High- and low-dose cyclophosphamide in Egyptian lupus nephritis patients: a multicenter retrospective analysis.

BACKGROUND: Lupus nephritis (LN) is a common serious presentation of systemic lupus erythematosus. Cyclophosphamide (CYC) and mycophenolate mofetil (MMF) are listed as the first-line drugs in induction therapy for LN. OBJECTIVE: This study aimed to compare high- and low-dose CYC in a cohort of Egyptian LN patients. PATIENTS AND METHODS: The data of 547 patients with class III/IV active LN who received CYC as induction therapy were retrospectively analyzed. Whereas 399 patients received 6­monthly 0.5-1 g/m2 CYC doses, 148 patients received six biweekly 500 mg CYC doses. Demographic data, laboratory test results, and disease activity index were recorded and compared at presentation and at 6, 12, 18, 24, and 48 months of follow-up. RESULTS: After 48 months, the proportion of patients maintaining normal creatinine levels was higher in the group receiving induction therapy with high-dose CYC (67.9%, 60.4%, p = 0.029), and these patients also had higher proteinuria remission at 36 (26.6%, 14.8%, p = 0.014) and 48 months (24.3%, 12.8%, p = 0.006). Comparison of patient outcomes according to both induction and maintenance therapy showed the best results in patients who received high-dose CYC and continued MMF as maintenance therapy. CONCLUSION: High- and low-dose CYC are comparable in early phases of treatment. However, after a longer duration of follow-up, high-dose CYC was associated with higher remission rates in the current cohort.

Development and initial validation of a composite disease activity score for systemic juvenile idiopathic arthritis.

OBJECTIVE: To develop a composite disease activity score for systemic JIA (sJIA) and to provide preliminary evidence of its validity. METHODS: The systemic Juvenile Arthritis Disease Activity Score (sJADAS) was constructed by adding to the four items of the original JADAS a fifth item that aimed to quantify the activity of systemic features. Validation analyses were conducted on patients with definite or probable/possible sJIA enrolled at first visit or at the time of a flare, who had active systemic manifestations, which should include fever. Patients were reassessed 2 weeks to 3 months after baseline. Three versions were examined, including ESR, CRP or no acute-phase reactant. RESULTS: A total of 163 patients were included at 30 centres in 10 countries. The sJADAS was found to be feasible and to possess face and content validity, good construct validity, satisfactory internal consistency (Cronbach's alpha 0.64-0.65), fair ability to discriminate between patients with different disease activity states and between those whose parents were satisfied or not satisfied with illness outcome (P < 0.0001 for both), and strong responsiveness to change over time (standardized response mean 2.04-2.58). Overall, these properties were found to be better than those of the original JADAS and of DAS for RA and of Puchot score for adult-onset Still's disease. CONCLUSION: The sJADAS showed good measurement properties and is therefore a valid instrument for the assessment of disease activity in children with sJIA. The performance of the new tool should be further examined in other patient cohorts that are evaluated prospectively.

Juvenile lupus and serum vitamin D levels: A cross-sectional study.

BACKGROUND: Juvenile systemic lupus erythematosus (JSLE) is usually associated with vitamin D deficiency and low bone mineral density. OBJECTIVES: To evaluate serum levels of 25-OH vitamin D in JSLE patients and to correlate these findings with disease activity and bone density. METHODS: This study was conducted on 100 patients with JSLE and 100 healthy children as controls. Disease duration and SLEDAI for disease activity were evaluated. CBC, anti-dsDNA, C3,C4,24hr urinary proteins, creatinine, estimated glomerular filtration rate(e-GFR),Ca,P,PTH, 25 (OH) D levels, and bone mineral density(BMD)Z score were measured. RESULTS: There were significant differences in mean 25(OH)D concentration between patients group (19.37 ± 9.72 ng/ml) and controls 35.90 ± 9.66 ng/ml(p < 0.05), with significant difference between active and inactive patients (p < 0.05).There were significant negative correlations between serum 25(OH)D and SLEDAI (r-0.545, p 0.001), steroid dose (r-0.561, p 0.001), anti-dsDNA (r-0.685, p 0.006), 24 hr-proteinuria (r-0.738, p 0.001) and PTH (r-0.335, p 0.001), significant positive correlations between 25(OH)D and C3 (r0.617, p 0.001),C4 (r0.544, p 0.001) serum Ca (r0.424, p 0.001) and Z score (r0.561, p 0.001),with non-significant correlations between 25(OH)D and serum P and both disease & steroid duration, (p > 0.05). CONCLUSION: Vitamin D deficiency is common in JSLE, it's correlated significantly with disease activity and bone mineral density.

