A juvenile dermatomyositis: demographics, characteristics and disease outcome in an Egyptian cohort.

OBJECTIVES: To study the demographics, characteristics, management and disease outcome of Egyptian children with juvenile dermatomyositis (JDM). METHODS: Retrospective analysis of the records of 134 JDM patients attending two centres in Cairo, Egypt from January 2010 to December 2019. A total of 128 patients were included in the study, all of which fulfilled either the Bohan and Peter criteria and/or the EULAR/ACR classification criteria of 2017. RESULTS: The mean age of disease onset was 5.9±2.8 years and the follow-up duration were 6±3.2 years. Female to male ratio was 2.2:1. Constitutional manifestations and cutaneous skin ulcers were common, while gut vasculopathy was rare in our patients. Heliotrope rash was the commonest skin manifestation. Lactate dehydrogenase enzyme was more frequently elevated than creatine kinase. Electromyography was the most frequently used diagnostic procedure, while muscle biopsy and muscle MRI were not commonly done in our patients. Glucocorticoids, methotrexate, hydroxychloroquine, mycophenolate mofetil and IVIG were the most frequently used medications. Sixty (46.9 %) of the patients had clinically inactive disease, at the last follow-up visit. Chronic skin disease, residual muscle weakness, calcinosis and growth failure were among the most common cumulative damage manifestations. The mortality rate was 1.6% over the follow-up period, one death was due to severe infection, and the other due to respiratory failure. CONCLUSIONS: Although our patients shared several similarities with their peers in the Middle East and in Europe, there were some striking differences. These differences can be attributed to the ethnic and environmental disparities.

Psoriatic arthritis among Egyptian patients with psoriasis attending the dermatology clinic: prevalence, comorbidities, and clinical predictors.

OBJECTIVES: Early diagnosis and treatment of psoriatic arthritis (PsA) help to prevent progressive joint involvement and disabilities. There is a problem in the early diagnosis of PsA worldwide, which may be attributed to the dermatologists missing PsA symptoms and signs and a lack of effective screening tools. AIM OF THE STUDY: The current study was designed to assess the prevalence, comorbidities, and clinical predictors associated with the development of PsA in psoriasis patients. MATERIAL AND METHODS: A cross-sectional observational study was performed. Screening questionnaires - the Psoriasis Epidemiology Screening Tool (PEST) and Early Arthritis for Psoriatic Patients (EARP) - were applied to 200 psoriasis patients; among them n = 22 (11% of all tested patients) were in developmental age. Those with positive questionnaires were classified as having PsA or not according to the classification for psoriatic arthritis criteria. Body surface area, psoriasis area and severity index, and psoriasis disability index tools were used for assessing psoriasis patients. A full rheumatological and dermatological evaluation were carried out for PsA patients. RESULTS: The prevalence of PsA was found to be 30%, with a mean age of 45.48 ±10.79 years. Further, psoriasis preceded the onset of PsA in 46 patients (76.6%), arthritis began before psoriasis in 6 individuals (10%), and both psoriasis and arthritis coincided in 8 (13.3%) patients. Obesity (OR 7.0, 95% CI: 2.61-18.85), nail psoriasis (OR 5.02, 95% CI: 2.02-12.476), and intergluteal cleft site (OR 12.659, 95% CI: 4.302-37.255) were associated with increased risk of PsA. However, classic plaque psoriasis (OR 0.149, 95% CI: 0.051-0.433) and flexure site (OR 0.238, 95% CI: 0.076-0.746) were linked with a decreased risk of PsA development. CONCLUSIONS: Screening for PsA in patients with psoriasis revealed a significant number of undiagnosed cases of PsA that should be treated early. Obesity, nail psoriasis, and psoriasis at the intergluteal sites can help predict the PsA development.

A comparative study between the disease characteristics in adult-onset and childhood-onset systemic lupus erythematosus in Egyptian patients attending a large university hospital.

