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Background/Aims Reporting of treatment-emergent adverse events (TEAEs) in rheumatoid arthritis (RA) clinical trials can be summarized as exposure-adjusted incidence rates (EAIRs) or exposure-adjusted event rates (EAERs). Censored EAIR (EAIR), weighing exposure up to a patient’s first event, is commonly reported; uncensored EAIR (EAIRu), using total exposure time for all patients, can also be used. For EAIR, exposure time can vary by event. In contrast to EAIR(u), the total number of events are used to calculate EAER. The three methods account for different exposures and/or multiple events, which can impact the outcome evaluation. Studies of filgotinib (FIL) in RA report safety data as EAIR/100 patient-years of exposure (PYE) for TEAEs, which is uncensored. Objective: To describe the outcome of long-term FIL integrated safety data in RA by applying different statistical methodologies: EAER, EAIRu and EAIR. Methods Integrated FIL safety data from seven clinical trials were assessed. Predefined adverse events of special interest (AESI) included serious infections (any), herpes zoster (HZ), major adverse cardiac events (MACE), malignancies (excluding nonmelanoma skin cancer [NMSC]), NMSC and venous thromboembolism (VTE). The number of patients with an event, number of events, EAER, EAIRu and EAIR were summarized. The data extraction date was January 2021 for the DARWIN 3 (NCT02065700) long-term extension (LTE) and November 2020 for the FINCH 4 (NCT03025308) LTE. Results In total, 3691 patients received ≥1 FIL dose for 8085 PYE. In this population, 176 serious infections were reported in 137 patients, 125 HZ events were reported in 112 patients, 39 MACE were reported in 33 patients, 20 cases of VTE were reported in 15 patients, 60 malignancies excluding NMSC were reported in 49 patients and 21 cases of NMSC were reported in 20 patients. Within each treatment arm (FIL 200 mg [FIL200], FIL 100 mg [FIL100] or combined FIL), rates for most AESI were similar when reported as EAER, EAIRu or EAIR. For serious infections, EAER was higher than EAIRu or EAIR. The total exposure time to first event (censored PYE) was high and comparable to total exposure (PYE) (>2700 years and >5100 years for the total populations in the FIL100 and FIL200 groups, respectively). Conclusion These data confirm that using different methods to analyze FIL safety data (EAER, EAIRu, EAIR) does not result in different safety outcomes, reinforcing the previously reported FIL safety profile in patients with RA. As the AESI reported in the long-term safety database with FIL are rare, patients commonly have long exposure times before experiencing an event, which are often associated with end of treatment. As such, EAIRu, EAIR and EAER are similar. Disclosure P. Durez: Member of speakers’ bureau; AbbVie, Galapagos, Lilly. E. Feist: Consultancies; AbbVie, Galapagos, Lilly, Novartis, Pfizer, Roche, and Sobi. Member of speakers’ bureau; AbbVie, Galapagos, Lilly, Pfizer, Roche, Novartis and Sobi. Grants/research support; Lilly, Pfizer and Roche. R. Blanco: Consultancies; AstraZeneca, Galapagos, Janssen, Novartis and Pfizer. Member of speakers’ bureau; AbbVie, Amgen, Bristol-Myers, Galapagos, Janssen, Lilly, MSD, Novartis, Pfizer, Roche and Sanofi. Grants/research support; AbbVie, Roche. V. Rajendran: Other; Galapagos. N. Verbruggen: Other; Galapagos. K. Van Beneden: Shareholder/stock ownership; Galapagos. Other; Galapagos. J. Galloway: Consultancies; AbbVie, Galapagos, Gilead, Janssen, Lilly, Novartis and Pfizer. Member of speakers’ bureau; AbbVie, Biogen, Galapagos, Gilead, Janssen, Lilly, Novartis, Pfizer, Roche and UCB. Grants/research support; AstraZeneca, Celgene, Gilead, Janssen, Medicago, Novavax and Pfizer.

