The impact of nicotine smoking on spondyloarthritis and rheumatoid arthritis.

OBJECTIVE: Nicotine has major side effects on human health through numerous mechanisms, one of which is the alteration of the immune system and its genetic components. Such alteration can be a predisposing factor for autoimmune diseases such as spondyloarthritis (SpA) and rheumatoid arthritis (RA). This review aims to shed light on the effects of nicotine smoking on the pathophysiology, clinical presentation, and management of SpA and RA. METHODS: This review looked into the studies, excluding case reports and series, which were cited by PubMed/MEDLINE. RESULTS: Patients with established autoimmune conditions may have a different underlying pathophysiology and disease course when exposed to nicotine through cigarette smoking. Through the involvement of several cytokines, endothelial dysfunction, and epigenetic mechanisms, the severity of SpA is more prominent in smokers. The global health status, pain, and fatigue are worse in SpA patients. The evidence on the effect of nicotine smoking on the treatment of SpA is still limited. Nicotine can contribute to RA via the disruption of cellular regulatory activity, inflammatory responses, morphological, physiological, biochemical, and enzymatic responses. As such, smokers with RA have higher disease activity and are more likely to be seropositive through the citrullination of peptides. In addition, these patients are at risk of achieving a suboptimal response to tumor necrosis factor inhibitors. CONCLUSIONS: Cigarette smoking can substantially affect the pathophysiology and clinical presentation of patients with SpA and RA. The impact of nicotine on the management of these diseases still needs to be further studied.

Intensive care unit deaths among neurosurgery patients in a tertiary hospital in South Western Nigeria

Neurosurgical patients are the most critical ICU admissions. While advancements in neurosurgical ICUs (NICU) have improved outcomes of care globally, ICU mortality remains a major clinical issue in developing nations. This study evaluates ICU mortalities of neurosurgical patients in a general ICU setting at the UNIOSUN Teaching Hospital, Osogbo, Nigeria. Method: Case records of neurosurgery patients who died in the ICU of UNIOSUN Teaching Hospital, Osogbo, South-Western, Nigeria from June 2012 to May 2022 were reviewed. Simple descriptive statistics of data on demographics, clinical diagnoses, management and outcome were done. Results: Mortality rate was 38.9% (84 of 216 admissions). Males were 67(79.8%) and the mean age was 41.5years (Range: 2-85years). The average duration of ICU stay was 3.5days (Range: 30minutes-20days). Most patients had severe traumatic brain injury (TBI) (62, 73.8%). This was followed by cerebrovascular diseases (12, 14.3%) and brain tumours (6, 7.1%). Two had brain abscess. One patient each had mixed subacute/chronic subdural haematoma and severe cervical spondylotic myelopathy. Of the 69 whose case files were found, 7(10.1%) had a diagnosis of brainstem death before eventual 'final' death after an average of 13.5 additional hours on mechanical life support. The identified secondary causes of death included raised ICP, sepsis, primary surgical haemorrhage, seizures, acute kidney injury, malignant hypertension, poor glycaemic control and aggressive blood pressure lowering. Only 1 patient had autopsy. Conclusion: Most ICU mortalities among neurosurgical patients were from severe TBI. The establishment of NICU is necessary to improve outcome of care of neurosurgical patients

Rheumatology research output in the Arab World: despite the challenges.

