Assessing hand and global disability in a cohort of Algerian patients with systemic sclerosis: Construct validities of the Arab Hand Function Index and the Arabic Health Assessment Questionnaire.

Objective: The study aimed to assess the construct validity of the Arab Hand Function Index and the Arabic Health Assessment Questionnaire in Algerian patients with systemic sclerosis. Methods: Consecutive Algerian patients who fulfilled the American College of Rheumatology/European League Against Rheumatism criteria for systemic sclerosis were included. In addition to disease characteristics, global disability and hand disability were assessed using the Arabic Health Assessment Questionnaire and the Arab Hand Function Index, respectively. Construct validity was assessed by convergent and divergent validity (Spearman's rank correlation coefficient) and factor analysis. The scale reliability was assessed using the Cronbach's alpha. Results: We evaluated 100 systemic sclerosis patients (83 females) of mean ± standard deviation age 46.7 ± 12.3 years, including 59 limited cutaneous systemic sclerosis and 41 diffuse cutaneous systemic sclerosis. Raynaud's phenomenon was detected in 99 patients and digital ulcers in 25. Gastrointestinal tract involvement and interstitial lung disease were detected in 86/100 (86%) and 46/72 (63.9%) patients, respectively. Anti-topoisomerase I and anti-centromere antibodies were detected in 33/76 (43.4%) and 23/76 (30.3%) patients, respectively. The Arab Hand Function Index had a good construct validity with a total score explaining 61% of the variance of the Arabic Health Assessment Questionnaire which also had a good construct validity. Factor analysis of the Arab Hand Function Index and the Arabic Health Assessment Questionnaire items extracted two factors explaining 64% of the variance for the Arab Hand Function Index and one factor explaining 55% of the variance for the Arabic Health Assessment Questionnaire. The Arab Hand Function Index and the Arabic Health Assessment Questionnaire were reliable questionnaires with a Cronbach's alpha >0.8. Conclusion: In Algerian patients with systemic sclerosis, Arab Hand Function Index and Arabic Health Assessment Questionnaire have a good construct validity and reliability.

Predictors of efficacy of ultrasound-guided intra-articular glucocorticoid injection in knee osteoarthritis: A prospective study.

BACKGROUND: Intra-articular glucocorticoid injection (IAGI) is widely used for treatment of knee osteoarthritis (OA) flares. Response rates are generally around 70%. Several studies have tried to identify predictors of good response, but response to ultrasound (US)-guided injection has not yet been investigated. This study aimed to identify the predictors of response to IAGI performed under US guidance in patients with primary knee OA. MATERIALS AND METHODS: A total of 116 patients (116 knees) presenting with unilateral or bilateral primary knee OA were enrolled for this prospective single-center study. All were aged >40 years and met the American College of Rheumatology (ACR) criteria for knee OA. Demographic, clinical, laboratory, and imaging data were collected, injection was performed using US guidance, and tolerance was assessed. The primary efficacy endpoint was ≥40% reduction in total WOMAC score (WOMAC40). Univariate and multivariate logistic regression analyses were conducted to identify the predictors of response. RESULTS: The mean age of the patients was 64.2 ± 9.4 years and mean BMI was 29.9 ± 3.8 kg/m2. Total WOMAC40 response rate was 61.2%. In multivariate analysis, the independent predictors of response were BMI.

Nakajo-Nishimura Syndrome: The First African Case.

Nakajo-Nishimura syndrome is a hereditary autoinflammatory disorder caused by an autosomal recessive homozygous mutation of the PSMB8 gene, which encodes the immunoproteasome subunit beta 5i. The clinical manifestations of NNS are mainly pernio-like skin rashes, nodular erythema, lipodystrophy, clubbed fingers, remittent fever, hepatosplenomegaly, and basal ganglia calcifications. Here we are reporting a case of NNS in an 11-year-old girl, who lives in eastern Algeria, born from a first-degree consanguineous marriage, she presented with erythematous patches on her face and her back, nodular erythema on her neck, swollen and painful fingers with acrocyanosis and recurrent fever that mainly occurred in cold weather. The patient received long-term treatment with low-dose glucocorticoids, along with immunomodulatory drugs (hydroxychloroquine with methotrexate), partial improvement clinically and biologically was observed. Colchicine was added to her treatment, with increased prednisone doses when she recently developed an AA amyloidosis. Our patient was diagnosed clinically with a probable NNS because she exhibited six of the eight characteristics. To the best of our knowledge, this is the first case of NNS in Africa.