Background/Aims In PREVENT (NCT02696031), secukinumab decreased sacroiliac joints (SIJ) bone marrow oedema (BMO) in patients with non-radiographic axial spondyloarthritis (nr-axSpA) through 52 weeks. We report radiographic progression and inflammation (BMO) assessed by X-ray and magnetic resonance imaging (MRI) of SIJ and spine over 2 years in PREVENT. Methods Patients (N = 555) were randomised (1:1:1) to secukinumab 150 mg, with loading dose (LD) or without (NL), or placebo. Switching to open-label secukinumab or standard of care (SoC) was permitted after Week (W) 20. By W52, all patients received open-label secukinumab or SoC. SIJ and spinal radiographs were collected at baseline and W104 and SIJ and spinal MR scans at baseline, W16, W52 and W104. Spinal radiographs were scored using the modified Stoke Ankylosing Spondylitis Spine Score (mSASSS) and SIJ radiographs using modified New York criteria (mNYC). SIJ and spinal BMO was assessed using Berlin Active Inflammatory Lesions Scoring and Berlin MRI scoring, respectively. Average scores of two blinded central readers were used. W104 reading data are presented as observed. Results Radiographs showed most patients (88% secukinumab, 86% placebo) had no SIJ progression (defined as change ≤ smallest detectable change [SDC, 0.46] in total mNYC score) by W104; no patient had an increase ≥2. At W104, screening radiographs of eligible patients were re-evaluated alongside post-baseline images; ∼25% of screening radiographs (68/277 secukinumab; 34/139 placebo) were re-evaluated as mNYC-positive by > 1 reader. Of these, 11/68 patients in the secukinumab (16.2%) and 5/34 in the placebo (14.7%) groups were evaluated as mNYC-negative at W104. Most patients who were mNYC-negative at screening remained mNYC-negative through W104 (202 secukinumab [96.7%]; 102 placebo [97.1%]). Almost all patients (98%) treated with secukinumab 150 mg (pooled LD and NL) showed no structural progression (change in total mSASSS ≤ SDC, [80% agreement level] of 0.76 over 2 years. At baseline, 62 patients (43 secukinumab, 19 placebo) presented with ≥1 syndesmophyte (≥1 vertebral unit scored by ≥ 1 reader). Of those 16 (9 secukinumab [20.9%]; 7 placebo [36.8%]) developed ≥1 new syndesmophyte by W104. Whilst of 354 patients (237 secukinumab, 117 placebo) without syndesmophytes at baseline, only 8 (4 secukinumab [1.7%]; 4 placebo [3.4%]) developed ≥1 new syndesmophyte by W104. Secukinumab reduced SIJ BMO score at W16 (-1.2) and W52 (-1.5) with sustained reduction through W104 (-1.7) in the overall population; greater reduction was observed in patients with baseline score >2 (W16, -4.0; W52, -4.7; W104, -5.4). Baseline spinal inflammation on MRI was low (mean Berlin score: 0.82, secukinumab; 1.07, placebo) with no meaningful change up to W104 (0.56, secukinumab). Conclusion Most patients showed no radiographic progression through 2 years. There was some discrepancy between SIJ eligibility and efficacy reads. Secukinumab reduced SIJ inflammation (BMO) on MRI in patients with active nr-axSpA. Disclosure H. Marzo-Ortega: Consultancies; H.M.O. has received consultancy fees from AbbVie, Celgene, Eli Lilly, Janssen, Novartis, Pfizer, Takeda and UCB. Member of speakers’ bureau; H.M.O. has received speakers bureau fees from AbbVie, Celgene, Eli Lilly, Janssen, Novartis, Pfizer, Takeda and UCB. Grants/research support; H.M.O. has received grant/research support from Janssen, Novartis and UCB. J. Braun: Consultancies; J.B. has received consultancy fees from AbbVie (Abbott), Amgen, BMS, Boehringer Ingelheim, Celgene, Celltrion, Centocor, Chugai, Eli Lilly, Medac, MSD (Schering-Plough), Mundipharma, Novartis, Pfizer (Wyeth), Roche, Sanofi-Aventis and UCB. Member of speakers’ bureau; J.B. has received speakers bureau fees from AbbVie (Abbott), Amgen, BMS, Boehringer Ingelheim, Celgene, Celltrion, Centocor, Chugai, Eli Lilly, Medac, MSD (Schering-Plough), Novartis, Pfizer (Wyeth), Roche, Sanofi-Aventis and