Rheumatology is a field in which diagnostic, pathophysiological, and therapeutic advancements occur daily. These developments are the result of research in basic sciences, translational sciences, and clinical sciences. Physical and financial support, provided by individuals and institutions, is essential for all types of research. The political and economic instability in the Arab world has impacted the advancement of healthcare and the output of research. This review seeks to evaluate the quantity and quality of rheumatology-related research conducted in the Arab world. This review examined the number of rheumatological clinical publications produced by Arab countries between 2017 and 2021 and cited by PubMed/MEDLINE. Publications with authors from multiple nations were disqualified. Publications were then categorized by type, including randomized controlled trials (RCTs), cohort studies, cross-sectional studies, systematic reviews, narrative reviews, and case reports. Publications were also organized according to the regions of the Arab world: North and East Africa, the Middle East, and the Arabian Peninsula. The review also used data from the world bank to evaluate the gross domestic product (GDP) and total population of Arabian nations in order to calculate a ratio of publications to GDP and publications to population. Egypt had the highest number of publications among north and east African countries and Arab countries in general, with 261 publications, including the highest number of RCTs, which were 23. With 81 publications, Saudi Arabia had the highest number of publications on the Arabian peninsula. However, no individual country on the Arabian peninsula published an RCT. Lebanon had the most publications in the Middle East, but the majority were reviews and case reports. Tunisia had the highest ratio of publications to GDP. Notably, the number of publications increased during the coronavirus disease 2019 era in some countries. The Arab world is still able to produce clinical rheumatology publications despite its political instability and lack of resources. The influence of such publications on the daily practice of rheumatology is still debatable.

Quality of life measures in Systemic Lupus Erythematosus: A systematic review.

In this study we systematically investigated the health-related quality of life (HRQoL) tools, which have been most often used over the last five years to evaluate the QoL in patients with systemic lupus erythematosus (SLE), focusing on their items and applications. A detailed literature search was conducted: the inclusion criteria were as follows: 1) studies including at least 50 patients; 2) studies including at least 25 patients with SLE; 3) quality of life testing with validated measures. The systematic review was based on 119 studies for a total of 32,449 SLE patients and 3092 controls. A total of 35 different patients-reported quality of life measures, applied in cohorts of patients with SLE, were retrieved with the 36-item Medical Outcome Short Form (SF-36) (63 studies of 119 =52.95%), Lupus Quality of Life (LupusQoL) (17 studies =14.3%) and Lupus Patient-Reported Outcome (LupusPRO) (12 studies =10%) being the most commonly used tools. Overall, this systematic review of the literature indicated that quality of life in patients with SLE appears to be poor and generally lower compared to both the general population and patients with other chronic conditions, as was shown by a few studies that used SF-36 and LupusPRO. The use of HRQoL scoring in SLE is gaining increasing interest and is used both in randomized controlled trials and in real-life. Future efforts are needed to improve the understanding of the impact of the disease burden on quality of life from the patient's perspective.

Postural tachycardia syndrome (POTS) and antiphospholipid syndrome (APS): What do we know so far?

As part of the non-criteria clinical manifestations, postural orthostatic tachycardia syndrome (POTS), a multisystem autonomic dysfunction, can co-exist with antiphospholipid syndrome (APS). Several pieces of evidence hint on the autoimmune basis of POTS, and its possible association with several autoimmune diseases, including APS. Indeed, the evidence exists in the etiologies, symptomatology, and treatment options. Although infections, viral ones in particular, stress, and pregnancy are etiologies to both POTS and APS, the exact pathophysiological connection is still to be studied taking into consideration the activity of cytokines in both diseases. Nevertheless, certain immunomodulatory treatments used for the catastrophic or obstetrical forms of APS, such as intravenous immunoglobulins (IVIG) and steroids, have been also used for the treatment of POTS resistant to classical treatments. Therefore, our review aims to highlight the association between POTS and APS, shedding light on the common etiologies explaining the pathophysiology of the two disorders, the diagnostic approach to POTS as a possible clinical criterion of APS, and the treatment of APS in the context of treating POTS.

Axial and peripheral spondyloarthritis triggered by sars-cov-2 infection: a report of two cases.

The severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection can show musculoskeletal symptoms such as peripheral arthritis. In rare cases, peripheral arthritis can develop after the resolution of SARS-CoV-2. We present two cases of spondyloarthritis induced by SARS-CoV-2; one case with axial and peripheral spondyloarthritis and the other with peripheral spondyloarthritis. Both cases refer to Lebanese patients who were HLA-B27 positive. These two cases highlight the possible predisposition of HLA-B27 positive patients to the development of spondyloarthritis symptoms triggered by SARS-CoV-2.

