Spasmophilia and entrapment nerve syndrome comorbidities in fibromyalgic patients: a possible neuromuscular pain generator.

This paper is aimed at investigating whether peripheral dysfunction at the neuromuscular level may represent a pain generator in fibromyalgia. We studied the prevalence of spasmophilia (SP), carpal tunnel syndrome (CTS) and ulnar neuropathy at the elbow (UNE) in a group of 40 subjects suffering from fibromyalgia. Clinical and electrophysiological data were obtained to ascertain whether comorbid conditions were present. For subjective evaluation of symptoms severity, validated questionnaires for CTS and UNE were completed by patients. Twenty subjects were positive for SP (50%); CTS was diagnosed in 12 subjects (30%); no patient suffered from UNE; 6 subjects were affected at the same time by SP and CTS (15%); 14 subjects (35%) were affected by SP alone. The prevalence of CTS and SP was higher in fibromyalgia subjects than in the general population. The scores of the questionnaires related to CTS were significantly higher in fibromyalgia subjects positive for CTS, with respect to the other subjects. In fibromyalgia, CTS and SP may be considered clinical entities in themselves, the importance of which lies in their acting as peripheral pain generators that enhance or initiate central sensitization, thereby contributing to chronic widespread pain. The amplification of pain is indeed a correctable/misguided message that occurs inside the brain of fibromyalgia subjects and identification and local treatment of pain generators would lessen the total pain burden. The magnitude of the overlap in symptoms between fibromyalgia and CTS/SP necessitates careful investigation of these conditions

Clodronate in the management of different musculoskeletal conditions

INTRODUCTION: Clodronic acid is a non-nitrogen-containing bisphosphonate largely used from some decades in the prevention and treatment of postmenopausal and secondary osteoporosis. in addition to antiresorptive activity, clodronate has shown anti-inflammatory and analgesic properties, and modulatory effects on bone and cartilage metabolism. EVIDENCE ACQUISITION: A literature review has been conducted to characterize the mechanism of action of clodronate and to retrieve available evidence about the use of clodronate in primary and secondary osteoporosis, and its potential role in other musculoskeletal conditions and orthopedic surgery. EVIDENCE SYNTHESIS: The efficacy and safety of the available clodronate formulations (oral, intravenous and intramuscular) in the prevention and treatment of postmenopausal and secondary osteoporosis, including corticosteroid-induced osteoporosis and bone mass loss secondary to endocrine, gastrointestinal and neoplastic diseases, have been demonstrated in a variety of clinical trials. The analgesic, anti-inflammatory, bone- and chondro-modulating properties of clodronate have allowed to expand its use in other musculoskeletal conditions to those currently approved. clodronate has proven to be beneficial in the treatment of osteoarthritis of the knee and of the hand, in the management of complex regional pain syndrome, and in the peri- and postoperative phase in subjects undergoing arthroplasty. CONCLUSIONS: The analysis of the available literature has shown that clodronate has relevant musculoskeletal effects beyond the antiresorptive activity. Further research is needed to better position clodronate therapy in the management of these conditions and to define the optimal formulation and dose regimen in any of the tested new indications

Prevalence of myositis specific and associated antibodies in a cohort of patients affected by idiopathic NSIP and no hint of inflammatory myopathies

