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WOS: 000505281300007, PubMed: 30680951, Objective Psoriatic arthritis (PsA) has a genetic background. Approximately 40% of patients with psoriasis or PsA have a family history of psoriasis or PsA, which may affect disease features. The aim of this study was to assess the effects of family history of psoriasis and PsA on disease phenotypes. Methods Data from 1,393 patients recruited in the longitudinal, multicenter Psoriatic Arthritis International Database were analyzed. The effects of family history of psoriasis and/or PsA on characteristics of psoriasis and PsA were investigated using logistic regression. Results A total of 444 patients (31.9%) had a family history of psoriasis and/or PsA. These patients were more frequently women, had earlier onset of psoriasis, more frequent nail disease, enthesitis, and deformities, and less frequently achieved minimal disease activity. Among 444 patients, 335 only had psoriasis in their family, 74 had PsA, and 35 patients were not certain about having PsA and psoriasis in their family, so they were excluded from further analysis. In the multivariate analysis, family history of psoriasis was associated with younger age at onset of psoriasis (odds ratio [OR] 0.976) and presence of enthesitis (OR 1.931), whereas family history of PsA was associated with lower risk of plaque psoriasis (OR 0.417) and higher risk of deformities (OR 2.557). Family history of PsA versus psoriasis showed increased risk of deformities (OR 2.143) and lower risk of plaque psoriasis (OR 0.324). Conclusion Family history of psoriasis and PsA impacts skin phenotypes, musculoskeletal features, and disease severity. The link between family history of psoriasis/PsA and pustular/plaque phenotypes may point to a different genetic background and pathogenic mechanisms in these subsets., Turkish Society for Rheumatology; Scientific and Technological Research Council of TurkeyTurkiye Bilimsel ve Teknolojik Arastirma Kurumu (TUBITAK), Supported by the Turkish Society for Rheumatology. Dr. Bakirci's work was supported by the Scientific and Technological Research Council of Turkey and the Turkish Society for Rheumatology.

Objectives: Behcet’s disease (BD) is a multi-systemic inflammatory disorder which can affect all types and sizes of vessels.The usage of TNF-α antagonists is increasing in different involvements of...

There is a need for better definition of polyarteritis nodosa (PAN) subphenotypes and the influence of ethnicity and geography. This study is aimed to study the demographic and clinical features of PAN cohorts from the UK and Turkey (TR) and to compare and contrast disease characteristics. A retrospective survey of databases from two vasculitis centres between 1990 and 2016 for PAN patients fulfilling the EMEA Vasculitis Classification algorithm. All paediatric-onset adult patients met the Ankara 2008 (EULAR/PReS endorsed) criteria for childhood PAN. Those with typical angiographic and/or histopathologic findings consistent with PAN were included. 93 (M/F: 51/42) patients (UK: 47, TR: 46) were included. Three were HBV-related, 20 (21.5%) had paediatric onset and 16 (16.5%), cutaneous PAN. TR patients had younger age of disease onset 44 (28.5-59.0) vs. 24.5 (11.8-40.5), p = 0.002. Twelve (26%) of TR patients had monogenic disease (Familial Mediterranean Fever association (n = 7), deficiency of adenosine deaminase 2, DADA2, (n = 5). No difference was found in phenotype between paediatric and adult onset patients except for frequency of cutaneous lesions (p = 0.002). During a median 67.5 (32-126) months follow-up, 13 patients died (12.7% in UK vs. 15.2% in Turkish cohorts). No difference was found between two cohorts in relation to relapse rate, death and vasculitis damage index. This study defined a diagnosis of PAN according to the EMEA algorithm. The TR group had a younger age of disease onset and more cases of monogenic disease; however, disease extent, relapse rate, damage index and death rates were similar between groups.

Aim To determine the frequency and define the causes of pulmonary hypertension (PH) in patients with Takayasu arteritis (TA). Method Sixty-four TA patients were evaluated by transthoracic echocardiography (TTE). Having an estimated systolic pulmonary arterial pressure (sPAP) ≥40 mm Hg by echocardiography or if performed, mean PAP ≥25 mm Hg in right heart catheterization was defined as PH. Clinical, imaging and laboratory results of the TA patients were obtained from hospital files. Result In total, seven (10.9%) patients had PH. Four patients had PH due to left-sided heart disease (group 2 PH), three patients due to pulmonary arterial involvement (PAI; group 4 PH) and one patient due to atrial septal defect (group 1 PH). In one patient, combination of PAI, aortic insufficiency and pulmonary venous return anomaly was present and he was considered to have both group 2 and group 4 PH. PAI was more frequent (42.9% vs 15.7%) in patients with PH but the difference was not statistically significant. The percentage of patients treated with cyclophosphamide and/or biologics was higher in the group with PH as compared to the group without PH (P = 0.015). One patient with group 4 PH had been on pulmonary arterial hypertension (PAH)-specific agents for 8 years. Conclusion Pulmonary hypertension is not infrequent in TA patients and all the potential causes of PH should be carefully evaluated. Patients with severe or treatment-resistant disease are prone to have PH. PAH-specific agents may be effective in patients with group 4 PH.

Background: Relapsing polychondritis (RP) is a rare autoimmune disorder, and myelodysplastic syndrome (MDS) is accompanied by RP at variable rates. Herein, we report a case with RP and MDS who responded dramatically to 5-azacitidine for MDS. Case presentation: With conventional immunosuppressive treatment, our patient had several episodes of different side effects, including infections. With the diagnosis of MDS and initiation of azacitidine treatment, all the manifestations of RP disappeared, and remission was achieved for MDS. Although he had relapses of either RP or MDS after several years of azacitidine treatment, all relapses were controlled well with the initiation of azacitidine treatment every time. Conclusions: Azacitidine should be kept in mind as a treatment option for RP patients with MDS.

Tezcan, Mehmet Engin/0000-0002-1753-4936; TUFAN, Abdurrahman/0000-0001-6244-9362; SARI, Ismail/0000-0001-7737-4180; YILMAZ, Sedat/0000-0002-4691-3417 WOS: 000436403100017 PubMed: 28992387 ObjectiveApproximately 30-45% of patients with familial Mediterranean fever (FMF) have been reported to have attacks despite colchicine treatment. Currently, data on the treatment of colchicine-unresponsive or colchicine-intolerant FMF patients are limited; the most promising alternatives seem to be anti-interleukin-1 (anti-IL-1) agents. Here we report our experience with the off-label use of anti-IL-1 agents in a large group of FMF patients. MethodsIn all, 21 centers from different geographical regions of Turkey were included in the current study. The medical records of all FMF patients who had used anti-IL-1 treatment for at least 6 months were reviewed. ResultsIn total, 172 FMF patients (83 [48%] female, mean age 36.2 years [range 18-68]) were included in the analysis; mean age at symptom onset was 12.6 years (range 1-48), and the mean colchicine dose was 1.7 mg/day (range 0.5-4.0). Of these patients, 151 were treated with anakinra and 21 with canakinumab. Anti-IL-1 treatment was used because of colchicine-resistant disease in 84% and amyloidosis in 12% of subjects. During the mean 19.6 months of treatment (range 6-98), the yearly attack frequency was significantly reduced (from 16.8 to 2.4; P < 0.001), and 42.1% of colchicine-resistant FMF patients were attack free. Serum levels of C-reactive protein, erythrocyte sedimentation rate, and 24-hour urinary protein excretion (5,458.7 mg/24 hours before and 3,557.3 mg/24 hours after) were significantly reduced. ConclusionAnti-IL-1 treatment is an effective alternative for controlling attacks and decreasing proteinuria in colchicine-resistant FMF patients.

The use of anti-tumor necrosis factor alpha (anti-TNF-α) agents has increased during the past decade in rheumatology practice. Opportunistic infections have been reported with anti-TNF-α agents in clinical trials and post-marketing usage. Aspergillus infection is a rare opportunistic infection that is associated with immunosuppression, and there are reported cases of pulmonary aspergillosis in various rheumatic diseases treated with anti-TNF-α agents. Here, we present the first case of pulmonary aspergillosis associated with infliximab treatment in a patient with Still’s disease.

The aim of this study was to determine the frequency of sarcopenia at the time of diagnosis in patients with rheumatoid arthritis (RA), assessing disease activity and factors that may be associated with sarcopenia and observe effects of treatment on sarcopenia.A prospective study was conducted with patients who have newly diagnosed RA. Patients were evaluated twice, at the time of diagnosis and 3 mo after the initiation of treatment. Demographic data, anthropometric measurements, disease activity scores, and sarcopenia status were recorded. Sarcopenia was evaluated with grip strength and bioelectric impedance. The results were compared with healthy volunteers.The age at onset of RA was 50.6 ± 14.6 y. Handgrip strength (P0.001), skeletal muscle mass (P = 0.009), and skeletal muscle mass index (P = 0.032) were reduced in patients with RA but not in the control group. The frequency of sarcopenia in RA at onset of diagnosis was 31.5%. There was a significant decrease in the rate of sarcopenia after 3 mo of treatment (31.5 versus 8.7%; P = 0.046).Sarcopenia was found in approximately one-third of the patients with newly diagnosed RA in our study. With treatment, sarcopenia improved significantly. Patients with RA should be evaluated in terms of sarcopenia in addition to evaluating joint and extraarticular findings at the time of diagnosis. Early detection and treatment planning may improve quality of life.

