Pericarditis in ANCA-Positive GPA

PericarditisECGVasculitis
Written By Sohaib Abdulqader

Clinical Context A 46-year-old male with a history of ANCA-positive Granulomatosis with Polyangiitis (GPA) presented with acute pleuritic chest pain


Diffuse PR depression. No ST depression except in aVR due to PR elevation.

ECG Findings

  • Global ST-segment elevation, consistent with acute pericarditis.

Bedside Imaging

  • Echocardiography revealed a small rim of pericardial effusion (<0.5 cm)

Laboratory Results

  • CRP: Elevated at 51 mg/L.

  • PR3-ANCA: Significantly elevated at 478.7 U/mL.

  • Urinalysis: Protein ++, blood +++.

Clinical Diagnosis

  • Pericarditis associated with GPA relapse, as evidenced by:

    • Rising PR3-ANCA levels.

    • Symptoms of chest pain and pericardial effusion.

    • Microscopic Hematuria


Pericarditis is a rare but recognized manifestation of ANCA-associated vasculitis, particularly GPA. It often presenting alongside pulmonary or renal involvement.


Management

  • NSAIDs and colchicine for symptom relief.

  • High-dose glucocorticoids and immunosuppressive agents (e.g., rituximab) are the mainstays of treatment.

Follow-up

2 years later patient presented with right-sided chest pain radiating to the back for 3 months, accompanied by a persistent cough, significant weight loss (14 kg), and fevers lasting 3 weeks


Aortitis

Significant soft tissue cuffing of a short segment of aortic arch surrounding an area of aortic wall calcification in keeping with aortitis

Discussion

The Birmingham Vasculitis Activity Score for Wegener granulomatosis (BVAS/WG) instrument can be used to quantify disease activity at the time of pericarditis presentation.

Disease manifestations that would qualify a patient as having severe disease include scleritis, nervous system involvement, alveolar hemorrhage, GI involvement, heart involvement, and glomerulonephritis.

Sohaib Abdulqader