Eosinophilic Orbital Myositis Superseding Ocular Myasthenia.

Various muscles can be involved in idiopathic eosinophilic myositis (IEM), with the ocular muscles being notably affected. Ocular eosinophilic myositis is a rare condition that typically affects the rectus muscles. A tissue biopsy stands as the gold standard for diagnosis. Different subtypes exist based on the extent of eosinophilic infiltration. Limited data is available about treatment, although glucocorticoids have shown successful outcomes. We present the case of a 60-year-old man who, a few years after being diagnosed with ocular myasthenia gravis, was diagnosed through a tissue biopsy with ocular eosinophilic myositis. Treatment with oral glucocorticoids significantly improved his symptoms.

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.

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.

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.

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.

Polymyalgia rheumatica-like presentation in a case of diffuse large B-cell lymphoma: a diagnostic pitfall.

Diffuse large B-cell lymphoma (DLBCL) commonly presents with systemic manifestations including fever, weight loss, and night sweats. Uncommonly, patients with DLBCL can present with musculoskeletal manifestations mimicking polymyalgia rheumatica (PMR). Herein, the case of a 61-year-old woman who presented with pain in the bilateral shoulders, arms, hands, knees, pelvic girdle, and neck with bouts of fever, is presented. Laboratory workup for infectious and connective tissue diseases was non-revealing, except for elevated inflammatory markers. A positron emission tomography (PET)/computed tomography (CT) scan was suggestive of PMR, but also revealed enlarged lymph nodes initially thought to be reactive in nature. However, a lymph node biopsy showed findings consistent with DLBCL. This case highlights the importance of a thorough investigational workup when cases with features of PMR do not meet the proper criteria for this diagnosis to be made, in order not to miss a hematopoietic neoplasm with a PMR-like presentation.

The Fibromyalgia Bladder Index in 100 consecutive women with fibromyalgia.

INTRODUCTION AND HYPOTHESIS: The Fibromyalgia Bladder Index (FBI) is a validated instrument to quantify bothersome bladder symptoms specifically in women with fibromyalgia syndrome (FMS). The FBI includes two sub-scales: one addressing urinary urgency and bladder pain (UP), the other addressing urinary frequency and nocturia (FN). The objectives of this study are to evaluate the FBI in a cohort of patients with FMS, to correlate it with certain characteristics in this cohort, and to compare it with controls. METHODS: We performed a case-control study of 100 women with FMS and 155 controls. Demographic data, comorbidities, and other characteristics were registered. Comparison between FBI scores of participants with and without FMS, as well as correlation of FBI scores with the characteristics of FMS patients, were undertaken using independent two-sample t test for continuous outcomes and Pearson's Chi-squared test for categorical outcomes. RESULTS: The mean UP subscale score of the FBI was significantly higher in the FMS group (10.29 ± 5.61) compared with the controls (1.65 ± 2.65; (p = 0.001). The mean FN subscale score was significantly higher in the FMS group (9.93 ± 5.37) compared with the controls (2.95 ± 3.27; p = 0.001). FMS patients diagnosed >3 years ago had a higher UP subscale score and a higher FN subscale score compared with FMS patients diagnosed <3 years ago (p = 0.020 and p = 0.024 respectively). Menopause and parity significantly increased the FBI scores. Smoking and a history of depression did not significantly affect any of the FBI subscale scores in the FMS group. CONCLUSION: Women with FMS suffer from bothersome bladder symptoms that can be readily identified and quantified.

The Use of Infliximab (Remicade®) for the Treatment of Rheumatic Diseases at a Tertiary Center in Lebanon: A 17-Year Retrospective Chart Review.