Evaluating the Effect of Delayed Diagnosis on Disease Outcome in Fibromyalgia: A Multi-Center Cross-Sectional Study.

Background: The impact of delayed diagnosis (DD) on fibromyalgia (FM) patients' symptomatology and disease outcome has not yet been systematically studied. Objective: To analyze the predictors of DD and the influence of DD on FM aggravation and disease evaluation measures. Methods: 370 FM patients were interviewed prospectively for this study. The following information was obtained: DD, widespread pain index (WPI), symptom severity scale (SSS), polysymptomatic distress scale (PDS) scale (SSS + WPI), and tender points. We identified three groups of patients: early diagnosis (ED: 2 years; 83 patients), late diagnosis (LD: > 2-7 years; 198 patients), and very late diagnosis (VLD: > 7 years; 89 patients). Results: The patients' average age was 33.9 (9.8) years, and 79% were female. The SSS, PDS, and tender point means were 7.8 (1.6), 16.46 (4.1), and 14.31 (2.3), respectively. The correlation between DD and SSS (r = 0.14), the PDS scale (r = 0.37), and FM tender points (r = 0.16) was significant, but not with WPI (r = 0.06). When the three groups were examined, the SSS mean was 7.54 (1.6), 7.73 (1.4), and 8.25 (1.7), respectively (P 0.008), while the PDS mean was 15 (3.8), 15.95 (3.8), and 18.96 (4.4), respectively (P 0.008). (P 0.001). Conclusion: Early FM diagnosis is associated with lower SSS, total severity scale, and FM tender points, indicating a less severe condition.

Towards a consensus on the clinical applications and interpretations of the nailfold capillaroscopy standards in clinical practice: An initiative by the Egyptian Society of Microcirculation.

Objectives: Based on the mainstream adoption of nailfold capillaroscopy as an investigative tool for rheumatologists, this work was carried out by a panel of experts in the field of capillaroscopy and microcirculation to issue a consensus view on capillaroscopic image acquisition and analysis standardization. Patients and methods: After the key clinical questions were identified by the core team, a systematic review of the published research was carried out focusing on variable capillaroscopic techniques, definitions, and characteristics, including capillary density (number of capillaries), capillary morphology (shape of each capillary), capillary dimensions (width of apical, arterial, and venous limb of the capillary), and the presence of hemorrhages. The expert panel attained a consensus and developed recommendations for the standardization of capillaroscopy in clinical practice. These included recommendations for normality and abnormality and the different capillaroscopic patterns. It also involved recommendations for scoring systems, reliability, and reporting. Results: A panel of 11 experts participated in the two rounds with a response rate of 100%. A total of nine recommendations were obtained. The agreement with the recommendations (a score of 7-9) ranged from 81.8 to 90.9%. A consensus (i.e., ≥75% of respondents strongly agreed or agreed) was reached on all the clinical standards. Conclusion: This work highlighted the main NFC indications, the technical equipment that should be used, how to carry out the procedure, standardization of the terminology of the parameters, and the interpretation of NFC findings. An evidence-based consensus incorporating the advice and experience of a diverse international expert panel was reached.

Targeted optimum care approach for osteoporotic fragility fractures: tailored strategy based on risk stratification to reduce incidents of falls-an initiative by the Egyptian Academy of bone health based on the FLS national register.

