Cardiac Involvement In Granulomatosis With Polyangiitis

Lucy McGeoch¹, Simon Carette², David Cuthberston³, Gary S. Hoffman⁴, Nader A. Khalidi⁵, Curry L Koening⁶, Carol A. Langford⁷, Paul A. Monach⁸, Larry W. Moreland⁹, Philip Seo¹⁰, Ulrich Specks¹¹, Steven R. Ytterberg¹², Carol McAlear¹³, Peter A Merkel¹⁴, Christian Pagnoux² and The Vcrc¹⁵, ¹Vasculitis clinic, Division of rheumatology, Mount Sinai Hospital, University Health Network, University of Toronto, Toronto, ON, Canada, ²Division of Rheumatology, University of Toronto, Toronto, ON, Canada, ³Biostatistics, University of South Florida, Tampa, FL, ⁴Center for Vasculitis Care and Research, Cleveland Clinic Foundation, Cleveland, OH, ⁵Internal Medicine/Rheumatology, McMaster University, Hamilton, ON, Canada, ⁶Division of rheumatology, George E. Wahlen Department of Veterans Affairs Medical Center Salt Lake City and University of Utah, University of Utah School of Medicine, Salt Lake City, UT, ⁷Center for Vasculitis Care and Research, Cleveland Clinic, Cleveland, OH, ⁸Rheumatology, Boston University, Boston, MA, ⁹Division of Rheumatology & Clinical Immunology, University of Pittsburgh, Pittsburgh, PA, ¹⁰Rheumatology Division, Johns Hopkins Vasculitis Center, Johns Hopkins University, Baltimore, MD, ¹¹Mayo Clinic, Rochester, MN, ¹²Rheumatology Division, Mayo Clinic, Rochester, MN, ¹³University of Pennsylvania, Philadelphia, PA, ¹⁴Division of Rheumatology, University of Pennsylvania and VA Medical Center, Philadelphia, PA, ¹⁵University of Pennsylvania and VA Medical Center, Philadelphia, PA

Meeting: 2013 ACR/ARHP Annual Meeting

Keywords: Heart disease, longitudinal studies, prognostic factors and vasculitis, Wegener's granulomatosis

Session Information

Title: Vasculitis I

Session Type: Abstract Submissions (ACR)

Background/Purpose: Prior cohort studies in Europe have found cardiac involvement to be rare in granulomatosis with polyangiitis (GPA) but associated with significant increases in mortality and risk of relapse. The aim of this study was to determine the frequency and associated clinical outcomes of cardiac disease in a large multicenter North American GPA cohort.

Methods: The data source was the Vasculitis Clinical Research Consortium (VCRC) Longitudinal Study of GPA which includes 517 patients satisfying the modified ACR criteria for GPA recruited into the cohort between 2006 and 2013. Information on cardiac disease was reported by expert investigators using a standardized protocol. The demographic and clinical characteristics of patients with GPA-related cardiac involvement at diagnosis or during follow-up were compared to those patients without cardiac disease.

Results: Seventeen (3.3%) patients with cardiac involvement were identified. Cardiac manifestations were observed both at diagnosis (n=9) or during subsequent disease course and/or relapse (n=8). Observed cardiac manifestations included pericarditis (n=8), cardiomyopathy (n=5, including 1 with pericarditis), coronary artery disease (n=4), and valvular disease (n=2, including 1 with CAD). There were no overt differences between patients with GPA with cardiac involvement and those without cardiac disease: 7/17 (41%) patients with cardiac disease were female (vs. 52% in those without cardiac disease; p= 0.36); mean age at diagnosis of patients with cardiac disease was 45.2 ± 19.9 yrs. (vs. 44.8 ± 16.5 yrs. for those without cardiac disease; p= 0.92); and 16/17 (94% [PR3 65%]) with cardiac disease were tested ANCA positive (vs. 81% [PR3 68%] in those without cardiac disease; p=0.33). Frequency of non-cardiac disease manifestations were comparable between the two groups and no significant differences were observed in the immunosuppressant agents. Lag time between symptom onset and diagnosis in the cardiac GPA group was comparable to the non-cardiac GPA group (18 ± 27 mo. vs. 14 ± 36 mo. respectively; p= 0.56).

Mean follow-up from diagnosis was 104 ± 68 mo. for cardiac GPA and 105 ± 71 mo. for non-cardiac GPA (p = 0.92). Rate of relapse was comparable between the 2 groups (53% of cardiac GPA patients experienced ≥1 GPA relapse vs. 66% of the non-cardiac GPA group; p= 0.25). At present, none of the patients with cardiac disease in the cohort have died.

Conclusion: This case series confirms that cardiac involvement in GPA is rare, heterogeneous, and can manifest at disease onset as well as during subsequent relapses. In contrast with previous reports, cardiac involvement was not associated in this population with a higher rate of relapse.

Disclosure:

L. McGeoch,
None;

S. Carette,
None;

D. Cuthberston,
None;

G. S. Hoffman,
None;

N. A. Khalidi,
None;

C. L. Koening,
None;

C. A. Langford,

Bristol-Myers Squibb,

Genentech and Biogen IDEC Inc.,

P. A. Monach,

Genentech and Biogen IDEC Inc.,

L. W. Moreland,
None;

P. Seo,
None;

U. Specks,
None;

S. R. Ytterberg,

Questcor,

Dynavax Technologies,

C. McAlear,
None;

P. A. Merkel,
None;

C. Pagnoux,
None;

T. Vcrc,
None.

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