UCB. Grants/research support; J.B. has received grant/research support from AbbVie (Abbott), Amgen, BMS, Boehringer Ingelheim, Celgene, Celltrion, Centocor, Chugai, Eli Lilly, Medac, MSD (Schering-Plough), Mundipharma, Novartis, Pfizer (Wyeth), Roche, Sanofi-Aventis and UCB. R. Blanco: Member of speakers’ bureau; R.B. has received speakers bureau fees from AbbVie, Bristol Myers Squibb, Eli Lilly, Janssen, MSD, Pfizer, Roche and UCB. Grants/research support; R.B. has received grant/research support from AbbVie, MSD and Roche. L.S. Gensler: Consultancies; L.S.G. has received consultancy fees from AbbVie, Eli Lilly, Gilead, Janssen, MoonLake, Novartis, Pfizer and UCB. Grants/research support; L.S.G. has received grant/research support from Janssen, Novartis and UCB. F. van den Bosch: Consultancies; F.v.d.B. has received consultancy fees from AbbVie, BMS, Celgene, Eli Lilly, Galapagos, Janssen, Merck, Novartis, Pfizer and UCB. Member of speakers’ bureau; F.v.d.B. has received speakers bureau fees from AbbVie, BMS, Celgene, Eli Lilly, Galapagos, Janssen, Merck, Novartis, Pfizer and UCB. Grants/research support; F.v.d.B. has received grant/research support from AbbVie, BMS, Celgene, Eli Lilly, Galapagos, Janssen, Merck, Novartis, Pfizer and UCB. S. Hall: Member of speakers’ bureau; S.H. has received speakers bureau fees from Eli Lilly, Janssen, Merck, Novartis, Pfizer and UCB; consultancy fees from Eli Lilly, Janssen, Merck, Novartis, Pfizer and UCB. Grants/research support; S.H. has received and grant/research support from AbbVie, Janssen, Merck and UCB. H. Kameda: Consultancies; H.K. has received consultancy fees from AbbVie, Eli Lilly, Gilead Sciences, Janssen, Novartis, Pfizer, Sanofi, Taisho and UCB. Member of speakers’ bureau; H.K. has received speakers bureau fees from AbbVie, Asahi-Kasei, AstraZeneca, Boehringer Ingelheim, BMS, Chugai, Eisai, Eli Lilly, Janssen, Mitsubishi-Tanabe, Novartis and Pfizer. Grants/research support; H.K. has received grant/research support from AbbVie, Asahi-Kasei, Boehringer Ingelheim, Chugai, Eisai, Mitsubishi Tanabe and Taisho. D. Poddubnyy: Consultancies; D.P. has received consultancy fees from AbbVie, Biocad, BMS, Eli Lilly, Gilead, MSD, Novartis, Pfizer, Samsung Bioepis and UCB. Member of speakers’ bureau; D.P. has received speakers bureau fees from AbbVie, BMS, Eli Lilly, MSD, Novartis, Pfizer and UCB. Grants/research support; D.P. has received grant/research support from AbbVie, Eli Lilly, MSD, Novartis and Pfizer. M.G.H. van de Sande: Consultancies; M.G.H.v.d.S. has received consultancy fees from AbbVie, Eli Lilly, Novartis and UCB. Member of speakers’ bureau; M.G.H.v.d.S. has received speakers bureau fees from Novartis and UCB. Grants/research support; M.G.H.v.d.S. has received grant/research support from Eli Lilly, Janssen, Novartis and UCB. D. van der Heidje: Other; D.v.d.H. has worked as a paid instructor for AbbVie, Bayer, BMS, Cyxone, Eisai, Eli Lilly, Galapagos, Gilead, Glaxo-Smith-Kline, Janssen, Novartis, Pfizer and UCB; and as a Director of Imaging Rheumatology at BV. T. Zhuang: Shareholder/stock ownership; T.Z. is a shareholder of Novartis. Other; T.Z. is an employee of Novartis. A. Stefanska: Shareholder/stock ownership; A.S. is a shareholder of Novartis. Other; A.S. is an employee of Novartis. A. Readie: Shareholder/stock ownership; A.R. is a shareholder of Novartis. Other; A.R. is an employee of Novartis. H. Richards: Shareholder/stock ownership; H.R. is a shareholder of Novartis. Other; H.R. is an employee of Novartis. A. Deodhar: Consultancies; A.D. has received consultancy fees from AbbVie, Amgen, Boehringer Ingelheim, Bristol Myers Squibb, Celgene, Eli Lilly, GSK, Janssen, Novartis, Pfizer and UCB. Member of speakers’ bureau; A. D. has received speakers bureau fees from AbbVie, Boehringer Ingelheim, Eli Lilly, Janssen, Novartis, Pfizer and UCB. Grants/research support; A.D. has received grant/research support from AbbVie, Eli Lilly, GSK, Novartis, Pfizer and UCB.

