What is the frequency of pericarditis in patients with Granulomatosis with Polyangiitis (GPA), formerly known as Wegener's granulomatosis?

Frequency of Pericarditis in Granulomatosis with Polyangiitis (GPA)

Pericarditis occurs in approximately 6-44% of patients with Granulomatosis with Polyangiitis (GPA, formerly Wegener's granulomatosis), making it one of the most common cardiac manifestations of this disease. 1, 2, 3

Epidemiology and Clinical Context

The reported frequency of cardiac involvement in GPA varies considerably across studies, with pericarditis being the predominant cardiac manifestation:

• Cardiac involvement overall affects 6-44% of GPA patients, with pericarditis representing the most frequent cardiac complication alongside coronary arteritis 1, 2, 3
• In one large historical series, cardiac involvement was documented in 12% of patients, primarily manifesting as pericarditis and coronary arteritis 1
• Echocardiographic abnormalities have been detected in up to 80% of GPA patients, suggesting that subclinical cardiac involvement may be substantially more common than clinically apparent disease 2

Clinical Presentations of Pericarditis in GPA

The pericardial involvement in GPA can manifest in several distinct patterns:

Acute Pericarditis

• Acute pericarditis may occur as part of active systemic vasculitis, directly related to the inflammatory process affecting the pericardium 1, 2
• Hemorrhagic pericarditis can develop, with the underlying inflammatory vasculitis playing the primary pathogenic role rather than uremia 2

Severe Complications

• Pericardial tamponade requiring pericardiocentesis has been reported, representing a life-threatening complication that demands urgent intervention 1, 2
• Constrictive pericarditis can develop, sometimes requiring surgical pericardiectomy even despite vigorous immunosuppression 1, 3
• Constrictive pericarditis may occur in 6-44% of cases with cardiac involvement, though distinguishing between uremic and vasculitic etiologies can be challenging in patients with concurrent renal failure 3

Recurrent Pericarditis

• Recurrent pericarditis can be an initial or presenting manifestation of GPA, sometimes preceding other classic features of the disease by years 4
• One case series documented a patient with 5 years of recurrent myopericarditis episodes before GPA diagnosis was established 4

Associated Cardiac Manifestations

Beyond pericarditis, clinicians should be aware of other cardiac complications:

• Coronary arteritis frequently accompanies pericarditis in GPA, and can manifest as rhythm disturbances including high-grade atrioventricular block and atrial tachycardia resistant to conventional therapy 1
• Myocardial involvement may occur concurrently, complicating the clinical presentation and requiring careful evaluation 5

Diagnostic Considerations

A critical pitfall is distinguishing pericarditis due to active GPA vasculitis from uremic pericarditis in patients with renal failure, as both conditions can coexist and the distinction impacts treatment decisions 1, 3

• Screening echocardiograms should be considered in all GPA patients given the high frequency of subclinical cardiac abnormalities and the potential for life-threatening complications like tamponade 2
• Regular cardiac monitoring with electrocardiography and echocardiography is recommended to detect both pericardial and myocardial involvement early 5, 6

Clinical Implications

The presence of pericarditis in GPA typically indicates active systemic disease requiring intensification of immunosuppressive therapy, as these patients often respond well to cyclophosphamide or rituximab-based regimens 1, 7

• Pericarditis in the context of systemic autoimmune vasculitides like GPA generally reflects the degree of activity of the underlying disease and should prompt comprehensive disease activity assessment 5
• Treatment should target control of the underlying systemic vasculitis rather than focusing solely on symptomatic pericardial management 5