Background Intravenous immunoglobulins (IVIg) have been used in management of severe Covid-19. Here in this study, we report our single-center experience regarding IVIg treatment in management of severe Covid-19. Materials and Method Among hospitalized adult Covid-19 patients between April 1 and December 31, 2020, patients with confirmed diagnosis of Covid-19 who had Brescia-COVID respiratory severity scale score ≥ 3, hyperinflammation and received IVIg treatment in addition to standard of care were retrospectively investigated. We grouped IVIg recipients into three according to reasons for IVIg administration: Group 1 patients requiring anti-inflammatory treatment but complicated with secondary infection and/or sepsis , group 2 patients with Covid-19 related complications including progressive disease refractory to other anti-inflammatory agents, myocarditis, adult multisystem inflammatory syndrome, hemophagocytic lymphohystiocytosis like syndrome and group 3 patients with other complications non-specific to Covid-19. Mortality and clinical data was compared among groups. Results A total of 46 IVIg recipients were enrolled. Group 1 comprised 17 (36.9%), group 2 comprised 18 (39.1%) and group 3 comprised 11 (23.9%) patients. No significant differences in means of age, gender and comorbidities were observed among groups. Mortality was significantly lower in group 3 when compared to group 1 (64.7% vs 18.2%, p = 0.016) and close to significance when compared to group 2 (50% vs 18.2% p = 0.087). Conclusions IVIg seemed to be used mostly in severe, refractory and complicated cases in our population. As a rescue agent in severe cases refractory to other anti-inflammatory strategies, 33.7% survival rate was observed with IVIg.

Background and Design: Psoriatic arthritis (PsA), enthesitis and/or soft tissue swelling accompany psoriasis in 6-30% of all psoriatic patients. Early recognition of PsA is crucial since it is a serious disabling comorbidity with irreversible complications. Materials and Methods: Patients, who were admitted to the outpatient clinic of Hacettepe University Faculty of Medicine Department of Dermatology between March 2014 and 2015 with plaque psoriasis lacking any prior PsA diagnosis, were enrolled for this study. Demographic data, previous treatment history, laboratory parameters and physical examination of the patients were collected. All patients were examined by the same physician with a rheumatologic questionnaire that consists of five questions about any accompanying rheumatologic complaints. All patients who had at least one positive answer were consulted with the department of rheumatology at the same center. Results: Two hundred and twenty-three patients were included, 58% (n=129) were male and 42% (n=94) were female. The mean age of the patients was 43.46±14.31 years. The mean Psoriasis Area and Severity Index score was 12.66±9.89 standard deviation. The most common complaint detected by the questionnaire was myalgia/arthralgia at rest in 28% (n=62) of the patients. 30% (n=69) of the patients were consulted to rheumatology for a positive answer on the questionnaire and 24% (n=53) of the patients were evaluated by a rheumatologist. 51% (n=27) of the evaluated patients were diagnosed with a rheumatologic disease which were PsA in 40% (n=21), sacroiliitis in 6% (n=3), ankylosing spondylitis in 4% (n=2), and unspecified connective tissue disease in one patient. Conclusion: The improvement of dermatologist’s skills about suspecting, questioning and examining PsA symptoms is crucial for early diagnosis. This study concludes that a standard rheumatologic questionnaire efficiently helps dermatologists to predict PsA in psoriatic patients.

Background: Behçet’s disease (BD) is a systemic vasculitis of all types of vessels of both the venous and arterial sites. Mean platelet volume (MPV) is the most commonly used measure of platelet (Plt) size and is also a potential marker of Plt activity. In this study, we wanted to evaluate the MPV value and the MPV/Plt ratio in patients with deep venous thrombosis (DVT) in BD. Methods: Overall, 228 patients – 49 BD patients with a thrombotic complication and 179 BD patients without a thrombotic complication (as the control group) – were included the study. Results: Of the 49 patients with thrombosis, there was a very significant difference between the genders: 41 (83.6%) were males while only eight (16.4%) were females (p Conclusions: Increased MPV can predict the risk of DVT development as well as the male sex among BD patients. The MPV/Plt ratio may also be used for predicting DVT.

Background/aim: Since the majority of the IgG4-related disease (IgG4-RD) patients in the literature are from the Far East and the United States, there is a lack of large series from other parts of the world. We aimed to identify the clinical characteristics and outcome of Turkish IgG4-RD patients from a tertiary center. Materials and methods: Fifty-two patients classified as having definite IgG4-RD according to comprehensive diagnostic criteria were included in the study. Patients not fulfilling the definite criteria due to lack of pathologic specimen and/or serum IgG4 levels were excluded (n = 47). Clinical, laboratory, and histopathological features and treatment approaches were analyzed. Results: Median age at diagnosis was 51.1 years and sex predominance was not observed (male/female: 26/26). Median follow-up duration was 18 (IQR 25–75: 8–35) months. Retroperitoneal fibrosis was the most frequent presentation. Twenty-four (46.1%) patients had localized involvement. Corticosteroids were the mainstay of treatment (92.5%). Rituximab had been used for cases resistant to previous treatment or with relapses in 19 (47.5%) patients. A complete response was achieved in 52.5% and partial response (

Background/aim: Abnormal capillaroscopic findings have been reported in vasculitic syndromes such as Behçet's disease, Henoch-Schönlein purpura, and Wegener's granulomatosis. This study was conducted to define the capillaroscopic changes in patients with Takayasu arteritis (TA). Materials and methods: We studied 28 TA patients (27 females). The nail folds from the 2nd to 5th fingers on both hands were examined with video capillaroscopy for all. A patient was defined as having an abnormal capillaroscopic examination if more than 1 morphologic abnormality was present in at least 2 nail folds. Results: The median capillary density of TA patients was 9 (range: 9-11). There were no patients with capillary disorganization or avascular areas. Tortuous capillaries were detected in all patients. The other common morphological capillary abnormalities included enlarged/dilated capillaries (39.3%), branching capillaries (35.7%), and hemorrhages (32.1%). Only 1 patient had giant capillaries with early scleroderma-like pattern. Overall, there were 11 (39.3%) patients with abnormal capillaroscopic findings. There were more patients with abnormal capillaroscopic findings in the subgroup of TA patients whose upper extremity blood pressure could not be measured as compared to those whose blood pressure could be measured (66.7% vs. 26.3% patients; P = 0.04). Conclusion: Capillaroscopic abnormalities are frequently seen in TA patients. We consider that abnormal capillaroscopic findings in TA patients reflect the impaired blood flow due to narrowed or occluded arteries rather than the primary capillary involvement of the disease process.

Background/aim Tumour necrosis factor inhibitors and anti-interleukin-6 (anti-IL-6) therapies are increasingly being used in Takayasu’s arteritis (TA) patients who are unresponsive to corticosteroids ± conventional immunosuppressive agents. The aim of this study is to assess the efficacy and safety of anti-IL-6 (tocilizumab) therapy in refractory TA patients in real life. Materials and methods Fifteen TA patients (86.7% were female) who received at least 3 cycles of tocilizumab therapy were retrospectively assessed by clinical, laboratory, and radiological evaluations before and after tocilizumab therapy. Results The median (min–max) age of the patients at evaluation was 35 (20–58) years and the median disease duration from diagnosis was 24 (12–168) months. The median (min.–max.) duration of follow-up after tocilizumab was 15 (3–42) months. There was a significant decrease in erythrocyte sedimentation rate (ESR), C-reactive protein (CRP), and patient global visual analogue scale (VAS) scores of patients after tocilizumab therapy. The median (min.–max.) ESR was 26 (5–119) vs. 3 (2–49) mm/h, P = 0.02; CRP was 39.8 (2.4–149.0) vs. 7.9 (0–92.9) mg/L, P = 0.017; and patient global VAS was 50 (0–90) vs. 30 (0–60), P = 0.027, respectively. In 8 patients, ESR and CRP levels were in the normal range in the last control. Imaging modality results after tocilizumab were available for 9 patients; 8 patients were radiologically stable and regression was seen in 1 patient. Comparable imaging modality results before and after tocilizumab were available for 5 patients; 4 patients were radiologically stable and regression was seen in 1 patient. Radiological findings were consistent with laboratory responses. Glucocorticoid dosages decreased from a mean dosage of 16.2 (9.1) mg/day at baseline to 7.1 (3.8) mg/day (P = 0.001) at the last follow-up visit. There was no increase in the steroid dosage in any of the patients. All patients tolerated tocilizumab well. Conclusion Based on retrospective real life data, anti-IL-6 (tocilizumab) appears to be an effective and tolerable treatment option in refractory TA patients.

