Session Information
Date: Sunday, October 21, 2018
Session Title: Vasculitis Poster I: Non-ANCA-Associated and Related Disorders
Session Type: ACR Poster Session A
Session Time: 9:00AM-11:00AM
Background/Purpose:
Immunoglobuline A (IgA) Vasculitis, formerly known as Henoch-Schönlein purpura, is a small-vessel leukocytoclastic vasculitis due to deposition of IgA1, a subclass of IgA. Although it is mainly a pediatric disease, it can affect adults with an incidence rate of 1-8 cases/1000000 patients/year and it is reported to have a more aggressive course and worse renal outcome. There are scarce studies analyzing this association, and none in our region.
The purpose of the study is to describe the different features of the disease (clinical, laboratory, treatment and prognosis) both in pediatric and adult patients, and evaluate the differences between these groups, especially regarding renal outcome.
Methods:
We performed a retrospective review of the electronic medical records of all the patients with IgA vasculitis that were followed in a university hospital between 01/01/2000 and 05/01/2018. We included all patients that fulfilled the EULAR/PRINTO/PRES 2010 criteria ( European League Against Rheumatism/Paediatric Rheumatology International Trials Organisation/Paediatric Rheumatology European Society), and collected and analyzed demographic, clinical, treatment and histopathologic information available on their medical records.
We considered pediatric patients those who were under 20 years when they were diagnosed.
Results:
One-hundred eighteen patients were included, being 107 pediatric and 11 adults.
Table 1 shows demographic information, clinical manifestations, predisposing factors, treatment used, relapses, kidney outcome and deaths in both groups.
In our cohort, the adult group had more renal involvement. Kidney biopsy was performed in 5/8 of the affected patients, with confirmatory histopathology for IgA vasculitis in all of them.
Since adults showed more severe clinical manifestations, significantly more corticosteroids and immunosuppressants were used in this group.
As a consequence of the vasculitis, 2 of the affected adults suffered from chronic kidney disease, one of them required kidney transplant. No pediatric patient presented chronic kidney disease.
Finally, 2 deaths were found, both in the adult group, but only one related to the disease.
Conclusion: In our cohort, adult patients with IgA vasculitis suffered a more aggressive disease, with more renal involvement and required more intense treatment.
Table 1.
| Pediatric Patients (n=107) | Adult Patients (n=11) | P | |
| Male, n (%, 95% CI) | 61 (57.0, 47.3-66.2) | 8 (72.7, 39.5-91.6) | 0.31 |
| Age at diagnosis, years, median (IQR) | 6.2 (4.4-9.5) | 39.7 (27.6-72.3) | <0.001 |
| Follow-up, years, median (IQR) | 7.3 (1.5-11.4) | 9.3 (0.3-15.6) | 0.47 |
| Initial Clinical Manifestations | |||
| – Purpura, n (%, 95% CI) | 107 (100) | 11 (100) | 1 |
| – Abdominal pain, n (%, 95% CI) | 62 (57.9, 48.3-67.0) | 5 (45.4, 19.1-74.6) | 0.43 |
| – Proctorrhagia, n (%, 95% CI) | 12 (11.2, 6.4-18.8) | 1 (9.1, 1.1-46.9) | 0.83 |
| – Intussusception, n (%, 95% CI) | 0 (0) | 0 (0) | 1 |
| – Scalp edema, n (%, 95% CI) | 45 (42.1, 32.9-51.7) | 1 (9.1, 1.1-46.9) | 0.03 |
| – Arthritis, n (%, 95% CI) | 30 (28.0, 20.2-37.4) | 2 (18.2, 4.2-52.9) | 0.48 |
| – Arthralgia, n (%, 95% CI) | 57 (53.3, 43.7-62.6) | 2 (18.2, 4.2-52.9) | 0.03 |
| – Renal involvement, n (%,95% CI) | 39 (36.4, 27.8-46.1) | 8 (72.7, 39.5-91.6) | 0.02 |
| Predisposing Factors | |||
| Upper respiratory tract viral infection, n (%, 95% CI) | 24 (44.4, 31.5-58.2) | 2 (33.3, 6.9-76.9) | 0.60 |
| Streptococcal infection, n (%, 95% CI) | 26 (48.2, 34.9-61.7) | 0 | 0.02 |
| None recognizable, n (%, 95% CI) | 4 (7.4, 2.7-18.6) | 4 (66.7, 23.1-93.0) | <0.001 |
| Unknown, n (%, 95% CI) | 53 (49.5, 40.0-59.1) | 5 (45.4, 19.1-74.6) | 0.79 |
| Initial Treatment | |||
| – Watch and wait, n (%, 95% CI) | 52 (48.6, 39.1-58.1) | 1 (9.1, 1.1-46.9) | 0.01 |
| – NSAIDs, n (%, 95% CI) | 35 (32.7, 24.4-42.3) | 2 (18.2, 4.2-52.9) | 0.32 |
| – Corticosteroids, n (%, 95% CI) | 36 (33.6, 25.2-43.2) | 9 (81.8, 47.0-95.8) | 0.002 |
| – Immunosuppressants, n (%, 95% CI) | 1 (0.9, 0.1-6.5) | 2 (18.2, 4.2-52.9) | 0.001 |
| Relapses, n (%, 95% CI) | 18 (16.8, 10.8-25.3) | 1 (9.1, 1.1-46.9) | 0.51 |
| Chronic kidney disease at the end of follow-up, n (%, 95% CI) | 0 | 2 (18.2, 4.2-52.9) | < 0.001 |
| Death, n (%, 95% CI) | 0 | 2 (18.2, 4.2-52.9) | < 0.001 |
To cite this abstract in AMA style:
Brom M, Gandino IJ, Scolnik M, Scaglioni V, Britos M, De Cunto C, Soriano ER. Immunoglobuline a Vasculitis: Comparison between Pediatric and Adult Population [abstract]. Arthritis Rheumatol. 2018; 70 (suppl 9). https://acrabstracts.org/abstract/immunoglobuline-a-vasculitis-comparison-between-pediatric-and-adult-population/. Accessed October 4, 2022.« Back to 2018 ACR/ARHP Annual Meeting
ACR Meeting Abstracts - https://acrabstracts.org/abstract/immunoglobuline-a-vasculitis-comparison-between-pediatric-and-adult-population/