Development of a guideline for the use of ultrasonography in rheumatoid arthritis: Preliminary recommendations.

AIM: To address the protocol of recommendations for the use of ultrasonography (US) in the management of rheumatoid arthritis (RA) in routine practice. METHODS: The present study is a protocol design for practical guidelines. Based on a systematic literature review, the scientific committee (composed of 6 experts on US) decided on key questions which will be used to develop recommendations. These recommendations will be submitted to a group of experts in US in rheumatic and musculoskeletal diseases using the Delphi method. This step will lead to preliminary recommendations. The next step will be to submit the preliminary guideline to an expanded group of US experts to check their relevance. The level of agreement of the experts will be recorded during a web-based meeting. RESULTS: Following two rounds of the Delphi method, a consensus will be addressed. The latter will i) Highlight the use of US for the diagnosis of RA in an early stage of the disease; ii) Define the role of US during follow-up; and iii) Underline the importance of US for the management of clinical remission. CONCLUSION: These recommendations will harmonize and optimize clinical practice and management of RA patients.

Ultrasonography of Shoulder in Rheumatoid Arthritis: A Reliability Exercise Using Consensual Definitions among Maghrebian Rheumatologists.

The shoulder may be affected in a large portion of patients with rheumatoid arthritis (RA) worldwide. However, this joint does not receive the attention required during follow-up. Indeed, although numerous clinical tests for diagnosis of a painful shoulder are available, differentiating articular from peri-articular lesions may be difficult in daily practice. Fortunately, the precise diagnosis of shoulder pain in RA has benefited from a reliable imaging modality used to detect its exact origin-ultrasonography (US). This study was aimed at assessing the intra- and inter-observer reliability of ultrasonographic findings for patients with established RA with shoulder pain in a patient-based exercise as a clinical challenge among Maghrebian rheumatologist experts in US. A total of 7 operators examined 10 patients in two rounds independently and blindly of each other. Before beginning the session, all of the rheumatologists reached a consensus on sites and US settings by performing a brief exercise on a normal shoulder. Outcome Measures in Rheumatology Clinical Trials (OMERACT) definitions of US-detected pathologies were used. Each patient underwent US scanning of the painful shoulder in predefined sites based on US technical guidelines of the European Society of Musculoskeletal Radiology: long head of biceps (LHB), subscapularis recess, posterior recess and axillary recess. The presence of subdeltoid or subcoracoid bursitis or full rupture of the suprasupinatus was identified if present. Intra- and inter-observer reliability measures were calculated using the κ coefficient. Intra-observer reliability was good for gray-scale (GS) synovitis in subscapularis and posterior recesses (κ = 0.77 and 0.73, respectively). It was moderate in the presence of GS synovitis and effusion in LHB (κ =0.53 and 0.40, respectively), posterior and subscapularis recess effusion (κ = 0.56 and 0.60, respectively) and GS and power Doppler (PD) synovitis in axillary recesses (κ = 0.58 and 0.49, respectively). Inter-observer reliability was good for PD for LHB signals (κ = 0.78). It was moderate for GS for LHB synovitis (κ = 0.54). Inter-observer agreement was poor for effusion and GS synovitis for subscapularis, posterior and axillary recesses, and very poor for PD signals in these recesses. US was a reliable imaging tool for detecting tenosynovitis in the LHB. However, reliability was moderate to poor in detecting synovitis in subscapularis, posterior and axillary recesses. These findings could be optimized by standardization of sites to assess.

Rheumatic diseases in Africa.

