S.A. Bilgen

Assessment of the HScore for reactive haemophagocytic syndrome in patients with rheumatic diseases

Objectives: 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. Method: 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. Results: 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. Conclusions: 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.

Inflammatory bowel diseases and Takayasu's arteritis: coincidence or association?

Takayasus arteritis (TA) and inflammatory bowel disease (IBD) are rare diseases but there are case reports presenting their co‐existence in the literature. The aim of this study was to investigate the relation between IBD and TA.

Aspirin resistance in systemic lupus erythematosus. A pilot study

Systemic lupus erythematosus (SLE) patients are at increased risk of thrombosis and cardiovascular diseases. Aspirin is an effective treatment option for these patients. The aim of this study was to investigate the presence of aspirin resistance in SLE patients. We studied aspirin resistance in 33 SLE patients and nine healthy controls by using a Multiplate® impedance aggregometer (Dynabyte GmbH, Munich, Germany). Twenty-six SLE patients were on regular aspirin treatment. Aspirin resistance was found in five (19.2%) out of 26 patients who were on aspirin treatment. When the tests were repeated by adding acetylsalicylic acid in the medium, all of these patients became responsive to the aspirin. SLE disease activity, body mass index, smoking status, and the presence of anticardiolipin antibodies or positive lupus anticoagulant test results were no different in patients with or without aspirin resistance. (p > 0.05 for all). Our results suggest that there may be a considerable number of SLE patients with aspirin resistance.

Rituximab Experience in Patients With Long-standing Systemic Sclerosis–Associated Interstitial Lung Disease: A Series of 14 Patients

Objectives The objective of this study was to report the experience with rituximab treatment in a case series of patients with long-standing systemic sclerosis–associated interstitial lung disease (SSc-ILD). Methods We reviewed the charts of 197 SSc patients. Fourteen patients who received rituximab for SSc-ILD participated in this analysis. Pulmonary function tests, high-resolution thorax computed tomography and modified Rodnan skin scores were evaluated at baseline and end of the follow-up. Results Median age was 53.2 years (interquartile range, 46.8–55.5 years), and median disease duration was 9.1 years (interquartile range, 5.1–13.6 years). At the end of median follow-up (15 months), although the median forced vital capacity value increased compared with baseline, the change was not statistically significant (52.5 vs. 58.0, P = 0.065). Forced vital capacity was improved in 4 patients and stabilized in 10 patients. High-resolution computed tomography was stable in 7 patients and worsened in 3 patients. Modified Rodnan skin scores remained stable at the end of follow-up (8.0 vs. 6.0, P = 0.6). Conclusions The improvement or stabilization of pulmonary disease was observed in most SSc patients with longer disease duration and worse pulmonary function. Rituximab might be useful in this patient group who is resistant to conventional immunosuppressive treatments.

Effect of capillaroscopic patterns on the pulse oximetry measurements in systemic sclerosis patients

Nailfold capillaroscopy is a simple method for determining microvascular damage in systemic sclerosis (SSc). The pulse oximeter is a noninvasive tool that is used to estimate a patients arterial blood oxygen saturation. The aim of this study was to investigate the effect of capillaroscopic patterns on pulse oximetry measurements in SSc patients. The nailfolds from 2nd to 5th fingers in both hands were examined in all subjects by using capillaroscopy. Patients were categorized according to their capillaroscopy findings in 3 groups as having early, active or late patterns. Oxygen saturation (SpO2) measurements were performed from 2nd to 5th fingers in both hands with a finger probe. We studied 53 SSc patients (F/M: 48/5). According to capillaroscopy findings 18 patients were classified as having early pattern (34.0%), 19 active pattern (35.8%), and 15 late pattern (28.3%). Only 1 (1.9%) patient had normal capillaroscopy findings. SpO2 could not be measured in 47 (11.0%) fingers and in 20 (37.7%) SSc patients. There were 20 (37.7%) patients with a ≥4% difference between the minimum and maximum SpO2 measurements among fingers. There were no difference between the groups of SSc patients defined by capillaroscopy findings in terms of mean maximal SpO2 or mean minimal SpO2 measured from fingers (p NS, for all). Assessment of SpO2 values in patients with SSc is challenging. We did not detect any effect of capillaroscopic patterns on mean SpO2 values. On the other hand ≥4% difference between minimum and maximum SpO2 values measured from fingers of a patient may be considered as an indirect sign of microvascular damage. Assessment of the highest measured SpO2 values among the fingers of a patient may be more suitable in practice.

