B Armagan

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.

Comparing polyarteritis nodosa in children and adults: a single center study

Polyarteritis nodosa (PAN) is a necrotizing vasculitis of medium/small arteries. We aimed to examine the characteristics of adult‐ and childhood‐onset PAN.

Multiple liver masses mimicking metastatic liver disease in an elderly patient

1Division of Gastroenterology, Department of Internal Medicine, Hacettepe University School of Medicine, Ankara, Turkey 2Division of Rheumatology, Department of Internal Medicine, Hacettepe University School of Medicine, Ankara, Turkey 3Department of Radiology, Hacettepe University School of Medicine, Ankara, Turkey 4Department of Nuclear Medicine, Hacettepe University School of Medicine, Ankara, Turkey 5Department of Pathology, Hacettepe University School of Medicine, Ankara, Turkey

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.

Comparing immunoglobulin A vasculitis (Henoch–Schönlein purpura) in children and adults: a single-centre study from Turkey

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.

A retrospective study comparing the phenotype and outcomes of patients with polyarteritis nodosa between UK and Turkish cohorts

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.

Remission of relapsing polychondritis after successful treatment of myelodysplastic syndrome with azacitidine: a case and review of the literature

Abstract 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.

Blood group ‘A’ may have a possible modifier effect on familial Mediterranean fever and blood group ‘0’ may be associated with colchicine resistance

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.

SAT0526 Is relapse rate of giant cell arteritis in real-life experience lower than in the controlled trials? results of a retrospective, multi-centre cohort study

Objectives Corticosteroids (CS) are accepted as the standard first-line treatment for giant cell arteritis (GCA). However, controlled trials of tocilizumab and abatacept demonstrated relapse rates of up to 70%–80% in patients on CS-only protocols in 12–24 months. Though level of evidence is low and not suggested by guidelines (except for methotrexate), conventional immunosuppressives (ISs) are also commonly used. We aimed to assess the relapse rates in patients with GCA in routine practice, retrospectively. Methods We assembled a retrospective cohort of patients with GCA from Turkey. All data was abstracted from records. Relapse was defined as any new manifestation or increased acute-phase response leading to the change of the CS dose or use of a new therapeutic agent by the treating physician. Results The study included 156 (F/M: 95/61) patients with GCA (table 1). The mean age at disease onset was 67.8±9.1 years. Polimyalgia Rheumatica was also present in 48 (30,8%) patients. Diagnosis was proven histopathologically in 99 patients.All patients received 1 mg/kg/day CS for remission induction, additional CS pulses were given to 36 (23.1%) patients. Conventional ISs including methotrexate and azathioprine were used in 89 (56.1%) and 26 (16.6%) patients respectively, while 10 (6.4%) patients received biologic treatments (8 tocilizumab,2 etanercept). Fourty-four (28.2%) patients used only CS during follow-up. Follow-up of at least 6 months was available for 132 patients, and median follow-up duration was 35 (6–268) months. Relapses occurred in 27 (20.5%) patients during follow-up. Mortality rate was 7.5% (n=10) during follow-up. VDI score was 2.4±1.7. Main causes of damage were related to CS treatments such as cataract, osteoporosis and diabetes mellitus. Conclusions In this first multi-centre series of GCA from Turkey, we observed that only one fifth of patients had relapses during a mean follow-up of 35 months. This lower relapse frequency suggests a different clinical spectrum in routine practice compared to patients included in controlled trials. Our results also suggest that there is a clear need for a steroid sparing agent in patients with GCA, that is a older aged population prone to CS side effects. Disclosure of Interest None declared

THU0452 Anti-interleukin-6 (TOCILIZUMAB) experience in takayasu’s arteritis patients

Background Targeted therapies such as tumour necrosis factor inhibitors (TNFi) and anti-interleukin 6 (anti-IL-6) are increasingly being used in Takayasu’s Arteritis (TA) patients who are unresponsive to corticosteroids±conventional immunosuppressives. Objectives The aim of this study was to evaluate the indications and efficacy of anti-IL-6 (tocilizumab) therapy in a case series of TA patients. Methods In the prospective database of the Hacettepe University Vasculitis Centre (HUVAM), 105 TA patients meeting the 1990 modified American College of Rheumatology (ACR) criteria were registered at the end of July 2017. Total 28 (26,7%) patients were treated with biological therapy and 22 (21.0%) of them were taking tocilizumab. Patients were assessed using a combination of clinical, laboratory and radiological evaluation before and after Tocilizumab therapy. The demographic and clinical characteristics of the patients, the pre-tocilizumab and end of the follow–up acute phase values (Erythrocyte sedimentation rate (ESR), C-reactive protein (CRP)), visual analogue scales (VAS) (Pain, Fatigue, Patient Global), and concomitant therapies were recorded. Other clinical, imaging and laboratory results of the TA patients were obtained from hospital files. The comparative imaging (Computed Tomography and Magnetic Resonance Imaging) results of the patients just before the Tocilizumab therapy and during follow-up were recorded from the hospital data system. Results Twenty-two patients (86.4% female) were included in to the analysis. The median (minimum-maximum) age of the patients was 4024–63 years and the median disease duration (from diagnosis) was 4812–168 months. Before tocilizumab therapy; cyclophosphamide (8 patients (36.4%), conventional immunosuppressives (21 patients, 95.4%) and TNFi (7 patients, 31.8%) were used in addition to corticosteroid therapy. Main indications for tocilizumab therapy was as follows; radiological progression in 9 patients (acute phase was normal in 2 of them), acute phase elevation in 8 patients (4 of them radiologically stable, 4 of them had no radiologic evaluation), physician’s decision and clinical symptoms in 3 patients (acute phase normal; 2 of them radiologically stable, one had no radiologic evaluation), in two patients tocilizumab therapy was started out of our centre and data before tocilizumab was not available. Fifteen patients (median follow-up time 153–42 months) were evaluated for response to tocilizumab treatment. There was a significant decrease in ESR, CRP, and VAS-patient-global of patients after tocilizumab therapy; median (min-max) ESR (265–119 vs. 3 (2–49) mm/s, p=0.02), CRP (39.8 (2.4–149.0) vs. 7.9 (0–92,9) mg/L, p=0,017), VAS-patient-global (50 (0–90) vs. 30 (0–60), p=0,027), respectively. Total of 9 patients had follow–up imaging; 8 of them was radiologically stable and regression was seen in one patient (figure 1). Table 1 Conclusions The use of biological therapies in more than a quarter of TA patients suggests that the role of conventional therapies in TA is limited. Although imaging modalities and acute phase elevations are the main factors in the decision of change in treatment, presence of symptoms and physician opinion are also important. Significant response was obtained with the treatment of tocilizumab. Disclosure of Interest None declared