Historically, rheumatic diseases have not received much attention in Africa, particularly in sub-Saharan Africa, possibly owing to a focus on the overwhelming incidence of infectious diseases and the decreased life span of the general population in this region. Global attention and support, together with better health policies and planning, have improved outcomes for many infectious diseases; thus, increasing attention is being turned to chronic non-communicable diseases. Rheumatic diseases were previously considered to be rare among Africans but there is now a growing interest in these conditions, particularly as the number of rheumatologists on the continent increases. This interest has resulted in a growing number of publications from Africa on the more commonly encountered rheumatic diseases, as well as case reports of rare diseases. Despite the limited amount of available data, some aspects of the epidemiology, genetics and clinical and laboratory features of rheumatic diseases in African populations are known, as is some detail on the use of therapeutics. Similarities and differences in these conditions can be seen across the multi-ethnic and genetically diverse African continent, and it is hoped that increased awareness of rheumatic diseases in Africa will lead to earlier diagnosis and better outcomes for patients.

Spondyloarthritis in North Africa: an update.

Spondyloarthritis (SpA) has been less well studied than rheumatoid arthritis in North Africa, due to a belief that it is rare and benign in certain populations. The main genetic trait of SpA is its association with human leukocyte antigen (HLA)-B27. The distribution of this allele largely explains the prevalence and severity of SpA. The prevalence of HLA-B27 in the general population of North Africa is estimated at about 4%, and rises to about 60% among people affected with SpA. Coxitis is one of the main features of North African SpA, but the response to treatment is comparable to the literature from the West. The major challenge in North Africa remains accessibility to specialized care and means of early diagnosis. Prevalent infections in North Africa do not seem to be a major obstacle to optimal treatment strategies.

African League Against Rheumatism (AFLAR) preliminary recommendations on the management of rheumatic diseases during the COVID-19 pandemic.

OBJECTIVES: To develop recommendations for the management of rheumatic and musculoskeletal diseases (RMDs) during the COVID-19 pandemic. METHOD: A task force comprising of 25 rheumatologists from the 5 regions of the continent was formed and operated through a hub-and-spoke model with a central working committee (CWC) and 4 subgroups. The subgroups championed separate scopes of the clinical questions and formulated preliminary statements of recommendations which were processed centrally in the CWC. The CWC and each subgroup met by several virtual meetings, and two rounds of voting were conducted on the drafted statements of recommendations. Votes were online-delivered and recommendations were pruned down according to predefined criteria. Each statement was rated between 1 and 9 with 1-3, 4-6 and 7-9 representing disagreement, uncertainty and agreement, respectively. The levels of agreement on the statements were stratified as low, moderate or high according to the spread of votes. A statement was retired if it had a mean vote below 7 or a 'low' level of agreement. RESULTS: A total of 126 initial statements of recommendations were drafted, and these were reduced to 22 after the two rounds of voting. CONCLUSIONS: The preliminary statements of recommendations will serve to guide the clinical practice of rheumatology across Africa amidst the changing practices and uncertainties in the current era of COVID-19. It is recognized that further updates to the recommendations will be needed as more evidence emerges. Key Points • AFLAR has developed preliminary recommendations for the management of RMDs in the face of the COVID-19 pandemic. • COVID-19 is an unprecedented experience which has brought new concerns regarding the use of some disease-modifying anti-rheumatic drugs (DMARDs), and these recommendations seek to provide guidelines to the African rheumatologists. • Hydroxychloroquine shortage has become rampart across Africa as the drug is being used as prophylaxis against COVID-19 and this may necessitate a review of treatment plan for some patients with RMDs. • Breastfeeding should continue for as long as possible if a woman is positive for SARS-CoV-2 as there is currently no evidence that the infection can be transmitted through breast milk.

The impact of COVID-19 on rheumatology practice across Africa.