AB0357 Pregnancy outcomes of rheumatic patients with pre/peri gestational leflunomide exposure

Background Leflunomide is a frequently used disease modifying drug in rheumatoid arthritis, psoriatic arthritis, vasculitis etc. Findings from animal studies have suggested that leflunomide may be a human teratogen and a wash out protocol is required for patients before planning pregnancy. Data about pregnancy in patients with leflunomide exposure is limited with case reports and case series. Objectives This study is aimed to evaluate the outcomes of pregnancies in patients with leflunomide exposure. Methods Hospital files between years of 2004-2012 were used for selecting patients who used leflunomide before or during pregnancy. In addition to demographic and clinical characteristics of patients, a study form consist of -drugs usedbefore pregnancy, elimination application, duration of pregnancy, drug use and pregnancy outcomes- is filled. Totally 33 patients (female /male: 24/9) were included into the study. Results In 12 of 33 patients (36.4%) no pregnancy occurred during more than 1 year follow up. Pregnancy took place in 16 (66.7%) of female and 5 (55.6%) of male patients’ wives while in others no pregnancy occurred Bloods levels of leflunomide were examined in 19 of 21 pregnancies. Leflunomide washout protocol with cholestyramine was applied in 15 patients. In 13 of female patients leflunomide was interrupted before pregnancy but in 3 patients it was stopped after detection of pregnancy. Results and the complications of pregnancies were shown in Table 1. Anti-Tumor necrosis factor (Anti-TNF) usage was positive in 4 of them – one of them had used during pregnancy. Majormalformations were not detected in any infants. Premature membrane rupture was observed in wife of a male patient. Conclusions Observation of none pregnancy in more than one third of patients during >1 year follow up, might reflect that rheumatic patients on leflunomide treatment had an increased reproductive problems. Wash out protocol and assessing blood drug level are used in most of patients before planning pregnancy. Althoughwe experienced no major malformations, the rate of complications such as premature rupture of membrane and low birth weight were higher. To give rise to more precise data, methods of contraception should be advised in patients receiving leflunomide. Disclosure of Interest None Declared

Increased psoriasis frequency in patients with familial Mediterranean fever

Abstract 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 < 0.05). Conclusion: Psoriasis was more common in FMF patients than in the normal population. Thus, FMF patients should be questioned and carefully examined for psoriasis lesions and psoriasis family history. Prospective multicenter studies may be important to find the incidence of psoriasis in FMF.

Therapeutic plasma exchange for refractory SLE: a comparison of outcomes between different subphenotypes

Objective Therapeutic plasma exchange (TPE) offers an alternative therapeutic modality for patients with systemic lupus erythematosus (SLE) and primary antiphospholipid syndrome (APS). However, there is conflicting evidence regarding its efficacy in different sub-phenotypes. This study aimed to investigate the main clinical characteristics and outcomes of patients with different phenotypes of SLE and APS treated with TPE at a tertiary care center. Methods The database of the Blood and Apheresis Unit between 2001 and 2013 was screened for patients with SLE and primary APS. SLE disease activity index (SELENA-SLEDAI), the indications for treatment, complications, and outcomes were obtained from a review of medical records and phone calls. A total of 24 patients (SLE: 20, APS: 4) were recruited for the study. Results Mean ages of SLE (M/F: 1/19) and primary APS (PAPS) patients (M/F: 2/2) were 32.4±12.89 and 52.0±10.7 years, respectively. The main indications for TPE were hematologic, neurologic, and pulmonary involvement and APS-related symptoms. TPE was preferred in eight patients because of leucopenia and co-infection. SLEDAI was significantly decreased after TPE (16.7±8.3 before vs. 8.8±3.1 after, p=0.001). Both primary APS and SLE-related catastrophic APS (CAPS) patients had completely responded to TPE. The success rate of TPE in patients with thrombocytopenia was lower than patients with hemolytic anemia. The median (IQR 25%-75%) number of TPE sessions was 6.5 (5-10.5). In total, five patients experienced TPE-related major adverse events (catheter infections in three patients, bleeding in one patient, and hypotension in one patient). The median (IQR 25%-75%) follow-up time was 33.5 (6.75-81.25) months. In total, four patients died during follow up, of which three died during the period of TPE administration. Conclusion Our data suggest that CAPS and other APS-related problems respond well to the TPE treatment. TPE should be kept in mind for the treatment of patients with other features of SLE, especially those resistant to other agents and in the presence of leucopenia.

FRI0612 IGG4-related disease in eastern mediterranean: clinical features and outcomes of a large cohort

