Cholesterol Pericarditis

Abstract

Background

Cholesterol pericarditis is a rare condition characterized by cholesterol crystal accumulation in the pericardium, often linked to hyperlipidemia and chronic inflammatory disorders.

Case Summary

A 50-year-old woman with a history of hypercholesterolemia presented with progressive dyspnea, chest pain, and fatigue. Clinical evaluation and imaging studies revealed a massive pericardial effusion. Pericardiocentesis was performed, and fluid analysis demonstrated the presence of cholesterol crystals. The patient was treated with pericardiocentesis, nonsteroidal anti-inflammatory drugs, and lipid-lowering therapy, leading to rapid symptom resolution.

Discussion

Cholesterol pericarditis remains an underrecognized cause of pericardial effusion. A high index of suspicion is required in patients presenting with unexplained pericardial effusion. Diagnosis is primarily based on pericardial fluid analysis. Management includes symptomatic relief with pericardiocentesis, anti-inflammatory therapy, and long-term lipid control to prevent recurrence.

Take-Home Messages

Cholesterol pericarditis should be considered in patients with unexplained pericardial effusion and dyslipidemia. Early diagnosis and treatment can lead to favorable outcomes.

Graphical Abstract

Pericarditis, or inflammation of the pericardium, can have diverse etiologies, including infectious, autoimmune, neoplastic, and metabolic causes.¹ Cholesterol pericarditis, or xanthogranulomatous pericarditis, is a rare form of pericardial disease characterized by the deposition of cholesterol crystals within the pericardium. This condition was first described more than a century ago and remains poorly understood owing to its rarity. The pathophysiology involves chronic inflammation and lipid metabolism abnormalities, leading to crystal deposition and recurrent effusion.^1,2 This report presents a case of cholesterol pericarditis, illustrating its clinical presentation, diagnostic evaluation, and management and emphasizing the importance of early recognition and treatment.

Take-Home Messages

• •Cholesterol pericarditis is a rare but important differential diagnosis for unexplained pericardial effusion.
• •Early recognition and treatment with pericardiocentesis and lipid-lowering therapy improve outcomes.
• •A high index of suspicion is required, especially in patients with dyslipidemia.

History of Presentation

Initial presentation

A 50-year-old woman with no known chronic illnesses presented to the emergency department with progressive dyspnea on exertion (NYHA functional class II to III over 3 months) and fatigue. She also reported chest discomfort but denied fever, weight loss, or other systemic symptoms. She had been treated empirically with antitubercular therapy for suspected tubercular pericarditis, which failed to alleviate her symptoms.

Examination

On physical examination, the patient appeared uncomfortable but not in acute distress. Raised jugular venous pressure and distant heart sounds were noted, and the lung examination was unremarkable. Vital signs were stable.

Past Medical History

The patient had a history of hypercholesterolemia, but no known cardiovascular disease, autoimmune disorders, or infectious conditions. She had not experienced prior episodes of pericarditis or other significant medical events. There was no history of diabetes, hypertension, or chronic inflammatory conditions. There had been a short course of antitubercular treatment without improvement.

Differential Diagnosis

The differential diagnosis included tuberculous pericarditis, given its prevalence in endemic regions and its known association with chronic pericardial effusions. Malignant pericardial effusion was considered owing to the large fluid accumulation, but the absence of malignant cells in the fluid analysis ruled it out. Autoimmune pericarditis was another possibility, as systemic inflammatory diseases can manifest with pericardial involvement, though the patient's rheumatologic markers were unremarkable. Lastly, idiopathic pericarditis was a potential consideration, but the presence of cholesterol crystals in the pericardial fluid confirmed the diagnosis of cholesterol pericarditis.

Investigations

Diagnostic work-up

Electrocardiogram showed low-voltage QRS complexes, but electrical alternans was not very impressive (Figure 1A). Chest x-ray showed massive pericardial effusion with an enlarged cardiac silhouette (Figure 1C). Echocardiography confirmed large pericardial effusion with signs of inflammation but no tamponade.

Figure 1.

Electrocardiogram and Chest X-Ray Before and After Pericardiocentesis

(A) Electrocardiogram before pericardiocentesis demonstrates low-voltage QRS complexes and electrical alternans, indicating a significant pericardial effusion. (B) Electrocardiogram after pericardiocentesis shows normalization of QRS voltage and resolution of electrical alternans following drainage of the pericardial effusion. (C) Chest x-ray before pericardiocentesis reveals a massive pericardial effusion causing an enlarged cardiac silhouette and blunted costophrenic angles. (D) Chest x-ray after pericardiocentesis shows significant reduction in cardiac silhouette size and improvement in lung fields following pericardial fluid drainage.

Pericardiocentesis was performed, draining 1.5 L of golden-yellow fluid, which was sent for analysis. Microscopic examination revealed multiple cholesterol crystals, confirming the diagnosis of cholesterol pericarditis. Laboratory investigations ruled out infection, malignancy, and autoimmune conditions.

Laboratory findings

Pericardial fluid analysis showed the following (Table 1):

• •Color: Golden Yellow
• •Protein: 4.2 g/dL
• •Cholesterol: 112 mg/dL
• •Differential cell count: Lymphocytes 80%, PMNs 20%
• •Gram and Ziehl-Neelsen stains: Negative

Table 1.

