Sarcoidosis as a cause of chronic back pain: a case report.

While sarcoidosis is typically a multisystem disease, it can, in some instances, exclusively affect the vertebrae, leading to back pain. Additionally, sarcoidosis may manifest with inflammation of the sacroiliac joints, not meeting the criteria for spondyloarthritis, yet contributing to back pain. In this report, we present a case involving a previously healthy 55-year-old woman who sought medical attention due to chronic back pain. She was subsequently diagnosed with spinal sarcoidosis, based on MRI, PET scan, and biopsy results. Furthermore, treatment with prednisolone monotherapy demonstrated substantial improvement in her symptoms.

Vacuolar Interface Dermatitis as a Histologic Reaction Pattern of Sjögren's Syndrome: A Case Report.

Sjögren's syndrome (SS) has been widely known for its dry mouth and dry eyes presentation. Extraglandular disease manifestations may be protean and pose a challenge for clinicians, especially when the typical known manifestations are absent. Skin involvement of SS is variable, and cutaneous signs and symptoms may be the initial presentation of this syndrome. Vacuolar interface dermatitis has been linked to dermatomyositis and systemic lupus erythematosus, but rarely to SS. Herein, we present the case of an 87-year-old man who presented for widespread itchy erythematous scaly plaques that were refractory to topical corticosteroids as well as discontinuation of possible offending medications. A biopsy demonstrated vacuolar interface dermatitis in the setting of strongly positive anti-SSA. Hydroxychloroquine treatment was effective in resolving the plaques.

Expert Opinion Guidance on the Detection of Early Connective Tissue Diseases in Interstitial Lung Disease.

There is a significant variation in symptoms and clinical presentation of connective tissue disorders (CTD) associated with interstitial lung disease (ILD) (CTD-ILD). This presents difficulties in the diagnosis and treatment of CTD-ILD. Early detection and treatment of CTD-ILD using a multidisciplinary approach have been shown to enhance patient outcomes. This exercise aims to explore clinical components to develop a screening tool for pulmonologists for early detection of CTD in ILD and to provide a framework for a multidisciplinary approach in managing CTD-ILD. This in turn will lead to early treatment of CTD-ILD in collaboration with rheumatologists. A panel of 12 leading rheumatologists from the Middle East and North Africa (MENA) region met virtually to select the most relevant clinical findings to aid in identifying CTD-ILD. Twelve panellists opted to investigate seven of the most common inflammatory autoimmune disorders. The panel discussed how to improve the early detection of CTD-ILD. Clinical characteristics were categorized, and a nine-item questionnaire was created. A biphasic algorithm was developed to guide early referral to a rheumatologist based on the presence of one of nine clinical features of CTD (Phase 1) or the presence of CTD-specific antibodies (Phase 2). A brief questionnaire has been developed to serve as a simple and practical screening tool for CTD-ILD detection. Additional research is needed to validate and evaluate the tool in longitudinal cohorts.

Sarcoidosis Mimicking a Malignant Metastatic Disease.

Sarcoidosis can have pulmonary and extrapulmonary clinical manifestations depending on the organ of involvement. Because multiple organs are involved by the disease, sarcoid can mimic metastatic diseases. Whenever clinical and radiological clues of metastasis are present, differentials other than cancer should not be missed. Herein, we present a case of a middle aged gentleman who presented to the oncology clinic for 1-month history of low back pain associated with a dry cough along with radiological findings of metastatic disease involving the lungs, liver, lymph nodes, axial spine, and adrenal gland. A biopsy of the liver lesion showed non-caseating granuloma. Elevated blood levels of angiotensin-converting enzyme confirmed the diagnosis of sarcoidosis.

Lupus, antiphospholipid syndrome, and stroke: An attempt to crossmatch.

