Anti-IL-1 treatment is an effective alternative for controlling attacks and decreasing proteinuria in colchicine-resistant FMF patients.

Patients with FMF whose symptoms start before 20 years of age seem to have severe symptoms and M694V mutation may be responsible for the early expression of the disease.

Objective Familial Mediterranean fever (FMF) is the most common autoinflammatory disease. Most of the identified disease-causing mutations are located on exon 10. As the number of studies about the effect of the exonal location of the mutation and its phenotypic expression is limited, we aimed to investigate whether the exonic location of the Mediterranean fever (MEFV) mutation has an effect on the clinical manifestation in patients with FMF. Methods Study population was derived from the main FMF registry that included 2246 patients from 15 different rheumatology clinics. We categorized the mutations according to their exon locations and retrieved the clinical and demographic information from the database. Results Patients having the MEFV mutations on exon 2 or 10 (n:1526) were divided into three subgroups according to the location of the MEFV mutations: Group 1 (exon 2 mutations), Group 2 (exon 10 mutations), and Group 3 (both exon 2 and exon 10 mutations). Group 2 patients were of a significantly younger age at onset, and erysipel-like erythema, arthritis, amyloidosis, and a family history of FMF were more common in this group. Conclusion Patients with FMF and exon 10 mutations show more severe clinical symptoms and outcome. Exon 2 mutations tend to have a better outcome.

BackgroundSeveral surveys indicate that the complementary and alternative medicine (CAM) use is especially prevalent in patients with chronic painful conditions like ankylosing spondylitis (AS). Despite good treatment options such as tumor necrosis factor alpha (TNFα) inhibitors in AS, it is seen that patients have applied for CAM use for many reasons including local regulatory funding requirements, potential risks and accessibility of biological treatments. Few studies have examined the frequency of CAM use, and associations between demographic and disease-related factors of it in AS.ObjectivesTo investigate the CAM usage of patients with AS and to determine the associated factors.MethodsTotal of 123 patients with AS, who were being followed in a tertiary rheumatology outpatient clinic, were included to the study. The demographic and clinical features along with the behaviors about the CAM usage of the patients agreeing to participate were recorded to the “Patient Assessment Form”. The activity of the disease were determined with doctor global assessment (numeric visual analog scale (nVAS; 0–10), and Routine Assessment of Patient Index Data (RAPID)-3 score. The treatment adherence of the patients was assessed with the Morisky Green Levine Scale.ResultsOne hundred eleven patients (%90.2) were male, and mean age was 36.5±8.8 years. The mean disease duration and mean delay in diagnosis were 10.9±6.4, and 3.7±3.9 years, respectively. The mean RAPID3 score, doctor and patient global assessment were; 9.9±5.3, 2.8±1.9, and 4.6±2.7, respectively. While 79 patients (%64.2) were on anti-TNF treatment, 76 patients were receiving NSAIDs, and 35 patients (%28.5) reported an adverse event related with the treatment. Forty-five patients (%36.6) reported to use any CAM (previous or current) (Table1). The reasons reported by the patients for the usage of CAM; media in %13, recommendations from family members or relatives in %10.6. It has been found that in married patients, the ones with lower the Morisky Green Levine Scale score (high adherence), CAM usage was statistically high (p<0.05). Receiving NSAIDs or anti-TNF agents was not statistically associated with CAM usage. The underlying expectations for the usage of CAM were; considering it might be helpful in %27.6; considering it might heal in %17.9; to relieve the pain in %14.6; and preventing to deteriorate the disease status in %12.2.Table 1.Types of CAM useCAM Typen*% Plants and herbs3125.2Massage1310.6Spa108.1Praying/spiritual approach64.9Cupping32.4Imagining21.6Naturapati21.6Acupuncture10.8CAM, complementary and alternative medicine. *There are patients marked the method more than one.ConclusionsIn our study, we found that approximately one third of our AS patients were using CAM. When compared with the literature related with other diseases, CAM usage in AS patients was somewhat lower. Our results have demonstrated that treatment adherence was higher in those using concomitantly CAM in their therapy.Disclosure of InterestNone declared

BackgroundPsoriatic arthritis (PsA) has a genetic background, approximately 40% of patients having a family history of psoriasis or PsA in first-degree relatives, which may impact the disease features.ObjectivesThe aim of this study was to evaluate the effects of family history of psoriasis or PsA on the disease phenotypes.MethodsThe demographic and clinical data were retrieved from the longitudinal, multicenter PsArt-ID (Psoriatic Arthritis-International Database). Family history of psoriasis and PsA were investigated for 1st and 2nd degree relatives separately. The effect of the family history of psoriasis and/or PsA on disease phenotypes and severity were analysed, calculating the relative risks (RR).Results1393 patients had the data for family history, 444 (31.9%) of whom was positive for psoriasis and/or PsA. The majority of the family history was only psoriasis (333/444; 75%) and 58.5% (260/444) of the patients had first-degree relatives affected. There was no differences in maternal or parental transmission rates however women had more psoriasis and/or PsA in their family (67.3% vs 32.7% p: 0.028). Patients with a family history had an earlier onset of age for psoriasis (29±14.8 vs 31±14.9 p: 0.007), more frequent nail involvement (50.7% vs 29.6% p: 0.032), more frequent enthesitis (28.2% vs 17.7% p<0.001) and deformities (25.2% vs 19.9% p: 0.05) and were able to achieve minimal disease activity (MDA) less often. (38.6% vs 49.5% p: 0.045). Plaque psoriasis was more common if the family history was positive for psoriasis whereas pustular psoriasis was more frequent when the family history was positive for PsA (figure 1). Family history of psoriasis were found as a risk factor for a younger onset (RR: 1.138), for nail disease (RR: 1.179), for enthesitis (RR: 1.504) and for not achieving MDA (RR: 1.246) whereas family history of PsA was a risk factor for having deformities (RR: 1.215) (table 1).Abstract THU0285 – Table 1Relative Risks in patients with or without family history of psoriasis or PsAFamily historypRR95% CI Onset of psoriasis before 40 years agePsoriasis<0.051.1381.063–1.219PsA>0.051.0230.879–1.191Nail involvement (ever)Psoriasis<0.051.1791.04–1.335PsA>0.051.1570.917–1.461Enhesitis (ever)Psoriasis<0.051.5041.192–1.898PsA>0.051.3500.871–2.092Not achieving MDAPsoriasis<0.051.2461.003–1.547PsA>0.051.0440.655–1.666Presence of deformitiesPsoriasis>0.051.2150.928–1.592PsA<0.051.7861.170–2.727PsA: Psoriatic Arthritis; MDA: Minimal Disease ActivityFigure: Distribution of skin lesions according to the family history in patients with PsA. Numbers are given as percentages. PsO: Psoriasis; PsA: Psoriatic ArthritisConclusionsThe family history of psoriasis and PsA has impacts on skin phenotypes, musculoskeletal features and the disease severity. The differences between family history of psoriasis and PsA and pustular vs plaque phenotypes may point out to a different genetic background and pathogenic mechanisms in these subsets.Disclosure of InterestNone declared