Background: Articular manifestations may differ in ulcerative colitis (UC) and Crohn’s disease (CD). Genetic and non-genetic factors like sex, smoking, and presence of HLA-B27 were previously shown to modify the expression of articular and other extraintestinal manifestations of IBD. Objectives: The aim of this study is to document disease features and factors affecting the expression of articular manifestations in Turkish patients with IBD-related (enteropathic) arthritis under treatment with disease modifying antirheumatic drugs (DMARDs). Methods: Data regarding enteropathic arthritis (EA) were collected from the TReasure database, a nation-wide multicenter observational registry of inflammatory arthritis patients. Results: Among 4066 patients with seronegative spondyloarthropaties (SpA), 156 (3.8%) had EA, not reflecting a true prevalence due to selection bias. Demographic and clinical features according to IBD groups were summarized in Table 1. Rates of presence of sacroiliitis were similar between patients with UC and CD (39.9% and 60.1%, p=0.086 respectively). Rates of HLA-B27 positivity were 31.6% and 7.1% in patients with and without radiographic sacroiliitis, respectively (p=0.101). Enthesitis, dactylitis, psoriasis, family history forSpA, ESR, CRP, BASDAI and aSDAS levels had similar distributions in patients with and without radiographic sacroiliitis. Rates of “never-smoked” (26.5% vs 64.7%) and “current smoking” (32.4% vs 17.6%) significantly differed in patients with and without sacroiliitis (overall p=0.012) Conclusion: Our data confirm an association between smoking status and disease manifestations, particularly radiographic sacroiliitis. References [1] Fries W. Clinical features and epidemiology of spondyloarthritides associated with inflammatory bowel disease. World J Gastroenterol2009; 15: 2449-2455. Disclosure of interests: Orhan Kucuksahin: None declared, abdulsamet Erden: None declared, Ufuk Ilgen: None declared, Sedat Kiraz: None declared, ali Ihsan Ertenli: None declared, Nazife Sule Yasar Bilge: None declared, Timucin Kasifoglu: None declared, Ediz Dalkilic Grant/research support from: MSD and abbvie, Consultant for: MSD, abbvie,Roche, UCB, Pfizer and Novartis, Speakers bureau: MSD, abbvie,Roche, UCB, Pfizer and Novartis, Cemal Bes: None declared, Nilufer alpay Kanitez: None declared, Hakan Emmungil Grant/research support from: MSD, Roche, Pfizer, abbvie, Consultant for: Novartis, Roche, Speakers bureau: MSD, Roche, Pfizer, abbvie,Celltrion, Novartis, Pamir atagunduz: None declared, Belkis Nihan Seniz: None declared, Burcu Yagiz: None declared, Suleyman Serdar Koca: None declared, Muhammet Cinar: None declared, askin ates: None declared, Servet akar Grant/research support from: MSD, abbvie, Roche, UCB, Novartis, Pfizer, amgen, Consultant for: MSD, abbvie, Roche, UCB, Novartis, Pfizer, amgen, Speakers bureau: Pfizer, Onay Gercik: None declared, Duygu Ersozlu: None declared, Veli yazisiz: None declared, Gezmis Kimyon: None declared, Muge aydin: None declared, Ridvan Mercan: None declared, Burak Oz: None declared, Zeynel abidin akar: None declared, Omer Karadag: None declared, Bahar Kelesoglu: None declared, Sedat Yilmaz: None declared, Yavuz Pehlivan: None declared, Ender Terzioglu: None declared, Levent Kilic: None declared, Sukran Erten: None declared, Koray Tascilar: None declared, Umut Kalyoncu Grant/research support from: MSD, Roche, UCB, Novartis and Pfizer, Consultant for: MSD, abbvie, Roche, UCB, Novartis, Pfizer and abdi Ibrahim, Speakers bureau: MSD, abbvie, Roche, UCB, Novartis, Pfizer and abdi Ibrahim

Background: Although cardiovascular (CV) diseases are very common in inflammatory arthritis like psoriatic arthritis (PsA), long-term impact of medication on CV outcomes is lacking. Objectives: The aim of our study was to evaluate the long-term effects of anti-TNF-a drugs on subclinical atherosclerosis assessed by the flow-mediated dilatation (FMD) and carotid intima media thickness (IMT). Methods: A total of 30 patients with PsA according to classification of psoriatic arthritis (CASPAR) criteria1 and 28 healthy controls were enrolled in this cross-sectional study between June 2011-July 2012. 22 out of 30 PsA patients completed the study. Demographic data (sex, age), PsA and psoriasis duration, joint pattern (monoarthritis, oligoarthritis or polyarthritis) and other PsA involvements (nail, enthesis, dactilytis) were noted. Tender joint count, swollen joint count and disease activity score (DAS)-28 were used for joint activity assessment. Erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) were used for acute phase reactants. Sex and age matched healthy controls were selected as the control group for this study. Flow mediated dilatation (FMD) from brachial artery and carotid intima media thickness (IMT) were measured by an experienced cardiologist both at initial and 5-year follow-up visits. Results: Psoriasis duration of PsA patients was 180±114 months. PsA disease duration was 108±33 months. The mean duration between two evaluations was 62±9 months. At first evaluation, 14 (63.6%) patients had peripheral joint, 1 (4.5%) patient had axial, and 7 (31.8%) patients had both peripheral and axial involvement among patients. Dactilytis in 6 (27.3%), enthesis in 7 (31.8%) and nail in 12 (54.5%) patients were other clinical involvements. FMD% was lower in PsA patients than healthy controls [9.3±3.9 vs 12.9±1.8, p Conclusion: Our results showed that there was a significant impact of anti-TNF-a drugs on progression of subclinical atherosclerosis at the vascular wall level, but no impact on the endothelial dysfunction. Further large-scale randomized studies are needed to confirm our findings. Disclosure of Interests: Abdulsamet Erden: None declared, Ugur Canpolat: None declared, Oguz Abdullah Uyaroglu: None declared, Cem Coteli: None declared, Levent Kilic: None declared, Ali Akdogan: None declared, Umut Kalyoncu Grant/research support from: MSD, Roche, UCB, Novartis and Pfizer, Consultant for: MSD, Abbvie, Roche, UCB, Novartis, Pfizer and Abdi Ibrahim, Speakers bureau: MSD, Abbvie, Roche, UCB, Novartis, Pfizer and Abdi Ibrahim, Omer Karadag: None declared, Ali Ihsan Ertenli: None declared, Sedat Kiraz: None declared, Kudret Aytemir: None declared, Sule Apras Bilgen: None declared

Background Immunoglobulin a vasculitis (IgAV) [formerly called Henoch– Schonlein purpura (HSP)] is an IgA mediated systemic vasculitis which primarily affects skin, gastrointestinal system and small vessels of kidneys. Exact pathogenesis of IgAV remains unknown. A fewclinicalstudiesevaluatedthe role of oxidativeinjury in thepathogenesis of vasculitis. Ischemia modified albumine (IMA) and Neopterin increased status of oxidative stress. Objectives The aims of the study are to investigate serum neopterin and IMA levels in patients with IgAV and evaluate the association of these markers with disease activity and relapse. Methods Thirty-four consecutive adult patients (24 males and 10 females) admitted to the rheumatology clinic of ankara Numune Training and Research Hospital meeting the IgAVAmerican College of Rheumatology (ACR) criteria were enrolled in this cross-sectional study. Demographic and clinical features of IgAV and control group were recorded into a predefined protocol. Disease activity was categorized as “remission” or “active” according to BVAS. BVAS ≥1 was accepted “active”.Serum neopterin levels, hsCRP and IMA were evaluated according to BVAS and compared to healthy control group. Results Serum median (IQR) neopterin, IMA levelsandhsCRPwerehigher in thestudygroupthan in controlgroup [2.01 (12.5) ng/mL vs. 1.77 (1.37) ng/mL, 0.67 (0.2) ng/mL vs. 0.43 (0.17) ng/mL, 5.6 (17.1) mg/L vs. 1.55 (1.6) mg/L, p=0.095, p Conclusion Oxidative stress is important in HSP pathogenesis. Roles of hsCRP,Neopterin and IMA as potential markers of diagnosis and disease activity seem to be worth studying in the future studies with larger study groups. Disclosure of interests None declared

Background: In rheumatoid arthritis (RA), biologic DMARDs are important treatment options in resistant patients. Inefficacy or side effects may cause switching between these drugs. Objectives: This study aimed to determine features of patients switching from one biologic DMARD to another in RA treatment and to investigate associated reasons for switching. Methods: This multicenter, prospective observational cohort study used the TReasure database in which web-based registration of RA and spondyloarthritis patients are being performed in 15 centers across different regions of Turkey. In this study, data of RA patients switching from one biologic agent to another were analyzed. Demographic and clinical data, follow-up duration, time to switch, and reasons for switching were retrieved from the database. Results: Of the included 2115 RA patients, 829 (39.2%) switched between biologic agents (switched group) and 1286 (60.8%) continued to receive their current therapies (continued group). The median follow-up duration of all patients was 3.7 years and the median time to switch was 1.1 years. In the switched group, the proportion of females and the median HAQ-DI score were higher as well as disease duration was longer (Table 1). Among the biologic agents used at first, 60.9% of the patients were receiving an anti-TNF agent and 39.1% of the patients were receiving other biologic agents (Table 2, figure 1). In the switched group (n=829), the main reasons for switching were secondary inefficacy (n=269), primary inefficacy (n=238), and side effects (n=178) followed by primary or secondary unknown inefficacy (n=30), patient’s demand (n=21), physician’s request (n=16), willing to be pregnant (n=7), other (n=31), and unknown (n=54). Conclusion: The patients in the Treasure database were followed-up approximately 4 years and about one-third of the patients had to switch from one biologic DMARD to another. The main reasons for this switching were primary (29.2%) and secondary (33.0%) inefficacy and 20% of the patients had to switch due to side effects. According to the switching pattern, about half of the patients using an anti-TNF agent at first switched to another anti-TNF agent and the other half switched to other biologic agents. Disclosure of Interests: Umut Kalyoncu Grant/research support from: MSD, Roche, UCB, Novartis and Pfizer, Consultant for: MSD, Abbvie, Roche, UCB, Novartis, Pfizer and Abdi Ibrahim, Speakers bureau: MSD, Abbvie, Roche, UCB, Novartis, Pfizer and Abdi Ibrahim, Ali Ihsan Ertenli: None declared, Abdulsamet Erden: None declared, Orhan Kucuksahin: None declared, Timucin Kasifoglu: None declared, Ediz Dalkilic Grant/research support from: MSD and Abbvie, Consultant for: MSD, Abbvie,Roche, UCB, Pfizer and Novartis, Speakers bureau: MSD, Abbvie,Roche, UCB, Pfizer and Novartis, Cemal Bes: None declared, Nilufer Alpay Kanitez: None declared, Hakan Emmungil Grant/research support from: MSD, Roche, Pfizer, Abbvie, Consultant for: Novartis, Roche, Speakers bureau: MSD, Roche, Pfizer, Abbvie,Celltrion, Novartis, Pamir Atagunduz: None declared, Belkis Nihan Coskun: None declared, Burcu Yagiz: None declared, Suleyman Serdar Koca: None declared, Muhammet Cinar: None declared, Askin Ates: None declared, Servet Akar Grant/research support from: MSD, Abbvie, Roche, UCB, Novartis, Pfizer, Amgen, Consultant for: MSD, Abbvie, Roche, UCB, Novartis, Pfizer, Amgen, Speakers bureau: Pfizer, Onay Gercik: None declared, Duygu Ersozlu: None declared, Veli Yazisiz: None declared, Gezmis Kimyon: None declared, Muge Aydin: None declared, Ridvan Mercan: None declared, Burak Oz: None declared, Nazife Sule Yasar Bilge: None declared, Zeynel Abidin Akar: None declared, Omer Karadag: None declared, Ayse Bahar Kelesoglu Dincer: None declared, Sedat Yilmaz: None declared, Ufuk Ilgen: None declared, Yavuz Pehlivan: None declared, Ender Terzioglu: None declared, Levent Kilic: None declared, Sukran Erten: None declared, Sedat Kiraz: None declared

