Characteristics of arthritis in patients with familial Mediterranean fever.

BACKGROUND: Many of the familial Mediterranean fever (FMF) patients present with arthritis during attacks, which may vary in its characteristics. AIMS: In this study, we aimed to describe and characterise arthritis in FMF patients. METHODS: We used our hospital's record system to retrospectively identify FMF patients with arthritis who presented to our clinic between 2005 and 2020. The prevalence, laboratory results of attack, remission periods, genetic mutations, demographic data, characteristics of attacks, characteristics of arthritis, comorbidities, treatments and treatment responses were recorded. RESULTS: Nine hundred fifty-four patients from a cohort of 2350 FMF patients had arthritis (40%). The male/female ratio was 0.49 in patients with arthritis. The frequency of at least one exon 10 mutation was high. The age of onset of symptoms was earlier for patients with arthritis. Monoarticular pattern was more frequent compared to oligo- and polyarticular patterns. Colchicine resistance was higher; the required colchicine dose for disease control and the frequency of use of biological agents were higher compared to general FMF population. CONCLUSION: Since M694V mutation is common and the colchicine dose required for disease control is high, we can conclude that the disease activity is high in FMF patients with arthritis. The frequency of sacroiliitis and spondyloarthropathy is significantly increased, especially in individuals with M694V mutation, suggesting that there may be a common pathway in their pathogenesis. FMF should be included in the differential diagnosis in patients presenting with arthritis in FMF endemic regions.

Screening of antigliadin and antitissue transglutaminase antibodies in patients with chronic plaque psoriasis: a case-control study.

BACKGROUND: In this study, we aimed to investigate different types of celiac antibodies in psoriasis patients and to see if the presenceof the antibodies was associated with other variables. METHODS: We included patients with plaque psoriasis who were followed up in our dermatology clinic between February 2019 and February 2021 and added a healthy control group for comparison. The antibodies studied were serum antitissue transglutaminase (tTG)-IgA, tTG-IgG, antigliadin antibody (AGA)-IgA, and AGA-IgG. The patients' records were used to note age, sex, the pattern of psoriasis involvement, psoriasis area and severity index (PASI), presence of hypertension, presence of type 2 diabetes mellitus, use of methotrexate, and use of biologic agents. RESULTS: Sixty-five psoriasis patients (31 F, 34 M, mean age: 38.9 ± 15.2) and 65 controls (42 F, 23 M, mean age: 40.7 ± 13.2) wereincluded in the study. There was no significant difference in antibody levels between the groups: tTG-IgA (2.4 U/mL vs 3.2 U/mL, p = 0.11), tTG-IgG (2.2 U/mL vs 3.2 U/mL, p = 0.74), AGA-IgA (2.4 U/mL vs 3.5 U/mL, p = 0.068), and AGA-IgG (3.2 U/mL vs 4.2 U/mL, p = 0.15). One patient from the psoriasis group only had borderline positive antibody levels whereas the rest of the psoriasis and control group had negative levels. Hypertensive psoriasis patients had significantly higher AGA-IgA titers compared to normotensive psoriasis patients (4.2 U/mL vs 2.3 U/mL, p = 0.005). DISCUSSION: There was no increase in the AGA-IgA/IgG and tTG-IgA/IgG levels in psoriasis patients compared to the healthy population. However, hypertensive psoriasis patients had higher AGA-IgA levels compared to normotensive ones.

Secukinumab after first-line tumor necrosis factor-alpha inhibitor therapy in psoriatic arthritis: A real-world retrospective cohort study.

Objectives: This study compared the secukinumab treatment responses and adverse effects in psoriatic arthritis patients who received secukinumab as second-line with those that received secukinumab after two or more tumor necrosis factor-alpha (TNF-α) inhibitors. Patients and methods: The retrospective study included 68 psoriatic arthritis patients followed up between October 2018 and October 2021. The patients were divided into two groups according to their anti-TNF-α treatment history. Group 1 consisted of 29 patients (11 males, 18 females; mean age: 45.3±13.3 years; range, 21 to 69 years) who had previously received one anti-TNF-α agent, while Group 2 included 39 patients (18 males, 21 females; mean age: 46.4±13.0 years; range, 24 to 70 years) who had been treated with two or more anti-TNF-α agents. Treatment responses of the groups were measured and compared using the Bath Ankylosing Spondylitis Disease Activity Index (BASDAI) and Visual Analog Scale (VAS). A posttreatment BASDAI score ≤4 was used as a criterion for remission. Results: The mean duration of secukinumab treatment was 16.6±12.7 months for Group 1 and 16.0±11.6 months for Group 2 (p=0.84). Both groups responded significantly to secukinumab in terms of BASDAI and VAS scores (p<0.001 and p<0.001, respectively). Group 1 had a greater decline in BASDAI and VAS scores than Group 2 (p=0.045 and p=0.032, respectively). Furthermore, the remission rate was greater in Group 1 compared to Group 2 (58% vs. 34%, p=0.03). The adverse effects of secukinumab treatment were an allergic reaction in Group 1 and one case of ulcerative colitis in Group 2. Conclusion: Second-line secukinumab treatment resulted in a greater decline in BASDAI and VAS scores. Moreover, secukinumab achieved a significantly higher rate of remission when it was used as second-line therapy after one anti-TNF-α agent.

