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].