Session Information
Session Type: ACR Poster Session C
Session Time: 9:00AM-11:00AM
Background/Purpose: Polyarteritis Nodosa (PAN) is a rare subgroup of the primary vasculitides. There are only two published cohorts describing demographic and clinical features of the disease. Furthermore, various subgroups of PAN have been described, such as hepatitis B virus (HBV)-related, cutaneous and monogenic forms. There is a paucity of information on the current phenotypes and geographic differences of PAN. This study aimed to compare disease characteristics between PAN cohorts from the UK and Turkey (TR).
Methods: A retrospective survey of databases from two vasculitis centres between 1990-2016 for PAN patients fulfilling the EMEA Vasculitis Classification algorithm. All paediatric-onset adult patients met the Ankara 2008 (EULAR/PReS endorsed) criteria for childhood PAN. Patients with typical angiographic and/or histopathologic findings consistent with PAN were included. We evaluated demographics, organ involvement, treatment, patient survival and time to first relapse
Results: 93 (M/F: 51/42) patients (UK: 47, TR: 46) were included in the study. Median age at disease onset was 36.0 (20.0-49.8) years. Three were HBV-related, 20 (21.5%) had paediatric onset and 16 (16.5%) had cutaneous PAN. TR patients had younger age of disease onset (p=0.002). Twelve (26%) of TR patients had a monogenic form of disease (Familial Mediterranean Fever association in seven, deficiency of adenosine deaminase 2, DADA2, in five). Cutaneous PAN was more frequent in the UK (12 vs. four patients, p=0.031) whereas renal involvement was higher in the TR group (76.1% vs. 40.4%,p<0.001).Further analyses showed female predominance in cutaneous PAN when compared to systemic involvement (68.8% vs. 40.3% p=0.037). No difference was found in phenotype between paediatric and adult onset patients except frequency of cutaneous lesions (100% vs. 64.3%, p=0.002). In both cohorts, most patients received a combination of glucocorticoids and cyclophosphamide. During a median 67.5 (32-126) months follow up, 13 patients died (12.7% in the UK vs. 15.2% in TR cohorts). No difference was found between the two cohorts in relation to relapse rate, death and vasculitis damage index. Five factor score was significantly related to mortality (p=0.019).
Conclusion: This is the largest study defining diagnoses of PAN according to the EMEA algorithm. The TR group had a younger age of disease onset and more cases of monogenic disease; however disease extent, relapse rate, damage index and death rates were similar between groups.
Table: Comparison of Demographic. clinical characteristics and outcomes between the UK and Turkish Cohorts
|
UK cohort (n:47) |
Turkish cohort (n: 46) |
p |
|
|
Age at onset, years |
44 (28.5-59.0) 43.0 (18.0 |
24.5 (11.8-40.5) 28.7 (17.8) |
0.002 <0.001 |
|
Paediatric onset,% |
17.0 |
26.0 |
0.287 |
|
Sex, male, % |
44.7% |
65.2% |
0.047 |
|
Time to diagnosis, months |
2 (1-5) |
3 (2-8) |
0.041 |
|
Constitutional symptoms, % |
87.1 |
88.1 |
0.898 |
|
Cutaneous Manifestations, % |
68.1 |
75.6 |
0.426 |
|
Musculoskeletal manifestations, % |
76.7 |
82.6 |
0.278 |
|
Neurologic manifestations, % |
32.5 |
54.3 |
0.089 |
|
Gastrointestinal manifestations, % |
48.8 |
45.0 |
0.733 |
|
Renal involvement, % |
40.4 |
76.1 |
<0.001 |
|
Testicular pain/tenderness (men only), % |
28.5 |
16.7 |
0.310 |
|
DEI |
5.3 (2.5) |
6.35 (2.3) |
0.05 |
|
Distribution of patients · FFS=0 · FFS=1 · FFS>=2 |
51.0% 36.2% 12.8% |
41.3% 43.5% 15.2% |
0.641 |
|
ESR, mm/hr |
29 (15.5-49) |
57 (35.3-78.3) |
0.035 |
|
CRP, mg/L |
24 (9.8-117.3) |
40.5 (18-40.5) |
0.128 |
|
WBC , /mm3 |
11450 (7500-15525) |
9875 (7800-12500) |
|
|
Haemoglobin, g/L |
12.6 (11.2-13.4) |
11.9 (10.4-13.2) |
0.238 |
|
Serum Creatinine, Umol/l |
73 (60.5-98) |
81.3 (64.0-88.4) |
0.41 |
|
Follow up, months |
79.0 (35.0-43.0) |
65 (29.5-108) |
0.669 |
|
Response to treatment, % · Complete · Partial · No response |
60.4 30.2 9.4 |
54.5 31.8 13.7 |
0.838 |
|
Any relapse,% |
58.9 |
52.9 |
0.675 |
|
Death, % |
12.7 |
15.2 |
0.733 |
|
VDI |
1 (0-1) |
1 (0-2) |
0.632 |
|
Values are labeled as mean (SD) or median (IQR 25%-75%). DEI: disease extent index; FFS: Five factor score, ESR: Eryhtrocyte Sedimentation Rate, CRP: C-reactive protein, WBC: White blood cells
|
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To cite this abstract in AMA style:
Karadag O, Erden A, Bilginer Y, Gopaluni S, Sari A, Armagan B, Ertenli I, Ozen S, Jayne D. A Retrospective Study Comparing the Phenotype and Outcomes of Patients with Polyarteritis Nodosa between UK and Turkish Cohorts [abstract]. Arthritis Rheumatol. 2017; 69 (suppl 10). https://acrabstracts.org/abstract/a-retrospective-study-comparing-the-phenotype-and-outcomes-of-patients-with-polyarteritis-nodosa-between-uk-and-turkish-cohorts/. Accessed .« Back to 2017 ACR/ARHP Annual Meeting
ACR Meeting Abstracts - https://acrabstracts.org/abstract/a-retrospective-study-comparing-the-phenotype-and-outcomes-of-patients-with-polyarteritis-nodosa-between-uk-and-turkish-cohorts/