The presence of interstitial lung disease (ILD) is a common and fearsome feature of idiopathic inflammatory myopathies (IIM). Such patients show radiological pattern of non-specific interstitial pneumonia (NSIP). The present study aimed to assess the prevalence of myositis-specific and myositis-associated antibodies (MSA and MAA) in a cohort of patients with a previous diagnosis of NSIP and no sign or symptom of IIM. Secondly, it will be assessed whether patients displaying MSA and/or MAA positivity have a worse or a better outcome than idiopathic NSIP. All patients affected by idiopathic NSIP were enrolled. MSA and MAA were detected using EUROLINE Autoimmune Inflammatory Myopathies 20 Ag (Euroimmun Lubeck, Germany), line immunoassay. A total of 16 patients (mean age 72 +/- 6.1 years old) were enrolled. Six out of 16 patients (37.5%) had significant MSA and/or MAA positivity: one displayed positivity of anti-PL-7 (+ +), one of anti-Zo (+ +), anti-TIF1 gamma (+ + +) and anti-Pm-Scl 75 (+ + +), one of anti-Ro52 (+ +), one of anti-Mi2 beta (+ + +), one of anti-Pm-Scl 75 (+ + +) and the latter of both anti-EJ (+ + +) and anti-Ro52 (+ + +).Two out of 7 seropositive patients showed a significant impairment of FVC (relative risk 4.8, 95% CI 0.78-29.5; p = 0.0350). Accordingly, among the 5 patients that started antifibrotic treatment during the observation time, 4 were seronegative. Our findings highlighted a potential autoimmune or inflammatory in idiopathic NSIP patients and also in those without significant rheumatological symptoms. A more accurate diagnostic assessment may ameliorate diagnostic accuracy as well as may provide new therapeutic strategy (antifibrotic + immunosuppressive). A cautious assessment of NSIP patients with a progressive and non-responsive to glucocorticoids disease course should therefore include an autoimmunity panel comprising MSA and MAA

VEXAS syndrome: a new paradigm for adult-onset monogenic autoinflammatory diseases

VEXAS (Vacuoles, E1 enzyme, X-linked, Autoinflammatory, Somatic) syndrome is a recently described pathological entity. It is an acquired monogenic autoinflammatory disease caused by somatic mutations of the UBA1 gene in blood cells precursors; the gene encodes one of the two E1 enzyme isoforms that initiates ubiquitylation in cell's cytoplasm. VEXAS syndrome leads to systemic inflammation, with all organs and tissues potentially involved. The clinical picture may be extremely heterogenous, mimicking different other systemic rheumatologic entities coexisting with haematological disorders, especially myelodysplastic syndrome. This new disease represents a very intriguing clinical condition in several respects: it accounts for the paradigm of adult-onset monogenic autoinflammatory diseases determined by a genetic mosaicism resulting in the development of a challenging multiorgan inflammatory condition. Moreover, VEXAS syndrome is perhaps not an exceptionally rare condition and represents an example of a systemic genetic autoinflammatory disease drawing its origin in bone marrow disorders. VEXAS syndrome should be strongly considered in each adult patient with an unexplained systemic inflammatory condition, especially when recurrent fevers, neutrophilic dermatosis, relapsing polychondritis, ocular inflammation and other systemic inflammatory symptoms accompanying myelodysplastic syndrome or other haematological disorders. The syndrome deserves a multidisciplinary approach to reach the diagnosis and ensure the best management of a potentially very challenging condition. To quickly describe the clinical course, long-term outcomes, and the optimal management of this new syndrome it is essential to join forces internationally. To this end, the international AutoInflammatory Disease Alliance (AIDA) registry dedicated to VEXAS syndrome has been developed and is already active. © 2023, The Author(s)

Intravitreal Dexamethasone Implant as an Adjunct Weapon for Severe and Refractory Uveitis in Behçet's Disease

Background: The evidence on the use of dexamethasone implants in the treatment of Behçet’s disease (BD)-related uveitis is limited to a few cases. Objectives: To evaluate the efficacy of dexamethasone implants on ocular functional, morphological, and clinical parameters in BD patients with severe refractory uveitis. Methods: Five eyes from five BD patients were enrolled. A single intravitreal dexamethasone injection was applied to each eye. Best corrected visual acuity (BCVA), central macular thickness (CMT) assessed with optical coherence tomography, retinal vasculitis assessed by fluorescein angiography, vitreous haze score (Nussenblatt scale), intraocular pressure (IOP), and lens status (LOCS III, Lens Opacities Classification System III) were recorded at baseline and at 1, 3, and 6 month follow-up visits. Results: At baseline, all eyes showed marked macular edema and 4/5 had concomitant active retinal vasculitis. Mean BCVA was increased from baseline at each control visit with a mean improvement of 0.26 ± 0.18 lines at 6 months follow-up. Mean CMT decreased from baseline at each control visit with a mean improvement at 6 months follow-up of 198.80 ± 80.08 µm. At the end of the study, none of the eyes showed macular edema and the mean CMT was 276.80 ± 24.94 µm. Retinal vasculitis resolved in all eyes. One eye experienced an IOP spike during treatment that resolved spontaneously, and one eye developed a clinically significant lens opacity at 6 months follow-up. Conclusions: Treatment with a dexamethasone implant in BD-uveitis and inflammatory macular edema was safe and effective as an additional treatment combined with systemic immunomodulatory drugs

Observations about subcalcaneal adventitial bursitis (heel fat pad inflammatory lesion) in rheumatoid arthritis. Comment on the article of Suzuki and Shirai.

