Session Information
Date: Sunday, October 21, 2018
Title: Vasculitis Poster I: Non-ANCA-Associated and Related Disorders
Session Type: ACR Poster Session A
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 103 (male/ female: 67/36) patients with adult IgA Vasculitis. The mean age was 42.6±17 years. Infection history within 6 weeks before presentation was present in 40 (38.8%) patients (32 upper respiratory tract, 3 urinary tract, 2 gastrointestinal, 3 others). Cutaneous manifestations and arthritis/arthralgia were the most common clinical manifestations (Table 1). Ninety-two (89.3%) patients were treated with oral glucocorticoids (GC). Pulse GC treatment was also given to 29 (28.1%) patients. As additional immunosuppressive agents, azathiopirine was given to 36 (34.9%) and pulse cyclophosphamide to 13 (12.6%) patients. Fifty-nine patients (58.2%) had follow-up of mean 35.6 months. Eleven (18.6%) patients relapsed during follow-up. While 5 relapses were major, six of them were minor relapses. At the last visit, disease status was evaluated as active or treatment failure by the treating physician in 7 (11.8 %) patients. The rate of chronic renal failure was 8.3 %(n=5). Mortality was 1.6% (n=1) during follow-up, due to pneumonia. The mean VDI score was 0.3 in the last visit. Twelve (20.3%) 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 and had at least one damage item at the end of follow-up, Although, 31% of patients had FFS≥1, the mortality rate was observed to be very low in the present study.
Table 1: Baseline vlinical characteristics of patients with adult Henoch Schönlein Purpura
| Adult Henoch Schönlein Purpura (n=103) | |
| Laboratory parameters | |
| Anemia (<12 mg/dl for female,<13 mg/dl for male)( n, %) | 36 (35%) |
| Erythrocyte Sedimentation Rate (mm/hour) * | 34.7 ± 22 |
| C-reactive protein (mg/l)† | 18 (1-297) |
| Proteinuria ( >300mg/24 hours) | 47 (45.6 %) |
| Creatinine (mg/dl)* | 0.8±0.3 |
| Hepatitis B positivity (n) | 8/97 (8.2 %) |
| Hepatitis C positivity (n) | 0/97 |
| ANA positivity | 21/97 (21.7%) |
| RF Positivity | 5/88 (5.7%) |
| c-ANCA positivity | 2/97 (2.1%) |
| p-ANCA positivity | 1/95 (1.1 %) |
| Clinical Manifestations, n/103 (%) | |
| Fever | 24 (23.3%) |
| Weight loss | 26 (25.2%) |
| Myalgia/Weakness/Leg tenderness | 44 (42.7%) |
| Arthritis and/or arthralgia | 87 (84.5%) |
| Neurologic manifestations | 1 (0.9%) |
| Testicular pain or tenderness | 4 (3.9%) |
| Recent onset or severe hypertension | 2 (1.9%) |
| Cutaneous Manifestations | 97 (94.2%) |
| Peripheral limb edema | 14 (13.6%) |
| Gastrointestinal manifestations | 71 (68.9%) |
| Cardiac manifestations | 5 (4.8%) |
| Ocular manifestations | 3 (2.9%) |
| Pulmonary manifestations | 3 (2.9%) |
| FFS=0 | 71 (68.9%) |
| FFS=1 | 24 (23.3%) |
| FFS=2 | 5 (4.9%) |
| FFS=3 | 3 (2.9%) |
| BVAS score at diagnosis* | 6± 3 |
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)
To cite this abstract in AMA style:
Gazel U, Omma A, Sari A, Uskudar Cansu D, Yazici A, Cefle A, Bes C, Karadag O, Direskeneli H, Alibaz-Oner F. Assessment of Damage and Prognosis in Patients with Adult IgA Vasculitis: Retrospective Multicentered Cohort Study [abstract]. Arthritis Rheumatol. 2018; 70 (suppl 9). https://acrabstracts.org/abstract/assessment-of-damage-and-prognosis-in-patients-with-adult-iga-vasculitis-retrospective-multicentered-cohort-study-2/. Accessed .« Back to 2018 ACR/ARHP Annual Meeting
ACR Meeting Abstracts - https://acrabstracts.org/abstract/assessment-of-damage-and-prognosis-in-patients-with-adult-iga-vasculitis-retrospective-multicentered-cohort-study-2/