Background/Purpose

In 2010 EULAR/PRINTO/PRES proposed new classification criteria for pediatric IgA vasculitis (IgAV). In pediatric population these criteria have a higher diagnostic sensitivity than the 1990 American College of Rheumatology (ACR) criteria, while they have thus far not been evaluated in adults. Our main objective was to compare the diagnostic sensitivity of the EULAR/PRINTO/PRES and ACR classification criteria in adult IgAV.

Methods

Adult IgAV cases fulfilling the 2012 revised International Chapel Hill Consensus Conference (CHCC) Nomenclature of Vasculitides definition of IgAV at a secondary/tertiary rheumatology referral center were critically reviewed in partially retrospective (from January 1, 2010 to December 31, 2012) and partially prospective (from January 1, 2013 to April 30, 2014) manner and we assessed whether these patients also fulfilled either the ACR or EULAR/PRINTO/PRES criteria. Biopsy samples were retrieved from the archive and were reevaluated by two pathologists.

Results

Between January 1, 2010 and April 30, 2014 (52 months observation period) 100 consecutive new adult IgAV cases fulfilling the CHCC Nomenclature of Vasculitides definition of IgAV were identified. There were 60 males and 40 females. Median age was 63.2 years (range 18–92, interquartile range (IQR) 40.1–77.2). 4/100 patients were ≤ 20 years old at disease presentation. The mean symptom duration was 14 days (range 1–150). Purpura was present in all cases, necrotic in 46/100 and bullous in 11/100 cases. Joints (arthralgia or arthritis) were involved in 46/100, gastrointestinal tract in 38/100, and kidneys in 58/100 patients. General symptoms were present in 18/100 cases. In all patients IgA deposition in vessel walls was documented on direct immunofluorescence staining. Leucocytoclastic vasculitis was observed in 97/100 cases. Granulocytes in vessel wall were found in 85/100 cases. The diagnostic sensitivity of the EULAR/PRINTO/PRES classification criteria was 100 %, and 90 % for the ACR classification criteria (Figure 1)

Figure 1. Venn diagram of fulfilled classification criteria items.

Conclusion

Our study supports the use of the 2010 EULAR/PRINTO/PRES criteria not only in children but also in the adult population and show that they are more sensitive than the 1990 American College of Rheumatology IgA vasculitis criteria..

References

1. Ozen S, Pistorio A, Iusan SM, et al. EULAR/PRINTO/PRES criteria for Henoch-Schonlein purpura, childhood polyarteritis nodosa, childhood Wegener granulomatosis and childhood Takayasu arteritis: Ankara 2008. Part II: Final classification criteria. Ann Rheum Dis 2010;69:798-806.

2. Mills JA, Michel BA, Bloch DA, et al. The American College of Rheumatology 1990 criteria for the classification of Henoch-Schönlein purpura. Arthritis Rheum 1990;33:1114–21.