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We describe the case of a 73-year-old man affected by pneumoconiosis, secondary to silica dust exposure, who was diagnosed with antineutrophil cytoplasmic antibody (ANCA)-positive microscopic polyangiitis (MPA)-related cervical myelitis. Pneumoconiosis is reported to trigger autoantibody production and the onset of different autoimmune diseases, including ANCA-associated vasculitis (AAV). MPA is an AAV of the small vessels that can often affect the nervous system, although involvement of the spinal cord in the form of myelitis is described as an anecdotal occurrence. Our experience suggests that an autoimmunity workup should be considered for patients with pneumoconiosis who present with neurological symptoms consistent with AAV.

Takayasu's arteritis is a rare large vessel vasculitis typically affecting young Asian women. It causes inflammation of the aorta and its major branches, leading to stenosis and aneurysmal dilations, and increasing cardiovascular morbidity due to accelerated atherosclerosis. Although glucocorticoids are effective for acute disease control and preliminary data on immunosuppressive drugs are promising, standardized treatment protocols are lacking. The use of prophylactic treatments with antihypertensives, antiplatelets, anticoagulants, and lipid-lowering drugs to prevent thrombotic and ischemic complications remains debated. This study reviews the evidence on the effectiveness of non-immunosuppressive medical therapy in Takayasu's arteritis. A search of the PubMed database identified eleven studies involving 204 patients. Antiplatelets: data on 68 patients were mixed, in fact low-dose aspirin did not prevent major cardiovascular events in 36 patients, but higher doses reduced ischemic complications in 24 patients. Anticoagulants: no data on new oral anticoagulants were available, and vitamin K antagonists in 9 patients did not alter cardiovascular complications. Antihypertensives: ACE-inhibitors controlled blood pressure in patients with renovascular hypertension but increased the risk of acute renal function decline, while β-blockers reduced the symptoms and the progression of myocardial hypertrophy in patients with heart failure and aortic regurgitation. Statins: data from two cohorts showed that while statins reduced the recurrence rate of arteritis in 30 patients, they did not affect recurrence rates or cardiovascular complications in 13 patients. Overall, current evidence, although not definitive, supports the use of non-immunosuppressive medical treatments to prevent long-term complications and damage in Takayasu's arteritis, considering the disease's pathophysiological mechanisms and increased cardiovascular risk. Further research is strongly encouraged., Competing Interests: Declaration of competing interest None., (Copyright © 2024 The Authors. Published by Elsevier B.V. All rights reserved.)

Objective: Tocilizumab (TCZ) is the only biologic drug approved for the treatment of giant cell arteritis (GCA), having clinical trials and real-life studies proved its efficacy and safety. However, the optimal duration of the treatment has yet to be determined, being its early interruption associated with an increased risk of relapse. Conversely, prolonged schemes of therapy may rise safety concerns. The aim of the study was to evaluate the incidence of adverse events (AEs) and remission/relapse rate in a cohort of GCA patients treated with TCZ and an accelerated steroid tapering scheme, followed for 24 months., Methods: We retrospectively included patients referring to our clinic from January 2019 to November 2021 who were diagnosed with GCA and started subcutaneous TCZ treatment (162 mg/week). They also received up to 62,5 mg of prednisone (PDN), tapered following an accelerated six-month scheme., Results: We collected 38 patients, with a mean age of 76,4 years, treated with TCZ for an average of 22,3 months. AEs occurred in 11 (29%) subjects, and only one serious AE was reported; 7 (18%) patients permanently discontinued TCZ. At the end of the follow-up, all the patients continuing treatment showed clinical remission, with a PDN dosage <5mg. We registered 3 (8%) minor relapses under TCZ, after an average of 15 months., Conclusion: Our data support the evidence of a safe and effective long-term use of TCZ in GCA patients, especially when combined with moderate GCs doses for the shortest possible duration., Competing Interests: Authors declare no conflicts of interest., (© 2024 Terribili et al.)

