Efficacy and Safety of Mepolizumab in Patients With Eosinophilic Granulomatosis With Polyangiitis: A Single-Center Experience.

Eosinophilic granulomatosis with polyangiitis (EGPA), previously known as Churg-Strauss syndrome, is a systemic vasculitis characterized by eosinophil-rich, necrotizing granulomatous inflammation primarily affecting the respiratory tract with necrotizing vasculitis of small- to medium-sized arteries. In this case series, we retrospectively evaluated the efficacy and safety of mepolizumab in seven patients diagnosed with EGPA who presented to the Department of Allergy and Clinical Immunology at Cleveland Clinic Abu Dhabi. The variables assessed before and after mepolizumab treatment included Birmingham Vasculitis Activity Score (BVAS), prednisolone dose, Asthma Control Test (ACT) score, and blood eosinophil count (BEC). We found a significant reduction in BVAS and prednisolone dosage with clinical improvements in asthma symptoms after treatment with mepolizumab. Our case series, the first from the Middle East on the use of mepolizumab in EGPA, demonstrates that mepolizumab is a safe and effective treatment for patients with EGPA.

From Sand to Excellence: A Deep Dive into Abu Dhabi's Rheumatology Landscape.

The Emirate of Abu Dhabi (AD) is the capital and largest emirate of the United Arab Emirates (UAE). The emirate's economic significance stems from non-oil and oil contributions to GDP. The 2022 GDP of Abu Dhabi was USD 230 billion. The government provides services to its residents through digital platforms such as official websites. The Abu Dhabi Health Insurance Law No. 23 of 2005 mandates that residents have access to necessary medical care and services. There is a paucity in the literature on the available rheumatology services in the Arab region. This review article aims to explore the status of rheumatology services in AD for both residents and visitors. It will include an overview of paediatric and adult rheumatology care, accessibility of diagnostic procedures, the integration of electronic medical records, access to medications, the status of postgraduate education, research, and suggestions on how to enhance rheumatology services in AD as a destination for medical tourism.

A Tangled Autoimmune Trio: Multiple Sclerosis, Systemic Lupus Erythematosus and Antineutrophil Cytoplasmic Antineutrophil Cytoplasmic Antibody Vasculitis.

The coexistence of multiple autoimmune diseases in the same individual is unusual and has received little attention in the literature. We present a young female patient with multiple sclerosis, systemic lupus erythematosus, and biopsy-proven renal proteinase 3 antineutrophil cytoplasmic antibodyassociated vasculitis who responded well to intravenous rituximab clinically and serologically.

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.

Comprehensive description of the prevalence, serological and clinical characteristics, and visceral involvement of systemic sclerosis (scleroderma) in a large cohort from the United Arab Emirates Systemic Sclerosis Registry.

Systemic sclerosis is an autoimmune condition characterized by a wide range of clinical presentations. Registries may serve to expand understanding about systemic sclerosis and aid in patient care and follow-up. The objective of this study was to analyze the prevalence of systemic sclerosis in a large cohort from the United Arab Emirates Systemic Sclerosis Registry and find the significant similarities and differences between the different subsets. All scleroderma patients in the United Arab Emirates were included in this multicenter national retrospective analysis. Data on demographics, comorbidities, serological characteristics, clinical aspects, and treatment were collected and analyzed, highlighting the most common traits identified. A total of 167 systemic scleroderma patients from diverse ethnic backgrounds were enrolled. Overall, 54.5% (91/167) of the patients were diagnosed with diffuse cutaneous systemic sclerosis, and 45.5% (76/167) with limited cutaneous systemic sclerosis. The prevalence of systemic sclerosis was 1.66 per 100,000 for the total registry and 7.78 per 100,000 for United Arab Emirates patients. Almost all patients in the diffuse cutaneous systemic sclerosis and limited cutaneous systemic sclerosis groups tested positive for the immunofluorescence antinuclear antibody. Antibodies against Scl-70 were significantly more associated with diffuse cutaneous systemic sclerosis, whereas anticentromere antibodies were significantly more associated with the limited cutaneous systemic sclerosis group (p < 0.001). Sclerodactyly, shortness of breath, and digital ulcers were more common in diffuse cutaneous systemic sclerosis patients compared with the limited cutaneous systemic sclerosis subtype in terms of clinical symptoms and organ involvement. Telangiectasia was much more common in the limited cutaneous systemic sclerosis group. Furthermore, diffuse cutaneous systemic sclerosis patients had more lung fibrosis (interstitial lung disease) than limited cutaneous systemic sclerosis patients (70.5% vs 45.7%), and pulmonary arterial hypertension was twice as common in limited cutaneous systemic sclerosis patients as it was in diffuse cutaneous systemic sclerosis patients. Local registries are paramount to understanding the clinical/serological characteristics of scleroderma. This study emphasizes the importance of raising disease awareness and distinguishing between the various systemic sclerosis subsets to implement patient-tailored strategies for early detection, better management, and higher quality of care.