Measuring Transition Readiness of Patients After Transfer from Pediatric to Adult Care in Rheumatology.

Transitional care is essential to maintain the continuity of care in younger patients with rheumatic diseases. In this study, we aimed to assess the transition readiness of rheumatology patients who had already transferred from pediatric to adult care using a questionnaire. We included young adult rheumatology patients who had already transferred to adult rheumatology care. The Transition Readiness Assessment Questionnaire (TRAQ) was used in the adult rheumatology clinic to assess the patients' readiness; a retrospective chart review was conducted to include diagnosis, age at diagnosis, age at transfer, and current age. Three hundred and ten patients (184 female and 126 male) participated in this study. The mean age at diagnosis, the mean age at transfer, and the mean age at the time of the study were 10.7 ± 4.29, 21.1 ± 1.69, and 24.0 ± 2.26 years, respectively. Most of the patients had familial Mediterranean fever, followed by arthritis, connective tissue disorders, and other diseases. Tracking health issues was the lowest-scored domain. Females scored significantly higher than males in the tracking health issue domain (P = .006) and managing health issue domain (P = .028) but not in the overall TRAQ score (P = .053). Patients in different diagnosis and transfer age groups scored similarly across the domains. In this study, females performed better than males in 2 domains of the TRAQ questionnaire. Diagnoses or transfer age groups were not associated with TRAQ outcomes.

When to start secukinumab treatment in patients with axial spondyloarthropathy: Before or after anti-TNFα treatment?

INTRODUCTION AND OBJECTIVES: This study aimed to evaluate the efficacy of secukinumab (SEC) in axial spondyloarthropathy (axSpA) in anti-TNFα naïve and anti-TNFα experienced patients. It also focused on the duration of SEC treatment and its side effects. PATIENTS AND METHODS: The patients with axSpA treated with SEC and followed up in our outpatient clinic from May 2018 through October 2021 were included in this study. All patients in the study also fulfilled the ASAS classification criteria for axSpA. Patients were separated into two groups according to whether they received prior anti-TNFα therapy. While anti-TNFα naïve patients comprised group 1, anti-TNFα experienced patients were included in group 2. Pre- and post-treatment BASDAI scores were reported and compared. RESULTS: Eighty-four axSpA patients (42 men; duration of the disease: 86.86±65.35 months in group 1 and 160.65±97.4 months in group 2) were treated with SEC. 45.5% of anti-TNFα naïve patients and 56.5% of anti-TNFα experienced patients were still on SEC therapy in October 2021. Duration of SEC treatment was 12.5±7.9 months in group 1 and 17.19±12 months in group 2 (p=0.098). The differences between pre-and post-treatment BASDAI scores were statistically significant in both groups (p<0.001). While patients in group 1 did not develop any adverse effects, three patients in group 2 experienced alopecia, uveitis, and recurrent pneumonia after SEC treatment. CONCLUSION: Our study's efficacy and safety data on the use of SEC were reassuring in both anti-TNFα naïve and anti-TNFα experienced patients. However, further studies are still needed to determine the appropriate timing to begin SEC treatment.

A case with febrile attacks and vasculopathy associated with ADA2 and MEFV pathogenic variants.

Deficiency of adenosine deaminase 2 (DADA2), caused by recessive mutations in the adenosine deaminase 2 (ADA2) gene, results in cutaneous or systemic vasculitis with variable clinical manifestations. There is only one other case in literature carrying both ADA2 and MEFV gene pathogenic variants. Here we report the second case that carries both ADA2 and MEFV pathogenic variants, presenting with characteristic phenotypes of both familial Mediterranean fever (FMF) and DADA2. A male patient, currently 29 years old, was initially diagnosed with FMF and developed livedo reticularis and nodular dermal lesions compatible with cutaneous polyarteritis nodosa (PAN) a year after diagnosis. His family history revealed a brother 2 years older than himself who was diagnosed with PAN and died at age 22 because of gut perforation secondary to acute mesenteric ischaemia. ADA2 gene mutation analysis on chromosome 22q11.1 was positive, and the patient responded to colchicine and infliximab.

Effectiveness of anti-interleukin-23 therapy in psoriatic arthritis: A pilot prospective real-world study.