Objective To assess the efficacy and safety of abatacept (ABA) in monotherapy (ABAMONO) vs combined ABA [ABA plus MTX (ABAMTX) or ABA plus non-MTX conventional synthetic DMARDs (csDMARDs) (ABANON-MTX)] in RA patients with interstitial lung disease (ILD) (RA-ILD). Methods This was a restrospective multicentre study of RA-ILD Caucasian patients treated with ABA. We analysed in the three groups (ABAMONO, ABAMTX, ABANON-MTX) the following outcome variables: (i) dyspnoea; (ii) forced vital capacity (FVC) and diffusion capacity of the lung for the carbon monoxide (DLCO); (iii) chest high-resolution CT (HRCT); (iv) DAS28-ESR; (v) CS-sparing effect; and (vi) ABA retention and side-effects. Differences between basal and final follow-up were evaluated. Multivariable linear regression was used to assess the differences between the three groups. Results We studied 263 RA-ILD patients (mean ± s.d. age 64.6 ± 10 years) [ABAMONO (n = 111), ABAMTX (n = 46) and ABANON-MTX (n = 106)]. At baseline, ABAMONO patients were older (67 ± 10 years) and took higher prednisone dose [10 (interquartile range 5–15) mg/day]. At that time, there were no statistically significant differences in sex, seropositivity, ILD patterns, FVC and DLCO, or disease duration. Following treatment, in all groups, most patients experienced stabilization or improvement in FVC, DLCO, dyspnoea and chest HRCT as well as improvement in DAS28-ESR. A statistically significant difference between basal and final follow-up was only found in CS-sparing effect in the group on combined ABA (ABAMTX or ABANON-MTX). However, in the multivariable analysis, there were no differences in any outcome variables between the three groups. Conclusion In Caucasian individuals with RA-ILD, ABA in monotherapy or combined with MTX or with other conventional-DMARDs seems to be equally effective and safe. However, a CS-sparing effect is only observed with combined ABA.

Objective Because carotid plaques predict the development of cardiovascular events in RA, we aimed to assess if the combined use of the systematic coronary risk evaluation (SCORE) and the QRISK3 algorithms allows for the identification of RA patients with carotid plaques in a defined population-based RA inception cohort. Methods A set of consecutive RA patients without a history of diabetes, chronic kidney disease or cardiovascular events were studied by carotid US between 2012 and 2019. Modified SCORE (mSCORE) for RA based on the 2015/2016 updated EULAR recommendations and QRISK3 algorithms were retrospectively tested using baseline data obtained at the time of the carotid US assessment. Results A total of 466 (54%) of 865 patients had carotid plaques. Using dichotomized QRISK3 and EULAR mSCORE, 73.2% (95% CI: 68.4.8, 77.6) of patients with QRISK ≥ 10% and EULAR mSCORE Conclusions . The combined use of QRISK3 and EULAR mSCORE allows for the identification of most RA patients at high risk of carotid plaques.