Pericardial Fluid Analysis

Color	Golden yellow
Appearance	Slightly turbid
Protein	4.2 g/dL
Albumin	3.0 g/dL
Sugar	127 g/dL
Chloride	590 mg/dL
Specific gravity	1,018
Total cell count	105/μL
Differential cell count	PMNs 20%; Lymphocytes 80%
ADA	9.8
Gram stain	Negative
ZN stain	Negative
Misc.	Multiple cholesterol crystals
Cholesterol	112 mg/dL (range <70 mg/dL)

ADA = adenosine deaminase; Misc. = miscellaneous; PMNs = polymorphonuclear leukocytes; ZN = Ziehl-Neelsen.

Serum investigations showed the following (Table 2):

• •Elevated C-reactive protein: 32 mg/L
• •Lipid profile: total cholesterol 240 mg/dL, low-density lipoprotein 210 mg/dL, high-density lipoprotein 30 mg/dL, triglycerides 240 mg/dL
• •Rheumatologic and thyroid profiles: within normal limits

Table 2.

Laboratory Investigations

Test	Value (Normal Range)
Rheumatoid factor	10.4 (<14)
Anti-CCP	1.6 (<5)
CRP	37.6 (0-5)
ANA	Not detected
Anti-dsDNA	5.10 (<20)
IGRA MTB	Negative
TSH	3.4
HbA1c	6.9%
Lipid profile
TAG	240 mg/dL
Cholesterol	240 mg/dL
LDL	210 mg/dL
HDL	30 mg/dL

ANA = antinuclear antibody; Anti-CCP = anti–cyclic citrullinated peptide; Anti-dsDNA = anti–double-stranded DNA; CRP = C-reactive protein; HbA1c = hemoglobin A1c; HDL = high-density lipoprotein; IGRA MTB = interferon- gamma release assay for Mycobacterium tuberculosis; LDL = low-density lipoprotein; TAG = triacylglycerol; TSH = thyroid-stimulating hormone.

Image findings

Figures 1A to 1D illustrate electrocardiogram, chest x-ray, and microscopic findings before and after pericardiocentesis, showing normalization of QRS complexes and reduction in cardiac silhouette size following fluid drainage.

Management

The patient underwent pericardiocentesis, which successfully drained 1.5 L of cholesterol-rich pericardial fluid, leading to significant symptomatic relief. Medical management included a combination of nonsteroidal anti-inflammatory drugs and colchicine to reduce inflammation and high-dose statins to address underlying dyslipidemia. Symptoms improved significantly during hospitalization, and the patient was discharged in stable condition.

Outcome and Follow-Up

The patient was closely monitored for complications, and serial follow-up assessments at 1 and 3 months showed no recurrence of pericardial effusion. The patient was counseled on long-term lipid control as a preventive measure; however, she was lost to follow-up thereafter.

Discussion

Cholesterol pericarditis is an exceedingly rare condition, with only a handful of reported cases. It is often associated with dyslipidemia and other metabolic or inflammatory disorders. The clinical presentation mimics other forms of pericarditis, necessitating a high index of suspicion. Diagnosis hinges on the identification of cholesterol crystals in pericardial fluid, alongside the exclusion of common causes such as tuberculosis, autoimmune diseases, and malignancy.³

The pathogenesis of cholesterol pericarditis involves the deposition of cholesterol crystals within the pericardium, leading to an inflammatory response and subsequent pericardial thickening.^4,5 The clinical presentation is often similar to other forms of pericarditis, with chest pain, dyspnea, and friction rub being common features. Diagnosing cholesterol pericarditis can be challenging, as it requires a high index of suspicion and confirmation through pericardial fluid analysis. Cholesterol pericarditis can be mistaken for other conditions, as seen in this case in which tuberculosis was initially suspected. Comprehensive fluid analysis is pivotal for accurate diagnosis. Microscopic examination of pericardial fluid reveals cholesterol clefts and multinucleated giant cells, characteristic of xanthogranulomatous inflammation.¹ Echocardiography is crucial in detecting pericardial effusion and assessing the hemodynamic impact.

A multimodal approach is essential for effective management, combining several therapeutic strategies. Anti-inflammatory therapy, including nonsteroidal anti-inflammatory drugs and colchicine, is pivotal in relieving symptoms and controlling inflammation. Lipid-lowering agents, particularly statins, are crucial for addressing dyslipidemia and reducing the risk of recurrence. In cases of symptomatic effusion, pericardiocentesis may be necessary to provide immediate relief. Early intervention is vital to prevent potential complications, such as constrictive pericarditis.

Cholesterol pericarditis is a rare pericardial disease manifestation requiring prompt recognition and appropriate management. Further studies are needed to understand the long-term outcomes and optimal management strategies for cholesterol pericarditis, including the role of aggressive lipid control and recurrence monitoring. This case highlights the importance of recognizing cholesterol pericarditis as a potential cause of pericardial effusion in patients with dyslipidemia. Prompt diagnosis through fluid analysis and a tailored treatment approach involving anti-inflammatory and lipid-lowering therapies can lead to successful management and prevention of complications.

Conclusions

Cholesterol pericarditis should be considered in patients with unexplained pericardial effusion, particularly individuals with dyslipidemia. Timely diagnosis through fluid analysis and a combination of anti-inflammatory and lipid-lowering therapy can result in successful management and prevent recurrence.

Visual Summary.

Cholesterol Pericarditis: Diagnostic and Therapeutic Snapshot

ECG = electrocardiogram; NSAID = nonsteroidal anti-inflammatory drug.

Funding Support and Author Disclosure

The authors have reported that they have no relationships relevant to the contents of this paper to disclose.