OBJECTIVES: Infliximab (Remicade®) was the first tumour necrosis factor-α (TNF) inhibitor to receive its initial marketing approval from the US Food and Drug Administration (FDA) for the treatment of Crohn's disease. Following that, infliximab became approved for several immune-mediated inflammatory diseases. No evidence exists in the Middle East and North Africa region on the experience with infliximab use over an extended period in terms of efficacy and safety. METHODS: The Rheumatology division at the American University of Beirut Medical Centre (AUBMC), one of the largest tertiary centres in the Middle East and North Africa region, has been using infliximab infusions for the treatment of certain rheumatic diseases for around two decades. By reviewing retrospectively medical charts at AUBMC, we investigate indications, safety and efficacy, rate of withdrawals, rate of switching to another biologic, and financial coverage of the drug to present data for practitioners and patients in the region considering infliximab for treatment of immune-mediated inflammatory diseases. RESULTS: A total of 198 patients were identified in the past 17 years to have taken infliximab. The largest proportion of treated patients had RA. Fourteen percent of the total cohort experienced serious adverse events, with 96.4% of those events being mild hypersensitivity reactions. Five patients withdrew the medication because of infectious complications, 4 of which were cases of tuberculosis reactivation. Despite that, around half of the patients were switched to another biologic agent such anti-TNF-α, anti-CD20, and anti-IL-6 due to partial response, and less than half were receiving add-on disease-modifying anti-rheumatic drugs (DMARDs) such as methotrexate, 70% of patients who used infliximab only or were switched achieved complete remission at their last hospital information. Around 98% of infliximab users were financially covered. CONCLUSION: According to our experience, infliximab has made remission and prevention of long-term disability realistic goals of therapy in the Middle East region.

Update on the management of colchicine resistant Familial Mediterranean Fever (FMF).

BACKGROUND: Familial Mediterranean Fever (FMF), an autoinflammatory disease, is characterized by self-limited inflammatory attacks of fever and polyserositis along with high acute phase response. Although colchicine remains the mainstay in treatment, intolerance and resistance in a certain portion of patients have been posing a problem for physicians. MAIN BODY: Like many autoimmune and autoinflammatory diseases, many colchicine-resistant or intolerant FMF cases have been successfully treated with biologics. In addition, many studies have tested the efficacy of biologics in treating FMF manifestations. CONCLUSION: Since carriers of FMF show significantly elevated levels of serum TNF alpha, IL-1, and IL-6, FMF patients who failed colchicine were successfully treated with anti IL-1, anti IL-6, or TNF inhibitors drugs. It is best to use colchicine in combination with biologics.

Disease burden and treatment challenges of psoriatic arthritis in Africa and the Middle East.

Psoriatic arthritis (PsA) is a chronic, inflammatory arthropathy occurring in up to 30% of patients with psoriasis, and is characterized by multiple manifestations including peripheral arthritis, enthesitis, dactylitis, spondylitis, and psoriatic skin and nail disease. This complex and heterogeneous disease is poorly understood and its diagnosis and treatment are suboptimal, particularly in Africa and the Middle East, where very few studies into the impact of PsA have been carried out. This article aims to highlight the disease burden of PsA in the region as well as to identify unmet clinical needs. A non-systematic review was carried out in the PubMed database and the most relevant publications were selected. Expert rheumatologists practicing in Africa and the Middle East provide an insight into the challenges of treating PsA in daily practice, along with recommendations for improvements.

Neutrophils contribute to vasculitis by increased release of neutrophil extracellular traps in Behçet's disease.

BACKGROUND AND OBJECTIVES: Behçet's disease (BD) is a multi-system inflammatory disorder that can cause vasculitis. Here we questioned whether Neutrophils in BD cause vasculitis via releasing Neutrophil Extracellular Traps (NETs), a process called NETosis. METHODS: Circulating neutrophils were isolated from a cohort of Middle Eastern BD patients with an active disease and healthy volunteers. The percentage of NETs release was monitored in neutrophils stimulated or not with BD serum, and treated or not with Colchicine, Dexamethasone, Cl-amidine or N-Acetyl Cysteine (NAC). The mRNA expression levels of PAD4 (a key enzyme in NETosis) was also assessed. The effect of NETs on the proliferation and cell death of endothelial cells was investigated using an in vitro co-culture model. The presence of NETs in skin tissues of BD patients was examined using immunolabeling of NETs associated proteins. RESULTS: Circulating Neutrophils from BD patients were more prone to release NETs in vitro and expressed higher levels of PAD4 compared to healthy volunteers. Spontaneous NETs formation in BD neutrophils was inhibited by Colchicine and Dexamethasone, two drugs used to treat BD. NETs formation was also inhibited by Cl-amidine, a specific PAD4 inhibitor, and by NAC, a ROS inhibitor. Interestingly, serum from BD patients stimulated circulating neutrophils from healthy volunteers to release more NETs and increased their mRNA PAD4 expression. Moreover, endothelial cells cultured in the presence of NETs from BD patients showed a decrease in proliferation and an increase in apoptosis and cell death. Finally, NETosis was predominantly identified around affected blood vessels in biopsies of vasculitis from BD patients. CONCLUSION: Our results provide evidence on the implication of NETosis in the pathophysiology of BD especially in inducing vasculitis.