Since falling is the third cause of chronic disability, a better understanding of the frequency, severity, and risk factors of falls across diagnostic groups is needed to design and implement customized, effective fall prevention, and management programs for these individuals, particularly those at risk of sustaining a fragility fracture. OBJECTIVE: (1) To assess the incidence of falls among osteoporotic patients with fragility fractures. (2) To evaluate the potential for stratifying the people at risk of falling in bone health setting aiming to provide targeted optimum care for them. METHODS: This was a multi-center, cross-sectional, observational study. Both men and postmenopausal women, admitted with an osteoporotic fracture (whether major osteoporosis or hip fracture), were consecutively recruited for this work and managed under Fracture Liaison Service. All the patients were assessed for their Fracture risk (FRAX), falls risk (FRAS), and sarcopenia risk (SARC-F) as well as functional disability (HAQ). Blood tests for bone profile as well as DXA scan were offered to all the patients. RESULTS: Four hundred five patients (121 males, 284 females) were included in this work. Mean age was 70.1 (SD = 9.2) years. The incidence of falls was 64.9%. The prevalence of falls was high (64.8%) in the patients presenting with major osteoporosis fractures and in those with hip fractures (61.8%). The prevalence of fragility fractures was positively correlated with HAQ score and the SARC-F score (p = 0.01 and 0.021 respectively). Falls risk score was positively correlated with FRAX score of major osteoporotic fractures, HAQ score, and SARC-F score (p = 0.01, 0.013, and 0.003 respectively). Seventy percent of the osteopenia patients who sustained fragility fracture had high falls risk and/or SARC-F score. CONCLUSION: This study highlighted the importance of falls risk stratification in osteoporotic patients presenting with fragility fractures. Identification of the patients at increased risk of falls should be a component of the standard practice.

Incidence and geographic characteristics of the population with osteoporotic hip fracture in Egypt- by the Egyptian Academy of Bone Health.

This work studies the epidemiology of hip fracture in Egypt. While the incidence of hip fracture in Egypt matches that of the Mediterranean region, there was geographic variation in osteoporotic hip fracture incidence between the north and south of Egypt. PURPOSE: To assess the incidence of hip fracture in Egypt, with special emphasis on the geographic and demographic variation among the Egyptian population. METHODS: The incidence of hip fractures treated in two Egyptian FLS centers was calculated for the period of February 2022-February 2023. Demographic information was recorded for every patient on the national register. All patients completed a baseline questionnaire, had clinical evaluation, fracture risk, falls, and sarcopenia risk assessment. A DXA scan was carried out for every patient. RESULTS: The annual incidence of low-energy hip fracture in individuals aged 40 years or more in Egypt in 2022-2023 was 123.34 per 100,000 in women and 55.19 per 100,000 in men. The incidence of hip fractures was higher in south Egypt (113.62) versus north Egypt (64.8). This was consistent for both genders. Bone mineral density was significantly (p < 0.01) lower in south Egypt at both the spine, distal forearm, as well as hip trochanters, whereas there was no significant difference between both locations in terms of the total hip and neck of the femur. Yet, falls risk, sarcopenia, as well as functional disability rates were significantly (p < 0.001) higher in the north. CONCLUSION: In Egypt, the hip fracture incidence was higher in the south compared to the north. Several modifiable factors contribute to fragility fracture risk independent of BMD, creating complex interrelationships between BMD, risk factors, and fracture risk.

Serum interleukin-23 levels: relation to depression, anxiety, and disease activity in psoriatic arthritis patients.

OBJECTIVES: Assessment of serum levels of IL-23 in PsA patients and its correlation with depression, anxiety, and disease activity. METHODS: Eighty psoriatic arthritis (PsA) patients and eighty healthy volunteers matched for age and gender were included in this observational case-control study. All participants suspected to detailed history, clinical assessment, PsA activity using Disease Activity Index for Psoriatic Arthritis (DAPSA) score, the severity and extent of psoriasis was assessed by the Psoriasis Area and Severity Index (PASI), and ultrasonographic assessments of the entheses were examined according to the Madrid Sonographic Enthesitis Index (MASEI). Depression and anxiety were assessed by Hospital Anxiety and Depression Scale (HADS). Serum IL-23 was measured and correlated with disease activity, depression, and anxiety. RESULTS: There was no significant difference between patients and controls regarding demographic data. Thirty-six PsA patients (45%) had anxiety and 28 patients (35%) had depression, while in the control group, 16 persons (20%) had anxiety and 12 (15%) had depression, with significant differences between the 2 groups (p < 0.0001). There were significant differences in HADS anxiety and depression scores between patients and controls with significant positive correlations between HADS depression, anxiety scores and IL-23, DAPSA, PASI, and MASEI scores (p < 0.05). IL-23 was positively correlated with DAPSA, PASI, and HADS scores; we observed that interleukin 23, higher DAPSA, and PASI were independently associated with depression and anxiety. CONCLUSION: Serum interleukin-23 levels were elevated in PsA patients and were found to be correlated with depression, anxiety, and disease activity. Key Points • Psoriatic arthritis is a multidimensional disorder with psychiatric drawbacks. • Interleukin-23 is a proinflammatory cytokines that was correlated with depression and anxiety in PsA patients. • Interleukin-23 was correlated with disease activity in PsA. • Depression and anxiety were positively correlated with disease activity in PsA.