INTRODUCTION: Disease features and laboratory abnormalities differ among adult-onset and childhood-onset systemic lupus erythematosus (aSLE and cSLE). Socioeconomic status both independent of, and in combination with, ethnicity influences the disease phenotype and outcome. OBJECTIVE: To compare the various disease features among patients with cSLE and aSLE in a limited monetary income Egyptian cohort attending a large free-of-charge university hospital. Patients and methods: Retrospective analysis of the medical records of 714 SLE patients attending Cairo University Hospitals from January 2000 to December 2019. Of them 602 (400 with aSLE and 202 with cSLE) were enrolled in the study. RESULTS: The mean age of disease onset was 28.27 ± 10.55 among aSLE patients compared to 12.88 ± 4.26 years among cSLE patients. Disease duration was 12.03 ± 5.05 and 4.14 ± 3.18 years in aSLE and cSLE, respectively. Female to male ratio was 15:1 among patients with aSLE, as compared to 2.67:1 among cSLE (<0.001). Arthritis (69%), oral ulcers (48.5%), neuropsychiatric (18.3%) and thrombotic manifestations of antiphospholipid syndrome (12%) were significantly more frequent in aSLE. On the other hand, renal (67.8%), serositis (49.6%), fever (49%), lymphopenia (40.6%), hemolytic anemia (38.6%), and discoid lupus (13.4%) were significantly more frequent in cSLE. Weight loss, malar rash, photosensitivity, thrombocytopenia, leucopenia and lymphadenopathy were not significantly different between the two groups. Hypocomplementemia, proteinuria, urinary sediments, hematuria were significantly more frequent in cSLE. For those patients with renal involvement, who underwent renal biopsy (58.3% in aSLE and 63.5% in cSLE), there was no significant difference with regard to the different histopathological classes. Anti-Smith, anti-cardiolipin antibodies and rheumatoid factor were significantly more frequent among aSLE patients, while anti-La antibodies were more frequent among cSLE patients. CONCLUSION: Arthritis was the most common clinical manifestation over time in aSLE compared to renal involvement in cSLE. Renal disease tends to be more active in cSLE. The differences in disease manifestations between this cohort and other studies can be attributed to the ethnic and socioeconomic disparities.

A Review of the Prevalence and Unmet Needs in the Management of Rheumatoid Arthritis in Africa and the Middle East.

Estimates of the global prevalence of rheumatoid arthritis (RA) range from 0.24 to 1%, but vary considerably around the globe. A variation in RA prevalence is also expected across Africa and the Middle East, due to ethnic, climate, and socioeconomic differences. To assess the prevalence of RA in Africa and the Middle East, we searched Medline (via PubMed) and databases of major rheumatology conferences. Seventeen journal articles and 0 abstracts met the inclusion criteria. Estimated prevalence ranged from 0.06 to 3.4%. Most studies reported values near or below 0.25%. Consistent with data from other regions, RA was more prevalent among urban than rural populations, and among women than men. The women:men prevalence ratio ranged from 1.3:1 to 12.5:1, which suggests notable differences from the global average of 2:1. Relative increases in prevalence were observed in North Africa and the Middle East (13% since 1990) and Western Sub-Saharan Africa (14%), whereas rates in Eastern, Central, and Southern Sub-Saharan Africa show decreases (4-12%). Low disease awareness, delays to visit rheumatologists, and socioeconomic factors appear to hinder early diagnosis and aggressive treatment. Few countries have developed RA-specific treatment guidelines, and many physicians and patients face limited access to even basic treatments. An improved understanding of the epidemiology and management of RA, and the related socioeconomic consequences is necessary, so that targeted attempts can be made to encourage early diagnosis and treatment.

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.

Challenges and opportunities in the early diagnosis and optimal management of rheumatoid arthritis in Africa and the Middle East.