Risk factors for incident shoulder soft tissue rheumatic disorders: a population-based case-control study in Lebanon.

BACKGROUND: Soft tissue rheumatic disorders (STRDs) are very common and impact enormously general population, working groups and physiotherapist practices. However, they do not have neither a clear case definition nor objective tests to be accurately diagnosed rendering them neglected with poorly-estimated burden. Shoulder is one of the most frequent sites for STRDs. AIM: The aim of this study was to identify risk factors for shoulder STRDs among Lebanese adults aged ≥ 15 years. METHODS: A case-control study was designed based on data from the Community Oriented Program for Control of Rheumatic Diseases (COPCORD) study conducted in Lebanon in 2009. Cases were defined as those who recently suffered from shoulder pain, tenderness or stiffness with duration not exceeding 12 months (52 cases). These were frequency-matched by age and gender with 208 controls who never experienced any musculoskeletal pain. RESULTS: Area of residence, physical activity, family history and stress-induced sleep difficulty were significantly associated with shoulder STRDs after adjusting for cigarette smoking, job nature and family monthly income. CONCLUSION: Factors associated with shoulder STRD among the Lebanese population include geographical location, psychosocial factors, physical activity and familial predisposition. Further longitudinal studies are needed to establish a temporal sequence and explore other potential determinants, especially among the working population.

Epstein-Barr virus DNA modulates regulatory T-cell programming in addition to enhancing interleukin-17A production via Toll-like receptor 9.

Infection with the Epstein-Barr virus (EBV) has been associated with several autoimmune diseases including rheumatoid arthritis (RA). We have previously reported that DNA from this virus enhances production of the pro-autoimmune interleukin 17A (IL-17A) in mice. In this study we assessed the effect of EBV DNA on regulatory T cell programming and examined whether it mediated its effects via Toll-like receptor 9 (TLR9) in mice; moreover, we evaluated whether EBV DNA in humans had similar effects to those seen in mice. For this purpose, we assessed the linearity of the correlation between EBV DNA and IL-17A levels in RA subjects and matched controls. A modulatory effect for the viral DNA was observed for regulatory T cell markers with an inhibitory effect observed for CTLA4 expression in the EBV DNA-treated mice. To examine whether TLR9 mediated the detection of EBV DNA and enhancement of IL-17A production, mouse peripheral blood mononuclear cells were treated with the DNA in the presence or absence of the TLR9 inhibitor ODN 2088. Subsequently, IL-17A production from these cells was assessed. Treatment with the TLR9 inhibitor resulted in a significant decrease in IL-17A production indicating that TLR9 is involved in this pathway. In human subjects, examining the linearity of the correlation between EBV DNA and IL-17A levels in RA subjects showed a propensity for linearity that was not observed in controls. Our data thus indicates that EBV DNA itself acts as a modulator of the Th17 compartment as well as that of regulatory T cell mechanisms. The involvement of TLR9 in the EBV DNA-triggered induction of IL-17A suggests therapeutic targeting of this endosomal receptor in EBV positive subjects with an autoimmune flare-up or possibly for prophylactic purposes.

A review of current management of vasculo-Behcet's.

PURPOSE OF REVIEW: To give an overview of recently published articles about the management of vasculo-Behcet's with particular emphasis on anticoagulation. RECENT FINDINGS: Biologic agents are emerging as a potential therapeutic option in refractory vasculo-Behcet with a good safety profile. Evidence further shows that following nonpulmonary aneurysm repair, there is a reduced risk of recurrent aneurysmal formation at the operative site in patients treated with immunosuppressants in addition to their surgery, than those undergoing surgical intervention alone. SUMMARY: Behcet disease patients are at risk of developing multiple vascular complications including thrombosis and aneurysms. Treatment should focus on reducing inflammation; and the role of anticoagulation is still debatable.

