significant improvements. Thereby, corticosteroids were added to his treatment regimen. At the same time, autoimmune-specific investigations for the etiology of the pericarditis, revealed high ANA and RF titers, and elevated anti-ds DNA antibody levels.
Anti-ds DNA antibody is an SLE-specific autoantibody. Thus, we calculated our patient’s score for SLE involvement based on the 2019 EULAR/ACR classification criteria for systemic lupus erythematosus [7]. ANA positive with a 1/80 titer, fever as a constitutional symptom (score weight: 2), acute pericarditis (score weight: 6), and positive anti-ds DNA antibody (score weight: 6), collectively made up a total of 12 score which made our SLE diagnosis definite.
Here after, we managed our patient with the consult of rheumatologists with the final diagnosis of large circumferential fibrinous constrictive pericarditis as the first presentation of SLE. NSAIDs were replaced by hydroxychloroquine for our patient. Our patient started to show progressive improvement from the second week of treatment and was completely symptom-free after 5 weeks of treatment with hydroxychloroquine and corticosteroids, with no need of pericardiocentesis.
The corticosteroid protocol used for our patients with the recommended starting dose was then adjusted and tapered based on our patient’s symptomatic and laboratory improvements as followed: 1) 30 mg prednisolone daily for 2 weeks; 2) 25 mg prednisolone for 4 weeks; 3) tapering and dose modifications with rheumatologist’s order reached to a 10 mg daily dose of prednisolone [2].
There are several remarkable aspects regarding this case. First of all, the diagnosis of constrictive pericarditis along with large pericardial effusion can be very tricky especially in acute phases. This is because constrictive features of acute pericarditis may not be represented in CT scans or magnetic resonance imaging (MRI). Therefore, it is crucial to execute a complete doppler echocardiography study for the patient.
The main concern respecting constrictive pericarditis is the missed diagnosis of its underlying cause. This could lead to a recurrent and/or chronic course of the disease to the point that the patient presents signs and symptoms indicating pericardiectomy, or more precisely epicardiectomy. Epicardium is the inner layer of pericardia, and responsible for constrictive features of constrictive pericarditis. Therefore, it is technically more troublesome to resect with a reported 6-12% mortality rate of the procedure [2]. This can go further with the involvement of other heart layers. In order to prevent these adverse consequences, a thorough and precise search for the etiology is essential so that the optimal treatment is provided [11]; as with our case which starting hydroxychloroquine resulted in remarkable resolution of our patient’s manifestations.
It is of great value to consider a full investigation for the etiology of constrictive pericarditis, as if the underlying cause is not treated promptly it can lead to a chronic course with probable eventual need for epicardiectomy and a more complicated disease course [11].