Objective: The main aim of the study was to evaluate the performance of 2 proposed criteria for difficult-to-treat (D2T) psoriatic arthritis (PsA) in a group of patients and to evaluate the agreement between the 2 sets of criteria., Methods: We performed a cross-sectional analysis of 2 longitudinal cohorts of patients with PsA fulfilling the Classification Criteria for Psoriatic Arthritis (CASPAR), with at least 1 year of follow-up. A detailed medical history was collected and a physical examination was performed for all recruited patients. The proposed criteria for patients with D2T PsA were applied in our group. To test the performance of the 2 sets of criteria, we used an external validator (absence of patient acceptable symptom state + physician global assessment ≥ 6 cm). Finally, the agreement between the 2 sets of criteria was assessed., Results: We evaluated 378 patients with PsA (219 male/159 female), with a mean age (range) of 58 (19-75) years. Seventy-five (19.8%) patients fulfilled the D2T criteria proposed by Perrotta et al and 58 (15.3%) the D2T criteria proposed by Kumthekar et al. Both criteria showed comparable performance, with low sensitivity (Perrotta: 37.8%, Kumthekar: 29.7%) but good specificity (Perrotta: 82.1%, Kumthekar: 86.2%). Finally, the agreement between the 2 sets of criteria is substantial (Fleiss [Formula: see text] 0.72), suggesting that both criteria identify nearly the same group of patients., Conclusion: Our study compared 2 published sets of criteria showing comparable performance and substantial agreement. This study may pave the way for further research in this field., (Copyright © 2024 by the Journal of Rheumatology.)

Fibromyalgia is characterized by chronic widespread pain, fatigue, and sleep disturbances. Recent theories attribute fibromyalgia to central sensitization syndromes, suggesting altered nociceptive processing leads to hyperalgesia and allodynia. Standardized effective treatments are currently lacking. Palmitoylethanolamide and melatonin have shown pain-relieving effects in chronic pain conditions, including fibromyalgia, with excellent safety. Our open-label study assessed the impact of a daily combination of 1200 mg of palmitoylethanolamide and 0.2 mg of melatonin on pain, sleep, and quality of life in fibromyalgia patients. Between June 2023 and March 2024, 50 patients (2016 ACR criteria) were treated and evaluated at baseline, 1 month, 3 months, and 4 months (1 month discontinuation). The assessments included VAS for pain, ISI for insomnia, HAQ for health assessments, and a tender points evaluation. The patients, averaging 54.12 years old with a 3:1 female-to-male ratio, showed significant improvements in VAS, ISI, and HAQ scores relative to their own baselines and a reduction in tender points at 1 and 3 months, which was maintained at 4 months. No adverse events were reported. This study is the first to demonstrate the efficacy of a palmitoylethanolamide and melatonin combination as an adjunct therapy in fibromyalgia, highlighting its potential to reduce pain and improve sleep and quality of life.

Objective: Inflammation has been associated with an increased risk for cancer development, while innate immune system activation could counteract the risk for malignancies. Familial Mediterranean fever (FMF) is a severe systemic inflammatory condition and also represents the archetype of innate immunity deregulation. Therefore, the aim of this study is to investigate the risk for cancer development in FMF., Methods: The risk ratio (RR) for malignancies was separately compared between FMF patients and fibromyalgia subjects, Still's disease patients and Behçet's disease patients. Clinical variables associated with cancer development in FMF patients were searched through binary logistic regression., Results: 580 FMF patients and 102 fibromyalgia subjects, 1012 Behçet's disease patients and 497 Still's disease patients were enrolled. The RR for the occurrence of malignant neoplasms was 0.26 (95% Confidence Interval [CI.] 0.10-0.73, p=0.006) in patients with FMF compared to fibromyalgia subjects; the RR for the occurrence of malignant cancer was 0.51 (95% CI. 0.23-1.16, p =0.10) in FMF compared to Still's disease and 0.60 (95% CI. 0.29-1.28, p =0.18) in FMF compared to Behçet's disease. At logistic regression, the risk of occurrence of malignant neoplasms in FMF patients was associated with the age at disease onset (β1 = 0.039, 95% CI. 0.001-0.071, p =0.02), the age at the diagnosis (β1 = 0.048, 95% CI. 0.039-0.085, p =0.006), the age at the enrolment (β1 = 0.05, 95% CI. 0.007-0.068, p =0.01), the number of attacks per year (β1 = 0.011, 95% CI. 0.001- 0.019, p =0.008), the use of biotechnological agents (β1 = 1.77, 95% CI. 0.43-3.19, p =0.009), the use of anti-IL-1 agents (β1 = 2.089, 95% CI. 0.7-3.5, p =0.002)., Conclusions: The risk for cancer is reduced in Caucasic FMF patients; however, when malignant neoplasms occur, this is more frequent in FMF cases suffering from a severe disease phenotype and presenting a colchicine-resistant disease., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2024 Vitale, Caggiano, Tufan, Ragab, Batu, Portincasa, Aragona, Sota, Conti, De Paulis, Rigante, Olivieri, Şahin, La Torre, Lopalco, Cattalini, Maggio, Insalaco, Sfikakis, Verrecchia, Yildirim, Kucuk, Kardas, Laymouna, Ghanema, Saad, Sener, Ercan Emreol, Ozen, Jaber, Khalil, Di Ciaula, Gaggiano, Malizia, Affronti, Patroniti, Romeo, Sbalchiero, Della Casa, Mormile, Silvaroli, Gicchino, Çelik, Tarsia, Karamanakos, Hernández-Rodríguez, Parronchi, Opris-Belinski, Barone, Recke, Costi, Sfriso, Giardini, Gentileschi, Wiesik-Szewczyk, Vasi, Loconte, Jahnz-Różyk, Martín-Nares, Torres-Ruiz, Cauli, Conforti, Emmi, Li Gobbi, Biasi, Terribili, Ruscitti, Del Giudice, Tharwat, Brucato, Ogunjimi, Hinojosa-Azaola, Balistreri, Fabiani, Frediani and Cantarini.)