Background Pseudotumor orbita, pachymeningitis, periaortitis could be seen in both ANCA-associated vasculitis and IgG4-RD. Sometimes it may be difficult to differentiate these two entities. The co-occurrence/concurrence of Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAV) and IgG4-related disease (IgG4-RD) was recently published by a collaborative EUVAS group [1]. Objectives Firstly, we aimed to investigate ANCA positivity of our IGG4-RD cohort. Secondly, a literature review of co-occurrence/concurrence of AAV and IgG4-RD was done. Methods Data of totally 62 patients with IgG4-RD in Hacettepe Vasculitis Center Database was used. Patients were diagnosed with IgG4-RD according to comprehensive diagnostic criteria [2]. Dataset of patients including demographic data, clinical characteristics, and imaging and laboratory findings of IgG4-RD was re-evaluated in terms of AAV and ANCA test. At next step, we performed a systematic literature review of the PUBMED database covering the time period until April 2018. Relevant publications were searched using the MeSH terms “IgG4-related disease and Eosinophilic Granulomatosis with Polyangiitis”, ‘‘IgG4-related disease and Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis” and “IgG4-related disease and Granulomatosis with Polyangiitis”. Results Totally 29 (46.7%) of our patients had ANCA results. Out of 29 patients 15 (51.7%) were considered as probable, 10 (34.5%) as definite and 4 as possible (13.8%) for IgG4-RD. Three (10.3%) of these patients had ANCA positivity. All of these ANCA titers were in low degree positivity (MPO-ANCA 1/100, MPO-ANCA 1/32 and PR3 ANCA 1/100). These three patients didn’t have any findings of vasculitis and didn’t have granuloma in their biopsy. When we evaluate these three patients with regards to meeting the Ig G4- RD criteria, 1 was definite, 1 was probable and 1 was possible. In literature review, we found 17 cases that having both features of IgG4-RD and AAV (Table). These cases were re-evaluated according to the ‘Comprehensive Diagnostic Criteria for IgG4-RD’. Diagnoses of IgG4-RD were definite in 11 cases (64.7%), probable in 2 cases (11.8%) and possible in 4 cases (23.5%). ANCA were positive in 15 of 17 patients (88%). ANCA were directed against proteinase 3 (PR3-ANCA) in 6 patients and were directed against myeloperoxidase (MPO-ANCA) in 5 patients. Other four cases had both MPO-ANCA and PR3-ANCA. All PR3-ANCA positive cases have high titers of ANCA, whereas only one MPO-ANCA positive case has high titers of ANCA. Conclusion None of our IgG4-RD patients have any overlap with ANCA-associated vasculitis. Only in 3 patients (10.3%), ANCA positivity was detected without any histopathologic evidence. Just two patients of literature review, seemed to be full compatible with both diseases. Even though ANCA-associated vasculitis and IgG4-RD share clinical features, we think this might be as co-occurrence instead of a histopathologic link. References [1] Danlos, FX. Autoimmun Rev, 2017. [2] Umehara, H. Mod Rheumatol, 2012. Disclosure of Interests None declared

Background Deficiency of adenosine deaminase 2 (DADA2) is an autosomal recessive autoinflammatory disorder caused by ADA2 mutations. Objectives We aimed to investigate the characteristics of DADA2 patients along with the ADA2 enzyme levels. Methods 24 DADA2 patients who admitted to the Adult and Pediatric Rheumatology, Pediatric Haematology, and Pediatric Immunology Departments were included. All exons of the ADA2 gene were screened by Sanger sequencing in all DADA2 patients. Serum ADA2 enzyme activity was measured by modified spectrophotometric method. Results 24 DADA2 patients were included; Group 1, 14 DADA2 patients with polyarteritis nodosa (PAN)-like phenotype; Group 2, 9 patients with Diamond-Blackfan anemia (DBA)-like features and one with immune deficiency. 14 PAN-like DADA2 patients did not have the typical thrombocytosis seen in classical PAN. Inflammatory attacks were evident in only Group 1 patients. Serum ADA2 was low in all DADA2 patients except one who was tested after hematopoietic stem cell transplantation. There was no significant difference in ADA2 levels between PAN-like and DBA-like DADA2 patients (Figure 1). ADA2 activities of heterozygote family members were about half the level of the control subjects. However, in heterozygote DADA2 patients, serum ADA2 levels were as low as the ones of homozygote DADA2 patients. ADA2 mutations were affecting the dimerization domain in Group 1 patients and in the catalytic domain in Group 2 patients (Table 1). Conclusion We suggest that enzyme activity of ADA2 should be assessed along with genetic analysis since there are heterozygote patients with absent enzyme activity. Our data confirms a possible genotype phenotype correlation where dimerization domain mutations are associated with a PAN-like phenotype whereas catalytic domain mutations are associated with hematological manifestations. References [1] Navon Elkan P, et al. Mutant ADA2 in a PAN vasculopathy. N Engl J Med 2014;370(10):921-931 [2] Zhou Q, et al. Early-onset stroke and vasculopathy associated with mutations in ADA2. N Engl J Med 2014;370(10):911-920 Acknowledgement This study was supported by Scientific and Technological Research Council of Turkey (TUBITAK) with grant numbers of 315S192. Prof. Nurten Akarsu performed the molecular studies of DBA patients and special thanks to her for great support. Disclosure of Interests Ezgi Deniz Batu: None declared, Ekim Z. Taskiran: None declared, Hatice Asuman Ozkara: None declared, Sule Unal: None declared, Naz Guleray: None declared, Abdulsamet Erden: None declared, Omer Karadag: None declared, Fatma Gumruk: None declared, Mualla Cetin: None declared, Yelda Bilginer: None declared, Hafize Emine Sonmez: None declared, Deniz Cagdas Ayvaz: None declared, Ilhan Tezcan: None declared, Seza Ozen Consultant for: Seza Ozen is receiving consultancy fees from Novartis, Speakers bureau: Roche

Background SLE phenotype, severity and prognosis varies widely, while its course, prognosis and pattern of severity cannot be predicted with confidence. Objectives We analyzed the phenotype and severity patterns of a SLE cohort in the Attica area of Greece, based in “Attikon” University Hospital, and assessed whether these patterns change over the course of the disease. Methods Retrospective cohort study of 512 Caucasian SLE patients fulfilling the ACR 1997 and/or SLICC 2012 criteria. Data on clinical course, pattern of severity and SLICC damage index (SDI) were recorded for each patient at the time of diagnosis and at last evaluation. Severity of disease was stratified based on BILAG manifestations and patients were assessed for progression to a more severe phenotype over their disease course. Patients with disease duration Results More than half patients (53.7%, 275/512) presented with mild disease, while in approximately 20% (20.8%, n=106) lupus presented with severe manifestations at diagnosis. Median (IQR) follow-up was 96.5 (144) months. Of 246 patients with initially mild disease, 126 (56.4%) retained their mild phenotype, 73 (29.7%) progressed to a moderate phenotype, while the remaining 47 (19.1%) eventually developed severe lupus. Also, 30 patients (29.4%, 30/102) who initially manifested moderately severe manifestations progressed to severe disease over time. At last evaluation, a nearly equal distribution in severity patterns was evident (mild 30%, moderate 34% and severe 36%). Independent factors for disease progression were older age at diagnosis (OR: 0.97 per 1-year, 95% CI 0.95-0.98), disease duration (OR: 1.10 per 1-year, 95% CI 1.06-1.13), positive anti-dsDNA (OR: 2.20, 95% CI 1.38-3.49) and presence of fever at diagnosis (OR: 1.67, 95% CI 1.00-2.77). By multivariate regression, only disease duration (OR: 1.09, 95% CI 1.05-1.12) and anti-dsDNA (OR: 1.73, 95% CI 1.05-2.85) were independently associated with disease progression. Ninety-two subjects (18%) had organ damage at the time of diagnosis, mainly due to neuropsychiatric and thrombotic events. At last visit, mean (SE) SDI was 0.67 (0.57). Two-hundred eight patients (59.8%) had no damage (SDI=0), while high damage (SDI ≥ 3) was measured in 25 subjects (7.2%). Conclusion In this SLE cohort of Caucasian patients, almost half of cases have mild disease at presentation, yet with significant damage accrual. Of patients with disease duration ≥1 year, 43.1% progressed to more severe phenotypes, with patients distributed evenly in the mild, moderate and severe categories. These data reiterate the rule of one third for severity observed in other autoimmune diseases. Disclosure of Interests Dionisis Nikolopoulos: None declared, Antigoni Pieta: None declared, Myrto Kostopoulou: None declared, Abdulsamet Erden: None declared, Pelagia Katsimbri: None declared, Dimitrios Boumpas: None declared, Antonis Fanouriakis Paid instructor for: Amgen, GSK, Speakers bureau: Abbvie, Enorasis, Genesis Pharma