OBJECTIVES: To identify the changes in rheumatology service delivery across the five regions of Africa from the impact of the COVID-19 pandemic. METHODS: The COVID-19 African Rheumatology Study Group created an online survey consisting of 40 questions relating to the current practices and experiences of rheumatologists across Africa. The CHERRIES checklist for reporting results of internet e-surveys was adhered to. RESULTS: A total of 554 completed responses were received from 20 countries, which include six in Northern Africa, six in West Africa, four in Southern Africa, three in East Africa and one in Central Africa. Consultant grade rheumatologists constituted 436 (78.7%) of respondents with a mean of 14.5 ± 10.3 years of experience. A total of 77 (13.9%) rheumatologists avoided starting a new biologic. Face-to-face clinics with the use of some personal protective equipment continued to be held in only 293 (52.9%) rheumatologists' practices. Teleconsultation modalities found usage as follows: telephone in 335 (60.5%), WhatsApp in 241 (43.5%), emails in 90 (16.3%) and video calls in 53 (9.6%). Physical examinations were mostly reduced in 295 (53.3%) or done with personal protective equipment in 128 (23.1%) practices. Only 316 (57.0%) reported that the national rheumatology society in their country had produced any recommendation around COVID-19 while only 73 (13.2%) confirmed the availability of a national rheumatology COVID-19 registry in their country. CONCLUSION: COVID-19 has shifted daily rheumatology practices across Africa to more virtual consultations and regional disparities are more apparent in the availability of local protocols and registries.

Prevalence of metabolic syndrome in Algerian rheumatoid arthritis patients. Correlation with disease activity and functional status.

BACKGROUND: There is evidence that rheumatoid arthritis (RA) patients have an over-risk of cardiovascular disease. This may be mainly due to an increase in the prevalence of metabolic syndrome (MetS). The prevalence of MetS among adults in Algeria is 19.1%. OBJECTIVES: The aim of the study was to evaluate the prevalence of MetS among RA patients in Algeria. Another aim was to evaluate the relationship between MetS, inflammation biomarkers and disease scores. METHODS: The study was performed on a cohort of 249 patients meeting the ACR/EULAR criteria for RA, followed in 11 Algerian centers. The diagnosis of MetS was based on the NCEP/ATP III (MetS+ if ≥3/5) definition. Prevalence of MetS was calculated, and patients were divided in two groups (MetS+ and MetS-). Comparison between the groups was performed using a t-test. RESULTS: Among the 249 RA patients, 213 were females and 36 males of a mean age of 50.1±14.5years and a mean disease duration of 8.4±7.8years. The overall prevalence of MetS was 13.9% (CI95%: 9.5-20.1%); it was 14.3% in males and 13.8% in females. The ESR level was significantly higher in MetS+ patients than in MetS- patients (p=0.036). CONCLUSION: In this multicenter study, unlike most studies on RA patients, the prevalence of MetS was as not higher in Algerian RA patients (13.9%) than in the Algerian general population (19.1%). Only ESR levels correlate with the presence of MetS, this may be due to the modest cohort size and needs to be confirmed.

Successful treatment of Acrodermatitis continua of Hallopeau associated with psoriatic arthritis with adalimumab.

Acrodermatitis continua of Hallopeau (ACH) is a rare form of pustular psoriasis, mainly affecting distal phalanges of hands and feet. Many therapeutic options exist; however, it tends to be resistant to treatment. We report a 26-year-old man presented with a very severe psoriatic arthritis associated with ACH. Although this patient was resistant to a first line treatment (glucocorticoids and methotrexate), a rapid and dramatic improvement was observed after adalimumab was introduced. The effectiveness and tolerance of the treatment were maintained during the 12-month period of follow-up. This is the first report of the efficacy of adalimumab on ACH in a patient presented with psoriatic arthropathy.

Characteristics of rheumatoid arthritis in Algeria: a multicenter study.

