New features of disease after diagnosis in 6 forms of systemic vasculitis

Peter C. Grayson, David Cuthbertson, Simon Carette, Gary S. Hoffman, Nader A. Khalidi, Curry L. Koening, Carol A. Langford, Kathleen Maksimowicz-McKinnon, Paul A. Monach, Philip Seo, Ulrich Specks, Steven R Ytterberg, Peter A. Merkel

Research output: Contribution to journalArticle

24 Citations (Scopus)

Abstract

Objective. To quantify the occurrence of features of vasculitis that initially present after diagnosis in 6 types of primary vasculitis. Methods. Standardized collection of data on 95 disease manifestations in 6 vasculitides, including granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), eosinophilic granulomatosis with polyangiitis (Churg-Strauss; EGPA), polyarteritis nodosa (PAN), giant cell arteritis (GCA), and Takayasu arteritis (TAK), was obtained within a set of multicenter longitudinal, observational cohorts. For each form of vasculitis, the frequency of disease-specific manifestations at diagnosis was compared to the cumulative frequency of each manifestation. The percentage of patients who initially developed severe manifestations after diagnosis, defined as organ- or life-threatening in the small and medium vessel vasculitides (GPA, MPA, EGPA, PAN) and as ischemic/vascular in the large vessel vasculitides (GCA, TAK), was reported. Results. Out of 838 patients with vasculitis, 490 (59%) experienced ≥ 1 new disease manifestation after diagnosis. On average, patients with vasculitis experienced 1.3 new manifestations after diagnosis (GPA = 1.9, MPA = 1.2, EGPA = 1.5, PAN = 1.2, GCA = 0.7, and TAK = 1.0). New severe manifestations occurred after diagnosis in 224 (27%) out of 838 patients (GPA = 26%, MPA = 19%, EGPA = 21%, PAN = 23%, GCA = 24%, and TAK = 44%). Timing of onset of new manifestations was not significantly associated with disease duration. Conclusion. A majority of patients with vasculitis develop new disease features after diagnosis, including a substantial number of new, severe manifestations. Ongoing assessment of patients with established vasculitis should remain broad in scope. The Journal of Rheumatology

Original languageEnglish (US)
Pages (from-to)1905-1912
Number of pages8
JournalJournal of Rheumatology
Volume40
Issue number11
DOIs
StatePublished - Nov 2013

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Systemic Vasculitis
Vasculitis
Granulomatosis with Polyangiitis
Microscopic Polyangiitis
Takayasu Arteritis
Polyarteritis Nodosa
Giant Cell Arteritis
Rheumatology
Blood Vessels

Keywords

  • EOSINOPHILIC GRANULOMATOSIS WITH POLYANGIITIS
  • GIANT CELL ARTERITIS
  • GRANULOMATOSIS WITH POLYANGIITIS
  • MICROSCOPIC POLYANGIITIS
  • POLYARTERITIS NODOSA
  • TAKAYASU ARTERITIS
  • VASCULITIS

ASJC Scopus subject areas

  • Rheumatology
  • Immunology
  • Immunology and Allergy

Cite this

Grayson, P. C., Cuthbertson, D., Carette, S., Hoffman, G. S., Khalidi, N. A., Koening, C. L., ... Merkel, P. A. (2013). New features of disease after diagnosis in 6 forms of systemic vasculitis. Journal of Rheumatology, 40(11), 1905-1912. https://doi.org/10.3899/jrheum.121473

New features of disease after diagnosis in 6 forms of systemic vasculitis. / Grayson, Peter C.; Cuthbertson, David; Carette, Simon; Hoffman, Gary S.; Khalidi, Nader A.; Koening, Curry L.; Langford, Carol A.; Maksimowicz-McKinnon, Kathleen; Monach, Paul A.; Seo, Philip; Specks, Ulrich; Ytterberg, Steven R; Merkel, Peter A.

In: Journal of Rheumatology, Vol. 40, No. 11, 11.2013, p. 1905-1912.