Cerebrovascular accidents (CVAs) or strokes are part of the common thrombotic manifestations of Systemic Lupus Erythematosus (SLEs) and Antiphospholipid syndrome (APS). Such neurological thrombotic events tend to occur in patients with SLE at a higher frequency when Antiphospholipid antibodies (aPLs) are present, and tend to involve the large cerebral vessels. The mechanism of stroke in SLE can be driven by complement deposition and neuroinflammation involving the blood-brain barrier although the traditional cardiovascular risk factors remain major contributing factors. Primary prevention with antiplatelet therapy and disease activity controlling agent is the basis of the management. Anticoagulation via warfarin had been a tool for secondary prevention, especially in stroke recurrence, although the debate continues regarding the target international normalized ratio (INR). The presence of either of the three criteria antiphospholipid antibodies (aPLs) and certain non-criteria aPL can be an independent risk factor for stroke. The exact mechanism for the involvement of the large cerebral arteries, especially in lupus anticoagulant (LAC) positive cases, is still to be deciphered. The data on the role of non-criteria aPL remain very limited and heterogenous, but IgA antibodies against ß2GPI and the D4/5 subunit as well as aPS/PT IgG might have a contribution. Anticoagulation with warfarin has been recommended although the optimal dosing or the utility of combination with antiplatelet agents is still unknown. Minimal data is available for direct oral anticoagulants (DOACs).

Erythema nodosum in Behçet's disease in remission: Think COVID-19?

The coronavirus disease has several manifestations related to the activation of the immune system. Because of such activation, autoimmune diseases, including vasculitis, have been reported to occur. Behçet's disease, a variable vessel vasculitis, has been discussed in the context of coronavirus disease. Rarely, the induction of Behçet's disease flare or exacerbation has been reported necessitating aggressive treatment. The presentation of Behçet's disease flares secondary to coronavirus disease is variable, including mucocutaneous lesions and eye or joint involvement. We highlight the case of a 35-year-old woman with pre-existing Behçet's disease in remission on colchicine presenting with new onset erythema nodosum-like lesions on her right shin being diagnosed with coronavirus disease infection a few days after. Despite treatment with systemic corticosteroid, the lesions did not resolve, necessitating the initiation of anti-interleukin-6 therapy.

Role of pro-inflammatory interleukins in osteoarthritis: a narrative review.

PURPOSE: This manuscript will summarize the role of pro-inflammatory cytokines and tackle newly discussed ones within the scope of OA pathogenesis as mentioned in the recent literature. This will allow for a better understanding of the mechanisms behind such a complicated disease. MATERIAL AND METHODS: Relevant articles were obtained by searching key terms including "pro-inflammatory cytokines," "inflammation," "pathophysiology," "cartilage damage," and "OA" in PubMed and Google Scholar databases. The year ranges set for the selection of the articles was between 2015 -2021. Inclusion criteria was based on the relevance and contribution to the field of the study. RESULTS: Osteoarthritis (OA) has a complex multifactorial pathophysiology which is attributed to molecular and biomechanical changes that disrupt the normal balance of synthesis and degradation of articular cartilage and subchondral bone. Pro-inflammatory cytokines, with their wide range of action and intricate signaling pathways, are the constant subject of new discoveries revolving around this inflammatory disease. The available literature indicates that some of these cytokines such as IL-33, IL-17, IL-6, and IL-22 have a direct relation to cartilage degradation, while others like IL-15, IL-1, IL-7, and IL-34 have an indirect one. CONCLUSIONS: Inflammation has an essential role in the manifestation of osteoarthritis clinical events. Specifically, certain cytokines exhibit pro-inflammatory properties that are markedly activated during the course of the disease and notably alter the homeostasis of the joint environment. However, clinical trials and observational studies remain insufficient to navigate the varying nature of this disease in humans.

ANCA-Associated Vasculitis following the First Dose of Pfizer-BioNTech COVID-19 Vaccine.

Coronavirus disease (COVID-19) vaccine can alter the body's immunological balance leading to autoimmune disease in rare cases. Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis is one of the autoimmune diseases which have been rarely reported to appear post-COVID-19 vaccine. Herein, we report the case of a 47-year-old woman who developed acute renal failure few days after receiving the first dose of the Pfizer-BioNTech COVID-19 vaccine. Corticosteroids along with azathioprine were used for the management.

Hematological manifestations of antiphospholipid syndrome: Going beyond thrombosis.

