Exploring S100A8/A9, Neopterin, and MMP3 in Familial Mediterranean Fever.

Familial mediterranean fever (FMF) is characterized by inflammatory attacks due to overactivation of pyrin inflammasome. This study aimed to investigate the reliability of S100A8/A9, neopterin, and matrix metalloproteinase 3 (MMP3) at monitoring subclinical inflammation and disease activity, and at differentiating FMF attacks from appendicitis, the most common misdiagnosis among FMF patients. Blood samples (n=75), comprising from FMF patients during an attack (n=20), the same FMF patients during the attack-free period (n=14), patients with appendicitis (n=24), and healthy volunteers (n=17) were obtained. Duplicate determinations of S100A8/A9, neopterin, and MMP-3 levels were conducted using the enzyme-linked immunosorbent assay (ELISA). FMF patients with and without attack and patients with appendicitis had significantly elevated S100A8/A9 levels compared to healthy volunteers (p-values: <0.001, 0.036, 0.002, respectively). Patients with appendicitis and FMF patients with and without attack had significantly increased serum neopterin levels compared to healthy volunteers (p-value: <0.001). MMP3 levels were significantly higher among patients with appendicitis and FMF patients during attack compared to healthy controls (p-values: <0.001, 0.001). Serum levels of S100A8/A9, neopterin, and MMP3 were increased significantly during attacks compared to attack-free periods among FMF patients (p-values: 0.03, 0.047, 0.007). S100A8/A9 emerges as a valuable marker for monitoring disease activity. Neopterin and S100A8/A9 might help physicians to monitor subclinical inflammation during the attack-free periods of FMF patients. MMP3 might aid in diagnosing FMF attacks when distinguishing between attack and attack-free periods is challenging.

Anxiety and depression among patients with familial Mediterranean fever.

BACKGROUND: Familial Mediterranean fever (FMF) is a systemic autoinflammatory disease that requires lifelong treatment and is associated with several comorbidities, including mental health disorders such as anxiety and depression. FMF and mental health necessitate further research; hence, this study aims to observe anxiety and depression and their relationship with several variables in patients with FMF. METHODS: As the study population, 360 FMF patients were surveyed between June and October 2022. Surveys included inventories assessing anxiety and depression, i.e., the Beck's Depression Inventory (BDI), the Beck's Anxiety Inventory (BAI), and the State-Trait Anxiety Inventory (STAI). RESULTS: Mean scores for STAI-Y1 (state), STAI-Y2 (trait), BAI, and BDI were 42.2 ± 12.0, 45.9 ± 10.6, 24.0 ± 13.9, and 13.1 ± 8.99, respectively. Medication-adherent patients had significantly lower scores on STAI-Y1 (41.5 ± 11.4 vs. 45.2 ± 14.0; p-value: 0.04). M694V homozygous patients exhibited significantly lower scores in the BDI (12.4 ± 9.37 vs. 13.2 ± 8.93; p-value: < 0.001) and BAI (17.0 ± 12.1 vs. 25.1 ± 13.9; p-value: 0.001). The patients with an exon-10 mutation demonstrated significantly lower scores compared to patients with an exon­2 mutation (17.9 ± 12.3, 29.6 ± 13.3; p-value: < 0.001). CONCLUSION: The patients with FMF had mild depression and moderate anxiety scores. A higher level of education and medication adherence were associated with lower levels of anxiety. Likewise, the patients with genotypes associated with severe disease courses had lower levels of anxiety. We suggest that physicians should be more attentive to patients with a milder disease course and ensure that these patients are provided with sufficient treatment and knowledge about their disease.

Clinical features of dermatomyositis patients with anti-TIF1 antibodies: A case based comprehensive review.

BACKGROUND AND OBJECTIVES: Dermatomyositis is chronic autoimmune disease primarily affecting skin and muscles. Antibodies are key players of pathogenesis and are in strong correlation with distinct clinical phenotypes. We present a case and a comprehensive review of the literature on dermatomyositis patients with Anti TIF1 antibodies. METHODS: PubMed and Web of Science databases were reviewed. 166 articles were identified; 95 of them were evaluated; 79 of them included to the study. 45 of the included articles were case reports 9 were case series and 25 were research articles. In total 1065 patients were identified but number of patients with available information for different clinical features varied. RESULTS: 69.6% of the patients with Anti TIF1-γ were female. Prevalence of malignancy was 42.6% among patients with Anti TIF1-γ. Muscle weakness (83%), Gottron sign (82.2%), heliotrope rash (73.7%), nailfold capillary changes (67.7%), dysphagia (38.4%), and joint involvement (31.1%) were the most common clinical features seen in patients with Anti TIF1-γ. Interstitial lung disease (ILD) was reported among 8.7% of patients with Anti TIF1-γ. Advanced age, male gender, dysphagia, and V-neck rash were significant risk factors for malignancy, whereas juvenile age, ILD, TIF1-ß antibodies and joint involvement were associated with a decreased risk for malignancy. Advanced age, malignancy, dysphagia, and muscle involvement were associated with an increased risk for mortality. CONCLUSIONS: Patients with advanced age, male gender, dysphagia, and V-neck rash require strict cancer screening. Patients with advanced age, malignancy, dysphagia, and muscle involvement have poor prognosis and should receive aggressive treatment.