ACR Meeting Abstracts

ACR Meeting Abstracts

Abstract Number: 2727

Assessment of Damage and Prognosis in Patients with Adult IgA Vasculitis: Retrospective Multicentered Cohort Study

Fatma Alibaz-Oner1, Ahmet Omma2, Alper Sari3, Omer Karadag4, Dondu Uskudar Cansu5, Cemal Bes6, Fatih Yildiz7, Mustafa Ferhat Oksuz8, Sema Yilmaz9, Atalay Dogru10, Ayse Balkarli8, Sibel Bakirci11 and Haner Direskeneli12, 1Marmara University, School of Medicine, Rheumatology, Istanbul, Turkey, 2Department of Internal Medicine, Division of Rheumatology, Ankara Numune Training and Research Hospital, Ankara, Turkey, 3Rheumatology, Hacettepe University, School of Medicine, Ankara, Turkey, 4Rheumatology, Hacettepe University Faculty of Medicine, Ankara, Turkey, 5Rheumatology, Osmangazi University, School of Medicine, Eskısehir, Turkey, 6Rheumatology, Bakırköy Dr. Sadi Konuk Training and Research Hospital, İstanbul, Turkey, 7Rheumatology, Van EAH, Adana, Turkey, 8PsART study group, Ankara, Turkey, 9Department of Rheumatology, Selcuk University School of Medicine, Konya, Turkey, 10Rheumatology, Suleyman Demirel University, School of Medicine, Isparta, Turkey, 11Fellow in Rheumatology, Antalya, Turkey, 12Rheumatology, Marmara University, School of Medicine, Rheumatology, Istanbul, Turkey

Meeting: 2017 ACR/ARHP Annual Meeting

Date of first publication: September 18, 2017

Keywords:

Session Information

Date: Tuesday, November 7, 2017

Title: Vasculitis Poster III: Other Vasculitis Syndromes

Session Type: ACR Poster Session C

Session Time: 9:00AM-11:00AM

Background/Purpose: IgA Vasculitis is a leukocytoclastic vasculitis involving small vessels with depositions of immune complexes containing IgA. IgA Vasculitis is a predominantly pediatric vasculitis. There is limited data for the prognosis of adult IgA Vasculitis, with also no damage assessment. In this study, we aimed to evaluate the clinical characteristics, treatment, outcome and damage of patients with adult IgA Vasculitis.

Methods: We assembled a retrospective cohort of patients with adult IgA Vasculitis from tertiary Rheumatology Centers in Turkey. The demographics, clinical characteristics, treatment and outcomes of patients were abstracted from medical records. Birmingham Vasculitis Activity Score (BVAS), prognostic Five Factor Score (FFS) and vasculitis damage index (VDI) were calculated.

Results: The study included 52 (male/ female: 40/12) patients with adult IgA Vasculitis The mean age was 42.2±17 years. Infection history within 6 weeks before presentation was present in 22 (42.3%) patients (18 upper respiratory tract, 3 gastrointestinal and one urinary tract). Cutaneous manifestations and arthritis/arthralgia were the most common clinical manifestations (Table 1). All patients were treated with oral glucocorticoids (GC). Pulse GC treatment was also given to 12 (23.1%) patients. As additional immunosuppressive agents, azathiopirine was given to 21 (40.4%) and pulse cyclophosphamide to 11 (21.2%) patients. Twenty-eight patients (53.9%) had follow-up of 28.6 months. Five (17.8%) patients relapsed during follow-up. While 3 relapses were major, 2 of them were minor relapses. At the last visit, disease status was evaluated as active or treatment failure by the treating physician in 6 (21.4%) patients. Mortality was 3.6% (n=1) during follow-up, due to pneumonia. The mean VDI score was 0.6 in the last visit. Nine (32.1%) patients had at least one damage item at the end of follow-up period.

Conclusion: Our results showed that approximately one fifth of patients with adult IgA Vasculitis had relapses during follow-up. At the end of follow-up, one third of patients had at least one damage item. Although, 45% of patients had FFS≥1, the mortality rate was observed to be low in the present study.

Table 1: Baseline vlinical characteristics of patients with adult Henoch Schönlein Purpura

Adult Henoch Schönlein Purpura

(n=52)

Laboratory parameters

Hemoglobin (g/dL)*

13.5±2

Erythrocyte Sedimentation Rate (mm/hour) *

32.7 ± 22

C-reactive protein (mg/l)†

25.2 (1-94.9)

Proteinuria ( >300mg/24 hours)

28 (53.9%)

Creatinine (mg/dl)*

0.9±0.4

Hepatitis B positivity (n)

4/47 (8.5%)

Hepatitis C positivity (n)

ANA positivity

RF Positivity

0/47

8/47 (17%)

0/48

c-ANCA positivity

1/48 (2.1%)

p-ANCA positivity

1/47(2.1%)

Clinical Manifestations, n/52 (%)

Fever

7 (13.5%)

Weight loss

14 (26.9%)

Myalgia/Weakness/Leg tenderness

24 (46.2%)

Arthritis and/or arthralgia

46 (88.5%)

Neurologic manifestations

1 (1.9%)

Testicular pain or tenderness

3 (5.8%)

Recent onset or severe hypertension

2 (3.8%)

Cutaneous Manifestations

48 (92.3%)

Peripheral limb edema

8 (15.4%)

Gastrointestinal manifestations

39 (75%)

Cardiac manifestations

2 (3.8%)

FFS=0

29 (55.8%)

FFS=1

15 (28.8%)

FFS≥2

8 (15.4%)

BVAS score at diagnosis*

4.1± 1.7

ANA: Anti nuclear antibody, FR: Rheumatoid factor, ANCA: Antineutrophilic cytoplasmic antibody, FFS:Five Factor Score, BVAS: Birmingham Vasculitis Activity score. *Mean ±SD †Median(Minimum-maximum)

Disclosure: F. Alibaz-Oner, None; A. Omma, Abbvie, 2,Merck; Pfizer, 5; A. Sari, None; O. Karadag, None; D. Uskudar Cansu, None; C. Bes, Roche Pharmaceuticals, 2; F. Yildiz, None; M. F. Oksuz, None; S. Yilmaz, None; A. Dogru, None; A. Balkarli, None; S. Bakirci, None; H. Direskeneli, None.

To cite this abstract in AMA style:

Alibaz-Oner F, Omma A, Sari A, Karadag O, Uskudar Cansu D, Bes C, Yildiz F, Oksuz MF, Yilmaz S, Dogru A, Balkarli A, Bakirci S, Direskeneli H. Assessment of Damage and Prognosis in Patients with Adult IgA Vasculitis: Retrospective Multicentered Cohort Study [abstract]. Arthritis Rheumatol. 2017; 69 (suppl 10). https://acrabstracts.org/abstract/assessment-of-damage-and-prognosis-in-patients-with-adult-iga-vasculitis-retrospective-multicentered-cohort-study/. Accessed .

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