Case Presentation Corner
Cardiac imaging of a patient with unusual presentation of granulomatosis with polyangiitis: A case report and review of the literature

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Abstract

Background

Recent evidence suggests that cardiac involvement in patients with granulomatosis with polyangiitis (GPA) occurs more frequently than previously reported. Multimodality cardiac imaging is gaining attention in the diagnosis, prognostication, and follow-up of such patients; however, the data remain scarce.

Results

2D-TTE was useful for initial screening; while both cardiac magnetic resonance imaging and 18F-fluoro-2-deoxyglucose positron emission tomography/computed tomography (18FDG-PET/CT) metabolic imaging with rubidium-82 PET perfusion imaging were useful for characterization of myocardial disease. 18FDG-PET/CT was very useful for the follow-up of cardiac disease activity following treatment.

Conclusion

18FDG-PET/CT is sensitive for the detection of cardiac involvement by GPA and is useful for the tissue characterization and follow-up of disease activity following treatment.

Section snippets

Case Report

A 21-year-old female, was referred to our cardiology clinic complaining of dyspnea on mild effort, atypical chest pain, and brief intermittent palpitations without hemodynamic compromise. She had been previously diagnosed with mild hyperactive airway disease for which she was not prescribed any treatment. Systemic enquiry revealed occasional epistaxis, stuffy nose, and frontal headaches. On examination, the pulse rate was 103 beats/minute and the blood pressure was 91/67 mmHg. Chest and

Granulomatosis with Polyangiitis (GPA)

GPA (formerly known as Wegner’s granulomatosis) is a multisystemic disorder characterized by ANCA-associated small-vessel necrotizing vasculitis with granulomatous infiltrates.5 The disease is uncommon, with a reported incidence of 5-10 cases per million population.6 It affects males and females equally and may occur at any age.5 GPA primarily affects the respiratory system, but involvement of other organs, including the heart, is well known.7,8 Based on the disease extent, GPA can be

New Knowledge Gained

18FDG-PET/CT metabolic imaging with rubidium-82 PET perfusion imaging is useful for the detection and characterization of myocardial disease in patient with GPA and for follow-up of disease activity after treatment.

Conclusion

Cardiac involvement by GPA is common, although subclinical in most patients, and produces a wide range of imaging findings. However, the literature concerning this topic remains limited to case reports and a few case series. The indications for and roles of different cardiac imaging modalities in screening and follow-up of patients with GPA have not been well delineated and are largely individualized. Given the high mortality associated with cardiac involvement in GPA, early detection of

Acknowledgements

This work was funded by the Cardiovascular Disease in Women Research Chair at the Princess Nourah bint Abdulrahman University (Grant No. CDW-2019-02).

Disclosure

Rabah Al-Mehisen, Khalid Alnemri, and Maha Al-Mohaissen have no conflicts of interest to declare.

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