Background: The vasculitides are a group of rare diseases with different manifestations and outcomes. New therapeutic options have led to the need for long-term registries. The Rheumatic Diseases Portuguese Register, Reuma.pt, is a web-based electronic clinical record, created in 2008, which currently includes specific modules for 12 diseases and > 20,000 patients registered from 79 rheumatology centres. On October 2014, a dedicated module for vasculitis was created as part of the European Vasculitis Society collaborative network, enabling prospective collection and central storage of encrypted data from patients with this condition. All Portuguese rheumatology centres were invited to participate. Data regarding demographics, diagnosis, classification criteria, assessment tools, and treatment were collected. We aim to describe the structure of Reuma.pt/vasculitis and characterize the patients registered since its development. Results: A total of 687 patients, with 1945 visits, from 13 centres were registered; mean age was 53.4 ± 19.3 years at last visit and 68.7% were females. The most common diagnoses were Behçet's disease (BD) (42.5%) and giant cell arteritis (GCA) (17.8%). Patients with BD met the International Study Group criteria and the International Criteria for BD in 85.3 and 97.2% of cases, respectively. Within the most common small-and medium-vessel vasculitides registered, median [interquartile range] Birmingham Vasculitis Activity Score (BVAS) at first visit was highest in patients with ANCA-associated vasculitis (AAV) (17.0 [12.0]); there were no differences in the proportion of patients with AAV or polyarteritis nodosa who relapsed (BVAS≥1) or had a major relapse (≥1 major BVAS item) during prospective assessment (p = 1.00, p = 0.479). Biologic treatment was prescribed in 0.8% of patients with GCA, 26.7% of patients with AAV, and 7.6% of patients with BD. There were 34 (4.9%) deaths reported.

Background Previous studies demonstrated functional and morphological microcirculatory abnormalities that may be relevant to the pathophysiology and clinical manifestations of fibromyalgia (FM). Objectives To ascertain and compare capillary morphology of FM patients with and without Raynaud's phenomenon (RP) using nailfold videocapillaroscopy (NVC). Methods Patients fulfilling the 2010 American College of Rheumatology Diagnostic Criteria for FM were allocated in 2 groups according to the presence or absence of RP (RP+ or RP-, respectively). All patients with secondary causes of RP other than FM were excluded. One blinded operator performed all the NVC using a 200x amplification digital microscope and analytical software. Capillary parameters evaluated in fingers II-V of both hands included: tortuosity, apex enlargement, branch enlargement, microhemorrhages, giant capillaries, capillary density, capillary branching and architectural derangements. Each parameter was rated 0-3 (0= no changes; 1= <33% abnormalities; 2=33-66% abnormalities; 3= >66% abnormalities) and the mean scores were calculated. The association of RP with the capillary parameters was assessed using multivariate linear regression adjusted for age, FM duration and occupation. Results Twenty FM patients were enrolled, 10 in each group, however 2 patients RP+ were excluded due to thyroid disease. Included patients were all female, had a mean age of 50.4 years and a mean duration of disease of 12.8 years. In both RP+ and RP- groups, the most frequently found abnormalities were minor dysmorphies, namely tortuosities, apex enlargement and branch enlargement. However, scoring of these parameters showed no significant difference between the 2 groups. More than half of RP+ and RP- patients presented slight focal decreases in capillary density, once again with no difference between groups. The only giant capillaries were found in a RP+ patient with an “early scleroderma pattern” as proposed by Cutolo et al. Hemorrhages were scarce and had a traumatic appearance, except in the previously mentioned patient. Interestingly, RP associated negatively with the capillary branching score. Table 1 summarizes the results. Table 1. Microcirculatory parameters assessed in fibromyalgia patients with and without Raynaud's phenomenon Parameters RP+, n=8 RP−, n=10 Adjusted p-value n (%) Score, median (IQR) n (%) Score, median (IQR) Tortuosity 8 (100) 1.17 (0.66) 10 (100) 1.25 (0.53) 0.99 Apex enlargement 7 (87.5) 0.31 (0.50) 10 (100) 0.14 (0.06) 0.26 Branch enlargement 7 (87.5) 0.13 (0.33) 10 (100) 0.03 (0.06) 0.06 Microhemorrhages 2 (25) 0 (0.06) 2 (20) 0 (0) 0.20 Giant capillaries 1 (12.5) 0 (0) 0 (0) 0 (0) 0.24 Capillary density 5 (62.5) 0.03 (0.09) 6 (60) 0.08 (0.19) 0.98 Capillary branching 3 (37.5) 0 (0.03) 5 (50) 0.03 (0.13) 0.04 Architectural derangements 2 (25) 0 (0.08) 3 (30) 0 (0.03) 0.27 IQR, interquartile range; RP, Raynaud's phenomenon. Conclusions No association was established between RP and microcirculatory abnormali...