Background In spondyloarthritis (SpA), biologic DMARDs are important treatment options in resistant patients. Inefficacy or side effects may cause switching between these drugs. Objectives This study aimed to determine features of patients switching from one biologic agent to another in SpA treatment and to investigate associated reasons. Methods This multicenter, prospective observational cohort study used the TReasure database in which web-based registration of rheumatoid arthritis and SpA patients are being performed in 15 centers across different regions of Turkey. In this study, data of SpA patients switching from one biologic agent to another were analyzed. Demographic and clinical data, follow-up duration, time to switch, and reasons for switching were retrieved from the database. Kaplan-Meier analysis was performed to show drug retention rates and Cox regression analysis was performed to investigate the factors affecting switching. Results Of the included 3138 SpA patients, 1165 (37.1%) switched to another biologic agent (switched group) and 1973 (62.9%) continued to receive their current therapies (continued group). The median follow-up duration of all patients was 3.8 years and the median time to switch was 1.0 years (0-13.4 years). According to the distribution of comorbidities, the rates of patients having diabetes mellitus, hyperlipidemia, asthma, gastrointestinal bleeding, and cancer were significantly higher in the switched group than those of in the continued group (8.4% vs. 5.8%, p=0.006; 14.5% vs. 9.2%, p In the switched group (n=1165), the main reasons for switching were secondary inefficacy (n=351), primary inefficacy (n=328), and side effects (n=267) followed by primary or secondary unknown inefficacy (n=57), physician’s request (n=45), patient’s demand (n=36), willing to be pregnant (n=9), other (n=37), and unknown (n=70). Conclusion In SpA patients, switching was frequent between anti-TNF agents and the median time to first switch was 1 year. Female gender, short disease duration, and lower BASDAI score were found to be the significant factors affecting switching from the anti-TNF agent used at first. The main reasons for this switching were primary (29.0%) and secondary (31.0%) inefficacy followed by side effects (23.6%). Switching between subcutaneous anti-TNF agents is generally less than switching from infliximab to another biologic agent. Disclosure of Interests Umut Kalyoncu Grant/research support from: MSD, Roche, UCB, Novartis and Pfizer, Consultant for: MSD, Abbvie, Roche, UCB, Novartis, Pfizer and Abdi Ibrahim, Speakers bureau: MSD, Abbvie, Roche, UCB, Novartis, Pfizer and Abdi Ibrahim, Sedat Kiraz: None declared, Abdulsamet Erden: None declared, Orhan Kucuksahin: None declared, Timucin Kasifoglu: None declared, Ediz Dalkilic Grant/research support from: MSD and Abbvie, Consultant for: MSD, Abbvie,Roche, UCB, Pfizer and Novartis, Speakers bureau: MSD, Abbvie,Roche, UCB, Pfizer and Novartis, Cemal Bes: None declared, Nilufer Alpay Kanitez: None declared, Hakan Emmungil Grant/research support from: MSD, Roche, Pfizer, Abbvie, Consultant for: Novartis, Roche, Speakers bureau: MSD, Roche, Pfizer, Abbvie,Celltrion, Novartis, Pamir Atagunduz: None declared, Belkis Nihan Coskun: None declared, Burcu Yagiz: None declared, Suleyman Serdar Koca: None declared, Muhammet Cinar: None declared, Askin Ates: None declared, Servet Akar Grant/research support from: MSD, Abbvie, Roche, UCB, Novartis, Pfizer, Amgen, Consultant for: MSD, Abbvie, Roche, UCB, Novartis, Pfizer, Amgen, Speakers bureau: Pfizer, Onay Gercik: None declared, Duygu Ersozlu: None declared, Veli Yazisiz: None declared, Gezmis Kimyon: None declared, Muge Aydin: None declared, Ridvan Mercan: None declared, Burak Oz: None declared, Nazife Sule Yasar Bilge: None declared, Zeynel Abidin Akar: None declared, Omer Karadag: None declared, Ayse Bahar Kelesoglu Dincer: None declared, Sedat Yilmaz: None declared, Ufuk Ilgen: None declared, Yavuz Pehlivan: None declared, Ender Terzioglu: None declared, Levent Kilic: None declared, Sukran Erten: None declared, Ali Ihsan Ertenli: None declared

Background/aim We aimed to assess the association between retinal vascular caliber (RVC) scores and disease activity in rheumatoid arthritis (RA) patients. Materials and methods Forty-seven RA patients, 32 systemic lupus erythematosus (SLE) patients, and 45 healthy people were enrolled. RA and SLE patients were subdivided into groups according to C-reactive protein (CRP) levels. RA patients were also grouped according to Disease Activity Score-28 (DAS-28). Fundus photography was performed for all patients. RVC was summarized as the central retinal artery and vein equivalents (CRAE and CRVE). Results Mean CRVE for RA patients was 213.3 ± 17.8 μm compared with 209.2 ± 14.1 μm for SLE and 217.5 ± 26.2 μm for the control group (P = 0.17). RVC scores did not differ between the CRP-high and CRP-low groups. As the RA disease activity increased, the widening of CRVE became more prominent and statistically significant. When the DAS-28 > 5.1 (CRVE, 220.4 (211.8–246.5) μm) group and DAS-28 ≤ 3.2 (CRVE, 214.4 (172.4–242.3) μm) group were compared, statistical significance was more pronounced (P = 0.03) than when comparing the DAS-28 > 3.2 and DAS-28 ≤ 3.2 groups (P = 0.05). Conclusions CRVE, which reflects systemic inflammation and possibly increased cardiovascular risk, was significantly increased in active RA patients. The association between retinal venular widening and disease activity, regardless of CRP, may be a sign that RA-related inflammation may have systemic vascular effects even with normal levels of CRP.

Objective Psoriatic Arthritis Impact of Disease 12-item questionnaire (PsAID-12) has been developed to be used in psoriatic arthritis in daily practice. The aim of the present study was to assess the change values of PsAID-12 in PsA patients continuing or discontinuing anti-TNF treatment. Methods We recruited patients from the Hacettepe University biological database (HUR-BIO). Overall, 70 PsA patients had PsAID-12 score before the initiation of the first anti-TNF treatment. Stopping or switching the anti-TNF treatment due to inefficacy was definitely considered a negative response. Changes were evaluated by the comparison with the baseline PsAID-12 score in compliance with the favorable and unfavorable responses to anti-TNF treatments. The standardized response mean (SRM) was used for determining the response. Results Seventy (78.6% female) patients were analyzed and their mean age was 45.5 years (12.0 years). The mean follow-up duration was 18.3 months (12.6 months). At baseline, the mean PsAID-12 score was 6.6 (1.5). Physicians stopped or switched the treatment in 28 patients (40.0%) due to the inefficacy of anti-TNF treatment. The ΔPsAID-12 score was 0.25 (1.71) in the patients discontinuing anti-TNF treatment and 3.52 (2.31) in the patients continuing their anti-TNF treatment (p

AIM The aims of the study are to investigate serum neopterin and ischemia modified albumin (IMA) levels in patients with immunoglobulin A vasculitis (IgAV) and evaluate the association of these markers with disease activity and relapse. METHOD Thirty-four consecutive adult patients (24 male and 10 female) admitted to the rheumatology clinic and met the IgAV American College of Rheumatology (ACR) criteria were enrolled in this cross-sectional study. Demographic and clinical features of IgAV and a control group were recorded into a predefined protocol. Disease activity was categorized as "remission" or "active" according to Birmingham Vasculitis Activity Score (BVAS). BVAS ≥ 1 was accepted as "active". Serum neopterin levels, high-sensitivity C-reactive protein (hsCRP) and IMA were evaluated according to BVAS and compared to the healthy control group. RESULTS Serum median (interquartile range) neopterin, IMA levels and hsCRP were higher in the study group than in control group (2.01 [12.5] ng/mL vs 1.77 [1.37] ng/mL, 0.67 [0.2] ng/mL vs. 0.43 [0.17] ng/mL, 5.6 [17.1] mg/L vs. 1.55 [1.6] mg/L, P = .095, P

WOS: 000507466904264, …

IgG4-related sclerosing disease is characterized by infiltration of various tissues and organs, including lung, kidney, pancreas, salivary glands, biliary tree, breast and vessels with IgG4-rich plasma cells. Fibroinflammatory infiltration of these tissues usually progress into fibrosis, eventually resulting in organ failure. In this case report, ultrasonography, computed tomography and positron emission computed tomography features of IgG4-related sclerosing disease involving the retroperitoneum and mesentery are presented together with histopathologic correlation.