Early diagnosis and early initiation of disease-modifying antirheumatic drug (DMARD) therapy slow the progression of joint damage and decrease the morbidity and mortality associated with rheumatoid arthritis (RA). According to the European League Against Rheumatism (EULAR) guidelines, treatment should be initiated with methotrexate and addition of biological DMARDs such as tumour necrosis factor (TNF) inhibitors should be considered for RA patients who respond insufficiently to methotrexate and/or other synthetic DMARDs and have poor prognostic factors. Africa and the Middle East is a large geographical region with varying treatment practices and standards of care in RA. Existing data show that patients with RA in the region are often diagnosed late, present with active disease and often do not receive DMARDs early in the course of the disease. In this review, we discuss the value of early diagnosis and remission-targeted treatment for limiting joint damage and improving disease outcomes in RA, and the challenges in adopting these strategies in Africa and the Middle East. In addition, we propose an action plan to improve the overall long-term outlook for RA patients in the region.

Suboptimal management of rheumatoid arthritis in the Middle East and Africa: could the EULAR recommendations be the start of a solution?

Although the prevalence of RA in the Middle East and Africa is comparable with that in other parts of the world, evidence indicates that its management in this region is suboptimal for a variety of reasons, including misconceptions and misunderstandings about the disease's prevalence and severity in the region, compounded by the lack of local epidemiological and health-economic data around the disease; the perception that RA is a low priority compared with other more prevalent conditions; delayed diagnosis, referral and treatment; and a lack of a region-specific, evidence-based management approach. In the absence of such an approach, the EULAR treatment recommendations may provide a useful starting point for the creation of guidelines to suit local circumstances. However, although agreement with the EULAR recommendations is high, many barriers prevent their implementation in clinical practise, including lack of timely referral to rheumatologists; suboptimal use of synthetic DMARDs; poor access to biologics; lack of awareness of the burden of RA among healthcare professionals, patients and payers; and lack of appropriate staffing levels.To optimise the management of RA in the Middle East and Africa, will require a multi-pronged approach from a diverse group of stakeholders-including local, national and regional societies, such as the African League of Associations in Rheumatology and International League of Associations for Rheumatology, and service providers-to collect data on the epidemiology and burden of the disease; to increase awareness of RA and its burden among healthcare professionals, payers and patients through various educational programmes; to encourage early referral and optimise use of DMARDs by promoting the EULAR treatment recommendations; to encourage the development of locally applicable guidelines based on the EULAR treatment recommendations; and to facilitate access to drugs and the healthcare professionals who can prescribe and monitor them.

The practical value of biologics registries in Africa and Middle East: challenges and opportunities.

Biologics, including tumor necrosis factor (TNF) inhibitors, are increasingly used for the treatment of inflammatory conditions such as rheumatoid arthritis (RA), psoriatic arthritis, and ankylosing spondylitis. The efficacy of these drugs has been demonstrated in randomized controlled trials (RCTs). However, these studies are conducted in controlled environments, and the results may not necessarily reflect clinical outcomes in daily clinical practice. In Europe and other western countries, numerous biologics registries that enroll and monitor patients receiving biologics have been established. These registries follow patients irrespective of whether they continue with the initial biologic drug. Thus, real-life efficacy data from these registries can be used to assess the long-term safety of biologics through longitudinal studies. In Africa and Middle East (AFME), such registries currently exist only in Morocco and South Africa. In light of the increasing availability of biologics and scarcity of long-term safety data of these agents in the AFME population, there is a need to establish biologics registries in other countries across the region. This review discusses the value of biologics registries versus RCTs as well as safety and efficacy data from observational studies presented as lessons from well-established biologics registries. In addition, the rationale for establishing such registries in the AFME region is also presented.

Prevalence and clinical presentations of hepatitis C virus among patients admitted to the rheumatology ward.