The aim of this study was to compare the epidemiology of rheumatoid arthritis (RA) in North Africa to that of Western countries. We have enrolled in a cross-sectional study all consecutive patients presenting with the diagnosis of RA according to the 1987 ACR criteria, and during a 5-month period, patients were included in 11 centers across northern Algeria. Demographics, clinical data, and health assessment questionnaires (HAQ) were collected for each patient. We have estimated means, standard deviations, and 95 % confidence intervals for all parameters. Of the 249 patients (213 females and 36 males) enrolled in the study, 10 (4 %) had juvenile onset of the disease. The mean age was 50.1 ± 14.5 years, and the mean duration of RA was 8.4 ± 7.8 years. In terms of comorbidities, 18.9 % of patients reported hypertension and 5.2 % had diabetes. The mean DAS28 at inclusion was 4.3 (95 % CI 4.1-4.5); 14.0 % were in remission (DAS28 ≤ 2.6). The mean HAQ score was 0.81 ± 0.82. Rheumatoid factor was positive in 78.5 % of cases, and anti-citrullinated protein/peptide antibodies, when measured, was positive in 69.0 % of cases. Seronegative patients were older and had a relatively less severe disease. For treatment, 89.7 % of patients were taking disease-modifying anti-rheumatic drugs and only 4 % were taking biologics (rituximab); 90.8 % of patients were taking glucocorticoids, and none of the patients satisfied the recommended calcium intake guidelines. RA in Algeria is more common in women. Compared to reports from Western countries, RA in Algeria appears to be less aggressive, with more dominant seronegative oligoarthritis forms. The remission rate is comparable to that of Western populations.

Successful treatment of pain in melorheostosis with zoledronate, with improvement on bone scintigraphy.

Melorheostosis is a very rare sclerosing bone disorder that involves frequently one limb. It may be asymptomatic, but pain and limb deformity may occur and can be very debilitating. Different reports have indicated efficacy of bisphosphonates (pamidronate and etidronate) on symptoms. We report an adult patient with a very painful melorheostosis, who  improved after treatment with zoledronate, either on symptoms or on bone scans.

Rituximab in rheumatoid arthritis and the risk of malignancies: report from a French cohort.

OBJECTIVE: To determine in real-life conditions the safety of treatment with rituximab (RTX) in patients with rheumatoid arthritis (RA) regarding malignancies. METHODS: Analysis of safety data from a cohort of RA patients who received at least one course of RTX. RA patients with previous malignancies were followed-up and compared to the group of patients with no history of malignancy. RESULTS: One hundred and eighty-six RA patients, 33 (17.7%) males, the mean age and disease duration were 55.8 ± 13.0 and 14.5 ± 11.1 years, respectively. The mean follow-up was 22.3 ± 15.1 months, corresponding to a follow-up of 346 pt-years of RTX exposure. Among these, 24 (12.9%) patients had a history of a prior malignancy. Five cancers were diagnosed during follow-up with four new malignancies (1 prostate, 1 breast, 1 colon and 1 cervical cancers) and one recurrence of a known breast cancer. The overall cancer rate was 1.45/100 pt-years (95%CI: 0.19 to 2.70), which is comparable to previously studied DMARD-treated cohorts. No new hematopoietic neoplasms were reported and the six lymphomas that have been in remission prior to RTX-therapy remained under follow-up. The baseline demographic and disease characteristics and the cancer-risk of the 24 patients who presented with a prior malignancy were similar to those with no cancer history (162 patients). CONCLUSIONS: Although based on a modest number of observed cancers, and despite selection bias (12.9% of prior malignancies in our RTX treated RA), this observational study suggests that RTX does not increase the cancer risk in RA patients.

Juvenile hyaline fibromatosis: focus on radiographic features in adulthood.

Juvenile hyaline fibromatosis (JHF) is a rare autosomal recessive hereditary disorder (less than 80 cases reported), characterized by multiple nodular lesions on the skin and musculoskeletal involvement, very debilitating because most adolescents and adults become bedridden. Only 10 cases have been reported on JHF in adulthood. We report the case of a 34-year-old male patient in whom clinical and histological findings were consistent with a mild JHF and focus on the radiographic features. The main purpose of this report is to increase the information available related to the radiographic manifestations and prognosis of JHF.