WOS: 000397050600018, PubMed ID: 27794533, Objective. The aim was to assess the characteristics of PsA, find out how well the disease is controlled in real life, demonstrate the treatments and identify the unmet needs. Methods. The PsA registry of Turkey is a multicentre Web-based registry established in 2014 and including 32 rheumatology centres. Detailed data regarding demographics for skin and joint disease, disease activity assessments and treatment choices were collected. Results. One thousand and eighty-one patients (64.7% women) with a mean (S.D.) PsA duration of 5.8 (6.7) years were enrolled. The most frequent type of PsA was polyarticular [437 (40.5%)], followed by oligoarticular [407 (37.7%)] and axial disease [372 (34.4%)]. The mean (S.D.) swollen and tender joint counts were 1.7 (3) and 3.6 (4.8), respectively. Of these patients, 38.6% were on conventional synthetic DMARD monotherapy, 7.1% were on anti-TNF monotherapy, and 22.5% were using anti-TNF plus conventional synthetic DMARD combinations. According to DAS28, 86 (12.4%) patients had high and 105 (15.2%) had moderate disease activity. Low disease activity was achieved in 317 (45.7%) patients, and 185 (26.7%) were in remission. Minimal disease activity data could be calculated in 247 patients, 105 of whom (42.5%) had minimal disease activity. The major differences among sexes were that women were older and had less frequent axial disease, more fatigue, higher HAQ scores and less remission. Conclusion. The PsA registry of Turkey had similarities with previously published registries, supporting its external validity. The finding that women had more fatigue and worse functioning as well as the high percentage of active disease state highlight the unmet need in treatment of PsA.

Reactive haemophagocytic syndrome (RHS) is a hyperinflammatory disorder often occurring in the background of several disorders such as infections, malignancies, and rheumatic diseases. Recently, a score known as the HScore was developed for the diagnosis of RHS. In the original study, most of the patients had underlying haematological malignancy or infection and the best cut-off value for the HScore was 169 (sensitivity 93%; specificity 86%). In this study we aimed to analyse the performance of the HScore in rheumatic disease-related RHS.The patients with rheumatic disorders evaluated in the Departments of Rheumatology and Paediatric Rheumatology at Hacettepe University, Ankara, Turkey between 2002 and 2014 were reviewed retrospectively. The first group (n = 30) consisted of patients with RHS; the control group (n = 64) included patients with active rheumatic diseases without RHS.In the RHS group, 14 (46.7%) had adult-onset Still's disease (AOSD), 10 (33.3%) systemic juvenile idiopathic arthritis (SJIA), and six (20%) systemic lupus erythematosus (SLE). The control group (n = 64) consisted of 32 (50%) AOSD, 13 (20.3%) SJIA, and 19 (29.7%) SLE patients. Applying the HScore to the RHS patients, the best cut-off value was 190.5 with a sensitivity of 96.7% and specificity of 98.4%. When we excluded the patients from the control group who had not had bone marrow aspiration (n = 23), the same cut-off (190.5) performed best (sensitivity 96.7%; specificity 97.6%). Applying the 2004 haemophagocytic lymphohistiocytosis (HLH-2004) criteria gave a sensitivity of 56.6% and a specificity of 100% in the whole study group.In our study, a cut-off value for the HScore different from the original study performed better. Further studies are warranted to determine optimum cut-off values in different studies.

Deniz Avci,1 Hatice Karagoz,2 Ozerhan Ozer,1 Kubra Esmeray,1 Kadir Bulut,1 Fatma Aykas,1 Ali Cetinkaya,1 Emine Uslu,1 Samet Karahan,1 Mustafa Basak,1 Abdulsamet Erden1 1Internal Medicine Department, Kayseri Training and Research Hospital, 2Internal Medicine Department, Acibadem Kayseri Hospital, Kayseri, Turkey Introduction: Preeclampsia (PE) is a pregnancy-related disorder characterized by hypertension (HT) and proteinuria noticeable after 20 weeks of gestation. PE is now considered as a cardiovascular disease risk factor and a number of studies have shown that experiencing PE increases the prevalence of various cardiovascular risk factors, such as metabolic syndrome and HT. In this study, we aimed to investigate any possible relationship between the ABO/Rh blood group system and PE in Turkey. In the second part of the study, we examined the relationship between the ABO blood group system and development of HT after PE. Patients and methods: A total of 250 patients with PE from Kayseri Training and Research Hospital between 2002 and 2012 were included in the study. Patients were classified according to blood groups (A, B, AB, and O) and Rh status (+/-). Results: There was a significant difference between the patients with PE and the control group in terms of distribution of ABO blood groups and the percentage of group AB was found to be higher in patients with PE compared to the control group (P=0.029). The risk of developing PE was significantly higher in group AB than other blood groups (P=0.006). The risk of developing HT after PE was significantly higher in group O than other blood groups (P=0.004). Discussion: In this study, we found that the patients with blood group AB have a higher risk for PE. The patients with PE of blood group O are at high risk of developing HT, and Rh factor was identified as another risk at this point and these patients should be closely followed postpartum. Keywords: ABO blood groups, Rh factor, preeclampsia, proteinuria, pregnancy, hypertension, postpartum

Background:Patients with rheumatological diseases can also apply to the emergency room due to acute attacks or complications. Especially in recent years, more patients, due to the increased use of immunosuppressant drugs in treatment, have applied to emergency services due to infection. On the other hand, data on applications to emergency services for rheumatological reasons are very few.Objectives:In this study, applications to emergency departments for inflammatory rheumatic diseases were investigated.Methods:2715 patients from Atatürk Training and Research Hospital Rheumatology Clinic who were followed-up with the diagnosis of inflammatory rheumatic diseases between 2014 and 2018 were included in this study. The clinical and laboratory information of the patients were achieved from the hospital file records and the hospital data bank. The patients were classified according to the 5-stage triage system (T1: resuscitation, T2: critical, T3: urgent, T4: less urgent, T5: non-urgent).Results:21.3% (577) of 2715 patients applied to the emergency department. Among the emergency admissions, the first three diseases were; rheumatoid arthritis 19.7%, ankylosing spondylitis 19.2%, and familial Mediterranean fever 15.9% [Vasculitis 8.8%, Behcet’s disease 7.6%, Gout 5.7%, Systemic lupus erythematosus 3.9%, Sjogren’s Syndrome 3.6%, Scleroderma 3.2%, Still Disease 1.5%, Polymyalgia rheumatica 0.6%, others 9%]. 343 patients (59.5%) were discharged from the emergency department. 36.8% (212) of the patients were hospitalized in services and 3.6% (21) of them in intensive care. The first three reasons of the applications to the emergency department were: fever, malaise and fatigue in 1. 150 patients (25.9%), musculoskeletal system complaints in 2. 124 patients (21.4%) and abdominal pain in 3. 89 patients (15.4%), respectively.Vasculitis was the most common cause of hospitalization in the service in 38 patients (17.9%), whereas scleroderma was the most common cause of hospitalization in intensive care in 7 patients (33.3%) (table). 16 (76.1%) of the patients followed in intensive care unit were hospitalized with the diagnosis of respiratory system diseases. Death was observed in 10 (1.7%) of 577 patients. Five (50%) of the ex-patients had scleroderma (table). 8 (80%) of the deaths were related to rheumatological disease and occurred after being hospitalized in intensive care unit.Conclusion:The patients with inflammatory rheumatic diseases usually apply to the emergency department with urgent or less urgent clinical pictures. Rheumatoid arthritis, one of the most common inflammatory rheumatic diseases in almost all societies, was also the most common diagnosis in our study. In the vast majority of applications, infectious causes and disease activations related to immunosuppressive treatments were in the foreground. In addition, although vasculitis is the most common reason for the in-patients in clinical service, since the most frequent in-patients in intensive care unit and death is seen in the scleroderma group; caution should be exercised in the emergency applications of patients with these two groups.References:[1]Rheumatology consultation in the emergency department: Annual results. RAED 2014Table.Examination of the diagnosis of 233 patients who were hospitalized and/or died from the emergency departmentParametersIn-patients in clinical service n, (%)In-patients in intensive care units n, (%)Died patients n, (%)Vasculitis38 (17.9)5 (23.8)1 (1)Ankylosing spondylitis33 (15.5)Rheumatoid arthritis31 (14.6)3 (14.2)2 (20)Behcet’s disease25 (11.7)2 (9.5)Systemic lupus erythematosus16 (7.5)1 (4.7)5 (50)Scleroderma10 (4.7)7 (33.3)Antiphospholipid syndrome10 (4.7)Psoriatic arthritis9 (4.2)Family Mediterranean fever8 (3.7)Sjogren’s Syndrome8 (3.7)Still Disease7 (3.3)1 (4.7)1 (10)Gout7 (3.3)Undifferentiated connective tissue disease7 (3.3)2 (9.5)1 (10)Dermatomyositis3 (1.4)Total212 (100)21 (100)10 (100)Disclosure of Interests:None declared

Muscle weakness is a nonspecific finding of myopathy of any etiology that include iatrogenic, toxic, endocrinological, infectious, immunologic, and metabolic disorders. Among the metabolic myopathies glutaric aciduria type II (GAII) is an autosomal recessively inherited rare disorder of fatty acid and amino acid metabolisms. The late onset form is heterogeneous in terms of symptomatology and severity and for the cases that chronic manifestations of lipid storage myopathy are the only clues for the disease, differential diagnosis can be challenging. Here we report two cases of GAII: the first one was 18-year old boy who presented with proximal muscle weakness and in another center, he was diagnosed as polymyositis and treated with immunosuppressive therapies. He admitted to our clinic with ongoing muscle weakness and symptoms that were related to the side effects of immunosuppressive therapies. The second case was also presented with muscle weakness. For both cases, muscle biopsies and urinary organic acid analyses were consistent with the diagnosis of GAII. To differentiate inflammatory myositis from non-inflammatory myopathies; rheumatic symptoms, accompanying complaints of the patient and autoantibody positivity can be helpful. To our knowledge this is the first report to underline the differential diagnosis of inflammatory myopathies from metabolic myopathies.