Background/Aims MAXIMISE, the first randomised controlled trial evaluating efficacy of a biologic for psoriatic arthritis (PsA) axial manifestations, showed that secukinumab 300 and 150 mg provided rapid and significant improvement in ASAS20 responses through Week 12. We report the effect of secukinumab on clinical and imaging outcomes through 52 weeks. Methods This Phase 3, double-blind, multicentre trial included 498 patients (≥18 years) with PsA who fulfilled CASPAR criteria presenting with spinal pain VAS ≥40/100, BASDAI ≥4 and inadequate response to ≥ 2 non-steroidal anti-inflammatory drugs. Patients were randomised to secukinumab 300 mg (N = 167), 150 mg (N = 165) or placebo (N = 166) weekly for 4 weeks and every 4 weeks thereafter. At Week 12, placebo patients were re-randomised to secukinumab 300/150 mg. The primary endpoint was ASAS20 with secukinumab 300 mg at Week 12. Exploratory assessments at Week 52 included ASAS20/40, BASDAI50, spinal pain (VAS) and improvement in Berlin magnetic resonance imaging (MRI) score for spine and sacroiliac joints. Results The primary endpoint was met. ASAS20/40 responses at Week 12 were 62.9%/43.6% (secukinumab 300 mg) and 66.3%/39.5% (secukinumab 150 mg) versus 31.2%/12.2% (placebo), respectively (P < 0.0001). ASAS20/40 responses improved further with secukinumab 300/150 mg from baseline through 52 weeks. 74.1%/74.7% and 63.0%/50.6% of placebo patients, re-randomised at Week 12 to secukinumab 300/150 mg, achieved ASAS20/40 at Week 52. At baseline, 59.5% (secukinumab 300 mg), 54.2% (secukinumab 150 mg) and 64.2% (placebo) of patients had positive MRI scores for the sacroiliac joints and/or the spine. Reductions in Berlin MRI scores for the entire spine and sacroiliac joints were sustained with secukinumab 300/150 mg from baseline through 52 weeks (Table 1). 64.6%, 69.1% and 33.6% of patients with inflammatory back pain at baseline, confirmed by ASAS, Calin et al. and Berlin criteria in the secukinumab 300 mg, 150 mg and placebo groups, respectively, achieved ASAS20 at Week 12. P184 Table 1:Endpoints at Week 52CriteriaSecukinumab 300 mg SC (N = 164)Secukinumab 150 mg SC (N = 157)Placebo to secukinumab 300 mg SC (N = 81)Placebo to secukinumab 150 mg SC (N = 80)Clinical endpointsASAS20, % responders (n/M)a75.5 (123/163)77.3 (119/154)74.1 (60/81)74.7 (59/79)ASAS40, % responders (n/M)a62.6 (102/163)60.4 (93/154)63.0 (51/81)50.6 (40/79)BASDAI50, % responders (n/M)b68.3 (95/139)58.5 (83/142)55.6 (40/72)54.1 (40/74)Spinal pain VAS, mean change from BL (SD), nb-42.4 (27.0), 140-43.8 (26.2), 142-43.1 (25.0), 72-36.4 (25.2), 74Imaging endpointBerlin MRI score for entire spine, mean change from BL (SD), nb-0.6 (2.3), 121-0.3 (1.3), 124-0.8 (2.7), 63-0.4 (1.3), 60Berlin MRI score for SIJ, mean change from BL (SD), nb-0.7 (2.2), 122-0.5 (1.7), 122-0.9 (2.4), 63-1.0 (2.7), 59N=total number of patients in the group; n=number of patients with response; M=number of evaluable patients. aIntermediate missing data as well as any data missing in the case of study discontinuation is imputed using LOCF; bObserved data. Patients with initial placebo treatment were re-randomised to secukinumab 300 or 150 mg at Week 12. ASAS, Assessment of SpondyloArthritis International Society; BASDAI, Bath Ankylosing Spondylitis Disease Activity Index; BL, baseline; LOCF, last observation carried forward; MRI, magnetic resonance imaging; SC, subcutaneous; SD, standard deviation; SIJ, sacroiliac joints; VAS, visual analogue scale. Conclusion Secukinumab improved signs and symptoms of axial disease (ASAS20/40) through 52 weeks with reduced inflammatory MRI lesions in the spine and sacroiliac joints in PsA patients with axial manifestations. Efficacy at Week 52 was comparable in patients who switched at Week 12 from placebo to secukinumab 300/150 mg. Disclosure X. Baraliakos: Consultancies; AbbVie, BMS, Celgene, Chugai, Merck, Novartis, Pfizer, UCB, Werfen. Member of speakers’ bureau; AbbVie, BMS, Celgene, Chugai, Merck, Novartis, Pfizer, UCB. Grants/research support; AbbVie, Novartis. L. Gossec: Consultancies; AbbVie, Amgen, Biogen, Celgene, Janssen, Lilly, Novartis, Pfizer, Sandoz, Sanofi-Aventis, UCB. Grants/research support; Lilly, Mylan, Pfizer, Sandoz. E. Pournara: Corporate appointments; Employee of Novartis. Shareholder/stock ownership; Novartis stock. S. Jeka: Grants/research support; AbbVie, Pfizer, Roche, Novartis, MSD, Sandoz, Lilly, Egis, UCB, Celgene. R. Blanco: Consultancies; AbbVie, Lilly, Pfizer, Roche, Bristol-Myers, Janssen, UCB Pharma, MSD. Grants/research support; AbbVie, MSD, Roche. S. D'Angelo: Consultancies; AbbVie, Biogen, BMS, Celgene, Lilly, MSD, Novartis, UCB. Member of speakers’ bureau; AbbVie, BMS, Celgene, Lilly, Novartis, Pfizer, Sanofi. G. Schett: Honoraria; AbbVie, BMS, Celgene, Janssen, Lilly, Novartis, Roche, UCB. B. Schulz: Corporate appointments; Employee of Novartis. M. Rissler: Corporate appointments; Employee of Novartis. Shareholder/stock ownership; Novartis stock. D. Whyms: Corporate appointments; Employee of Novartis. C. Perella: Corporate appointments; Employee of Novartis. Shareholder/stock ownership; Novartis stock. L.C. Coates: Consultancies; : AbbVie, Amgen, Biogen, Celgene, Pfizer, UCB, Boehringer Ingelheim, Novartis, Lilly, Janssen, Sun Pharma, Prothena, Gilead. Grants/research support; AbbVie, Janssen, Lilly, Novartis, Pfizer, Amgen.