Background Since majority of IgG4-related disease (IgG4-RD) patients in the literature are from Far East and United States, there is a lack of large series from other parts of the world. Objectives We aimed to identify the clinical characteristics and outcome of Turkish IgG4-RD patients from a tertiary center. Methods In step 1, clinical and histopathological features of patients having any disease under the clinical spectrum of IgG4-RD were retrospectively reviewed. For step 2, patients prospectively diagnosed are enrolled into the study. Patients fulfilling the “definite” diagnosis according to comprehensive diagnostic criteria were recruited (enrolled (n=52), excluded (n=47). Results Median age was 51.1 years and no gender predominance was observed (Male/female: 26/26). Retroperitoneal fibrosis is the most frequent presentation; others were lymphadenopathy, orbital pseudotumor, pancreas and salivary glands in decreasing order (Table). Twenty-four (46.1%) of patients had localized involvement. Corticosteroids were mainstay of treatment in 92.5% of patients, and in 57.5% with any immunosuppressive agents as first line treatment. Rituximab has been used for cases resistant to previous treatment or with relapses in 19 (47.5%) of patients. A complete response was achieved in 52.5% of patients and partial response (<50% of regression) in 40%. Two patients deceased due to IgG4-RD attributed problems and no malignancy was observed (median follow up: 18 months).Table 1. Distribution of clinical findings and organ involvement (n=52) Constitutional symptoms, n (%)  Fatigue 31 (59.6)  Tiredness 16 (30.7)  Night sweats 15 (28.8)  Weight loss 14 (26.9)  Fever 13 (25) Retroperitoneal fibrosis, n (%) 23 (44.2) Lymphadenopathy, n (%) 20 (39.2) Any cardiovascular involvement, n (%) 15 (28.8)  Periaortitis 12 (23.1)  Pericardium 5 (9.6)  Coronary periarteritis 4 (7.7)  Abdominal aort aneurysm 1 (1.9) Orbital pseudotumor, n (%) 12 (23.1)  Orbitalmass/proptosis 6 (11.5)  Extraoculary muscles 6 (11.5) Pancreas, n (%) 12 (23.1) Major salivary glands, n (%) 11 (21.2) Lacrimal glands, n (%) 9 (17.3) Mediastinal fibrosis, n (%) 6 (11.5) Ear, nose, sinuses, n (%) 5 (9.6) Lung fibrosis, n (%) 5 (9.6) Skin , n (%) 4 (7.7) Pleura, n (%) 4 (7.7) Gall bladder and Biliary ducts, n (%) 4 (7.7) Thyroid, n (%) 3 (5.8) Liver, n (%) 3 (5.8) Kidney (mass) , n (%) 3 (5.8) Pachymeningitis, n (%) 2 (3.8) Breast involvement: (n=1), tubulointerstitial nephritis (n=1). Conclusions We observed similar features with previous European cohorts however no male predominance was seen. Even though conventional immunosuppressives were used in more than half of patients, treatment had switched to rituximab ∼50% patients owing to resistance or relapses. Disclosure of Interest None declared

AB0562 Demographic Characteristics and Distribution of The Vasculitides Frequencies: A Real- Life Experience of Vasculitis Center from Eastern Mediterranean

Background Vasculitides are a heterogeneous group of diseases. The distribution of the type of vasculitis changes not only with age and sex but according to the geographic area as well. The prevalence of each vasculitis is important in guiding the local medical education and raising awareness of doctors and policy makers etc. Objectives This study is aimed to present the distribution of vasculitides in the Pediatric and Adult Vasculitis patient Groups. Methods Hacettepe University is one of the main referral centers in the country. Executive committee of our Vasculitis center consist of physicians from Pediatric and Adult Rheumatology, Nephrology, Radiology and Pathology Departments. Multidisciplinary approach improves management of our patients. All patients starting from October 2014 have been included and defined according to the 2012 revised Chapel Hill nomenclature criteria. Results A total of 882 patients had been newly diagnosed/followed in this period. 205 (23.2%) of them were pediatric patients. The leading vasculitis among adult patients was Behcets Disease whereas in pediatric patients it was HSP (Figure). More than half of the adult Behcets patients (58.4%) had uveitis/neurologic/vascular involvement. Takayasu arteritis was more frequent than giant cell arteritis among the adult patients. There was a female predominance in patients with large vessel vasculitis. Granulomatous polyangiitis was the most common small vessel vasculitis in adults. Finally 23 adult cases of Immunglobulin G4 related disease have been seen.Table 1. Demographic characteristics of each vasculitis Adult patients Pediatric patients Mean age (SD) Mean age (SD) Behçets disease 42.8 (12.9) 11.8 (4.0) Female, % 52.5% 46.1% Takayasus arteritis 38.9 (14.2) 14.3 (4.1) Female, % 91.6% 100% Giant cell (temporal) arteritis 69.5 (8.2) – Female, % 74.2% Poliarteritis nodosa 40.1 (15.3) 11.3 (7.3) Female, % 39.1% 50.0% Kawasaki disease – 4.7 (3.0) Female, % 26.4% Eosinophilic granulomatosis with polyangiitis 52.9 (19.3) 10 Female, % 60% 100% Granulomatosis with polyangiitis 54.1 (15.9) 8.5 Female, % 46.5% 50% IgA Vasculitis (Henoch-Schoenlein purpura) 38.3 (18.5) 7.3 (4.9) Female, % 46.1% 47.7% IgG4related disease 47.8 (17.8) – Female, % 52.2% Others (secondary vasculitis, Buerger, cryoglobulinemic vasculitis, aortitis, etc.) 47.4 (15.6) 13 (5.1) Female, % 50% 50% Conclusions We describe the demographic characteristics and distribution of vasculitides in the eastern Mediterranean. Behcets disease has a high frequency as expected. Also Takayasu arteritis is more frequent than Giant cell arteritis. Microscopic polyangiitis is very rare. Among children Kawasaki was less and Behçet disease markedly more when compared to western Europe figures. The observed differences may be due to genetic modifiers or environmental factors. Disclosure of Interest None declared