Hematological malignancies in connective tissue diseases.

Chronic inflammation has profound tumor-promoting effects. Inflammatory cells are the key players in immunosurveillance against tumors, and immunosuppression is known to increase the risk of tumors. Autoimmune diseases, which manifest as loss of self-tolerance and chronic immune dysregulation, provide a perfect environment for tumor development. Aside from managing the direct inflammatory consequences of autoimmune pathogenesis, cancer risk profiles should be considered as a part of a patient's treatment. In this review, we describe the various associations of malignancies with autoimmune diseases, specifically systemic lupus erythematosus, rheumatoid arthritis, systemic sclerosis, and Sjögren's syndrome, as well as discuss the mechanisms contributing to the pathogenesis of both disorders.

Antiphospholipid syndrome and infertility.

Antiphospholipid syndrome is a systemic autoimmune disease associated with obstetric complications along with vascular events affecting multiple organ systems in patients having positive titers of antiphospholipid antibodies. Eight to 20% of infertility cases have an unknown cause, part of which could be due to antiphospholipid syndrome. Although still debatable, many studies have addressed the relation between reproductive failure and antiphospholipid antibodies through the relation between antiphospholipid antibodies and unexplained infertility as well as the effect of antiphospholipid antibodies on the outcome of in vitro fertilization-embryo transfer. Few studies and cases have associated the presence of antiphospholipid antibodies with male infertility, describing morphofunctional penile abnormalities and testicular infarction. There are not enough data to support the routine practice of testing antiphospholipid antibodies in patients with infertility.

Characteristics of lupus and lupus nephritis at a tertiary care center in Lebanon.

INTRODUCTION: Systemic lupus erythematosus affects 4.8-78.5 people per 100,000 worldwide, 90% of whom are females. Geography and ethnicity have been shown to significantly affect the prevalence and natural history of the disease. Lupus nephritis affects around half of patients with systemic lupus erythematosus. Data about systemic lupus erythematosus and lupus nephritis in the Middle East are still scarce. In this study, we aimed to describe the characteristics of systemic lupus erythematosus and lupus nephritis at a tertiary care center in Lebanon. METHODS: This is a retrospective chart review of all biopsy-proven lupus nephritis patients admitted to the American University of Beirut medical center between January 2000 and December 2018. Patients above 12 years of age who had any International Society of Nephrology/Renal Pathology Society (ISN/RPS) class of lupus nephritis on their renal biopsy were included in the study. RESULTS: The study included 55 patients with lupus nephritis. Upon presentation of systemic lupus erythematosus, the most common clinical feature was arthritis, seen in 83% of patients, followed by anemia (82%) and malar rash (48%). In total 93% had positive ANA, 89% had positive anti-dsDNA and 98% of patients had proteinuria. The most common ISN/RPS class of lupus nephritis in our series was IV (49%). At the time of the biopsy 15% of patients underwent dialysis. At 6 months, 11/27 had complete remission, 6/27 had partial remission and 10/27 had no remission. At 1 year, 8/23 had complete remission, 4/23 had partial remission and 11/23 had no remission. During the study period, 15 out of 35 patients available for analysis had chronic kidney disease (CKD) and six out of 34 patients developed end-stage kidney disease requiring renal replacement therapy. In comparison to other series in the region, our series had more males affected, higher creatinine at the time of biopsy and greater degree of proteinuria. CONCLUSION: Our study provided insight on the demographics, characteristics, and outcomes of lupus nephritis in Lebanon. Interestingly, male gender was present in a quarter of patients. This warrants further investigation and confirmation. We are hoping to expand this experience into a national prospective registry to further characterize this entity in our region.

Hydroxychloroquine in the primary thrombosis prophylaxis of antiphospholipid antibody positive patients without systemic autoimmune disease.

