Background/Purpose
In our population IgA vasculitis (IgAV) has an annual incidence rate of 5.1 cases per 105 adults increasing with patients’ age which makes it by no means uncommon contrary to common belief. Little data is available in the literature about the clinical picture and prognosis of adult IgAV. Available data is most often limited to adult IgAV cases with kidney disease. Our aim was to determine short-term outcomes in an unselected adult IgAV population diagnosed at a single secondary/tertiary rheumatology center.
Methods
We performed an electronic and paper chart review of all adult IgAV cases, diagnosed in our center between January 1, 2010 and December 31, 2013. Appropriate descriptive statistical methods and post hoc tests were used describe our cohort (e.g. Mann-Whitney test, Fisher exact test).
Results
During the observation period 96 new adult IgAV cases were identified. Clinical characteristics at presentation are shown in Table 1. Disease severity was higher in males (median BVAS3 13 vs. 9, p=0.027), and in patients with generalized purpura in contrast to purpura limited to lower limbs (median BVAS3 14 vs. 9, p=0.014). Treatment of IgAV consisted of systemic glucocorticoids (oral 67/96; additional i.v. metil prednisolone pulses 13/96), intravenous cyclophosphamide (9/96), hyperimmune gammglobulins (4/96), plasma exchange (2/96) and mycophenolate mofetil (1/96). During acute phase of the disease 3/96 patients died. Two deaths were attributed to vasculitis (intractable GI hemorrhage in the first; and alveolar hemorrhage in the second patient), while one succumbed following generalized CMV infection and heart failure attributable to immunosuppressive treatment. 18/93 (19.4%) survivors were lost to follow up. The remaining 75/93 (80.6%) patients were followed for a median of 8.4 (IQR 4.8–19.2) months. IgAV relapsed in 13/75 cases (one, two and three times in 8/75, 4/75 and 1/75 cases, respectively). 8/13 (76.9%) patients had a single organ, and 5/13 a multi-organ relapse. Skin was involved in 11, joints in 3, GI tract in 2, and kidney in 2 cases. At last visit urinary abnormalities were present in 13/68 patients. 13/68 had microhameturia (1+, 2+ and 3+ in 6, 3 and 4 cases, respectively); 4/68 had mild proteinuria and 1 patient had proteinuria >1 g per day. Renal function remained stable in 67/68, and worsened in 1/68 case. 7/75 (9.3%) patients died during follow up (cause of death: infection 2; cardiovascular disease 4; unknown 1). In 2/68 survivors malignancy was diagnosed (1 hematologic; 1 teratoma).
Table 1. Clinical characteristic of IgAV patients at presentation and at last follow up
|
Clinical characteristic of IgAV |
at presentation N=96 |
at last follow-up visit N=68 |
|
% male |
60 |
60 |
|
Age (median; IQR) |
63.4 (40.8–77.3) |
/ |
|
Purpura # (%) |
96 (100) |
6 (8.8) |
|
necrotic # (%) |
43 (44.8) |
0 |
|
Joint involvement # (%) |
44 (45.8%) |
0 |
|
arthralgia # (%) |
21 (42.7) |
0 |
|
arthritis # (%) |
41 (21.9) |
0 |
|
GI tract involvement # (%) |
35 (36.4) |
2 (2.9) |
|
Kidney involvement # (%) |
56 (58.3) |
13 (19.1) |
|
nephritic or nephrotic syndrome # (%) |
10 (10.4) |
1 (1.5) |
|
BVAS3 (median; IQR) |
12 (6–17) |
3 (1-3)* |
|
*patients in remission not included Legend: GI gastrointestinal tract |
||
Conclusion
Our findings suggest that even in short-term IgAV might not be as benign as perceived thus far. From our data it is impossible to conclude whether this is due to the nature of the IgAV or due to the fact that IgAV seems to be more common in older adults, who also have more comorbidities.
Disclosure:
A. Hočevar,
None;
Z. Rotar,
None;
J. Ostrovrsnik,
None;
M. Tomsic,
None.
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ACR Meeting Abstracts - https://acrabstracts.org/abstract/follow-up-of-an-unselected-iga-vasculitis-henoch-schonlein-purpura-population-at-single-rheumatology-center/