Medium-Sized Vasculitides May Be Distinct Diseases

July 17, 2017

A direct comparison of their clinical characteristics helps distinguish one from another.

Polyarteritis nodosa, cutaneous arteritis, and gastrointestinal vasculitis are likely 3 distinct diseases and not variations on a single disease process, researchers found.

The clinical characteristics of polyarteritis nodosa, cutaneous arteritis, and gastrointestinal vasculitis have been reported separately but have not been compared to each other.

Fatma Alibaz-Oner and colleagues at the Mayo Clinic, Rochester, Minnesota, pointed out that since the revised 2012 International Chapel Hill Consensus Conference, where vasculitis definitions and nomenclature were agreed upon, differentiation among the medium-sized vasculitides has not been considered.

The authors presented the results of their study comparing the 3 diseases in a recent edition of Arthritis Care & Research.

The study

The retrospective cohort study included 108 patients with vasculitis at the Mayo Clinic in Rochester, Minnesota, between 1980 and 2014. Included were 48 patients with polyarteritis nodosa, 41 with cutaneous arteritis, and 19 with gastrointestinal vasculitis.

Results

• The most common features in the polyarteritis nodosa group were constitutional symptoms (71%), musculoskeletal symptoms (69%), neurologic symptoms (56%), and skin involvement (56%).

• Constitutional and musculoskeletal symptoms were less common in patients with cutaneous arteritis and gastrointestinal vasculitis than in those with polyarteritis nodosa.

• 61% of patients with cutaneous vasculitis had subcutaneous nodules.

• 89% of patients with gastrointestinal vasculitis complained of ischemic abdominal pain.

• The presence of abnormal angiograms, histologic necrotizing vasculitis involving medium-sized vessels, arterial stenosis, and microaneurysms was highly variable among the 3 groups.

• Pulse steroid therapy was employed in 18% of patients with gastrointestinal vasculitis and 15% of those with polyarteritis nodosa.

• 94%, 93%, and 67% or patients with polyarteritis nodosa, cutaneous arteritis, and gastrointestinal vasculitis, respectively, were given oral steroids.

• Birmingham Vasculitis Activity Scores were higher in patients with polyarteritis nodosa than in those with gastrointestinal vasculitis or cutaneous arteritis.

• More patients with gastrointestinal vasculitis had prognostic Five-Factor Scores ≥ 1 than in the polyarteritis nodosa group (58% to 42%, P<0.001).

• Relapse rates at 5 years were 45.2% and 9.6% in the cutaneous vasculitis and polyarteritis nodosa groups, respectively.

•The Vasculitis Damage Index scores were similar between the polyarteritis nodosa and gastrointestinal vasculitis groups at last follow-up but higher than those of the cutaneous arteritis group (P=0.030).

• In the polyarteritis group, the 1-, 5-, and 10-year survival rates were 92.5%, 82.5%, and 66.2% respectively.

• All deaths in the gastrointestinal vasculitis group occurred in the first year.

• Causes of death in the gastrointestinal vasculitis group in order of commonality were: uncontrolled vasculitis, unknown causes, cancer, and infection.

• Causes of death in the polyarteritis nodosa group in order of commonality were: cancer, uncontrolled vasculitis, and unknown causes.

• Low Birmingham Vasculitis Activity Scores were associated with relapse but without significance when adjusted for cutaneous arteritis diagnosis (P=0.19).

• Mortality was significantly related to older age (hazard ratio, 1.05), low hemoglobin (hazard ratio, 0.68), weight loss (hazard ratio, 13.79), and arteriographic abnormalities (hazard ratio, 4.94).

Implications for physicians

• Polyarteritis nodosa is distinguished from other vasculitides by the presence of systemic necrotizing vasculitis of the medium-sized vessels.

• In this study, most cases of polyarteritis nodosa were idiopathic.

• Cutaneous arteritis and gastrointestinal arteritis, while also involving the medium-sized vessels, have distinct clinical courses compared with polyarteritis nodosa.

• Progressing from cutaneous arteritis to systemic polyarteritis nodosa is very rare.

• The damage caused by polyarteritis nodosa and that with gastrointestinal arteritis are comparable.

• Relapse is less common in polyarteritis nodosa than in cutaneous arteritis, possibly because of less frequent use of immunosuppressive treatment in the latter.

Disclosures:

No funding was disclosed.

References:

Fatma Alibaz-Oner, Matthew J. Koster, Cynthia S. Crowson, et al. “Clinical Spectrum of Medium-Sized Vessel Vasculitis.” Arthritis Care Res (Hoboken). 2017;69:884-891. doi: 10.1002/acr.23007. Epub 2017 Apr 24.