BackgroundThe vasculitides are a group of relatively rare diseases with different manifestations and outcomes. New therapeutic options have led to the need for long-term registries. The Rheumatic Diseases Portuguese Register, Reuma.pt, is an electronic clinical record, created in 2008, which currently includes specific protocols for 11 diseases and >16000 patients registered from 79 national and international rheumatology centres. Since October 2014, a dedicated protocol to vasculitis has been created as part of the European Vasculitis Society initiative of having compatible European registries.ObjectivesTo describe the structure of Reuma.pt/Vasculitis and characterize the patients registered over the last two years.MethodsWe developed a dedicated web-based software to enable prospective collection and central storage of anonymised data from patients with vasculitis. All Portuguese rheumatology centres were invited to participate. Data regarding demographics, diagnosis, classification criteria, imaging and laboratory tests, outcome measures of prognosis, damage, disease activity and quality of life, and treatment were collected. We performed a cross-sectional descriptive analysis of all patients registered up to January 2017.ResultsA total of 492 patients, with 1114 visits, from 11 centres were registered in Reuma.pt/Vasculitis. The mean age was 53±20 years at last visit; 68% were females. The diagnoses followed the 2012 Chapel Hill Consensus nomenclature (Table 1). The most common diagnoses were Behçet's disease (BD) (39%) and giant cell arteritis (GCA) (20%). Patients with BD met the International Study Group 1990 criteria, the International Criteria for BD 2006 and 2013 in 84%, 95% and 95% of cases, respectively. Patients with GCA met the 1990 American College of Rheumatology criteria in 95% of cases. Data on vascular ultrasound was available in 74% of patients; 73% compatible with the diagnosis. Assessment of the Birmingham Vasculitis Activity Score (BVAS) and Vasculitis Damage Index (VDI) was available for all vasculitides and the Five Factor Score calculation of survival rate for ANCA associated vasculitis (AAV) and polyarteritis nodosa (PAN). The mean BVAS at first visit was 18±7 for AAV and 15±9 for PAN; the mean VDI at last visit was 3±2 for AAV and 2±2 for PAN. Health related quality of life assessments (SF-36, EQD5, FACIT and HADS) were also collected. Treatment registry with the disease assessment variables shown in graphics was available for all patients; only 6% were under biologic treatment.ConclusionsReuma.pt/Vasculitis is a registry adapted for routine care, allowing an efficient data repository at a national level with the potential to link with other international databases. It facilitates research, trials recruitment, service planning and benchmarking.Disclosure of InterestNone declared

Disease Area Index), o PDAI (Pemphigus Disease Area Index) ou o MMPDAI (Mucous Membrane Pemphigoid Disease Area Index), que permitem uma avaliação mais objetiva das lesões e caracterizar melhor a sua evolução. 10 CONCLUSÃOO rituximab é cada vez mais usado em doenças mediadas por autoanticorpos. Apesar de haver poucos casos descritos do uso de rituximab no penfigoide bolhoso, a grande maioria são situações de sucesso e com resposta sustentada, como neste doente. Assim, os autores pretendem relembrar que este fármaco deve ser considerado em casos de penfigoide bolhoso refratários ao tratamento convencional ou mesmo como primeira linha nos casos mais agressivos. AGRADECIMENTOSOs autores gostariam de agradecer a Trigo Faria e Francisco Esteves pela sua colaboração neste caso. CONFLITOS DE INTERESSEOs autores declaram não terem qualquer conflito de interesse relativamente ao presente artigo. FONTES DE FINANCIAMENTOOs autores declaram não ter recebido subsídios ou bolsas para a elaboração do artigo. REFERÊNCIAS RESUMOA doença intersticial pulmonar ocorre em até cerca de 25% dos doentes com síndrome de Sjögren e em 2% -8% dos doentes com lúpus eritematoso sistémico. Os corticosteróides permanecem como pilar de tratamento do lúpus eritematoso sistémico mas podem associar-se a complicações neuropsiquiátricas, sobretudo com doses de prednisolona superiores a 40 mg/dia. Apresentamos o caso de uma doente de 51 anos com síndrome depressiva, lúpus eritematoso sistémico e síndrome de Sjögren secundária que desenvolveu envolvimento pulmonar grave evidente quatro anos após o diagnóstico, com tomografia computadorizada de tórax a revelar padrão de neofibrose e vidro despolido. Com o aumento da prednisolona para 60 mg/dia a doente iniciou um quadro maníaco com necessidade de internamento e que foi admitido no contexto de corticoterapia em dose elevada. Foi excluído envolvimento neurológico central por doença orgânica. Iniciou ciclofosfamida endovenosa mensal durante seis meses seguida de micofenolato de mofetil, tendo-se reduzido prednisolona até 10 mg/dia e mantido hidroxicloroquina 400 mg/dia, com controlo da atividade da doença.