Thrombotic complications are a hallmark of antiphospholipid syndrome (APS). These vascular - arterial, venous, and/or small vessel - complications are well described and known to hematologists and healthcare providers caring for patients with this disease. In this review, we shed light on other hematological manifestations of the disease, including bleeding, thrombocytopenia, autoimmune hemolytic anemia, and thrombotic microangiopathy syndromes. While these manifestations are not bona fide clinical criteria for the diagnosis of APS, they frequently interact and contribute to the complexity of clinical management of APS.

Multi-ancestry genome-wide association analyses identify novel genetic mechanisms in rheumatoid arthritis.

Rheumatoid arthritis (RA) is a highly heritable complex disease with unknown etiology. Multi-ancestry genetic research of RA promises to improve power to detect genetic signals, fine-mapping resolution and performances of polygenic risk scores (PRS). Here, we present a large-scale genome-wide association study (GWAS) of RA, which includes 276,020 samples from five ancestral groups. We conducted a multi-ancestry meta-analysis and identified 124 loci (P < 5 × 10-8), of which 34 are novel. Candidate genes at the novel loci suggest essential roles of the immune system (for example, TNIP2 and TNFRSF11A) and joint tissues (for example, WISP1) in RA etiology. Multi-ancestry fine-mapping identified putatively causal variants with biological insights (for example, LEF1). Moreover, PRS based on multi-ancestry GWAS outperformed PRS based on single-ancestry GWAS and had comparable performance between populations of European and East Asian ancestries. Our study provides several insights into the etiology of RA and improves the genetic predictability of RA.

Lebanese Hospital-Based Rheumatoid Arthritis Registry: Characteristics of Patients and Comparison with Other Populations.

Objectives: The aim of the Lebanese hospital-based Rheumatoid Arthritis (RA) registry, initiated in 2011, is to evaluate the safety and efficacy of biologic agents among patients seeking care at the American University of Beirut Medical Center (AUBMC). We aimed to characterize the demographic and clinical profile of RA patients included in the Lebanese registry. We compared our results with those issued from Middle Eastern and non-Middle Eastern registries. Methods: 195 Patients enrolled in the RA registry from 2011 to 2018 were considered in this study. Patients enrolled in the registry were eligible to be treated with biologics, but 56 patients remained biologics naïve. Patients were reassessed every six months. Results: The highest proportion of patients were female (81%). The mean age was 55.53±15 years, and the disease duration was 11.38±7.7 years. RA was diagnosed at a mean age of 44.13±16 years. Almost one-third of RA patients were smokers (29.2%) and 15% consumed alcohol. Comorbidities included cardiac diseases (30.8%), hypertension (24.6%), hyperlipidemia (11.8%), diabetes mellitus (9.2%), and Hypothyroidism (6.2%). Three cases of cancer and seven cases of tuberculosis were reported. The mean of the Disease Activity Score (DAS28) was 3.75 ± 2.28 with no difference according to gender; the mean of the Health Assessment Questionnaire (HAQ) score showed a significant difference between females and males (1.02 ± 0.84 and 0.61 ± 0.7 respectively). Methotrexate was the most commonly used medication. There was non-significant difference in taking biologics according to gender. Conclusion: Our findings are similar to other studies in terms of gender distribution. The higher mean age at diagnosis compared to other populations could indicate a delay in seeking appropriate care. The Lebanese RA registry provides valuable data on pharmacological interventions used and an opportunity to follow up to examine the effectiveness of different therapeutics and to monitor their side effects.

The psychological impact of Behçet's disease.

BACKGROUND: Bechet's disease (BD), a chronic multiorgan involving disease, has a significant impact on quality of life in spite of effective treatment modalities. Disease manifestations such as arthritis, orogenital ulcerations, rashes, angiitis, and neurological involvement affect health-related quality of life (HRQoL) through its impact on depression, anxiety, and fatigue. OBJECTIVES: We aimed explore the psychological impact of BD, taking into consideration the effect on the HRQoL, as well as the association with depression, anxiety, wellbeing, and fatigue. METHODS: This is a narrative review of the literature that looks into the association of BD on the HRQoL including all studies that have assessed such as association. RESULTS/FINDINGS: Depression and anxiety are prevalent among patients with BD, and contribute significantly to fatigue, a common symptom among BD patients. In addition, the psychological wellbeing is affected by the disease, however, more studies are needed to assess this relationship. CONCLUSION: Depression and anxiety are strongly associated with BD, and contribute significantly to fatigue, a common symptom among BD patients. In addition, the psychological wellbeing is affected by the disease, however, more studies are needed to assess this relationship. Besides, the controlling factors of the psychological impact are still to be deciphered.