Objective The objective of this study was to determine the efficacy of hydroxychloroquine (HCQ) in the primary thrombosis prevention of antiphospholipid antibody (aPL)-positive patients with no other systemic autoimmune diseases. Methods Under the auspices of Antiphospholipid Syndrome Alliance for Clinical Trials and International Networking, a multicenter, international, randomized controlled trial (RCT) was initiated, in which persistently aPL-positive but thrombosis-free patients without systemic autoimmune diseases were randomized to receive HCQ or no treatment in addition to their standard regimen. The primary objective was the efficacy of HCQ in preventing the first thrombosis. The secondary objectives were the thrombosis incidence rate, and the effects of HCQ on aPL profile and mortality rate. Patients were risk-stratified based on antiplatelet agent use. The goal was to follow patients every 6 months for 5 years. Results We recruited 20 persistently aPL-positive patients (female: 19, mean age: 46.6 ± 9.9 years, and baseline antiplatelet medication: 14); 9/20 were randomized to HCQ. During the mean follow-up of 1.7 years, no patients developed thrombosis or a serious adverse event. The study was terminated early due to the low recruitment rate, exacerbated by the prolonged manufacturing shortage and significant price increase of HCQ in the United States. Conclusion Given that a small number of patients with a relatively short follow-up were enrolled in our RCT, and no patients developed thrombosis, we cannot accurately assess the effectiveness of HCQ for primary thrombosis prevention in persistently aPL-positive patients with no other systemic autoimmune diseases. Our experience suggests that conducting an international RCT, especially without pharmaceutical support, is an extremely challenging undertaking.

Adaptation of the 2015 American College of Rheumatology treatment guideline for rheumatoid arthritis for the Eastern Mediterranean Region: an exemplar of the GRADE Adolopment

BACKGROUND: It has been hypothesized that adaptation of health practice guidelines to the local setting is expected to improve their uptake and implementation while cutting on required resources. We recently adapted the published American College of Rheumatology (ACR) Rheumatoid Arthritis (RA) treatment guideline to the Eastern Mediterranean Region (EMR). The objective of this paper is to describe the process used for the adaptation of the 2015 ACR guideline on the treatment of RA for the EMR. METHODS: We used the GRADE-Adolopment methodology for the guideline adaptation process. We describe in detail how adolopment enhanced the efficiency of the following steps of the guideline adaptation process: (1) groups and roles, (2) selecting guideline topics, (3) identifying and training guideline panelists, (4) prioritizing questions and outcomes, (5) identifying, updating or conducting systematic reviews, (6) preparing GRADE evidence tables and EtD frameworks, (7) formulating and grading strength of recommendations, (8) using the GRADEpro-GDT software. RESULTS: The adolopment process took 6 months from January to June 2016 with a project coordinator dedicating 40% of her time, and the two co-chairs dedicating 5% and 10% of their times respectively. In addition, a research assistant worked 60% of her time over the last 3 months of the project. We held our face-to-face panel meeting in Qatar. Our literature update included five newly published trials. The certainty of the evidence of three of the eight recommendations changed: one from moderate to very low and two from low to very low. The factors that justified a very low certainty of the evidence in the three recommendations were: serious risk of bias and very serious imprecision. The strength of five of the recommendations changed from strong to conditional. The factors that justified the conditional strength of these 5 recommendations were: cost (n = 5 [100%]), impact on health equities (n = 4 [80%]), the balance of benefits and harms (n = 1 [20%]) and acceptability (n = 1 [20%]). CONCLUSION: This project confirmed the feasibility of GRADE-Adolopment. It also highlighted the value of collaboration with the organization that had originally developed the treatment guideline. We discuss the implications for both guideline adaptation and future research to advance the field.(AU)

Hughes syndrome and epilepsy: when to test for antiphospholipid antibodies?

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.

Musculoskeletal manifestations of the antiphospholipid syndrome.

