AB0524 ANCA ASSOCIATED VASCULITIS IN GRAN CANARIA: THE IMPORTANCE OF THE INTERSTITIAL LUNG DISEASE
Authors: F. J. Nóvoa Medina, F. Rubiño, B. Tejera-Segura, B. Romero Díaz, S. Machín García, I. Rua-Figueroa
Journal: Annals of the Rheumatic Diseases (2020)

Background: ANCA-associated vasculitis (AVV) are a heterogeneous group of systemic diseases that needs a better knowledge and approach due to the high mortality it presents. Objectives: Describe the clinical characteristics of patients with AAV assessed by the Rheumatology services in two university hospitals in Gran Canaria in the last decade, as well as clinical differences between the AAV subtypes. Methods: Characteristics of 34 patients diagnosed with AAV between January 2011 - December 2018 were collected retrospectively. The patients met ACR classification criteria and consensus criteria from Chapell Hill-2012. Variables are compared using the χ2 test for dichotomous variables or the t-Student test for continuos variables. For non-continuous variable, Mann-Whitney U or a logarithmic transformation was used Results: 21 (61.7%) patients were women. We found 14 granulomatosis with polyangeiitis (GPA 41.2%), 10 microscopic polyangeiitis (MPA) and 10 eosinophilic granulomatosis with polyangeiitis (EGP) (29.4%). They presented an average follow-up time (±SD) of 46.3 months (±26.8). Patients with MPA presented an older age at diagnosis and a higher proportion were diagnosed with age&gt; 65 years (p = 0.003). The mean (±SD) of the BVAS index of activity at diagnosis was 15,7 (± 7.9). 80,5% of the patients presented positivity against ANCA: 34,4% c-ANCA and 65.5% (Clinical manifestations in Table 1). TABLE 1. INITIAL CLINICAL MANIFESTATIONS ALL THE PATIENTS (n=34) GPA (n=14) MPA (n=10) EGP (n=10) p value Otolaryngological involvement 13(92.9%) 2(20%) 6(60%) 0.001 Interstitial lung disease 5 (14.7%) 0 5 (50%) 0 Renal involvement 0.027 - Reno-pulmonary syndrome 6 (17,6%) 3 (21.4%) 2 (20%) 1 (10%) - Renal Involvement - glomerulonephritis 10 (29.4%) 3 (21.4%) 6 (60%) 1 (10%) - Basal proteinuria &gt;1 gr/24 hs 13 (38.2%) 4 (28.5%) 7 (70%) 1 (10%) Alveolar pulmonary hemorrhage not associated with renal involvement 2 (5.8%) 0 1 (10%) 1 (10%) Manifestations Peripheral Nervous System 10 (29.4%) 2 (14.2%) 4 (40%) 4 (40%) Cardiomyopathy 3 (8.8%) 0 0 3 (30%) Eye involvement (scleritis/conjunctivitis/keratitis/uveitis) 6 (17.6%) 6 (42.8%) 0 0 0.01 21 (61.7%) patients received cyclophosphamide and 3 (8.8%) patients received rituximab as induction treatment. Azathioprine was the most commonly used maintenance treatment (41,1%). 16 (47%) patients had renal involvement. An improvement in proteinuria was observed, both in GPA (p=0.008) and in MPA (p=0,03) (Renal outcomes in Table 2). No patient received kidney transplant. TABLE 2. RENAL OUTCOMES GPA (n= 6) MPA (n=8) p VALUE Basal serum creatinine, mean (SD), mg/dl 2.07 (1.1) 3.08 (2.06) 0.3934 Basal proteinuria, mean (SD), mg/dl 2264 (1391.5) 2731.2 (1334.7) 0.8348 Last serum Creatinine, mean (SD), mg/dl 2,2 (1.4) 2,1 (1.5) 0.5577 Last proteinuria, mean (SD), mg/dl 485 (457.9) 326 (110.4) 0.4704 Interestingly, 5 patients (14.7%), all of them MPA, presented interstitial lung disease (ILD), 3 of them (60%) prior to systemic involvement (9, 10 and 82 months). 3 patients had an usual interstitial pneumonitis (UIP) pattern, none had a non-specific interstitial pneumonia (NSIP) pattern and two had other patterns. 15 patients had 17 relapses. Five (14.7%) patients had serious infections. Eight (23.5%) patients died: 4 due to progression of ILD, 2 due to vasculitis manifestations. Conclusion: ILD can be considered a relatively frequent manifestation of this group of diseases. A high percentage of patients had recurrences. Mortality remains high in AAV and in our series ILD is a frequent cause of death. Disclosure of Interests: Francisco Javier Nóvoa Medina Speakers bureau: I have been paid as a speaker for a few medical talks, Francisco Rubiño: None declared, Beatriz Tejera-Segura Speakers bureau: I have been paid as a speaker for a few medical talks, Beatriz Romero Díaz: None declared, Sergio Machín García: None declared, Iñigo Rua-Figueroa: None declared