Rheumatoid Cerebral Vasculitis in a Patient in Remission.

Cerebral vasculitis is a very rare extra-articular complication of rheumatoid arthritis (RA) that is often challenging to diagnose. Elevated titers of rheumatoid factor (RF), anti-cyclic citrullinated peptide antibodies (anti-CCP), and antinuclear antibodies (ANA) have been linked with severe complications. The absence of highly elevated titers of RF, anti-CCP, and ANA can complicate the diagnosis of RA-associated cerebral vasculitis. We report the case of a 59-year-old woman with long-standing arthritis maintained on rituximab and leflunomide who developed sudden headaches and altered level of consciousness. Laboratory work-up revealed normal lymphocyte count and mildly elevated total serum protein and anti-CCP with negative RF and ANA and no evidence for viral or bacterial infections. Cerebrospinal fluid analysis (CSF) showed slightly elevated anti-CCP with normal levels of CXCL-13 and interleukin 6 (IL-6). Brain magnetic resonance imaging (MRI) showed ill-defined lesion of high T2 signal. Using MR angiogram, MR perfusion, and MR spectroscopy, the diagnosis of rheumatoid cerebral vasculitis was confirmed. The patient was treated with intravenous methyl-prednisolone with fast complete improvement. We conclude that adequate immunosuppression in RA might not be able to prevent rare extra-articular manifestations such as rheumatoid cerebral vasculitis.

Musculoskeletal Manifestations of Sarcoidosis.

Since its initial description in the late 19th century, sarcoidosis has been extensively studied. Although the general mechanism of immune activation is known, many details especially in the context of disease associations are still missing. One of such associations is the musculoskeletal complications that are widely variable in terms of presentation and response to treatment. Sarcoidosis can involve the joints leading to acute and, less commonly chronic, arthritis. While acute arthritis is mostly self-resolving in nature, chronic arthritis may lead to deformity and destruction of the joint. Sarcoidosis can also involve the muscles, leading to different pathologies primarily categorized according to the clinical presentation, despite the efforts to find a new classification based on imaging, histological, and clinical findings. The bones can be directly and indirectly affected. Different types of bone lesions have been described, although around half of these patients remain asymptomatic. Osteoporosis, increased risk of fractures, hypercalcemia, and hypercalciuria are examples of the indirect effect of sarcoidosis on the bones, possibly contributed to elevated levels of calcitriol. Nevertheless, sarcoidosis can be associated with small-vessel, medium-vessel, and large vessel vasculitis, although it is frequently difficult to differentiate between the co-existence of a pure vasculitis and sarcoidosis and sarcoid vasculitis.

Thalassemia and autoimmune diseases: Absence of evidence or evidence of absence?

The thalassemias are a group of inherited disorders of hemoglobin synthesis that continue to pause a global public health concern. The complex molecular and pathogenetic pathways involved in disease process lead to an array of comorbidities that require lifelong management. The disease and its treatment can also lead to alterations in immune function and a link to various autoimmune diseases has been frequently suggested. However, most data stem from single case reports and small studies that do not allow proper assessment of causal associations. Still, the high morbidity in thalassemia makes patients vulnerable to the added burden of coexisting autoimmune diseases, and special management considerations in this patient population are warranted. In this review, we explore insights and data from the literature on various autoimmune disease that have been observed in patients with thalassemia. The role of the thalassemia carrier state in modifying outcomes of patients with autoimmune diseases is also discussed.

Fibromyalgia among University Students: A Vulnerable Population.

