Chronic pain with neuropathic characteristics is probably more prevalent in the general population than was previously thought and is generally more severe than other chronic pain.

This study represents the second validation DN4 in a language different from the original after the Spanish adaptation. These results support the high discriminatory value of the DN4 questionnaire for identification of NP.

Pachydermoperiostosis, or primary hypertrophic osteoarthropathy (PHO), is an inherited multisystem disorder, whose features closely mimic the reactive osteoarthropathy that commonly accompanies neoplastic and inflammatory pathologies. We previously described deficiency of the prostaglandin-degrading enzyme 15-hydroxyprostaglandin dehydrogenase (HPGD) as a cause of this condition, implicating elevated circulating prostaglandin E(2) (PGE(2)) as causative of PHO, and perhaps also as the principal mediator of secondary HO. However, PHO is genetically heterogeneous. Here, we use whole-exome sequencing to identify recessive mutations of the prostaglandin transporter SLCO2A1, in individuals lacking HPGD mutations. We performed exome sequencing of four probands with severe PHO, followed by conventional mutation analysis of SLCO2A1 in nine others. Biallelic SLCO2A1 mutations were identified in 12 of the 13 families. Affected individuals had elevated urinary PGE(2), but unlike HPGD-deficient patients, also excreted considerable quantities of the PGE(2) metabolite, PGE-M. Clinical differences between the two groups were also identified, notably that SLCO2A1-deficient individuals have a high frequency of severe anemia due to myelofibrosis. These findings reinforce the key role of systemic or local prostaglandin excess as the stimulus to HO. They also suggest that the induction or maintenance of hematopoietic stem cells by prostaglandin may depend upon transporter activity.

Well-recognized for their role in vascular homoeostasis, platelets may play a major role in inflammation and immunomodulation. Substantial data are emerging on the pathogenic involvement of platelets in inflammatory arthritis and autoimmune diseases, indicating the existence of crosstalk between the coagulation and inflammation system. Upon activation, platelets release pro-inflammatory platelets microparticles, which interact with leucocytes leading to joint and systemic inflammation in rheumatoid arthritis. Platelets activation by immune complexes activate dendritic cells promoting the secretion of interferon alpha, which has a key role in the development of systemic lupus erythematous. In this review, we discuss the current data on the role of platelets in the pathophysiology of inflammatory arthritis and various autoimmune diseases, such as rheumatoid arthritis, systemic lupus erythematosus, and systemic sclerosis.

Platelet-rich plasma (PRP) is an autologous blood product with platelets above circulating levels and releases several growth factors after activation. PRP may help to decrease joint inflammation by modulating synovial cell proliferation and differentiation and inhibition of catabolic pathways in various articular conditions. Though PRP has shown good efficacy in osteoarthritis and other musculoskeletal conditions such as synovitis, epicondylitis, skeletal muscle injuries, and tendinopathy, there is limited experience for the use of PRP in patients with rheumatoid arthritis. Precise mechanisms of action of PRP are not known. We present clinical experience for treatment with PRP (2–4 ml) in four patients with rheumatoid arthritis who had inadequate response and persistent pain and inflammation with intra-articular steroids. Irrespective of past and ongoing treatments and duration of disease, all patients showed improvement in the visual analog scale and disease activity score of 28 joints at 4 and 8 weeks after injection. There was an improvement in joint inflammation on ultrasound imaging in some patients. These effects were sustained for up to 1 year. No adverse effects were reported in any patient. PRP may be a safe and useful therapy in patients with rheumatoid arthritis who fail to respond to one or more established treatment options.

Epilepsy and seizures are reported among the neurological manifestations of antiphospholipid syndrome (APS) at a prevalence rate of approximately 8%, which is nearly 10 times the prevalence of epilepsy in the general population. The association of seizures with antiphospholipid antibodies (aPL) is even more significant in the presence of systemic lupus erythematosus (SLE). In this review, we discuss the epidemiological, pathophysiological, laboratory, clinical, and radiological aspects of this association, and derive suggestions on when to consider testing for aPL in epileptic patients and how to manage seizures secondary to APS based on literature data. Epilepsy due to APS should be considered in young patients presenting with seizures of unknown origin. Temporal lobe epilepsy seems to be particularly prevalent in APS patients. The pathogenesis is complex and may not only involve micro-thrombosis, but also a possible immune-mediated neuronal damage. Patients with seizures and positive aPL tend to develop thrombocytopenia and livedo racemosa more frequently compared with those without aPL. Magnetic resonance imaging (MRI) remains the imaging modality of choice in these patients. The presence of SLE and the presence of neurological symptoms significantly correlate with the presence of white matter changes on MRI. In contrast, the correlation between aPL positivity and the presence of white matter changes is very weak. Furthermore, MRI can be normal in more than 30-40% of neuropsychiatric lupus patients with or without aPL. aPL testing is recommended in young patients presenting with atypical seizures and multiple hyper-intensity lesions on brain MRI in the absence of other possible conditions. New MRI techniques can better understand the pathology of brain damage in neuro-APS. The therapeutic management of epileptic APS patients relies on anti-epileptic treatment and anticoagulant agents when there is evidence of a thrombotic event. In the absence of consensual recommendations, the decision of lifelong anticoagulation is discussed on a case-by-case basis. The anti-thrombotic benefit of hydroxychloroquine and statins is supported by several studies.

To evaluate the perceptions of patients with rheumatoid arthritis (RA) about self-assessment of their disease activity after watching an educational video. Consecutive patients with RA consulting their rheumatologist in six Middle Eastern Countries were invited to watch an educational video developed to teach self-assessment using Disease Activity Score (DAS-28). Then, a rheumatology nurse conducted a semi-structured interview and collected the patients’ perception about the understanding of the video, feasibility, capability and confidence in performing self-assessment using Likert-type items. The degree of confidence with self-assessment was correlated to the patients’ socio-demographic characteristics. Sixty-two patients were included and had an overall positive reaction to the video. It was easy to understand in 96% and helped facilitate self-assessment in 92% of cases. Self-assessment was considered totally feasible in 74%, and 66% of patients were capable of always doing it, with a confidence of 60% (always) to 34% (sometimes). Confidence was associated with a higher educational level. Nevertheless, 77% of patients felt that the self-assessment would not fully replace the physician’s visit. Open-ended questions identified five themes: better understanding of the disease, easier communication with the rheumatologist, less consultation time, difficulty with the scoring part and importance of practice. Patients with RA felt that self-assessment was feasible and helpful in understanding RA, improving communication with the rheumatologist and shortening the visit time. Supplementary Information The online version contains supplementary material available at 10.1007/s00296-021-04799-2.

Vaccine hesitancy is considered a major barrier to achieving herd immunity against COVID-19. While multiple alternative and synergistic approaches including heterologous vaccination, booster doses, and antiviral drugs have been developed, equitable vaccine uptake remains the foremost strategy to manage pandemic. Although none of the currently approved vaccines are live-attenuated, several reports of disease flares, waning protection, and acute-onset syndromes have emerged as short-term adverse events after vaccination. Hence, scientific literature falls short when discussing potential long-term effects in vulnerable cohorts. The COVAD-2 survey follows on from the baseline COVAD-1 survey with the aim to collect patient-reported data on the long-term safety and tolerability of COVID-19 vaccines in immune modulation. The e-survey has been extensively pilot-tested and validated with translations into multiple languages. Anticipated results will help improve vaccination efforts and reduce the imminent risks of COVID-19 infection, especially in understudied vulnerable groups.