Consensus Evidence-Based Clinical Practice Recommendations for the Diagnosis and Treat-To-Target Management of Osteoporosis in Chronic Kidney Disease Stages G4-G5D and Post-transplantation: An Initiative of Egyptian Academy of Bone Health.

Objective: The aim of this study was to reach a consensus on an updated version of the recommendations for the diagnosis and Treat-to-Target management of osteoporosis that is effective and safe for individuals with chronic kidney disease (CKD) G4-G5D/kidney transplant. Methods: Delphi process was implemented (3 rounds) to establish a consensus on 10 clinical domains: (1) study targets, (2) risk factors, (3) diagnosis, (4) case stratification, (5) treatment targets, (6) investigations, (7) medical management, (8) monitoring, (9) management of special groups, (10) fracture liaison service. After each round, statements were retired, modified, or added in view of the experts' suggestions, and the percent agreement was calculated. Statements receiving rates of 7-9 by more than 75% of experts' votes were considered as achieving consensus. Results: The surveys were sent to an expert panel (n = 26), of whom 23 participated in the three rounds (2 were international experts and 21 were national). Most of the participants were rheumatologists (87%), followed by nephrologists (8.7%), and geriatric physicians (4.3%). Eighteen recommendations, categorized into 10 domains, were obtained. Agreement with the recommendations (rank 7-9) ranged from 80 to 100%. Consensus was reached on the wording of all 10 clinical domains identified by the scientific committee. An algorithm for the management of osteoporosis in CKD has been suggested. Conclusion: A panel of international and national experts established a consensus regarding the management of osteoporosis in CKD patients. The developed recommendations provide a comprehensive approach to assessing and managing osteoporosis for all healthcare professionals involved in its management.

Consensus evidence-based clinical practice guidelines for the diagnosis and treat-to-target management of osteoporosis in Africa: an initiative by the African Society of Bone Health and Metabolic Bone Diseases.

The objective of this consensus statement is to inform the clinical practice communities, research centres and policymakers across Africa of the results of the recommendations for osteoporosis prevention, diagnosis and management. The developed guideline provides state-of-the-art information and presents the conclusions and recommendations of the consensus panel regarding these issues. PURPOSE: To reach an African expert consensus on a treat-to-target strategy, based on current evidence for best practice, for the management of osteoporosis and prevention of fractures. METHOD: A 3-round Delphi process was conducted with 17 osteoporosis experts from different African countries. All rounds were conducted online. In round 1, experts reviewed a list of 21 key clinical questions. In rounds 2 and 3, they rated the statements stratified under each domain for its fit (on a scale of 1-9). After each round, statements were retired, modified or added in view of the experts' suggestions and the percent agreement was calculated. Statements receiving rates of 7-9 by more than 75% of experts' votes were considered as achieving consensus. RESULTS: The developed guidelines adopted a fracture risk-centric approach. Results of round 1 revealed that of the 21 proposed domains, 10 were accepted whereas 11 were amended. In round 2, 32 statements were presented: 2 statements were retired for similarity, 9 statements reached consensus, whereas modifications were suggested for 21 statements. After the 3rd round of rating, the experts came to consensus on the 32 statements. Frequency of high-rate recommendation ranged from 83.33 to 100%. The response rate of the experts was 100%. An algorithm for the osteoporosis management osteoporosis was suggested. CONCLUSION: This study is an important step in setting up a standardised osteoporosis service across the continent. Building a single model that can be applied in standard practice across Africa will enable the clinicians to face the key challenges of managing osteoporosis; furthermore, it highlights the unmet needs for the policymakers responsible for providing bone health care together with and positive outcomes of patients' care.

