Background/Purpose: IgA vasculitis (IgAV) is the most common vasculitis in children, with a defined epidemiology (14 cases/ 105children), clinical picture and generally favourable prognosis. In adults IgAV is less well characterized. It is assumed to be rare, with a higher incidence of renal involvement but overall a benign disease. Our aim was to better define IgAV in our adult patient population.
Methods: A retrospective chart analysis of adult patients diagnosed with IgAV at our rheumatological department between June 2010 and June 2013. The demographics, clinical data, treatment and outcome were recorded.
Results: 205 new cases of vasculitis were identified (Table 1). In 71 (34.6%) IgAV was histologically confirmed, 42 males (59.2%), 29 females (40.8%), mean age 58.2 ± 21.5. Prior infection was detected in 26/71 (36.6%) cases. Leukemia and primary biliary cirrhosis was coincidently diagnosed in one and two patients respectively. In 11 cases a new drug was introduced before the IgAV episode (mostly antibiotics).
Skin involvement was seen in all patients (purpura 71/71 cases, skin necrosis 32/71 (45.1%), bullous lesions 7/71 (9.8%)) and there was an isolated manifestation in 15 cases (21.1%). Arthritis was present in 16/71 (22.5%) and gastrointestinal involvement in 29/71 (40.8%; severe with bleeding or ileus in 9/71 (12.6%). 43/71 (60.6%) had renal involvement. Lung, heart and testicular involvement was rare – one case of each. The most common combination of symptoms was skin, gastrointestinal and renal involvement (18/71 cases; 25.3%).
No treatment (13/71) or local glucocorticoids (6/71) were sufficient in the isolated purpura. 52 patients (73.2%) received systemic glucocorticoid, 10/71 (14.1%) needed additional immunosuppression (9 cyclophosphamide (CyC), 1 mycophenolate mofetil), four received IVIG and two patients plasmapheresis in addition to CyC. None needed dialysis.
Two patients died due to active vasculitis, lung and gastrointestinal manifestation respectively and one due to cytomegalovirus infection. Two patients died shortly thereafter due to heart failure and pneumonia respectively.
Conclusion: In our adult patient population IgAV is the second most common vasculitis. Adult IgAV patients frequently need systemic immunosuppression. The most common clinical manifestation is concomitant skin, gastrointestinal and renal involvement. Heart and lung involvement is rare.
|
TYPE |
number |
% |
|
Takayasu arteritis |
1 |
0.5 |
|
GCA |
77 |
37.6 |
|
Isolated aortitis |
3 |
1.5 |
|
PAN |
2 |
1.0 |
|
Localized PAN |
3 |
1.5 |
|
AAV (GPA and MPA) |
18 |
8.8 |
|
EGPA |
3 |
1.5 |
|
Cryoglobulinemic vasculitis |
12 |
5.9 |
|
IgA vasculitis |
71 |
34.6 |
|
Anti-GBM disease |
1 |
0.5 |
|
Behçet disease |
5 |
2.4 |
|
Cogan syndrome |
2 |
1.0 |
|
SVV secondary to infection |
3 |
1.5 |
|
Undetermined SVV |
2 |
1.0 |
|
PCNSV |
1 |
0.5 |
|
Vasculitis secondary to RA |
1 |
0.5 |
|
TOTAL |
205 |
|
Table 1. Newly diagnosed vasculitis from June 2010 to June 2013
GCA – giant cell arteritis; PAN – polyarteritis nodosa; AAV – ANCA associated vasculitis; GPA -granulomatosis with polyangiitis; MPA – microscopic polyangiitis; EGPA – eosinophilic granulomatosis with polyangiitis; SVV – small vessel vasculitis; PCNSV – primary central nervous system vasculitis; RA – rheumatoid arthritis
Disclosure:
A. Hocevar,
None;
J. Ostrovrsnik,
None;
V. Jurcic,
None;
M. Tomsic,
None.
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ACR Meeting Abstracts - https://acrabstracts.org/abstract/iga-vasculitis-in-adults-a-rare-and-benign-disease/