Risk factors for lung function decline in systemic sclerosis-associated interstitial lung disease in a large single-centre cohort.

OBJECTIVES: The aim of this study was to identify risk factors of percent predicted forced vital capacity (ppFVC) decline in patients with SSc-associated interstitial lung disease (SSc-ILD). METHODS: We identified 484 patients with SSc who had HRCT Chest, of which 312 with ILD. Those with serial pulmonary function tests were included in a longitudinal analysis (n = 184). Linear mixed effect models were fitted to assess the decline in ppFVC over time, and to explore the effect of demographics and baseline characteristics on ppFVC decline. RESULTS: The majority of SSc-ILD patients were female (76.3%) and 51.3% had diffuse cutaneous subset. The mean (s.d.) age was 53.6 (12.7) years, median disease duration since first non-RP symptoms was 2.6 years, and 48.4% of the patients had ILD extent >20% on HRCT. In the univariate analysis, longer disease duration (>2.37 years), ILD extent >20%, and anti-topoisomerase I (ATA) positivity were significantly associated with ppFVC decline. In the multivariate analysis, the only statistically significant variable associated with ppFVC decline was ATA positivity. The overall group's mean decline in ppFVC was -0.28% (P-value 0.029), with -0.13% (n = 163) in those who were alive and -8.28% (P-value 0.0002 for the change in ppFVC trajectory) in patients who died within 2 years. CONCLUSION: Our study confirms that ppFVC is a marker of survival in SSc-ILD, supporting its use for risk stratification to identify patients who may benefit from earlier interventions and treatment. Our study also supports the role of ATA positivity as a predictive marker for ppFVC decline in this population.

Long-Term Efficacy and Safety of Omalizumab Monotherapy in a Patient With Normocomplementemic Urticarial Vasculitis.

Normocomplementemic urticarial vasculitis is a rare autoimmune disorder characterized by leukocytoclasia, fibrin deposits, and extravasated erythrocytes affecting multiple organ systems. Current treatment modalities, including corticosteroids and immunosuppressive agents, are of limited efficacy and an expansive side effect profile. Omalizumab has been reported to be effective in urticarial vasculitis, but its long-term effectiveness and tolerability have not yet been evaluated. We report a case of long-standing normocomplementemic urticarial vasculitis treated with omalizumab only, for almost 3 years. The patient reported a significant improvement in quality of life after the first few doses with a significant improvement in the urticaria control test. The treatment was well tolerated and no adverse events were reported after 3 years. Our patient was treated with 300 mg of omalizumab, as it was previously linked with a better improvement in quality of life. We were able to extend our patient’s treatment intervals, suggesting that this is feasible in patients treated with omalizumab who achieve a complete response. We recommend that larger and long-term studies are conducted to assess the efficacy and effectiveness of omalizumab in patients with urticarial vasculitis. J Drugs Dermatol. 2022;21(10):1124-1126. doi:10.36849/JDD.6739.