Objective: To assess the efficacy of tocilizumab (TCZ) in refractory uveitis of Behcet's disease (BD). Methods: Multicentre study of patients with BD-associated uveitis. Patients were refractory to conventional and biologic immunosuppressive drugs. The main outcome measures were intraocular inflammation, macular thickness, visual acuity and corticosteroid-sparing effects. Results: We studied 11 patients (7 men) (20 affected eyes); median age 35 years. Uveitis was bilateral in nine patients. The patterns of ocular involvement were panuveitis (n = 8, with retinal vasculitis in 4), anterior uveitis (n = 2) and posterior uveitis (n = 1). Cystoid macular oedema was present in seven patients. The clinical course was recurrent (n = 7) or chronic (n = 4). Before TCZ, patients had received systemic corticosteroids, conventional immunosuppressants and the following biologic agents: adalimumab (n = 8), infliximab (n = 4), canakimumab (n = 1), golimumab (n = 3), etanercept (n = 1). TCZ was used as monotherapy or combined with conventional immunosuppressants at 8 mg/kg/i.v./4 weeks (n = 10) or 162mg/s.c./week (n = 1). At TCZ onset the following extraocular manifestations were present: oral and/or genital ulcers (n = 7), arthritis (n = 4), folliculitis/pseudofolliculitis (n = 4), erythema nodosum (n = 2), livedo reticularis (n = 1) and neurological involvement (n = 2). TCZ yielded rapid and maintained improvement in all ocular parameters of the patients, with complete remission in eight of them. However, this was not the case for the extraocular manifestations, since TCZ was only effective in three of them. After a mean (S.D.) follow-up of 9.5 (8.05) months, TCZ was withdrawn in two cases, due to a severe infusion reaction and arthritis impairment, respectively. Conclusion: TCZ could be a therapeutic option in patients with BD and refractory uveitis.

Objective Cutaneous vasculitis (CV) encompasses a wide group of entities characterized by inflammation of skin blood vessels. The term single-organ vasculitis was recently coined by the 2012 Chapel Hill Consensus Conference (CHCC) to define vasculitis affecting a single organ. To our knowledge there are no published reports on single-organ cutaneous small vessel vasculitis (SoCSVV). Our aim was to characterize this entity from a wide series of patients with CV. Methods We analysed cases of SoCSVV from a series of 766 patients with CV from a single university referral centre. According to 2012 CHCC, the following conditions were required to define SoCSVV: (i) skin biopsy showing characteristic leucocytoclastic vasculitis and (ii) vasculitis limited to skin. Results We included 60 patients (26 women and 34 men) with a mean age of 56 years. The main precipitating factors for SoCSVV were drugs [26 patients (52%)] and previous infection [17 patients (34%)]. The main clinical manifestations were palpable purpura (81.7%) and fever (18.3%). The most frequent laboratory findings were leucocytosis and elevated ESR. Nearly one-quarter of patients with SoCSVV required pharmacological therapy. Corticosteroids (15%) and NSAIDs (13.3%) were the main agents prescribed. After a median follow-up of 4 months, complete recovery was observed in all the patients, although relapses occurred in 8% of patients. Conclusion SoCSVV defined according to the 2012 CHCC may be considered a benign disease usually associated with drugs and/or a previous infection.

