Familial Mediterranean fever (FMF) is characterized by recurrent episodes of fever and serositis. Blood-based biomarkers determined in FMF patients during attack-free periods could be used to predict the risk of amyloidosis and the severity of the disease. The recently defined pan-immune-inflammation value (PIV) comprises four distinct subsets of blood cells and serves as an easily accessible and cost-effective marker. The objective of this study was to assess the role of PIV in predicting amyloidosis and moderate-to-severe disease. Clinical characteristics and laboratory values during the attack-free period were retrospectively analyzed in 321 patients over 18 years of age diagnosed with familial Mediterranean fever (FMF). In our tertiary adult rheumatology outpatient clinic, disease severity and laboratory markers were evaluated during the first attack-free interval. At baseline, patients with amyloidosis were excluded. Patients were categorized based on the presence of amyloidosis and the severity of the disease. When focusing on amyloidosis in receiver operating characteristic (ROC) analysis, optimal cut-off values for pan-immune-inflammation value (PIV), neutrophil-to-lymphocyte ratio (NLR), and platelet-to-lymphocyte ratio were determined as ≥518.1, ≥2.3, and ≥127.2, respectively. In multivariate analysis, PIV, C-reactive protein (CRP), and the presence of the M694V homozygous mutation emerged as independent risk factors for both amyloidosis and moderate-to-severe disease. Additionally, NLR was identified as an independent risk factor for amyloidosis, while red blood cell distribution width was associated with moderate-to-severe disease. In patients with FMF, especially in the presence of the M694V homozygous mutation, CRP and PIV may be useful in predicting both amyloidosis and moderate-to-severe disease.
Familial Mediterranean Fever (FMF) is characterized by recurrent febrile attacks and serositis. While colchicine is the primary treatment for FMF, some patients present resistance or intolerance with respect to this drug. Anakinra—an IL-1 receptor antagonist—has demonstrated efficacy in colchicine-resistant or -intolerant FMF patients. Background and Objectives: This study aimed to evaluate the clinical characteristics, treatment duration, response to therapy, dose interval modifications, and long-term outcomes in FMF patients treated with anakinra. Materials and Methods: We retrospectively analyzed data from 68 FMF patients who were colchicine-resistant or -intolerant and received anakinra treatment. Results: The median patient age was 40.2 years, with a predominance of female patients (57.3%). The median follow-up duration for patients treated with anakinra was 34.2 months. Anakinra dosing was successfully extended in 30.8% of patients. Eight patients discontinued anakinra due to remission, with a median remission duration of 18.4 months. In a subgroup analysis of 57 patients treated with anakinra for at least 12 months, a significant decrease was observed in Pras scores at 0 months, 3 months, and 12 months, as well as in Erythrocyte Sedimentation Rate, C-reactive protein, and Serum Amyloid A values (all p < 0.001). Statistically significant decreases in 24 h proteinuria values were found between 0 and 3 months, 3 and 12 months, and 0 and 12 months (p = 0.011, p = 0.006, and p = 0.007, respectively). Anakinra use in pregnancy and kidney transplant recipients was well tolerated. Dose extension and treatment discontinuation in remission are feasible strategies. Conclusions: These findings support the use of anakinra as a good treatment option in selected patients.
3 August 2023 in Euroasian Journal of Hepato-Gastroenterology
Spontaneous bacterial peritonitis (SBP) is an important reason for mortality in cirrhosis. This study aimed to identify the factors associated with mortality in SBP. A total of 69 patients with cirrhosis and 74 with SBP attacks that occurred in this group were assessed. Demographic data, symptoms at admission, comorbidities, laboratory parameters, treatment protocols, causes of cirrhosis, scoring characteristics, cirrhosis complications, and mortality were analyzed. Model for end-stage liver disease (MELD; p = 0.001), sodium-MELD (p = 0.001), and Child-Pugh-Turcotte (CTP) (p < 0.001) scores were correlated with mortality in patients with SBP episodes. Hepatorenal syndrome (p = 0.001) and esophageal variceal bleeding (p < 0.001) related to mortality. Serum lactate dehydrogenase (LDH) (p = 0.007), serum leukocyte (p = 0.017), and serum hemoglobin (p = 0.010) values had a statistically significant effect on mortality in multivariate regression analysis. The mortality rate can be reduced by identifying factors influencing death in patients with SBP episodes. Ocak T, Gülten M. Retrospective Investigation of Factors Affecting Mortality in Spontaneous Bacterial Peritonitis. Euroasian J Hepato-Gastroenterol 2023;13(1):5-9.
Background: Secukinumab is a monoclonal antibody against interleukin 17 approved for patients with axial spondyloarthritis (axSpA), psoriatic arthritis (PsA), and psoriasis. Treating axSpA and PsA patients with a history of malignancy is a challenge. While initial results on the applicability of secukinumab in this patient group are positive, the number of studies on this topic remains limited. This study aimed to investigate the drug’s survival time and the efficacy and safety of secukinumab treatment in this specific patient group. Methods: This retrospective study included 30 patients with a history of malignancy who were followed up in rheumatology outpatient clinics in 12 centers throughout Turkey and treated with secukinumab between May 2018 and March 2024 with a diagnosis of axSpA and PsA. Results: The mean follow-up time was 29.8 ± 19.3 months. The drug retention rate was 89.7% after 12 months and 80.6% after 24 months. The most common tumor in our study was papillary thyroid carcinoma (n = 5, 16.7%). During follow-up, local tumor recurrence was observed in a patient with urothelial carcinoma of the bladder. Conclusions: In the largest cohort reported to date, treatment with secukinumab in axSpA and PsA patients with a history of malignancy was not shown to cause oncologic recurrence except for one local tumor recurrence. Drug retention rates were also high, and disease activation and function improved compared to baseline. Therefore, secukinumab could be a safe and effective option for this patient group.
29 January 2022 in Turkish Journal of Internal Medicine
Introduction: Chronic inflammatory diseases (CIDs) are lifelong complex disorders that affect quality of life, and this study aimed to summarize 5 years of experience with a multidisciplinary approach for these complex diseases as a result of medical council meetings. Methods: Hospital-based, medical records review study was conducted. A total of 45 monthly medical council meetings were held between 2014-2019 with the participation of the rheumatology, dermatology and gastroenterology departments of the same university. Patients with complex conditions that were seen in each department's own polyclinic composed the council. This study only included 308 patients referred by the rheumatology group. Results: Females made up 66.5 % of the 308 patients. The median age was 45 (19-77). PsA (psoriatic arthritis) and other SPA (spondyloarthritis) patients composed 49.3% of the total. A total of 68.18% of the patients were presented only to consult with the dermatology department. The most common reason for presenting patients was to discuss options for treatment (41.5%). The diagnosis of psoriasis was confirmed in 48 of 67 (71.6%) patients who presented with a pre-diagnosis. The diagnosis was changed in 34.74% of the patients, whereas the diagnosis became completely different in 11.36% of the patients. Conclusions: Many patients with challenging diagnosis and treatment processes are encountered in daily practice. The combination of different disciplines makes it possible to provide more rapid and effective solutions. In this study, we aimed to emphasize the increasing importance of such multidisciplinary approaches