Background: Fibromyalgia (FM), a complex musculoskeletal disorder, can affect individuals from different genders having different genetic and psychosocial backgrounds. The prevalence of FM depends specifically on the age, gender, and level of stress of the individual. Since the university student body tackles high levels of academic and non-academic stress, we aimed to assess the prevalence and characteristics of FM among such a vulnerable population. Methods: A survey was sent to participants from two major English-speaking private universities in Lebanon; the American University of Beirut (AUB) and the Lebanese American University (LAU). The survey included the modified American College of Rheumatology (ACR) 2016 criteria, the widespread pain index (WPI), the symptoms severity score (SSS), and the duration of presence of such FM symptoms. In addition, the survey evaluated the presence of other specific musculoskeletal disorders among participants. Nevertheless, a 12-item general healthy questionnaire (GHQ-12) was used to assess the presence of anxiety, depression, social dysfunction, and loss of confidence among participants. Results: The survey was sent to a total of 2178 students with 184 complete responses (8.45% response rate). The prevalence of FM among the respondents was 13.6%. Students with FM had a significant personal history of a musculoskeletal disorder other than FM and a significant family history of musculoskeletal disorders. The mean SSS score of the target population, including those with FM and those without FM, was 4.5. Patients with FM were significantly in distress and highly symptomatic as measured by GHQ-12 (Unadjusted OR 3.23 [95% CI 1.32-7.95]). Conclusion: Fibromyalgia seems to be prevalent among university students; in particular, those with other musculoskeletal disorders, those with a family history of musculoskeletal disorders, and those with severe depression and anxiety.

COVID-19 and antiphospholipid antibodies: A position statement and management guidance from AntiPhospholipid Syndrome Alliance for Clinical Trials and InternatiOnal Networking (APS ACTION).

Coronavirus disease 2019 (COVID-19) is associated with a high rate of thrombosis. Prolonged activated partial thromboplastin times (aPTT) and antiphospholipid antibodies (aPL) are reported in COVID-19 patients. The majority of publications have not reported whether patients develop clinically relevant persistent aPL, and the clinical significance of new aPL-positivity in COVID-19 is currently unknown. However, the reports of aPL-positivity in COVID-19 raised the question whether common mechanisms exist in the pathogenesis of COVID-19 and antiphospholipid syndrome (APS). In both conditions, thrombotic microangiopathy resulting in microvascular injury and thrombosis is hypothesized to occur through multiple pathways, including endothelial damage, complement activation, and release of neutrophil extracellular traps (NETosis). APS-ACTION, an international APS research network, created a COVID-19 working group that reviewed common mechanisms, positive aPL tests in COVID-19 patients, and implications of COVID-19 infection for patients with known aPL positivity or APS, with the goals of proposing guidance for clinical management and monitoring of aPL-positive COVID-19 patients. This guidance also serves as a call and focus for clinical and basic scientific research.

Development of an Educational Video for Self-Assessment of Patients with RA: Steps, Challenges, and Responses.

OBJECTIVES: The primary objective was to develop an educational video to teach patients with rheumatoid arthritis (RA) self-assessment of their disease activity. Secondary objectives were to validate the video, identify the challenges in producing it, and the responses to these challenges. METHODS: Rheumatologists from 7 Middle Eastern Arab countries (MEAC) discussed unmet needs in the education of patients with RA. They reviewed pre-existing educational audiovisual material and drafted the script for a new video in Arabic. The video was produced in collaboration with a technical team, then validated by patients using a standardized interview. At each step of production, challenges were identified. RESULTS: Twenty-three rheumatologists from MEAC identified unmet needs in patients' education. A video was produced, explaining the concepts of treat-to-target and showing a patient performing self-assessment using DAS-28. Sixty-two patients were interviewed for validation and found the video to be useful and easy to understand, albeit not replacing the physician's visit. Most common challenges encountered included acceptance of patient empowerment, agreement on DAS-28 as composite measure, production of a comprehensible written Arabic text, and addressing the population cultural mix. CONCLUSION: Despite challenges, the video was well accepted among patients and can be used for clinical and research purposes. It is particularly useful in pandemic periods where social distancing is recommended.