AB0741 Severe granulomatosis with polyangiitis in argentinian patients, clinical diferences with severe patients described in wegener etanercept trial
Authors: A. Allievi, A.O. Orden, S.A. Muñoz, M.C. Basta, A. Kostianovsy, M.C. Furattini
Journal: Annals of the Rheumatic Diseases (2013)

<h3>Background</h3> The EUVAS group classified patients using the following disease states:localized, early systemic, generalized disease and severe renal involvement. WGET group introduced a distinction between limited and severe disease on the basis of consensus definitions <h3>Objectives</h3> To report clinical characteristics of 37 Argentinian patients with severe granulomatosis with polyangiitis (SGPA) and to explore their differences with severe patients described in Wegener Etanercept Trial (WGET) (1) <h3>Methods</h3> We performed a retrospective study that included 37 consecutive SGPA patients seen at two tertiary centers in Buenos Aires. <i>Eligibility:</i> All patients included in the study met at least two of the five modified classification criteria of the ACR. <i>Disease assessment:</i> Disease activity was measured at baseline by means of the modified BVAS/WG. <i>Definitions:</i> Organ involvement definition was based on clinical and/or laboratory criteria, according the “EULAR Recommendations”. <i>Statistical analysis:</i> In order to explore differences between the actual 37 case series and the 128 severe patients reported in WGET trial, one-sample Z test of proportion was used. The proportions reported in the WGET trial were considered as population parameter <h3>Results</h3> Mean age was 48.5 (± 12.01) years, 51.3% were male. Follow up 6.2 (± 5.7) years, C –ANCA was positive in 91,9%, and P-ANCA in 5.4%. BVAS/WG mean was 22.8±5.7. 14/37 patients died, (37.8%), six during the first month of follow up.Patients over 50 years old had double mortality rate.Respiratory failure was present in 27%, and end stage renal disease in 10.8%. Regarding treatments 37 patients received monthly glucocorticoids (GC) pulses and oral or endovenous cyclophosphamide until remission. Thirty-one patients (83%) reached remission; 24 patients complete remission, and 7 patients (19%) partial remission. Seven patients with refractory disease required additional treatments such as IVIG, dialysis, plasma exchange and rituximab. Patients who achieved remission received maintenance therapy (steroids and azathioprine or methotrexato or sodium mycophenola) WGET % -n:128-/vs. Present work % -n:37- (P value) Constitutional symptoms 70.3%/89.18% (0.0001) Alveolar hemorrhage 25%/54.5% (0.0001) Arthralgias/arthritis 64.8%/37.83% (0.0001) Ear, nose, throat. (ENT) 71.9%/48.64% (0.003) Renal damage 64.1%/81.08% (0.005) Oral ulcers 11.7%/0.00% (0.006) Scleritis 2.3%/18.91% (0.006) Cutaneous 21.9%/40.54% (0.012) <h3>Conclusions</h3> With this presentation we should like to rebound disagreement between different series of severe patients even though our patients were collected in a retrospective analysis and compared with a referral trial. Our patients presented more alveolar hemorrhage and renal damage and less granulomatous lesions than WGET patients <h3>References</h3> Limited Versus Severe Wegener’sGranulomatosis J.H.Stome MD Arthritis &amp; Rheum Vol 48,N 8 August 2003;2299-2309 <h3>Disclosure of Interest</h3> None Declared