Alper Sari

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.

The clinical features and outcomes of Turkish patients with IgG4-related disease:a single-center experience

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 (<50% regression) in 40%. Conclusion: This large and first cohort of IgG4-RD patients from Turkey showed similar clinical features to European cohorts, except for the male predominance in previous cohorts. Corticosteroids and rituximab are effective in IgG4-RD but there is still uncertainty about the usage of corticosteroid-sparing agents.

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.

Should our approach to diuretic using in patients with gout change

We read with interest the recent article by Ranieri et al. [1]. The intention of the authors was to investigate the impact of diuretic therapy on the response of urate-lowering drugs (ULDs) in patients with gout. According to the results of this study, diuretic therapy did not impair response to ULDs. Also, there was no significant effect of diuretic use on the achievement of serum urate (SU) targets or on the maximum doses of ULDs. However, it is known that the European League Against Rheumatism (EULAR) has reported its concern in regard to the use of diuretics in patients with gout and recommends substitution of diuretic to losartan or calcium channel blockers if gout is diagnosed in a hypertensive patient receiving loop or thiazide diuretics [2]. For this reason, we believe that the results of this study, which reported the incompatibility with the recommendations of known and accepted organizations, should be interpreted carefully as it may bring a question mark to daily clinical practice. First, the retrospective design of the study led to the heterogeneity of the patient population. Given the baseline characteristics of enrolled patients, 137 (65.9%) patients in the allopurinol group and 30 (85.7%) in the febuxostat group had hypertension. In addition, 107 (51.4%) patients in the allopurinol group and 20 (57.1%) patients in the febuxostat group had dyslipidemia. But in this study, we did not find any information about the drugs used for hypertension and dyslipidemia which may have an effect on urate metabolism. A large epidemiological study reported that calcium channel blockers and losartan are associated with a lower risk of incident gout among patients with hypertension [3]. It is well known that fenofibrate and statins have uricosuric properties [4, 5]. Second, in the study, alcohol use and dietary habits of patients may directly affect the SU levels. But there is no detailed information on alcohol use and dietary habits of patients. All of these factors may have affected the results of the study. Lastly, given diuretic indications, 63% of patients using diuretics had pure hypertension without heart failure or chronic kidney disease. In these patients, although there are alternative anti-hypertensive drugs, the reason for the continuation of diuretics after diagnosis of gout is a matter to be discussed. In conclusion, the results of this study, which can change our clinical practice, consisting of a retrospective and heterogeneous population, should be interpreted with caution. We believe that, as the authors emphasize, there is a need for prospective well-designed studies about the effects of diuretics on the necessary doses of ULDs to achieve the targeted SU level.

AB0953 Psaid-12 can be used to determine the anti-tnf treatment decision in the psoriatic arthritis registry

Background Psoriatic Arthritis Impact of Disease (PsAID-12) score has 12 questions and each question has its own weight. PsAID-12 is developed to be used in daily practice. However, in the daily practice, there has been no information on the utilisation of determining the response of the biological DMARD treatment. Objectives The assessment of utilisation of PsAID-12 for PsA patients on determination of the efficiency and inefficiency of anti-TNF treatment in a biological registry. Methods In this study patients were taken from Hacettepe University biological database (HUR-BIO). Since January 2013 PsAID-12 score was built in HUR-BIO database. PsAID-12 score was known for 116 patients before starting off the first anti-TNF treatment and 88 patients whose PsAID-12 score was 4 and above were included in the enquiry. Overall, 70 PsA patients included to analysis. Demographic data before anti-TNF treatment of PsA patients were noted. The decision of continuation, stopping or switching to another anti-TNF drugs were performed by both clinicians and the patients agreement. According to baseline evaluation, decrease of 20 mm and above on pain-VAS score and PGA, improvement of 0.22 unit and above on HAQ-DI score, or decrease of 1.2 unit and above on DAS-28 score were considered favourable to the anti-TNF treatment. Stopping or switching the anti-TNF treatments due to inefficiency was definitely a negative response. Pain-VAS score being under 15 mm or below, global-VAS score being 20 mm and below, HAQ-DI score being 0.5 and below, DAS-28 score being 2.6 and below were determined as targeted goals. Changes were analysed comparing to baseline level in PsAID-12 score, in compliance with the favourable and unfavourable responses to Anti-TNF treatments. In determining the response of the treatment, standardised response mean (SRM) was used. Results Seventy (78.6% female) patients were analysed, mean age was 45.5 (12.0). Mean follow-up duration was 18.3 (12.6) months, and total of 213 clinical visits were performed, median 31–8 control visits were done. At baseline, the mean (SD) DAS-28 4.07 (1.22), HAQ-DI 0.86 (0.53), pain-VAS 6.9 (2.1), PGA-VAS 6.4 (1.7), and PsAID-12 6.6 (1.5) were as follows. Anti-TNF treatments were stopped due to inefficacy in 43/210 (20.5%) outpatient visit during the follow-up period. The results of anti-TNF stopped and continuing patients; Δ PsAID-12 were 0.38 (1.71), and 3.12 (2.14), respectively and PsAID-12 baseline/control visits 0.96 (0.29) vs 0.50 (0.33), respectively. Level of favourable response and achieving to goal according to ΔPsAID-12 and PsAID-12 Baseline/control visit were shown table 1. On the follow up visits, among measured parameters one of the highest SRM was detected in PsAID-12; PsAID-12 (1.10), DAS-28 (1.14), PGA (0.88), Pain (0.85), and HAQ-DI (0.51), respectively.Abstract AB0953 – Table 1 Goals and responses of Anti-TNF treatment Conclusions Having 3.5 unit or 50% decrease in the PsAID-12 score indicates a favourable response to anti-TNF treatment, 4 unit or 70% decrease indicates level of targeted response. PsAID-12 appears to be an effective composite index for retaining the response of the treatment in biological registry. Disclosure of Interest None declared