The scope of clinical and laboratory manifestations of the antiphospholipid syndrome (APS) has increased dramatically since its discovery in 1983, where any organ system can be involved. Musculoskeletal complications are consistently reported in APS patients, not only causing morbidity and mortality, but also affecting their quality of life. We reviewed all English papers on APS involvement in the musculoskeletal system using Google Scholar and Pubmed; all reports are summarized in a table in this review. The spectrum of manifestations includes arthralgia/arthritis, avascular necrosis of bone, bone marrow necrosis, complex regional pain syndrome type-1, muscle infarction, non-traumatic fractures, and osteoporosis. Some of these manifestations were reported in good quality studies, some of which showed an association between aPL-positivity and the occurrence of these manifestations, while others were merely described in case reports.

Interstitial granulomatous dermatitis associated with systemic lupus erythematosus: case report and review of the literature.

Systemic lupus erythematosus (SLE) is a chronic multisystem autoimmune disease of unknown etiology that most frequently involves the skin and the musculoskeletal system. In addition to the more common cutaneous manifestations, interstitial granulomatous dermatitis (IGD) may rarely occur in association with SLE or even be the first sign of the disease. We describe a 40-year-old man with SLE-associated IGD, and review all cases of SLE-associated IGD in the literature.

Hughes syndrome and multiple sclerosis.

Multiple sclerosis (MS) and antiphospholipid syndrome (APS) share common clinical, laboratory and radiological features. We reviewed all the English papers on MS and APS published in the literature from 1965 to 2014 using PubMed and Google Scholar. We found that APS can mimic antiphospholipid antibodies (aPL)-positive MS in many ways in its clinical presentation. Nevertheless, APS diagnosis, clinical manifestations and management differ from those of MS. aPL were found in MS patients with titers ranging from 2% to 88%. The distribution and volume of lesions on magnetic resonance imaging (MRI) may help to differentiate MS from primary APS. In addition, atypical MS presentation can guide physicians toward an alternative diagnosis, especially when features of thrombosis and/or history of connective tissue disease are present. In that case, an anticoagulation trial could be worthwhile.

Lumbar disc herniation in young children.

AIM: This article explores lumbar disc herniation in young children through focusing on matters relevant to patient presentation, physical examination, differential diagnosis, imaging and treatment. METHODS: Major databases were searched for studies that addressed lumbar disc herniation in young children. RESULTS: Diagnosis of lumbar disc herniation in young children is usually delayed because of the rarity and lack of experience with this entity and the difficulty in extracting a reliable medical history. Nevertheless, lumbar disc herniation should be considered in the differential diagnosis of any young child presenting with a chief complaint of back pain and/or radiculopathy, especially in the setting of recent trauma. This should be coupled with a directed physical examination to elicit signs and narrow the differential diagnosis. Imaging studies, mainly magnetic resonance imaging, will help establish a diagnosis; yet radiographs are still required to exclude other spinal lesions. The initial management of lumbar disc herniation in children is the same as that in adults and consists of conservative treatment unless lumbar disc herniation affects the patient's motor and neurological functions in which case, early surgical treatment must be undertaken. Although the latter remains more difficult, current experience suggests a favourable outcome. CONCLUSION: Awareness of lumbar disc herniation will help the paediatrician extract a relevant medical history, perform a directed physical examination, and order appropriate imaging studies. This will aid in initiating early intervention, be it conservative or operative, and achieving a favourable outcome.

Childhood stroke in a child with familial Mediterranean fever carrying several prothrombotic risk factors.

Familial Mediterranean fever (FMF) was recently associated with a hypercoagulable state. However, clinically overt thrombosis remains a rare event limited to patients with other predisposing factors. We herein present a child with FMF who experienced a stroke. An extensive thrombophilia work-up revealed multiple inherited and acquired risk factors. In areas with high prevalence of prothrombotic mutations and in children who are products of consanguineous marriages, early screening for concurrent thrombotic risk factors is warranted; as this may help design an optimal management plan and prevent unfavourable outcomes.