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.

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.

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.

Extensive drug resistant Acinetobacter baumannii: a comparative study between non-colistin based combinations.

Background The Gastrointestinal Surgery Center (GISC)-Mansoura University, faced a series of extensive drug resistant (XDR) A. baumannii cases, that were microbiologically resistant to penicillins, cephalosporins, fluoroquinolones, aminoglycosides, carbapenems and tigecycline. Colistin would have been a last resort therapy in such situation, however, intravenous polymyxins E (colistin) is relatively unavailable in Egypt. Many practitioners tried to form antibiotic combinations from the available antibiotics to overcome the resistance mechanisms of the pathogen. Objective Evaluate the clinical outcomes of these combinations retrospectively. Setting The study took place at the GISC, which is an academic specialized center affiliated with Mansoura University-Egypt. Method Clinical data were collected from the patients' files, where the subjects were classified into two major groups according to the therapeutic intervention. Group 1 included 24 patients divided into 4 subgroups. The first was treated by a Cephalosporin with a Fluoroquinolone (1A), The second was treated by a Carbapenem with a Fluoroquinolone (1B), The third was treated by a B-lactam with an Aminoglycoside (1C) and the fourth was treated by Carbapenem with a Glycylcycline (1D). Group 2 included 6 patients, treated with Tigecycline and Ampicillin-Sulbactam. Main outcome measure Primary outcomes are the A. baumannii microbiological culture negativity after 14 days of therapy and the 30 days' survival after the antibiotic course, while the secondary outcomes are the expected therapies' side effects. Results Group 2 is associated with significant higher primary outcomes without a significant difference regarding the secondary outcomes. Conclusion The combination of Tigecycline and Ampicillin-Sulbactam, appears to be a clinically effective therapy against XDR A. baumannii, despite each agent being resistant alone, without alerting adverse effects.

Overtreatment and undertreatment of hyperlipidemia in the outpatient setting.

BACKGROUND: National guidelines recommend lipid-lowering therapy for patients with coronary heart disease (CHD), its equivalent (eg, diabetes mellitus, peripheral arterial disease, cerebrovascular disease), and those at high risk of CHD. Quality-of-care studies demonstrate that patients at high risk of CHD are undertreated. Overtreatment of patients at low risk of CHD remains relatively unexplored. Our study aimed to determine patient characteristics associated with under- and overtreatment of hyperlipidemia. METHODS: We conducted a retrospective chart review of patients aged 35 to 80 years attending an inner-city ambulatory teaching clinic. We noted patients' 10-year cardiovascular risk based on the Framingham Heart Study equation, other patient demographics, pretreatment lipid levels, and whether they received a prescription for lipid-lowering therapy. RESULTS: Of 676 patients included, 46% were at high (>15% for 10 years) and 37% were at low (<5%) risk. Of the patients at high risk for CHD, 34% received no drug therapy, including 5% of patients with known CHD; 37% of patients with diabetes mellitus; and 59% of patients without CHD equivalents. Undertreatment was associated with lower low-density lipoprotein (LDL; odds ratio [OR] per 30 mg/dL 2.7, confidence interval [CI] 2.0-3.6), fewer risk factors (OR per risk factor 1.5 CI 1.1-2.1), and not receiving other preventive care interventions (OR 2.1, CI 1.0-4.5). Of 247 patients at low risk for CHD, 8% received drug therapy. Overtreatment was associated with higher LDL (OR per 30 mg/dL 3.0, CI 1.7-5.3) and more cardiac risk factors (OR per risk factor 3.1, CI 1.4-6.7). Age, race, sex, and specific risk factors were not associated with overtreatment or undertreatment. CONCLUSIONS: Both overtreatment and under-treatment are common. Physicians' decisions appear to reflect LDL values and number of risk factors rather than calculated cardiovascular risk.