AIM: This study aimed to show the effectiveness of interleukin (IL)-23 inhibitors in psoriatic arthritis (PsA) at weeks 12 and 24 in a real-world setting. MATERIALS AND METHODS: Forty-three patients with active PsA were enrolled in this study. These patients were treated with either guselkumab (n = 20) or risankizumab (n = 23). Treatment responses at the 12th and 24th weeks were evaluated with the parameters of the number of joints with active arthritis, Psoriasis Area Severity Index (PASI) response rate, Bath Ankylosing Spondylitis Disease Activity Index (BASDAI) score, Disease Activity Index for Psoriatic Arthritis (DAPSA) score, and C-reactive protein (CRP) value. The study's primary endpoint was BASDAI ≤ 4 and DAPSA ≤ 14 at week 24, and the secondary endpoint was the absence of joints with clinically active arthritis signs at week 24. RESULTS: IL-23 inhibition significantly improved all treatment response parameters at the 12th and 24th weeks (P < 0.001). While 90% of patients reached the primary endpoint with anti-IL23 therapy, 74% achieved the secondary endpoint. Both biologic-naïve and biologic-experienced patients responded significantly to anti-IL-23 therapy. Also, no adverse events related to anti-IL-23 agents were observed. CONCLUSIONS: The response parameters indicating the severity of PsA (the number of joints with active arthritis, BASDAI score, DAPSA score, and CRP value) and a parameter indicating the severity of skin involvement, that is, PASI score, significantly improved with anti-IL-23 therapy at weeks 12 and 24. Moreover, significant improvement was achieved at week 24 compared to week 12 in all response parameters.

Rituximab in the management of retroperitoneal fibrosis: A single tertiary rheumatology care center experience.

AIM: To investigate the clinical and radiological outcomes and glucocorticoid-sparing effect of rituximab therapy in 13 patients with retroperitoneal fibrosis (RPF). METHODS: We analyzed the data of both glucocorticoid-naive and glucocorticoid-resistant RPF patients who were treated with rituximab. Demographic features, positron emission tomography computed tomography (PET-CT) findings, and clinical and histopathologic outcomes were collected retrospectively. RESULTS: We evaluated the data of 13 RPF patients (8M/5F). The median follow-up duration was 28 months (interquartile range [IQR] 24.5-55.5 months) and median age at the time of diagnosis was 50.8 years (IQR 46.5-54.5 years). PET-CT scans showed that following the rituximab therapy, the craniocaudal diameter of the RPF mass reduced from 74 mm (IQR 50.5-130 mm) to 52 mm (IQR 35-77 mm; p = .06), and periaortic thickness of the RPF mass reduced from 14 mm (5.5-21.9 mm) to 7 mm (4.5-11 mm; p = .12). The maximum standardized uptake value (based on body weight) of the RPF mass decreased from 5.8 (4.3-9.7) to 3.1 (2.8-5.3) after the therapy (p = .03). The number of patients with hydronephrosis reduced from 11 to 6 following rituximab therapy (p = .04). Before rituximab, nine patients received a median dose of 10 mg (IQR 0-27.5 mg) prednisolone per day. After the rituximab treatment, we discontinued prednisolone treatment for four out of nine patients and reduced the daily dose for the remaining patients. At the time of the final evaluation of the patients, the median prescribed prednisolone dose was 5 mg/day (IQR 2.5-7.5 mg/day; p = .01). CONCLUSION: Our study shows that rituximab may be a favorable treatment option for glucocorticoid-refractory RPF patients with high disease activity on PET-CT scans.

Portal hypertension in familial Mediterranean fever patients.

INTRODUCTION: Familial Mediterranean fever (FMF) is an autoinflammatory disease characterized by polyserositis and arthritis attacks, which are mediated by increased plasma levels of cytokines. Our hypothesis was that the increase in specific cytokines can also lead to portal hypertension, even in the absence of overt hepatic steatosis. METHODS: We designed a comparative cross-sectional study with 41 patients and 30 healthy individuals to show if there is a relationship between portal hypertension and FMF. B mode ultrasound and Doppler ultrasound were utilized to evaluate liver echogenicity, portal vein diameter, peak portal blood flow velocity, and portal vein flow direction, which are important diagnostic criteria for portal hypertension. RESULTS: Spleen and liver sizes and portal vein diameters of the FMF patients and the healthy controls were not significantly different. Imaging of 4 patients revealed periportal cuffing and one patient with periportal cuffing also had a fine granular appearance of the liver. The peak portal blood flow velocity of the FMF patients was lower than that of the control group (p<0.007). CONCLUSION: The FMF group had significantly lower peak portal blood flow velocity than the control group, indicating the existence of portal hypertension. However, the differences between the other findings that correlate with portal hypertension were not significant.