Research output: Contribution to journalArticle

Grayson, PC, Cuthbertson, D, Carette, S, Hoffman, GS, Khalidi, NA, Koening, CL, Langford, CA, Maksimowicz-McKinnon, K, Monach, PA, Seo, P, Specks, U, Ytterberg, SR & Merkel, PA 2013, 'New features of disease after diagnosis in 6 forms of systemic vasculitis', Journal of Rheumatology, vol. 40, no. 11, pp. 1905-1912. https://doi.org/10.3899/jrheum.121473
Grayson PC, Cuthbertson D, Carette S, Hoffman GS, Khalidi NA, Koening CL et al. New features of disease after diagnosis in 6 forms of systemic vasculitis. Journal of Rheumatology. 2013 Nov;40(11):1905-1912. https://doi.org/10.3899/jrheum.121473
Grayson, Peter C. ; Cuthbertson, David ; Carette, Simon ; Hoffman, Gary S. ; Khalidi, Nader A. ; Koening, Curry L. ; Langford, Carol A. ; Maksimowicz-McKinnon, Kathleen ; Monach, Paul A. ; Seo, Philip ; Specks, Ulrich ; Ytterberg, Steven R ; Merkel, Peter A. / New features of disease after diagnosis in 6 forms of systemic vasculitis. In: Journal of Rheumatology. 2013 ; Vol. 40, No. 11. pp. 1905-1912.
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abstract = "Objective. To quantify the occurrence of features of vasculitis that initially present after diagnosis in 6 types of primary vasculitis. Methods. Standardized collection of data on 95 disease manifestations in 6 vasculitides, including granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), eosinophilic granulomatosis with polyangiitis (Churg-Strauss; EGPA), polyarteritis nodosa (PAN), giant cell arteritis (GCA), and Takayasu arteritis (TAK), was obtained within a set of multicenter longitudinal, observational cohorts. For each form of vasculitis, the frequency of disease-specific manifestations at diagnosis was compared to the cumulative frequency of each manifestation. The percentage of patients who initially developed severe manifestations after diagnosis, defined as organ- or life-threatening in the small and medium vessel vasculitides (GPA, MPA, EGPA, PAN) and as ischemic/vascular in the large vessel vasculitides (GCA, TAK), was reported. Results. Out of 838 patients with vasculitis, 490 (59{\%}) experienced ≥ 1 new disease manifestation after diagnosis. On average, patients with vasculitis experienced 1.3 new manifestations after diagnosis (GPA = 1.9, MPA = 1.2, EGPA = 1.5, PAN = 1.2, GCA = 0.7, and TAK = 1.0). New severe manifestations occurred after diagnosis in 224 (27{\%}) out of 838 patients (GPA = 26{\%}, MPA = 19{\%}, EGPA = 21{\%}, PAN = 23{\%}, GCA = 24{\%}, and TAK = 44{\%}). Timing of onset of new manifestations was not significantly associated with disease duration. Conclusion. A majority of patients with vasculitis develop new disease features after diagnosis, including a substantial number of new, severe manifestations. Ongoing assessment of patients with established vasculitis should remain broad in scope. The Journal of Rheumatology",
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AU - Langford, Carol A.

AU - Maksimowicz-McKinnon, Kathleen

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N2 - Objective. To quantify the occurrence of features of vasculitis that initially present after diagnosis in 6 types of primary vasculitis. Methods. Standardized collection of data on 95 disease manifestations in 6 vasculitides, including granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), eosinophilic granulomatosis with polyangiitis (Churg-Strauss; EGPA), polyarteritis nodosa (PAN), giant cell arteritis (GCA), and Takayasu arteritis (TAK), was obtained within a set of multicenter longitudinal, observational cohorts. For each form of vasculitis, the frequency of disease-specific manifestations at diagnosis was compared to the cumulative frequency of each manifestation. The percentage of patients who initially developed severe manifestations after diagnosis, defined as organ- or life-threatening in the small and medium vessel vasculitides (GPA, MPA, EGPA, PAN) and as ischemic/vascular in the large vessel vasculitides (GCA, TAK), was reported. Results. Out of 838 patients with vasculitis, 490 (59%) experienced ≥ 1 new disease manifestation after diagnosis. On average, patients with vasculitis experienced 1.3 new manifestations after diagnosis (GPA = 1.9, MPA = 1.2, EGPA = 1.5, PAN = 1.2, GCA = 0.7, and TAK = 1.0). New severe manifestations occurred after diagnosis in 224 (27%) out of 838 patients (GPA = 26%, MPA = 19%, EGPA = 21%, PAN = 23%, GCA = 24%, and TAK = 44%). Timing of onset of new manifestations was not significantly associated with disease duration. Conclusion. A majority of patients with vasculitis develop new disease features after diagnosis, including a substantial number of new, severe manifestations. Ongoing assessment of patients with established vasculitis should remain broad in scope. The Journal of Rheumatology

AB - Objective. To quantify the occurrence of features of vasculitis that initially present after diagnosis in 6 types of primary vasculitis. Methods. Standardized collection of data on 95 disease manifestations in 6 vasculitides, including granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), eosinophilic granulomatosis with polyangiitis (Churg-Strauss; EGPA), polyarteritis nodosa (PAN), giant cell arteritis (GCA), and Takayasu arteritis (TAK), was obtained within a set of multicenter longitudinal, observational cohorts. For each form of vasculitis, the frequency of disease-specific manifestations at diagnosis was compared to the cumulative frequency of each manifestation. The percentage of patients who initially developed severe manifestations after diagnosis, defined as organ- or life-threatening in the small and medium vessel vasculitides (GPA, MPA, EGPA, PAN) and as ischemic/vascular in the large vessel vasculitides (GCA, TAK), was reported. Results. Out of 838 patients with vasculitis, 490 (59%) experienced ≥ 1 new disease manifestation after diagnosis. On average, patients with vasculitis experienced 1.3 new manifestations after diagnosis (GPA = 1.9, MPA = 1.2, EGPA = 1.5, PAN = 1.2, GCA = 0.7, and TAK = 1.0). New severe manifestations occurred after diagnosis in 224 (27%) out of 838 patients (GPA = 26%, MPA = 19%, EGPA = 21%, PAN = 23%, GCA = 24%, and TAK = 44%). Timing of onset of new manifestations was not significantly associated with disease duration. Conclusion. A majority of patients with vasculitis develop new disease features after diagnosis, including a substantial number of new, severe manifestations. Ongoing assessment of patients with established vasculitis should remain broad in scope. The Journal of Rheumatology

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KW - POLYARTERITIS NODOSA

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