ANCA-associated vasculitides (AAV) from the gender point of view

Although renal disease was common only a minority required long-term renal replacement therapy.It is not over-represented among indigenous people.Conclusion.– AASV is an uncommon. The morbidity and mortalityremains significant.

Reference[1] Watts RA et al. Ann Rheum Dis 2007;66:222-7.

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P49Microscopic polyangiitis in the setting of usualinterstitial pneumoniaK. Keogh1, M. Baqir1, U. Specks1, G. Aughenbaugh1, G. Battaglia2,F. Londrino2, A. Tironi2, S. Monti2, G. Gina2

1. Mayo Clinic, Rochester, Mn, USA2. University of Brescia, Brescia, Italy

Introduction.– Usual interstitial pneumonia (UIP) is a specific radio-graphic and histolopathologic pattern of interstitial lung disease. WhenUIP occurs in isolation (UIP-IPF), it has a poor prognosis. UIP can occur inassociation with connective tissue disease and has been reported invasculitis. The relationship between microscopic polyangitis (MPA) andUIP remains ill defined.Methods.– We conducted a collaborative observational cohort studybetween two tertiary referral centers. Directed search strategies of clinicaldatabases identified patients with concurrent diagnoses of UIP and MPAbetween 1989 and 2011. Inclusion criteria for UIP were: radiographicfindings and/or biopsy evidence of UIP. The MPA diagnosis was basedon the Chapel Hill Consensus Conference definition. We describe the clinicalfeatures including radiographic findings and survival data in this cohort.Results.– We identified 68 patients with a diagnosis of both MPA andUIP. MPA was diagnosed first in nine patients, UIP in 14, and in 45, thediagnoses were made concurrently. Mean age was 68 � 10 years.Sixty-two of 67 were P-ANCA positive. One was C-ANCA positive. MeanBVAS/WG at diagnosis 4.9 � 3.3. Seventy-eight percent had renalinvolvement. Twenty-three percent had alveolar hemorrhage con-firmed (n = 12) or suspected (n = 3). Forty of 68 received cyclopho-sphamide. Median survival from diagnosis of UIP in patients whoreceived cyclophosphamide was 138.2 months (92–175) versus 51.0months (28–86) for those who did not (P = 0.0008). The use ofcyclophosphamide was associated with a 0.32 fold lower risk of death(OR 0.32; 95%CI 0.16–0.64).Discussion.– In this cohort of UIP and MPA, the two diagnoses wereestablished concurrently in most cases, suggesting that UIP changesusually develop first. Lack of specific data on causes of death is a limitationof our study.Conclusion.– Our survival data suggest that in contrast to UIP-IPF,immunosuppression improves prognosis in patients with UIP in thesetting of MPA. Whether UIP is a possible disease manifestation of MPAor an unrelated co-occurrence remains unclear.

http://dx.doi.org/10.1016/j.lpm.2013.02.120

P50Incidence of granulomatosis with polyangiitis(Wegener’s) among GreenlandersM. Faurschou, M. Helleberg, N. Obel, B. BaslundDepartment of Infectious Diseases and Rheumatology, CopenhagenUniversity Hospital Rigshospitalet, Copenhagen, Denmark

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Introduction.– Previous studies suggest that the incidence of granulo-matosis with polyangiitis (Wegener’s; GPA) increases along a south-north gradient in the northern hemisphere with an incidence rate of8.0/million/year reported for the population of northern Norway. Inthe present study, we estimated the incidence of GPA among personsborn in Greenland.Methods.– Greenlandic patients with severe rheumatic diseases areroutinely referred to university hospital departments in Denmark fortreatment. The Danish National Hospital Register was established in1977 and contains information on all admissions to Danish hospitals. Inthe Register, we identified all patients treated under a diagnosis of GPAduring 1977–2011. Those born in Greenland were identified throughthe Danish Civil Registration System. For each Greenlandic-born patient,the GPA diagnosis was validated by medical files review.Results.– Three Greenlandic GPA patients were identified. Two deve-loped GPA in Greenland, and one developed GPA while living inDenmark. The mean annual incidence of GPA among Greenlandersliving in Greenland was 1.2/million (95% CI 0.1–4.4) with no differ-ences observed between genders. Annual incidences of 0.8/million(95% CI 0.02–4.4) and 2.8/million (95% CI 0.07–15.4) were calculatedfor the age groups 0–44 and 45–99 years, respectively. The annualincidence of GPA among Greenlanders living in Denmark was estimatedto be 2.8/million (95% CI 0.07–15.5).Conclusion.– The occurrence of GPA is lower in the predominantly Inuitpopulation of Greenland than among Caucasians living in arctic parts ofNorway. This observation demonstrates that the risk of GPA variesacross ethnic groups populating the northern-most regions of the world.