Inhibiting Janus Kinases (JAK) is a crucial therapeutic strategy in rheumatoid arthritis (RA). However, the use of JAK inhibitors has recently raised serious safety concerns. The study aims to evaluate the safety profile of JAKi in patients with RA and identify potential risk factors (RFs) for adverse events (AEs). Data of RA patients treated with JAKi in three Italian centers from January 2017 to December 2022 were retrospectively analyzed. 182 subjects (F:117, 64.3%) underwent 193 treatment courses. 78.6% had at least one RF, including age ≥ 65 years, obesity, smoking habit, hypertension, dyslipidemia, hyperuricemia, diabetes, previous VTE or cancer, and severe mobility impairment. We identified 70 AEs (28/100 patients/year), among which 15 were serious (6/100 patients/year). A high disease activity was associated with AEs occurrence (p = 0.03 for CDAI at T0 and T6; p = 0.04 for SDAI at T0 and T6; p = 0.01 and p = 0.04 for DAS28ESR at T6 and T12, respectively). No significant differences in AEs occurrence were observed after stratification by JAKi molecules (p = 0.44), age groups (p = 0.08) nor presence of RFs (p > 0.05 for all of them). Neither the presence of any RFs, nor the cumulative number of RFs shown by the patient, nor age ≥ 65 did predict AEs occurrence. Although limited by the small sample size and the limited number of cardiovascular events, our data do not support the correlation between cardiovascular RFs-including age-and a higher incidence of AEs during JAKi therapy. The role of uncontrolled disease activity in AEs occurrence should by emphasized., (© 2024. The Author(s).)

Introduction: No head-to-head study has assessed the superiority of tocilizumab versus methotrexate in giant cell arteritis (GCA), and few studies have demonstrated its effectiveness in terms of ultrasonographic findings, but without a control group. The primary endpoint was to assess whether tocilizumab was superior to methotrexate in inducing normalization of US findings, whereas the secondary endpoint was to assess the effectiveness of precocious withdrawal of glucocorticoids., Methods: We prospectively enrolled all the patients with active GCA at our clinic. The inclusion criteria were clinical diagnosis of GCA; active disease; and clinical, laboratory, and US data, evaluated using the halo count (HC) and OMERACT GCA Ultrasonography Score (OGUS). Evaluations were repeated at 3, 6, and 12 months., Results: Twenty patients were treated with Tocilizumab and 9 with Methotrexate. All but three tocilizumab-treated patients achieved remission at six months, whereas at 12 months, all patients were in glucocorticoid-free remission. Up to three of the nine methotrexate patients experienced a lack of efficacy or minor relapses. Tocilizumab-treated patients showed a statistically significant difference between baseline and all follow-ups in terms of OGUS and HC, whereas the difference in the Methotrexate group was significant after 1 year. The mean glucocorticoid dosage significantly decreased in both groups. No severe adverse events or major relapses were reported., Conclusion: Our study demonstrates the superiority in terms of rapidity of a tocilizumab-based scheme over a methotrexate-based scheme in inducing clinical and US remission. Precocious withdrawal of glucocorticoids did not increase the risk of relapse., Competing Interests: All authors have no conflicts of financial interest, direct or indirect, that may be perceived as affecting the conduct or reporting of the work submitted., (© 2023 Grazzini et al.)