Background: Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) and immunoglobulin G4-related disease (IgG4-RD) have some common features. The co-occurrence/concurrence of AAV and IgG4-RD was recently published by the collaborative European Vasculitis Study Group. First, we aimed to investigate ANCA positivity of our IgG4-RD cohort. Second, a literature review of co-occurrence/concurrence of AAV and IgG4-RD was done. Methods: Data of 62 patients with IgG4-RD in Hacettepe Vasculitis Center Database were used. Patient dataset was designed to include demographic data, clinical characteristics, imaging and IgG4-RD, AAV and ANCA test results. At the next step, we performed a systematic literature review in PUBMED database covering the time period from 1976 until April 2018. Relevant publications were searched using these MeSH terms ''IgG4-related disease and Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis'', "IgG4-related disease and Eosinophilic Granulomatosis with Polyangiitis", "IgG4-related disease and Microscopic Polyangiitis" and "IgG4-related disease and Granulomatosis with Polyangiitis". Results: Three (10.3%) of 29 patients had low titer ANCA positivity. These three patients didn't have any findings of vasculitis and no granuloma was seen in biopsy. In the literature review, we found 17 cases had features of both IgG4-RD and AAV. These cases were re-evaluated according to the Comprehensive Diagnostic Criteria for IgG4-RD. ANCA were positive in 15 of 17 patients (88%). Conclusion: None of our IgG4-RD patients overlapped with AAV. Only two patients in the literature review seemed to be fully compatible with both diseases. Even though AAV and IgG4-RD share similar clinical features, we think this might be a co-occurrence instead of a histopathological link.

Objectives Takayasu arteritis and Spondyloarthritis are two distinct inflammatory diseases that affect the same age periods. Increasing number of reports on co-incident Takayasu arteritis-spondyloarthritis cases in literature raised the hypotheses about their association. The purpose of this study is to evaluate the incidence of spondyloarthropathy spectrum diseases in Takayasu arteritis patients. Methods Detailed clinical and demographic features of Takayasu arteritis patients were recorded and all were screened meticulously for the presence of spondyloarthropathy features following recommendations of Assessment of SpondyloArthritis international Society. Patients were questioned for inflammatory back pain, enthesitis, uveitis, inflammatory bowel disease, peripheral arthritis, and investigated accordingly with HLA-B27, plain X-rays and sacroiliac magnetic resonance imaging. Results A total of 69 Takayasu arteritis patients (65 female, 94.2%) were enrolled. After detailed investigation, 14 (20.3%) Takayasu arteritis patients fulfilled the Assessment of SpondyloArthritis international Society criteria for Spondyloarthropathy. Two of 14 (14.2%) spondyloarthropathy patients were positive for HLA-B27. Type 1 and type 2 Takayasu arteritis were more common in patients with diagnosis of both Takayasu arteritis and spondyloarthropathy than those without spondyloarthropathy. Most of patients with diagnosis of both these diseases required biologic therapies than patients with diagnosis of Takayasu arteritis alone (64.3% vs 29.1%, P = 0.014) due to refractory Takayasu arteritis. Conclusion Our results suggest a significant association between Takayasu arteritis and spondyloarthropathy. Possible shared genetic or immunopathogenic processes may explain this association, which merits further investigations.

Minimal disease activity (MDA) is an important target in patients with psoriatic arthritis (PsA), however it is also criticised for having a low threshold for patient reported outcomes (PRO).The aim of the study was to assess the prevalence of MDA and its components in patients with PsA and to evaluate disease characteristics and patterns in patients with or without MDA (MDA+ or MDA-).PsArt-ID (Psoriatic Arthritis-International Database) is a prospective, multicentre web-based registry. PsA patients who had at least 1 year of disease duration and had full data for MDA were included for this analysis (n=317). Patients were considered in MDA+ when they met at least 5/7 of the MDA criteria.MDA was achieved in 46% patients. Within MDA- patients, body surface area (51.2%) and swollen joint count (53.5%) domains could still be achieved in the majority and 93.5% of them had no enthesitis using the Leeds enthesitis index. Of 170 patients with MDA-, 90 patients did not fulfill all 3 PROs of MDA. Mono-arthritis subtype (RR: 2.01), absence of enthesitis (RR: 1.570) and absence of distal interphalangeal (DIP) joint disease (RR: 1.1) were associated with higher probability of achieving MDA.The MDA criteria provide an objective target for treatment in trials and clinical practice; however, in real life PROs are the most significant barriers to achieve MDA. The presence of DIP joints disease makes it difficult to reach MDA due to active PROs.

Polyarteritis nodosa is a necrotising vasculitis of predominantly medium size vessels. The present study aimed to summarise the characteristics of PAN patients, and also analyse the trend of decreasing PAN frequency in the last 25 years.PAN patients followed up between 1990 and 2015 were included. The demographics, clinical findings and outcomes were retrospectively evaluated.One hundred thirty-three patients, including 66 children, were enrolled in the study. Among 133 patients, 86 (64.7%) had fever, 108 (81.2%) had skin involvement, 54 (40.6%) had renal involvement, 43 (32.3%) had neurological involvement, 32 (24.1%) had gastrointestinal involvement, 10 (7.5%) had cardiac involvement, 6 (4.5%) had pulmonary involvement. The median (minimum-maximum) leukocyte count, erythrocyte sedimentation rate and C-reactive protein levels at the time of diagnosis were 10400 (6100-32000)/mm3, 58 (2-132) mm/h and 5.22 (0-46) mg/dL, respectively. All patients were ANCA negative. Hepatitis serology was analysed in 121 patients and found positive in 13 of them. MEFV mutations were screened among 65 patients, 24 of them had mutations in at least one allele. Biopsy was performed in 109 patients and angiography was performed in 92 patients. The number of PAN patients declined significantly after 2010. 9 patients were re-categorised as DADA2 after 2014 and no patient were diagnosed with FMF+PAN after 2008.Our results suggest a decrease in PAN in our country which may be due to improved healthcare and dissecting mimicking diseases. Further prospective studies with prolonged follow-up could help us to better understand the disease characteristics.

Aim/purpose: Our aim was to investigate the association between blood groups and colchicine resistance in familial Mediterranean fever (FMF) patients. Methods: This is a single-center, cross-sectional study. Between January and December 2016, 385 FMF patients were assessed by the Adult and Pediatric Rheumatology outpatient clinics and 297 patients had blood groups (ABO and Rh) results. The patients were grouped into two groups: colchicine-responsive patients (Group CR) and colchicine-unresponsive patients (Group CUR). Results: Patients with blood group A had 1.5-fold higher FMF compared with non-A blood group (OR: 1.50 [95% CI: 1.11–1.87]), particularly having a Rh (+) blood group (OR: 1.47 [95% CI: 1.13–1.91]). Furthermore, patients with blood group A had a better response to colchicine treatment than non-A blood group; (OR: 2.21 [95% CI: 1.15–4.27]). Patients with blood group O were prominently associated with colchicine resistance. Conclusion: ABO blood phenogroups may be used in combination with other risk factors to identify FMF patients and patients at high risk for colchicine resistance.

Background Immune-mediated necrotising myopathies are characterised clinically by the subacute onset of proximal limb weakness, accompanied by elevated creatinine kinase levels. They are distinguished from other myopathies by the absence of prominent infiltration of the muscle with inflammatory cells in the biopsies. Case presentation A 44-year-old man presented with upper extremity weakness and dysphagia. Laboratory tests included a creatinine kinase level of 4362 U/L (normal: 52–336 U/L). Rheumatological markers were all negative. A muscle biopsy showed multiple necrotic fibres with minimal inflammatory infiltration. One gram of methylprednisolone (IV) was given, followed by 1 mg/kg of methylprednisolone daily by the oral route. Intravenous immunoglobulin (0.4 mg/kg/day) was given for five days. Muscle weakness regressed and dysphagia disappeared with treatment. The patient remains well in the 23rd month of treatment, taking 5 mg/day prednisolone and monthly intravenous immunoglobulin. Conclusion Treatment of immune-mediated necrotising myopathy can be challenging as evidence-based therapeutic options are limited. It is generally accepted that early and extensive immunosuppression, including glucocorticoids as first-line agents, may be required.

The objective of this study was to assess the frequency of comorbidities and multimorbidities in rheumatoid arthritis (RA) patients under biologic therapy and their effects on biological disease modifying antirheumatic drugs (DMARDs) choice, timing, and response. Hacettepe University Biologic Registry (HUR-BIO) is single center biological DMARD registry. Cardiovascular, infectious, cancer, and other comorbidities were recorded with face to face interviews. Multimorbidity is defined as >1 comorbidity. Disease duration, initial date of biological DMARDs, initial and overall biological DMARD choice were recorded. Disease activity score-28 (DAS-28) responses were compared to comorbidity presence and multimorbidity. Total of 998 RA patients were enrolled into the study. The mean age was 53.1 (12.5) and mean disease duration (standard deviation [SD]) was 11.7 (7.5) years. At least 1 comorbidity was detected in 689 (69.1%) patients, 375 (37.9%) patients had multimorbidity. Patients had mean 1.36 ± 1.32 comorbidity. The median durations of first biological DMARDs prescription were 60 (3–552) months after RA diagnosis. For multimorbidity patients, the median first biological prescription duration was longer than the duration for patients without multimorbidity (72 [3–552] vs 60 [3–396] months, P