To study the prevalence of anti-HCV antibodies among patients admitted to the rheumatology department, Cairo University hospitals, in 6-month period as well as to determine whether chronic HCV infection was the primary cause of their admission or just a concomitant association with the rheumatic disease. One hundred and fifty-seven patients were included in this study. They represent all patients admitted to the rheumatology inpatient department of Cairo University hospitals during the study period. Preset questionnaire including detailed demographic data, cause of admission and clinical manifestations of their disease was obtained for every patient. All patients were screened for HCV antibodies using ELISA technique. Other laboratory and imaging investigations were done according to the patient's diagnosis. Twenty-nine patients (18.5%) were positive for HCV antibody. Eleven patients of them (38%) were admitted due to rheumatic manifestations directly related to chronic HCV infection, which represent 7% of all admitted patients (11/157). HCV antibodies were found in 17.6 and 6.7% among patients with rheumatoid and systemic lupus erythematosus. Arthritis, palpaple purpura, digital gangrene and mononeuritis multiplex were the most common causes of admission related to chronic HCV infection. HCV antibodies were found in 18.5% among admitted patients to the rheumatology ward. The rheumatic manifestations of chronic HCV represent the primary cause of admission in 7% of all admitted patients. HCV screening should be included in the routine investigations for patients presenting to rheumatology departments in countries with high prevalence of chronic HCV infection.

MEFV mutations in Egyptian patients suffering from familial Mediterranean fever: analysis of 12 gene mutations.

The objective of the study is to screen 12 MEFV gene mutations in Egyptian patients with familial Mediterranean fever (FMF) and to study the initial hypothesis that the phenotypic expression of the disease may be attributable to the existence of a particular mutation. We enrolled 136 Egyptian patients (74 males, and 62 females) with a clinical diagnosis of FMF. DNA was amplified by PCR and subjected to reverse hybridization for the detection of 12 MEFV gene mutations. The phenotypic expression of the disease was compared in two subgroups according to the presence of homozygote E148Q and M694V gene mutations. The most frequent gene mutations in the studied group were V726A, M694V, M680I, E148Q and M694I in 41.2, 32.4, 29.4, 25 and 20.6%, respectively. At least one of these main five founder mutations was present in 132 patients (97.1%). Thirty-two patients (23.5%) were homozygote for one of the main five founder mutations. The most common homozygote gene mutations were E148Q and M694V, each in 12 patients (8.8%). Significant increase in abdominal pain and arthritis was found in patients with homozygote M694V mutation compared to those with E148Q mutation. All patients with amyloidosis had M694V gene mutation. The increased frequency of V726A gene mutation and the rarity of amyloidosis in this study suggest that Egyptian patients may have a milder form of FMF compared to other populations. The five main founder mutations account for the vast majority of cases of FMF. M694V gene mutation may be associated with increased frequency of abdominal pain, arthritis and the presence of amyloidosis.

Human leukocyte antigen and autoantibodies association with juvenile systemic lupus erythematosus.

In this study we investigated the association between autoantibodies production and MHC class II alleles in fifty three Egyptian children patients with systemic lupus erythematosus (SLE). A significant association was found between expression of HLA-DR4 and HLA-DR13 genes and the generation of anti-ribonucleoprotein and IgG cardiolipin antibodies respectively, in contrast to the negative association of antinuclear antibodies (ANA) with HLA-DR8 and HLA-DR14. Analysis of HLA-DR alleles and autoantibodies frequencies in relation to different clinical manifestations revealed significant association between HLA-DR13 and vasculitis, while, HLA-DR1 and HLA-DR3 were significantly associated with seizures. In contrast, HLA-DR8, HLA-DR4 and HLA-DR52 alleles were associated with significant protection from arthritis, abnormal kidney function and neuropsychiatric disorders, respectively. SLE autoantibodies, namely anti-DNA antibodies were significantly associated with disturbed kidney function tests and the occurrence of seizures. In contrast, nucleosome antibodies showed no association with renal involvement in childhood onset systemic lupus erythematosus.

A cross-cultural study of pain intensity in Egyptian and Dutch women with rheumatoid arthritis.