Consensus evidence-based recommendations for treat-to-target management of immunoglobulin A vasculitis.

IgA vasculitis (IgAV), formerly known as Henoch-Schönlein purpura, is the most common cause of systemic vasculitis in childhood. Given its potential life-threatening systemic complications, early and accurate diagnosis as well as management of IgAV represent a major challenge for health care professionals. This study was carried out to attain an evidence-based expert consensus on a treat-to-target management approach for IgAV using Delphi technique. The preliminary scientific committee identified a total of 16 key clinical questions according to the patient, intervention, comparison, and outcomes (PICO) approach. An evidence-based, systematic, literature review was conducted to compile evidence for the IgAV management. The core leadership team identified researchers and clinicians with expertise in IgAV management in Egypt upon which experts were gathered from different governorates and health centers across Egypt. Delphi process was implemented (two rounds) to reach a consensus. An online questionnaire was sent to expert panel (n = 26) who participated in the two rounds. After completing round 2, a total of 20 recommendation items, categorized into two sections were obtained. Agreement with the recommendations (rank 7-9) ranged from 91.7-100%. Consensus was reached (i.e. ⩾75% of respondents strongly agreed or agreed) on the wording of all the 20 clinical standards identified by the scientific committee. Algorithms for the diagnosis and management have been suggested. This was an expert, consensus recommendations for the diagnosis and treatment of IgAV and IgA vasculitic nephritis, based on best available evidence and expert opinion. The guideline presented a strategy of care with a pathway to achieve a state of remission as early as possible. PLAIN LANGUAGE SUMMARY: Given its potential life-threatening systemic complications, early and accurate diagnosis of immunoglobulin A vasculitis represents a major challenge for health care professionals. This work provided cornerstone principles for the management of the condition. Adopting PICO approach and implementing Delphi process a consensus was reached on evidence-based treat-to-target treatment recommendations. This will endorse enhancement and consistency of care of this cohort of patients in standard practice.

Correction to: In shoulder adhesive capsulitis, ultrasound-guided anterior hydrodilatation in rotator interval is more effective than posterior approach: a randomized controlled study.

The authors of the original version of the above article requested to add the name of the instrument that was used in the Intervention section.

In shoulder adhesive capsulitis, ultrasound-guided anterior hydrodilatation in rotator interval is more effective than posterior approach: a randomized controlled study.

Shoulder adhesive capsulitis, also called frozen shoulder, is a musculoskeletal disorder associated with pain and functional disability. This study aimed to compare the effectiveness of shoulder ultrasound-guided hydrodilatation with corticosteroid, via rotator interval (RI) anteriorly, versus posterior approach, in adhesive capsulitis patients. All patients received exercise program following injection. PATIENTS AND METHODS: A prospective randomized controlled study among 60 consecutive adhesive capsulitis patients was randomized into two equal groups. Group I received ultrasound-guided hydrodilatation with corticosteroid, saline, and local anesthetic via posterior intra-articular approach; group II received the same ultrasound-guided hydrodilatation via anterior rotator interval approach. Both groups received guided stretching exercises for 3 months after injection. Baseline and 3 months evaluation of pain by visual analogue scale (VAS), shoulder pain and disability index (SPADI), and range of motion (ROM) had been recorded for all patients. RESULTS: Both groups showed significant improvement 3 months after hydrodilatation regarding VAS pain, external rotation, and SPADI. Only in group II (RI anterior approach) improvement was observed regarding flexion and abduction. There was no improvement regarding extension or internal rotation in either group. When comparing the improvement in both groups after hydrodilatation, group II (anterior approach) showed a statistically significant higher level of improvement regarding VAS pain (p = 0.003), SPADI, flexion, abduction, and external rotation, compared to group I (p < 0.001). Extension, internal rotation, and adduction were not different. CONCLUSIONS: Ultrasound-guided anterior rotator interval hydrodilatation for adhesive capsulitis, followed by guided exercise, is clinically and functionally more effective than the conventional posterior approach.