In patients with systemic sclerosis (SSc) gastrointestinal (GI) involvement, nutritional status and medications may lead to cobalamin (Vit B12) deficiency. We aimed to determine the frequency and the potential causes of Vit B12 deficiency in SSc patients.We conducted a cross-sectional analysis of 62 SSc patients in a single center in 1 year period. Medical history and physical examination of patients were reevaluated. Data about organ involvements were obtained from hospital file records. The nutritional status of the patients was assessed with Malnutrition Universal Screening Tool (MUST). Vit B12, homocysteine (except in three patients) and Helicobacter Pylori Immunoglobulin G (H. Pylori IgG) levels were measured in all patients. Vit B12 deficiency was considered as serum Vit B12 level200 pg/mL or being on Vit B12 replacement therapy. Serum Vit B12 levels of the patients were also grouped as low (200 pg/mL), borderline (200-300 pg/mL) and normal (300 pg/mL). Plasma homocysteine levels of the patients were classified as elevated (9 μmol/L) and hyperhomocysteinemia (15 μmol/L). Mann-Whitney U and Kruskal-Wallis tests were used to compare parameters among the groups. Correlation was tested by Spearman's correlation coefficient.Forty-four (71.0%) patients were defined as Vit B12 deficient; 22 had Vit B12 level200 pg/mL (four were on Vit B12 replacement therapy) and the remaining 22 had Vit B12200 pg/mL and were already on Vit B12 replacement therapy. The percentage of the patients with hyperhomocysteinemia was significantly higher in the group with Vit B12200 pg/mL as compared to other groups (P = 0.004) but only 33.3% (7/21) of the patients with Vit B12200 pg/mL had hyperhomocysteinemia. There were no statistically significant differences between patients with and without Vit B12 deficiency regarding age, mean disease duration, MUST scores, mean hemoglobin levels, H. Pylori IgG positivity and organ involvements (P0.05 for all).Vit B12 deficiency is frequent in SSc and has multiple causes. All patients should be monitored for Vit B12 deficiency. The homocysteine levels seem unlikely to be helpful for confirmation of Vit B12 deficiency.

Objective: Familial Mediterranean fever (FMF) is a periodic fever syndrome caused by MEFV mutations. FMF may be associated with psoriasis in some cases. The prevalence of psoriasis in the normal Turkish population is 0.42%. We aimed to investigate the prevalence of psoriasis among FMF patients and their relatives. Methods: FMF patients followed at Hacettepe University Adult and Pediatric Rheumatology Departments between January and August 2016 were included. FMF patients/their relatives were accepted to have psoriasis if the diagnosis was made by a dermatologist. Results: A total of 351 FMF patients (177 adults; 174 children) were included. The median (min–max) age of adult and pediatric patients was 35 (19–63) and 10 (2–18) years, respectively. Thirteen (3.7%) FMF patients (11 adults, 2 children) had psoriasis. Psoriasis was more common in adult than pediatric patients (p = 0.02). Psoriasis was present in 22 (12.4%) of adult and 9 (5.2%) of pediatric patients’ relatives (p = 0.023). The frequency of psoriasis in ≥1 relatives of FMF patients was found to be 8.8%. Abdominal pain and fever were significantly higher, and arthralgia, arthritis, pleural chest pain, and pericarditis were significantly less frequent in the pediatric group than in adults (p

Behcet’s disease (BD) is one of the reasons of acquired visual impairment among young adults. Ocular involvement is a significant cause of disability in BD. The objective of this study is to assess the prevalence of BD among a group of adults who has visual impairment. Ankara Metropolitan Municipality Education and Technology Center is one of the official institutions which records and follows the demographic data of visually impaired people in Turkey. In November 2014, there were 675 visually impaired people recorded at this center. Medical history was taken from 294 adults by phone in November and December of 2014. Participants were asked if the visual impairment had been either acquired or congenital. If the patients had BD or suspicious BD, they were recalled for detailed investigation which would be carried out by an internist, a rheumatologist and an ophthalmologist. Two hundred thirteen of 294 (72.4%) visually impaired adults were male. One hundred nine of 294 (37.1%) had acquired visual impairment. Six (5.5%) of those 109 patients had BD. Overall prevalence of BD among study group was 2.04%.The median age of people with BD was 35 years. The median age at BD diagnosis was 16.5 years and the median duration from diagnosis to visual loss was 2.5 years. BD is still one of the causes of acquired visual impairment in Turkey. In this study, BD prevalence among a visually impaired adult group was 2.04%. BD accounted for 5.5% among adults who had acquired visual impairment. In a study of 1965, BD prevalence among people with acquired blindness was 12%. However, this study was conducted at pre-immunosuppressive period. Our prevalence is obviously lower than those studies. Extended population-based studies are needed for population estimations.

Objective: Cardiovascular disease (CVD) is more prevalent in almost all patients with chronic inflammatory musculoskeletal diseases than in their healthy counterparts. The aim of this study was to assess the presence of subclinical atherosclerosis in patients with psoriatic arthritis (PsA) in comparison with patients with rheumatoid arthritis (RA) and healthy controls. Methods: A total of 30 patients with PsA, 30 patients with RA, and 30 healthy controls were enrolled in this parallel group study. Demographic, clinical, and laboratory data of the groups were recorded. The Disease Activity Score-28 tool was used for joint assessment. The erythrocyte sedimentation rate and C-reactive protein level were measured as acute phase reactants. Flow-mediated dilatation (FMD) and carotid intima media thickness (CIMT) were also measured in all participants. Results: The median duration of disease in patients with PsA was 60 months (range: 8–216 months). A total of 22 of 30 (73.3%) PsA patients had a diagnosis of psoriasis and 13 (48.1%) had active disease. The study groups were similar with regard to age, gender, and body mass index data. In all, 23 (76.7%) of the PsA patients and 5 (16.7%) of the RA patients were using an anti-tumor necrosis factor alpha therapy (p

Objective: Immunoglobulin A vasculitis/Henoch–Schönlein purpura (IgAV/HSP) is a systemic vasculitis involving small vessels with the deposition of immune complexes containing IgA. It is the most common primary systemic vasculitis of childhood and is much less common in adults. Our aim was to investigate the differences and similarities between adult and paediatric patients with IgAV/HSP. Method: We retrospectively evaluated the medical records of 35 adult and 159 paediatric (˂ 18 years old) patients with a clinical diagnosis of IgAV/HSP who were seen at the Departments of Rheumatology and Pediatric Rheumatology, Hacettepe University, Ankara, Turkey. The paediatric and adult patients were classified with IgAV/HSP according to the Ankara 2008 and American College of Rheumatology 1990 criteria, respectively. Results: Upper respiratory tract infection was a common predisposing factor for both adults (34.3%) and children (21.4%). Creatinine and C-reactive protein were higher; and skin biopsy, hypertension, renal involvement, haematuria, proteinuria, and renal insufficiency at diagnosis were more frequent in adults than in children. Thrombocyte count was higher in children than in adults. Follow-up without treatment and complete recovery were more frequent in children, while persistent haematuria, chronic renal failure, relapse, and the use of corticosteroids/azathioprine were more frequent in adults. The only independent predictive factor for relapse was persistent haematuria. Conclusion: Various clinical and laboratory characteristics differ between children and adults with IgAV/HSP. Overall, IgAV/HSP has a self-limiting course in children but represents a more severe form of disease in adults, with more severe renal involvement. Persistent haematuria is a predictive factor for relapse.

Çalışmada 25 yazar bulunmaktadır. Bu yazarlardan sadece Bursa Uludağ Üniversitesi mensuplarının girişleri yapılmıştır. Psoriatic arthritis (PsA) may affect different joints, including the spine. The prevalence of spinal involvement is variable depending on the definition and a subset of patients have been identified in cohorts that do not have clinical features of axial disease and yet have imaging findings. Still, there is not a consensus on how and when to screen axial disease. In this study, we aimed to investigate factors associated with being underdiagnosed for axial psoriatic arthritis (axPsA) and its impacts on outcomes. Disease features and outcomes of axPsA according to the physician (n=415) were compared with patients with imaging findings only (sacroiliitis fulfilling the modified New York criteria, n=112), using data from a real-life PsA registry. Patients with imaging findings only were more frequently women (83/220 (37.7%) vs 29/122 (23.8%); p=0.008). This group also had higher peripheral disease activity (imaging only vs clinical AxPsA: mean (SD) tender joint count 5.3 (6.1) vs 3.3 (4.7), swollen joint count 1.9 (2.9) vs 1.2 (2.4); p

Secondary amyloid A (AA) amyloidosis is a late and serious complication of poorly controlled, chronic inflammatory diseases. Rheumatoid arthritis (RA) patients with poorly controlled, longstanding disease and those with extra-articular manifestations are under risk for the development of AA amyloidosis. Although new drugs have proven to be significantly effective in the treatment of secondary AA amyloidosis, no treatment modality has proven to be ideal. To date, only in small case series preliminary clinical improvement have been shown with rituximab therapy for AA amyloidosis secondary to RA that is refractory to TNF-&alpha, inhibitors (TNF-i) therapy. In these case series, we assessed the efficacy and safety of rituximab therapy for patients with RA and secondary amyloidosis. Hacettepe University Biologic Registry was developed at 2005. The data of the RA patients who were prescribed a biological drug were recorded regularly. Patients with biopsy proven AA amyloidosis patients were screened. Of 1022 RA patients under biologic therapy, 0.7% patients had clinically apparent histologically confirmed amyloidosis. Four of seven patients who were prescribed rituximab at least one infusion enrolled to those case series. Two of four patients showed significant clinical improvement and one of them also had decrease in proteinuria and the other one had stable renal function and proteinuria. The main goal for the treatment of AA amyloidosis is to control the activity of the underlying disorder. In this study, we showed that rituximab may be an effective treatment in RA patients with amyloidosis who were unresponsive to conventional disease modifying anti-rheumatic drugs (DMARDs) and/or TNFi.