http://dx.doi.org/10.1016/j.lpm.2013.02.121

P51ANCA-associated vasculitides (AAV) from thegender point of viewA. Radice1, L. Bianchi2, S. Glionna2, B. Trezzi2, R.A. Sinico2

1. Microbiology Institute, San Carlo Borromeo Hospital, Milan, Italy2. Nephrology and Clinical Immunology Unit, San Carlo Borromeo

Hospital, Milan, Italy

Introduction.– The awareness that gender not only influence thesusceptibility to autoimmune diseases (AIDs), but also the course,the response to treatment, the short- & long-term side effects, isincreasingly.AAV are immune-mediated, multi-factorial diseases, in which differentrisk factors act. Different from other AIDs, AAV do not show a clear sex-prevalence. Although several clinical trials have been realized, datahave never been evaluated according to sex.Methods.– Evaluation of published literature.Results.– AAV & pregnancy: because the disease peak is > 50, preg-nancy in , with AAV is rarely observed. According to a recent systematicreview of 567 PSV pregnancies (AAV 79), these conditions should bemanaged very carefully because both, disease complications andtreatments may have negative effects on maternal and foetal health.Long-term survival: in a recent, large EUVAS study, the 1, 2 and 5 yrsurvival of AAV pts treated with current regimens were 88%, 85% &78%, respectively. Compared to an age & sex-matched general popula-tion there was a mortality rate ratio of 2.6. Unfortunately, data werecollected and analysed for the whole group, and no gender-relatedevaluations were available.Stratta et al. reported that pt and renal survival considerably improvedover time. In particular, life expectancy of young , with vasculitis wouldhave improved, approaching that of the matched-general population:

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relative survival (RS) in the cohort of , < 60 was lower in the 1st period(10 yrs = 0.339), increased in the 2nd (0.929), as it did during the latest5 yrs (0.916); the opposite was for <of all ages, showing a RS at 5 yrsfrom 0.491 to 0.546.Long-term side effects: the impact of cyclophosphamide treatment onlong-term ovarian function is poorly understood, despite the extensiveuse of this agent in up to > 80% of , with vasculitis.Conclusion.– All things considered we urgently need to look at theseentities from a gender point of view for the best placement andmanagement of AAV.

http://dx.doi.org/10.1016/j.lpm.2013.02.122

P52Anti-neutrophil cytoplasmic antibodies associatedvasculitis frequency in Dr. Manuel Quintelauniversity hospitalN. Rodriguez Sisniega, E. Araujo, C. Buzzi, N. Garcia, P. Lopez,C. Montenegro, R. Balleste, L. BorcheHospital de Clínicas, Clinical Laboratory department, Inmunology Unit,‘‘Dr. Manuel Quintela’’, Facultad de Medicina, Montevideo, Uruguay

Introduction.– The anti-neutrophil cytoplasmic autoantibodies (ANCA),are directed against constituents of the primary granules of neutrophiland monocyte lysosomes. The mayor antigens, with correlation to ANCAassociated Vasculitis (AAV) that are sought in the currently laboratorypractice are myeloperoxidase (MPO) and proteinase 3 (PR3). There areinteresting differences in the prevalence of AAV between differentgeographical areas, as well as for MPO and PR3-ANCA.Objective.– To determine the number of patients with AAV that under-went ANCA test in our laboratory from 2002 to 2008.Patients.– Study population: Serum samples from patients with ANCArequest by the physician that arrived to the Laboratory-ImmunologyUnit, from March 2002 to December 2008, who were positive byIndirect Immunofluorescence (IIF) and Enzyme Linked ImmunosorbentAssay (ELISA). Then we proceeded to revise the medical records togather histological diagnosis information. Statistical Analysis: datafound were processed by the Microsoft Excel program.Results.– One thousand eight hundred and eighty-eight sera underwentANCA test, 166 (8.8%) met the selection criteria, corresponding to 61patients that were classified in three groups:– AAV (66%);– connective tissue disorders (CTD) (18.9%);– gastro-intestinal disorders (GID) (15.1%).Fifty-one percent of AAV group had histological confirmation, 62.8%were MPO and 37.2% PR3, with a 1.7 ratio.Conclusion:– although the scarce number of samples with AAV, the 1.7 ratio (MPO/PR3) obtained is similar to the Mediterranean results, unlike to thesouthern hemisphere region latitude gradient suggested in other re-ports. A genetic rather than a ‘‘latitude’’ reason may explain thedifferent results obtained;– all patients of our GID group had PR3;– last but not least, like many other reports, our findings remarks theneed of a suitable clinical suspicion of AAV to reach the ANCA’s highpositive predictive value, taking into account that many other non AAVconditions can be positive ANCA.

Further readingsGang Xin, Ming-hui Zhao. Detection rate and antigenic specificities ofAnti-neutrophil Cytoplasmic Antibodies in Chinese patients with clinicallysuspected vasculitis. Clin Diagn Lab Immunol 2004; 11(3):559–562.

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