Consensus statements for pharmacological management, monitoring of therapies, and comorbidity management of psoriatic arthritis in the United Arab Emirates.

OBJECTIVE: Psoriatic arthritis (PsA), a chronic inflammatory disease characterized by heterogeneous clinical manifestations, substantially impacts the quality of life of affected individuals. This article aims at developing consensus recommendations for the management of PsA and associated comorbidities and screening and monitoring requirements of PsA therapies in the United Arab Emirates (UAE) population. METHODS: An extensive review of present international and regional guidelines and publications on the pharmacological management, monitoring of therapies in the context of PsA was performed. Key findings from guidelines and literature were reviewed by a panel of experts from the UAE at several meetings to align with current clinical practices. Consensus statements were formulated based on collective agreement of the experts and members of Emirates Society for Rheumatology. RESULTS: The consensus recommendations were developed to aid practitioners in clinical decision-making with respect to dosage recommendations for pharmacological therapies for PsA, including conventional drugs, non-biologic, and biologic therapies. Consensus recommendations for therapeutic options for the treatment of PsA domains, including peripheral arthritis, axial disease, enthesitis, dactylitis, psoriasis, and nail disease, were developed. The panel emphasized the importance of monitoring PsA therapies and arrived at a consensus on monitoring requirements for PsA therapies. The expert panel proposed recommendations for the management of common comorbidities associated with PsA. CONCLUSION: These consensus recommendations can guide physicians and healthcare professionals in the UAE in making proper treatment decisions, as well as efficiently managing comorbidities and monitoring therapies in patients with PsA.

Consensus statements for evaluation and nonpharmacological Management of Psoriatic Arthritis in UAE.

OBJECTIVE: Psoriatic arthritis (PsA), a chronic inflammatory arthropathy, is often underdiagnosed in Middle Eastern countries, substantially impacting the treatment of affected individuals. This article aims to highlight current unmet clinical needs and provide consensus recommendations for region-specific evaluation methods and nonpharmacological therapies in the United Arab Emirates (UAE). METHOD: An extensive literature review was conducted, focusing especially on global and regional guidelines for the evaluation and treatment of PsA. These form the basis of the consensus statements formulated. Additionally, an expert panel of key opinion leaders from the UAE reviewed these guidelines and available literature at an advisory board meeting to identify unmet needs, bridge clinical gaps in the UAE, and develop consensus statements for the evaluation and treatment of PsA. RESULT: The consensus statements were developed based on overarching principles for the management of PsA, evaluation of patients with PsA, and nonpharmacological approaches for the management of PsA. The overarching principles included adopting a targeted, multidisciplinary approach, along with collaboration between rheumatologists and dermatologists in cases of clinically significant skin involvement. The panel also highlighted the value of composite disease severity measures for characterizing clinical manifestations of PsA. In terms of nonpharmacological management approaches, lifestyle modification (comprising dietary change, exercise, and cessation of smoking) and psychotherapy were recommended. CONCLUSION: The consensus statements will aid healthcare professionals in clinical decision-making in the context of PsA.

Predictors of not Achieving Remission or Low Disease Activity in Axial Spondyloarthritis Patients from Middle Eastern Countries: A Prospective, Multicenter, Real-world Study.