UNLABELLED: It has been suggested that patients from Mediterranean cultures tend to report more intense pain than their Northern or Western European counterparts in comparable medical conditions. However, empirical data to support this hypothesis are limited. The goals of the present study were to examine differences in pain intensity reports between Dutch and Egyptian women with rheumatoid arthritis (RA) and to examine the influence of possible confounding variables using multivariate analyses. We performed a cross-sectional study in 30 Dutch and 42 Egyptian women with comparable RA, matched for age and disease duration. Pain intensity was measured on a 100-mm graphic rating scale. Additionally, we assessed physical function, radiographic joint damage, progression of RA, disease activity, number of swollen and tender joints, medication, rheumatoid factor, and socioeconomic variables. The progression of RA and radiographic damage were not significantly different between Egyptian and Dutch patients. However, the Egyptian population reported significantly worse pain and physical function and demonstrated higher disease activity. Multiple linear regression analysis showed that the country of residence and the number of tender and swollen joints were significant independent determinants of pain reports. The results provide some support for the idea that there are ethnocultural differences in pain reports between Egyptian and Dutch women with RA, although the mechanisms underlying these differences remain unclear. PERSPECTIVE: This article shows that after controlling for differences in demographic, socioeconomic, and clinical variables, Egyptian women with RA reported more pain than Dutch women with RA. Clinicians and investigators should recognize that cultural or ethnic factors may play an important role in patients' pain reports.

The validity and reliability of the graphic rating scale and verbal rating scale for measuring pain across cultures: a study in Egyptian and Dutch women with rheumatoid arthritis.

OBJECTIVE: To compare the validity and reliability of a graphic rating scale (GRS) and a verbal rating scale (VRS) for measuring pain intensity in young female Egyptian and Dutch patients with rheumatoid arthritis (RA). METHODS: Data were obtained in a cross-cultural study of 42 Egyptian and 30 Dutch female outpatients with stable RA. Construct validity was assessed by correlating the scales with other core measures of disease activity in RA. Test-retest reliability was assessed over a 1-week interval. RESULTS: The GRS and the VRS were strongly intercorrelated in the total study cohort and in the Egyptian and Dutch subgroups. In the individual subgroups, only the GRS demonstrated the expected pattern of correlations with other disease activity measures. Test-retest reliability of the GRS was adequate in both Egyptian and Dutch patients (intraclass correlation coefficient 0.78 vs. 0.83, respectively), whereas reliability of the VRS was unsatisfactory in the Egyptian subgroup (weighted kappa 0.60 vs. 0.82 in the Netherlands). DISCUSSION: The study confirmed that the GRS and VRS were reliable and valid in the total study cohort. Within the individual countries, the GRS seemed to perform better than the VRS.

Camptodactyly, arthropathy, coxa vara, and pericarditis syndrome among egyptians.

OBJECTIVE: To highlight the clinical, radiological, and pathological presentations of 10 Egyptian patients with camptodactyly, arthropathy, coxa vara, and pericarditis (CACP) syndrome. Methods. Ten cases underwent a full history, complete clinical examination, laboratory and radiological investigations (and magnetic resonance imaging, MRI, of knee joints); arthroscopic histopathological synovial studies were performed in 6 cases. Results. Camptodactyly and large joint arthropathies were present in all cases. The onset was at birth in 5 cases, and consanguinity was present in all cases. Laboratory investigations were normal in all cases (normal complete blood cell count, erythrocyte sedimentation rate, absent rheumatoid factor, and antinuclear antibody). Synovial fluid analyses were acellular in 3, hypocellular in 4, and moderately cellular in 2 cases. Histopathology revealed noninflammatory synovial hyperplasia in the 6 synovial biopsies obtained, with multinucleated giant cells in 4 of them. Plain hip radiology revealed short broad femoral neck and widening of joint space in all cases, with coxa vara in 9 cases. MRI of the knees showed rim-like enhancement of the lining of the fluid filled bursae in 7 cases, and homogenous enhancement pattern in 2 cases. No evident cartilage destruction existed in any case. CONCLUSION: Our cases represent a familial syndrome of noninflammatory arthropathies associated with camptodactyly and coxa vara. The complete picture of the syndrome may be related to disease duration, and MRI is a useful tool in diagnosis. Physicians should be aware of the syndrome, to avoid misdiagnosis with other pediatric connective tissue diseases.