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J Rheumatol. 2013 Nov;40(11):1905-12. doi: 10.3899/jrheum.121473. Epub 2013 Aug 1.

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

Author information

1
From The Vasculitis Center, Section of Rheumatology, and the Clinical Epidemiology Unit, Boston University School of Medicine, Boston, Massachusetts; Department of Biostatistics, University of South Florida, Tampa, Florida; Division of Rheumatology, Mount Sinai Hospital, Toronto, Ontario; and the Center for Vasculitis Care and Research, Cleveland Clinic, Cleveland, Ohio; Division of Rheumatology, McMaster University, Hamilton, Ontario, Canada; Division of Rheumatology, University of Utah, Salt Lake City, Utah; Division of Rheumatology, University of Pittsburgh; Division of Rheumatology, Johns Hopkins University, Baltimore, Maryland; Division of Pulmonary and Critical Care Medicine, Mayo Clinic College of Medicine, Rochester; Division of Rheumatology, Mayo Clinic College of Medicine, Rochester, Minnesota; and Division of Rheumatology, University of Pennsylvania, Philadelphia, Pennsylvania, USA.

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.

KEYWORDS:

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

Comment in

PMID:
23908447
PMCID:
PMC4292850
DOI:
10.3899/jrheum.121473
[Indexed for MEDLINE]
Free PMC Article
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