Determining the severity of systemic sclerosis related interstitial lung disease (SSc-ILD) is based on clinical and radiological findings, inflammation marker levels, and carbon monoxide diffusing capacity of the lung (DLCO). Recently studies are ongoing for objective and easy markers. Neutrophil/lymphocyte ratio (NLR) is shown to be a good marker for inflammation in recent clinical trials. In this study, we aimed to identify the possible relationship between NLR and carbon monoxide transfer coefficient (KCO) of SSc-ILD patients. Fifty-nine patients with systemic sclerosis (SSc) were enrolled in the study. We used high-resolution computed tomography for diagnosis and used DLCO to evaluate degree of lung involvement. Complete blood cell counts and acute phase reactants were included as laboratory assessments. NLR values were significantly higher in the SSc-ILD group (3.66 ± 1.32 vs. 2.85 ± 1.12, P = 0.01) and correlated negatively with KCO. The NLR cut-off value was 3.21, its sensitivity was 81%, and its specificity was 81%. NLR level may serve as a marker of lung involvement in the presence of ILD in patients with SSc.

Objective: The aim of this study is to assess the efficacy and safety of nintedanib (NTD) therapy in a real world population of patients with connective tissue diseases related interstitial lung disease (CTDs-ILD). Methods: Our multicenter retrospective study included patients with a CTD-ILD diagnosis who started NTD treatment due to the development of PPF during follow-up. The results of the percentage predicted forced vital capacity (%pFVC) and percentage predicted diffusion capacity (%pDLCO) of patients before NTD treatment and 6, 12 and 18 months after the start of NTD treatment were evaluated. In addition, the patients were divided into two groups, SSc-ILD and other CTDs-ILD, and compared in terms of the efficacy and safety of the NTD. Results: In all patients (n = 66), %pFVC and %pDLCO values stabilised after 6, 12 and 18 months compared to baseline values. All patients received at least one immunosuppressive therapy in combination with NTD treatment. The most common side effect after NTD treatment was diarrhoea (n = 20, 30.3%). When we divided the patients into two groups, SSc-ILD (n = 35) and other CTDs-ILDs (n = 31), no significant difference was found between the groups in the change in %pFVC (p values = 0.498, 0.595 and 0.376, respectively) and in the change in %pDLCO (p values = 0.817, 0.185 and 0.399, respectively) at 6, 12 and 18 months follow-up. Again, there was no statistically significant difference between the two groups in terms of adverse events and safety data after NTD treatment (p > 0.05). Conclusion: In summary, the use of NTD in combination with immunosuppressive therapies was effective and safe in SSc-ILD patients as well as in other CTDs-ILD patients. Key Points• This multicenter study provides real-world data on the use of nintedanib in patients with connective tissue disease-interstitial lung disease.• Nintedanib treatment is as effective and safe in patients with other connective tissue disease as in patients with systemic sclerosis.

Objective: Familial Mediterranean fever (FMF) is the most common autoinflammatory disease. Most of the identified disease-causing mutations are located on exon 10. As the number of studies about the effect of the exonal location of the mutation and its phenotypic expression is limited, we aimed to investigate whether the exonic location of the Mediterranean fever (MEFV) mutation has an effect on the clinical manifestation in patients with FMF. Methods: Study population was derived from the main FMF registry that included 2246 patients from 15 different rheumatology clinics. We categorized the mutations according to their exon locations and retrieved the clinical and demographic information from the database. Results: Patients having the MEFV mutations on exon 2 or 10 (n:1526) were divided into three subgroups according to the location of the MEFV mutations: Group 1 (exon 2 mutations), Group 2 (exon 10 mutations), and Group 3 (both exon 2 and exon 10 mutations). Group 2 patients were of a significantly younger age at onset, and erysipel-like erythema, arthritis, amyloidosis, and a family history of FMF were more common in this group. Conclusion: Patients with FMF and exon 10 mutations show more severe clinical symptoms and outcome. Exon 2 mutations tend to have a better outcome. Cite this article as: Yaşar Bilge Ş, Solmaz D, Şenel S, Emmungil H, Kılıç L, Yılmaz Öner S, et al. Exon 2: Is it the good police in familial mediterranean fever? Eur J Rheumatol 2019; 6(1): 33-6.

In this paper we want to demonstrate whether higher than normal levels of RDW, and lower than normal levels of MPV can be used as indicators of subclinical inflammation and tools for treatment decision in FMF or not. The participants in this study included 89 patients with FMF during attack-free periods and 30 healthy controls. The RDW and platelet counts were significantly higher, while the MPV was significantly lower in the patients with FMF group than healthy control group (; ; , resp.). In the attack-free FMF group, a negative correlation was found between the MPV and RDW values (, ). The positive correlation was found between the RDW and ESR (, ). And the negative correlation was found between the MPV and CRP (, ). Consequently, our results suggest that low MPV and high RDW levels may provide additional information about subclinical inflammation in FMF patients. But other strong predisposing factors affecting subclinical inflammation in FMF should be considered. Further studies with large numbers of patients are needed. Treatment of FMF should include not only prevention of acute attacks but also decreasing of the subclinical inflammation.

Sir, We report the case of a 23-year-old woman with adult-onset Still’s disease (AOSD) who became pregnant while taking LEF and MTX. LEF was stopped after 7 weeks and MTX was stopped after 3 weeks of gestation according to the last menstrual period.

Objective: TURKBIO registry, established in 2011, is the first nationwide biological database in Turkey. This study aimed to provide an overview of TURKBIO data collected by June 2018. Methods: The registry included adult patients with rheumatoid arthritis (RA), ankylosing spondylitis (AS), nonradiographic axial spondyloarthritis (nr-AxSpA), and psoriatic arthritis (PsA). Demographic and clinical features, disease activity markers, and other follow-up parameters, current and previous treat- ments, and adverse events were registered electronically at each visit using open-source software. The registration of patient-reported outcome measures was carried out electronically by the patients using touch screens. Results: TURKBIO registry included a total of 41,145 treatment series with biologicals. There were 2,588 patients with axSpA (2,459 AS and 129 nr-axSpA), 2,036 with RA, and 428 with PsA. The total number of patients, including those with other diagnoses, was 5,718. In the follow-up period, the number of patients and also visits steadily increased by years. The yearly mean number of visits per patient was found to be 2.3. Significant improvements in disease activity and health assessment parameters were observed following the biological treatments. Biologics were often given in combination with a con- ventional synthetic disease-modifying antirheumatic drug in patients with RA. Infections were the most commonly seen adverse events, followed by allergic reactions. Tuberculosis was observed in 12 patients, malignancy in 18, and treatment-related mortality in 31. Conclusion: TURKBIO provided a valuable real-life experience with the use of biologics in rheumatic diseases in Turkey. Cite this article as: O€ nen F, Can G, C apar S, et al. A real-life analysis of patients with rheumatologic diseases on biological treatments: Data from TURKBIO Registry.Eur J Rheumatol. 2022;9(2):82-87.