OBJECTIVE: The aim of this study was to assess the efficacy of anti-TNF-α therapy in refractory uveitis due to Behcet's disease (BD). METHODS: We performed a multicentre study of 124 patients with BD uveitis refractory to conventional treatment including high-dose corticosteroids and at least one standard immunosuppressive agent. Patients were treated for at least 12 months with infliximab (IFX) (3-5 mg/kg at 0, 2 and 6 weeks and then every 4-8 weeks) or adalimumab (ADA) (usually 40 mg every 2 weeks). The main outcome measures were degree of anterior and posterior chamber inflammation, visual acuity, macular thickness and immunosuppression load. RESULTS: Sixty-eight men and 56 women (221 affected eyes) were studied. The mean age was 38.6 years (s.d. 10.4). HLA-B51 was positive in 66.1% of patients and uveitis was bilateral in 78.2%. IFX was the first biologic agent in 77 cases (62%) and ADA was first in 47 (38%). In most cases anti-TNF-α drugs were used in combination with conventional immunosuppressive drugs. At the onset of anti-TNF-α therapy, anterior chamber and vitreous inflammation was observed in 57% and 64.4% of patients, respectively. In both conditions the damage decreased significantly after 1 year. At baseline, 50 patients (80 eyes) had macular thickening [optical coherence tomography (OCT) >250 μm] and 35 (49 eyes) had cystoid macular oedema (OCT>300 μm) that improved from 420 μm (s.d. 119.5) at baseline to 271 μm (s.d. 45.6) at month 12 (P < 0.01). The best-corrected visual acuity and the suppression load also showed significant improvement. After 1 year of follow-up, 67.7% of patients were inactive. Biologic therapy was well tolerated in most cases. CONCLUSION: Anti-TNF-α therapy is effective and relatively safe in refractory BD uveitis.

Objective. Coding variants in Toll-like receptor 4 (TLR4) have been reported to be associated with inflammatory diseases. The aim of this study was to determine whether two of these polymorphisms (+896 A/G and +1196 C/T) are associated with susceptibility and clinical features of GCA. We also attempted to correlate the functional consequences of these polymorphisms. Methods. A total of 72 patients with GCA and 126 age-matched controls were genotyped using allele-specific PCR and restriction fragment length polymorphism analysis. TLR4 expression was studied on peripheral blood mononuclear cells by flow cytometry and TLR4 function was assessed by stimulating monocytes in vitro with a specific ligand. Results. There was no significant difference in allele frequency or genotype of TLR4 (+896 A/G and +1196 C/T) between GCA patients and controls. The clinical characteristics of these patients were unrelated to the presence of these polymorphisms. Furthermore, we did not observe an association with TLR4 expression or a distinct phenotype of TLR4 response with the +896 A/G and +1196 C/T genotypes. Conclusion. Our results do not support the association of these TLR4 variants with GCA. Studies including a larger number of patients and patient populations from different geographical origin are needed.

The objective was to assess the efficacy of therapy with danazol in refractory immune thrombocytopenia associated with different rheumatic diseases. Patients with severe immune thrombocytopenia (platelet counts40 x 10(9)/l) with a bone marrow biopsy showing megakaryocytes in normal or increased number and normal morphology were included if they fulfilled at least one of the following criteria: (a) thrombocytopenia refractory to prednisone (or = 1 mg/kg/day duringor = 4 weeks); (b) patients requiring an unacceptably high dose of prednisone for2 months (prednisone doseor = 20 mg/day); (c) no response to at least another drug besides corticosteroids. Other causes of thrombocytopenia were excluded. They were treated with danazol (100-200 mg q.i.d.) and followed for at least 12 months. Four patients diagnosed with systemic lupus erythematosus, two with rheumatoid arthritis and one with primary antiphospholipid syndrome met the inclusion criteria. All of them achieved acceptable platelet counts within the first 4 weeks of danazol therapy that allowed the prednisone dosage to be tapered. No important side-effects related to danazol therapy were observed. Danazol therapy seems to be a useful and well-tolerated treatment for refractory immune thrombocytopenia associated with different rheumatic diseases.