Idiopathic Granulomatous Mastitis Presenting as a Breast Pseudotumor: Case Reports with Review of the Literature.

Idiopathic granulomatous mastitis is a rare benign inflammatory breast disease that affects women of childbearing age with a history of breastfeeding. It usually presents as an enlarging breast mass that can greatly mimic breast cancer. Moreover, it does not have a specific radiographic finding, so the only way to reach a definitive diagnosis is by core biopsy and histology. Furthermore, a consensus regarding the best treatment modality has not been reached yet. In this report, we describe the cases of two patients who suffered from this disease, and to our knowledge, such a report is the first of its kind to address this topic in this region. Therefore, because of its uncommon nature and obscure presentation, we hereby report two cases of idiopathic granulomatous mastitis. The clinical presentation, treatment, and pathological findings are described, and a literature review on idiopathic granulomatous mastitis will be reported.

Fibromyalgia: epidemiology and risk factors, a population-based case-control study in Lebanon.

AIM: To investigate the epidemiology of fibromyalgia (FM) and assess its risk factors. METHODS: Using data from the 2009 Community Oriented Program for Control of Rheumatic Diseases (COPCORD) study conducted in Lebanon, a population-based case control study was performed. The sample included 34 FM patients, frequency matched with 136 controls free from any musculoskeletal complaints and randomly sampled from the population. The controls were frequency matched with cases by age and gender. RESULTS: The 34 female FM cases were prevalent cases which existed for a long period of time and all those who consulted a doctor were previously misdiagnosed. Family history of joint problems (OR = 4.93, 95% CI: 1.56-15.58) and working status (OR = 2.69, 95% CI: 1.04-6.93) were significant risk factors for FM, after adjusting for body mass index, distress level, smoking status and residence location. CONCLUSION: This was the first study to address the epidemiology of FM in Lebanon and the region. The chronic nature of FM that is characterized by frequent bouts of intense disabling pain and symptoms constitutes a significant health and economic burden. Clustering of cases in coastal areas was partially explained by other factors such as body mass index, distress level, smoking and work status. The high burden of FM found in our study calls for further investigation of potential risk factors of this condition.

How to treat ankylosing spondylitis and nonradiographic axial spondyloarthritis. Key practical messages from the 2015 American College of Rheumatology recommendations.

A panel of experts commissioned by the American College of Rheumatology have recently reviewed the literature related to the treatment of patients with ankylosing spondylitis and nonradiographic axial spondyloarthritis. They published a set of recommendations for the management of common clinical questions for both active and stable disease, including the appropriate use of nonsteroidal anti-inflammatory drugs, tumor necrosis factor inhibitors, rehabilitation, education, and preventive care. This article summarizes these recommendations and provides key practical messages for physicians taking care of these patients.

Mania induced by adalimumab in a patient with ankylosing spondylitis.

Objectives Monoclonal antibodies such as antagonists of tumor necrosis factor-alpha have been shown to have beneficial effects on the well-being of patients with inflammatory illnesses. However, mood episodes triggered by such agents have been reported. We herein report the case of mania induced by adalimumab treatment in an adult with ankylosing spondylitis, which later resolved once adalimumab was discontinued and mood stabilizers were initiated. Methods A 25-year-old man, with prior history of dysthymia, was diagnosed with ankylosing spondylitis and started on adalimumab. He gradually developed manic symptoms over seven to eight months, while maintained on adalimumab. As his condition did not improve with outpatient management, the patient was admitted to the Psychiatry inpatient unit. Results Valproate and aripiprazole were initiated, and adalimumab was substituted with non-steroidal anti-inflammatory agents. Mood symptoms resolved within days, and the patient was discharged. Upon follow-up, the patient was euthymic and compliant to his psychotropic medications. He was started on certolizumab, a different immunomodulatory, for his ankylosing spondylitis. Conclusions Immunological modulation might be a key factor in triggering, maintaining, or treating mood symptoms. Further research in this field is warranted to better understand the pathophysiology of mania. To our knowledge, manic symptoms induced by adalimumab have not been previously reported in the literature, which is why our case report can have an impact in recognizing this important clinical adverse effect.