Session Information
Date: Tuesday, November 10, 2015
Title: Vasculitis Poster III
Session Type: ACR Poster Session C
Session Time: 9:00AM-11:00AM
Background/Purpose: Polyarteritis nodosa (PAN) is a rare systemic necrotizing
vasculitis predominantly targeting medium-sized visceral arteries. Cutaneous
arteritis (CA) is generally limited to the medium-sized vessels in the skin.
Although previously categorized as cutaneous PAN, it is now included as a form
of single organ vasculitis in the revised 2012 International Chapel Hill
Consensus Conference Nomenclature of Vasculitides. In this study, we aimed to
evaluate and compare the demographics, clinical characteristics, treatment, and
outcome of patients with systemic PAN and CA.
Methods: Retrospective cohorts of patients with PAN and CA evaluated in 1986-2014
were assembled. The demographics, clinical characteristics, treatment and outcomes
of patients were abstracted from medical records. Birmingham Vasculitis
Activity Score (BVAS) and the prognostic Five Factor Score (FFS) were
calculated. Chi-square and rank sum tests were used to compare characteristics
between the groups.
Results: The study cohort included 29 patients with PAN and 39 patients with CA. Only
1 patient presenting as CA evolved to systemic PAN during disease course. Sixteen
of 29 patients with PAN and all patients with CA had histologically proven vasculitis
involving medium-sized vessels. Mean follow-up duration was 6.0±5.9 years in
PAN group, 5.8±4.4 years in the CA group. Angiographic abnormalities consistent
with PAN were present in 16 patients. Most cases of PAN were idiopathic. Three
patients in the PAN group and 2 patients in CA group had hepatitis B. One patient
in the PAN group had Hepatitis C. Clinical characteristics of the groups are
presented in Table 1. All patients with PAN and 90% of patients with CA were
treated with glucocorticoids (GC). Additional immunosuppressive (IS) agents
were used in 62% of PAN and 33% of CA. Dapsone was used in 8 (20.5%) patients
in CA group. Twenty patients with PAN and 16 patients with CA were followed for
at least 6 months. BVAS and FFS scores were higher among patients with PAN. However,
the relapse rate was significantly higher in the CA group compared to PAN (9.8
vs 0.9 per 100 person-years; Rate ratio:7.88; 95% confidence interval:1.81-112.73).
While no deaths were observed in the CA group, survival in the PAN group was
66% at 10-years.
Conclusion: Our results suggest that CA is a distinct disease rather than
a limited expression of systemic PAN. Progression of CA to systemic PAN is
rare. Patients with CA have a higher relapse rate compared to those with
systemic PAN, possibly due to lower use of IS agents in CA.
|
Table 1: Baseline clinical and laboratory characteristics |
|||
|
|
Systemic PAN (n=29) |
Cutaneous Arteritis (n=39) |
P value |
|
Demographics |
|
|
|
|
Age at diagnosis, years* |
56.7±18.2 |
48.7±19.3 |
0.10 |
|
Female |
13 (45%) |
25 (64%) |
0.11 |
|
Laboratory parameters |
|
|
|
|
Sedimentation rate (mm/hour)* |
55.3±41 |
32.7±26.6 |
0.042 |
|
C-reactive protein(mg/l)† |
9.1(0.6-117) |
6.9(0.7-42) |
0.53 |
|
Proteinuria( >400gm/24 hours) |
4/27 (15%) |
0/34 |
0.02 |
|
Constitutional symptoms |
|
|
|
|
Fever |
9/29 (31%) |
6/39 (15%) |
0.12 |
|
Weight loss |
10/29 (34%) |
2/38 (5%) |
0.002 |
|
Clinical manifestations |
|
|
|
|
Musculoskeletal |
19/29 (66%) |
20/39 (51%) |
0.24 |
|
Neurologic |
15/29 (52%) |
2/39 (5%) |
<0.001 |
|
Urologic |
4/28 (14%) |
0/38 |
0.001 |
|
Hypertension |
8/29 (28%) |
0/39 |
<0.001 |
|
Cutaneous |
16/29 (55%) |
39/39 (100%) |
<0.001 |
|
Gastrointestinal |
12/29 (41%) |
0/39 |
<0.001 |
|
Cardiac |
1/29 (3%) |
0/39 |
0.24 |
|
Vascular |
3/29 (10%) |
1/39 (3%) |
0.18 |
|
Ocular |
1/27 (4%) |
0/36 |
0.24 |
|
Pulmonary |
1/29 (3%) |
0/39 |
0.24 |
|
ENT |
1/26 (4%) |
0/39 |
0.22 |
|
Disease assessment |
|
|
|
|
BVAS score* |
11.3±5.2 |
2.5±1.8 |
<0.001 |
|
Five Factor Score =0 |
16/29 (55%) |
38/39 (97%) |
<0.001 |
|
=1 |
11/29 (38%) |
1/39 (3%) |
|
|
≥2 |
2/29 (7%) |
0/39 |
|
| |
|||
To cite this abstract in AMA style:
Alibaz-Oner F, Koster MJ, Crowson CS, Makol A, Ytterberg SR, Matteson EL, Warrington KJ. Polyarteritis Nodosa and Cutaneous Arteritis: Are They Distinct Diseases? [abstract]. Arthritis Rheumatol. 2015; 67 (suppl 10). https://acrabstracts.org/abstract/polyarteritis-nodosa-and-cutaneous-arteritis-are-they-distinct-diseases/. Accessed .« Back to ACR/ARHP Annual Meeting 2015
ACR Meeting Abstracts - https://acrabstracts.org/abstract/polyarteritis-nodosa-and-cutaneous-arteritis-are-they-distinct-diseases/