Objectives: We sought to identify the predictors of not achieving remission or low disease activity (LDA) among axial spondyloarthritis (SpA) patients in four Middle Eastern countries. Methods: In this multicenter prospective real-world study, adult patients with axial SpA diagnosed clinically during January-June 2019, and who met the Assessment of SpondyloArthritis International Society classification criteria for axial SpA, were enrolled from the participating centers of four countries-Lebanon, Oman, Qatar, and the UAE. Patient demographics, disease history, comorbidities, treatment, and compliance data were obtained at baseline. The primary outcome was to determine the percentage of patients who did not achieve the clinical target of remission or LDA as indicated by Ankylosing Spondylitis Disease Activity Score-C-reactive protein (ASDAS-CRP) < 2.1 after a three-month follow-up period. Secondary outcomes were assessing the demographic and clinical characteristics of 'achievers' and 'non-achievers' and to study the predictors of ASDAS-CRP ≥ 2.1 in different clinical subsets. Results: The participants were 309 patients of both sexes, with a median age of 43 years. Women had a slight majority (53.7%). At the end of the study, 72.2% of patients achieved the clinical target of ASDAS-CRP < 2.1. Non-achievers were significantly more likely to have enthesitis, positive human leukocyte antigen B 27 status, psoriasis, peripheral involvement, fibromyalgia, and a lower score on Compliance Questionnaire for Rheumatology (CQR). Multiple regression analysis showed that low CQR score, enthesitis, psoriasis, and family history of SpA were independent predictors of ASDAS-CRP ≥ 2.1. Conclusions: This real-world study suggests that low compliance, positive human leukocyte antigen B 27 status, peripheral involvement, and presence of enthesitis, psoriasis, and fibromyalgia are predictors of not achieving remission or LDA in axial SpA patients.

Intravenous immunoglobulin in the management of refractory lupus profundus.

Lupus profundus, often known as lupus panniculitis, is a rare form of persistent cutaneous lupus erythematosus. It usually manifests as painful plaques or nodules that can ulcerate and cause atrophy and scarring. It may respond to topical treatments and antimalarials, although treatment might be difficult at times, necessitating immunosuppressive medications. A 36-year-old woman from the United Arab Emirates presented with multiple painful disfiguring nodules involving the face and shoulders. The disfiguring skin nodules were resistant to systemic glucocorticoids, hydroxychloroquine, azathioprine, mycophenolate mofetil, and cyclosporine, but they significantly improved with monthly intravenous immunoglobulin over a 6-month period.

Primary Sjögren's Syndrome Presenting as Recurrent Ischemic Strokes.

Primary Sjogren's Syndrome (PSS) is an autoimmune exocrinopathy, with protean manifestations affecting multiple organ systems. Neurological manifestations are documented in about 20% of PSS cohorts in literature, with peripheral manifestations being commoner. Central nervous system manifestations of PSS (CNS-SS) encompass ischemic strokes, demyelinating lesions, aseptic meningitis, encephalitis, cerebellar ataxia, cognitive impairment and movement disorders. Ischemic stroke as presenting manifestation of PSS is extremely rare. We hereby describe a 50-year-old male, who presented for evaluation of 2 episodes of discrete focal neurological deficits over a duration of 6 weeks, with neuro-imaging findings revealing evidence of acute-subacute bihemispheric infarcts. Further evaluation revealed evidence of strongly positive anti phospholipid antibodies (aPL), indirect immunofluorescence antinuclear antibody (IIF-ANA), anti Sjögren's syndrome-A (SS-A/Ro) and anti-Ribonuclear protein (RNP) antibodies, with histopathological evidence of periductal and periacinar lymphocytic infiltration as well as acinar atrophy and interstitial fibrosis of minor salivary glands on lip biopsy, consistent with a diagnosis of Sjögren's syndrome, constituting a diagnosis of Antiphospholipid syndrome (APS) associated with PSS.

B Cell Signatures Distinguish Cutaneous Lupus Erythematosus Subtypes and the Presence of Systemic Disease Activity.