Not availabl

Adalimumab-Based Treatment Versus Disease-Modifying Antirheumatic Drugs for Venous Thrombosis in Behçet's Syndrome: A Retrospective Study of Seventy Patients With Vascular Involvement

Objective: Since Behçet's syndrome (BS) is the prototype of inflammation-induced thrombosis, immunosuppressants are recommended in place of anticoagulants. We undertook this study to assess the clinical efficacy and the corticosteroid-sparing effect of adalimumab (ADA)–based treatment versus disease-modifying antirheumatic drug (DMARD) therapy in a large retrospective cohort of patients with BS-related venous thrombosis. Methods: We retrospectively collected data on 70 BS patients treated with DMARDs or ADA-based regimens (ADA with or without DMARDs) because of venous complications. Clinical and imaging evaluations were performed to define vascular response. We explored differences in outcomes between ADA-based regimens and DMARDs with respect to efficacy, corticosteroid-sparing role, and time on treatment. We also evaluated the role of anticoagulants as concomitant treatment. Results: After a mean ± SD follow-up period of 25.7 ± 23.2 months, ADA-based regimens induced clinical and imaging improvement of venous thrombosis more frequently (P = 0.001) and rapidly (P < 0.0001) than did DMARDs. The mean dose of corticosteroids administered at the last follow-up visit was significantly lower with ADA-based regimens than with DMARDs (P < 0.0001). The time on treatment was significantly longer with ADA plus DMARDs than with DMARDs alone (P = 0.002). No differences were found in terms of efficacy and time on treatment between DMARDs or ADA-based regimens among patients who received anticoagulants and those who did not. Conclusion: In this large retrospective study, we have shown that ADA-based regimens are more effective and rapid than DMARDs in inducing resolution of venous thrombosis in BS patients, allowing reduction of steroid exposure. Moreover, our findings suggest that anticoagulation does not modify the efficacy of either ADA-based regimens or DMARDs for venous complications

Doppler and Spectral Ultrasound of Sacroiliac Joints in Pediatric Patients with Suspected Juvenile Spondyloarthritis

Background: Power Doppler ultrasound (PDUS) with spectral wave analysis (SWA) has been compared with magnetic resonance imaging (MRI) in documenting active sacroiliitis in early spondyloarthritis (SpA) but, to date, PDUS/SWA has not been yet applied to the study of sacroiliac joints (SIJs) in children. Methods: A group of 20 children (13 F/7 M, mean age 14.2 y) with suspected juvenile SpA (jSpA) underwent PDUS/SWA and, subsequently, MRI of the SIJs. SIJs PDUS scoring and resistance index (RI) of the SIJs flows were recorded. The accuracy of PDUS/SWA for the diagnosis of active sacroiliitis was evaluated, with MRI as the gold standard. Results: PDUS signals were detected in 19 patients and 30 SIJs. Bone marrow edema (BME) lesions on MRI were detected in 12 patients (diagnosed as jSpA) and 22 SIJs. PDUS scoring on SIJs were higher in patients with a final diagnosis of jSpA (p = 0.003). On SWA, the mean RIs in patients with or without final diagnosis of active sacroiliitis were, respectively, 0.604 and 0.767 (p = 0.005) at joint level. A RI < 0.55 and PDUS > 1 showed the higher specificity for sacroiliitis (AUROC curve 0.854 for PDUS and 0.920 for RI). SIJs PDUS/SWA showed an overall concordance of 82.35%, with substantial agreement (k = 0.627) with MRI on the diagnosis of sacroiliitis. Conclusions: In children with sacroiliitis, PDUS demonstrates a rich vascularization into SIJs and low RIs (<0.55) have high specificity for this condition. SIJs PDUS/SWA could be useful as a screening method in children with suspected jSpA