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

26 Scopus citations

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

Keywords

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

ASJC Scopus subject areas

  • Rheumatology
  • Immunology and Allergy
  • Immunology

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