Cutaneous lupus erythematosus (CLE) is a chronic inflammatory skin disease characterized by a diverse cadre of clinical presentations. CLE commonly occurs in patients with systemic lupus erythematosus (SLE), and CLE can also develop in the absence of systemic disease. Although CLE is a complex and heterogeneous disease, several studies have identified common signaling pathways, including those of type I interferons (IFNs), that play a key role in driving cutaneous inflammation across all CLE subsets. However, discriminating factors that drive different phenotypes of skin lesions remain to be determined. Thus, we sought to understand the skin-associated cellular and transcriptional differences in CLE subsets and how the different types of cutaneous inflammation relate to the presence of systemic lupus disease. In this study, we utilized two distinct cohorts comprising a total of 150 CLE lesional biopsies to compare discoid lupus erythematosus (DLE), subacute cutaneous lupus erythematosus (SCLE), and acute cutaneous lupus erythematosus (ACLE) in patients with and without associated SLE. Using an unbiased approach, we demonstrated a CLE subtype-dependent gradient of B cell enrichment in the skin, with DLE lesions harboring a more dominant skin B cell transcriptional signature and enrichment of B cells on immunostaining compared to ACLE and SCLE. Additionally, we observed a significant increase in B cell signatures in the lesional skin from patients with isolated CLE compared with similar lesions from patients with systemic lupus. This trend was driven primarily by differences in the DLE subgroup. Our work thus shows that skin-associated B cell responses distinguish CLE subtypes in patients with and without associated SLE, suggesting that B cell function in skin may be an important link between cutaneous lupus and systemic disease activity.

Demographic and clinical patterns of severe asthma in the Middle East.

BACKGROUND: Severe asthma is a major burden on health-economic resources; hence, knowing the epidemiology of these patients is important in planning and provision of asthma care. In addition, identifying and managing the comorbidities helps improve symptoms and reduce associated morbidity and mortality. OBJECTIVES: Epidemiology of difficult asthma has not been well studied in the Middle East, so in this study, we present the demographic and clinical characteristics of severe asthma in the United Arab Emirates (UAE). METHODS: We retrospectively reviewed the notes of severe asthma patients attending three tertiary care hospitals between May 2015 and December 2019. Data on baseline demographics, asthma characteristics, treatment, and comorbidities were collected. RESULTS: We reviewed the notes of 458 patients (271 females and 187 males) that fulfilled the 2019 Global Initiative for Asthma guidelines for the diagnosis of severe asthma. The mean age was 47.7 (standard deviation 17.2) years. Males had significantly higher asthma control test scores (17.9 vs. 16, P = 0.01) and mean blood eosinophils (0.401 vs. 0.294, P <0.01) than females. The most common comorbidity observed was allergic rhinitis (52.2%) followed by gastroesophageal reflux disease (27.1%). In total, 109 (23.8%) patients were on biological therapies with most patients being on omalizumab and dupilumab (29 and 18 patients, respectively). Most patients were nonsmokers (97.2%), and majority were of TH2-high phenotype (75.7%). CONCLUSIONS: In this first report of severe asthma characteristics in the UAE, we found a pattern of female preponderance and most patients having a Th2-high phenotype. The findings are likely to help optimize asthma care in the region in the era of biologic therapies.

The treatment journey for patients with axial spondyloarthritis in North Africa and the Middle East: From diagnosis to management.

The management of axial spondyloarthritis (axSpA) is challenging worldwide, and the particular challenges shared by North Africa and the Middle East are mainly related to early diagnosis and standardized management. We believe there are several reasons for these challenges, including: (a) limited awareness of the disease manifestations and prevalence in the region among physicians; (b) the concept of nonradiographic axSpA, while accepted with some difficulty by the rheumatology community, may not be well understood by the referring primary care physicians; (c) access to, and training in magnetic resonance imaging varies greatly between countries in the North Africa and Middle East region, and this may have a large impact on early diagnosis; (d) country-specific treatment guidelines are unavailable; and (e) economic and cultural factors influence patients' attempts to seek and continue treatment. In this review, we will discuss the prevalence of axSpA in North Africa and the Middle East, as well as the challenges to diagnose and treat patients in this region. As rheumatologists practicing in North Africa and the Middle East, we also provide suggestions to assist physicians, other healthcare professionals, and researchers in facilitating early, accurate diagnosis and treatment of axSpA.