Multifocal cardiac infiltration with predominant mitral valve involvement in granulomatosis with polyangiitis

Q: What is the clinical evidence from this study?

Study design: Case Report. Population: Granulomatosis with polyangiitis with cardiac infiltration (n=1). Intervention: Rituximab. Primary outcome: Clinical and imaging response.

Q: What does this research mean for the field?

Rituximab therapy can lead to sustained multi-year asymptomatic survival with stable cardiac imaging and preserved valvular function in ANCA-negative granulomatosis with polyangiitis presenting with extensive cardiac infiltration. Novelty: ClaimNovelty.INCREMENTAL. Consensus alignment: ConsensusAlignment.NEUTRAL.

doi:10.33963/v.phj.114156

August 10, 2026Kardiologia PolskaOpen Access

Multifocal cardiac infiltration with predominant mitral valve involvement in granulomatosis with polyangiitis

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Key Points

To describe an uncommon presentation of extensive multifocal cardiac and mitral valve infiltration in an ANCA-negative patient with granulomatosis with polyangiitis.
Evaluated a 69-year-old ANCA-negative patient diagnosed with granulomatosis with polyangiitis based on histopathological evidence of vascular necrosis and progressive multiorgan involvement.
Performed multimodal cardiac assessments using positron emission tomography, transthoracic echocardiography, and cardiac magnetic resonance imaging during immunosuppressive therapy over 3 years.
Imaging identified extensive infiltration across both mitral leaflets with mild regurgitation, extending into the aortic valve, annuli, atrio-ventricular septum, and descending aorta, while left ventricular systolic function remained normal.
Troponin and natriuretic peptide levels remained within normal limits throughout clinical presentation.
Treatment with rituximab achieved long-term disease control, with the patient remaining asymptomatic and exhibiting unchanged, stable valvular masses on cardiac magnetic resonance and echocardiography at 3-year follow-up.

Study Design

Type

Case Report (n=1)

Structured PICO

P

Population

A 69-year-old patient with ANCA-negative granulomatosis with polyangiitis presenting with extensive multifocal cardiac infiltration predominantly involving the mitral valve, treated with rituximab and followed for 3 years.

I

Intervention

Immunosuppressive therapy (initial steroids and methotrexate, followed by mycophenolate mofetil, then cyclophosphamide, and finally rituximab).

O

Outcome

Clinical symptom resolution and imaging response (echocardiography and cardiac magnetic resonance) of cardiac infiltrates.

This case highlights a rare presentation of ANCA-negative granulomatosis with polyangiitis involving extensive multifocal cardiac and mitral valve infiltration, which was successfully stabilized with rituximab therapy.

Limitations

Single case report
Lack of cardiac biopsy due to preserved valve function and lack of disease activity

Implication

Cardiac GPA may feature rare mitral infiltration; case reports leave open questions on prevalence and management.

Abstract

Granulomatosis with polyangiitis (GPA) is a rare form of vasculitis characterized by the inflammation of small-and medium-sized vessels and formation of inflammatory tissue masses (granulomas).It most commonly affects upper respiratory tract, lungs, and kidneys, but can involve any organ.Heart involvement affects 6%-12% patients [1].

Discussion

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Cite This Study

A 2026 study conducted a case report in Granulomatosis with polyangiitis with cardiac infiltration (n=1). Rituximab was evaluated on Clinical and imaging response.

synapsesocial.com/papers/6a7d75ad2b0e0cff3f63e85e https://doi.org/10.33963/v.phj.114156

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Multifocal cardiac infiltration with predominant mitral valve involvement in granulomatosis with polyangiitis

Key Points

Study Design

Structured PICO

Limitations

Implication

Abstract

Discussion

Cite This Study

Multifocal cardiac infiltration with predominant mitral valve involvement in granulomatosis with polyangiitis

Key Points

Study Design

Structured PICO

Limitations

Implication

Abstract

Discussion

Cite This Study

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