Search

Background: Many children with rheumatic and musculoskeletal diseases are unrecognized. Identifying these children requires health care provider awareness, knowledge, and skills to recognize disease features and how (and when) to refer to specialist care. The aim of this paper is to highlight the need for better access to health care, review the essential role that education and virtual care play to address unmet need in low resource areas and especially to expand workforce capacity. Using collaborative partnerships, virtual platforms, and innovative assessment methods, musculoskeletal care and education can be delivered to reach a greater audience than ever before. Increased awareness through multiple initiatives and readily available resources are imperative to improve global rheumatology care. Conclusion: The needs of children with rheumatic diseases and musculoskeletal conditions are vastly underserved around the world resulting in preventable morbidity and mortality. Expanded implementation of virtual education and e-health care platforms provides an opportunity to increase access to care for children globally. [ABSTRACT FROM AUTHOR]

Juvenile idiopathic arthritis (JIA) is the most prevalent chronic rheumatic disease in children and young people (CYP) and a major cause of pain and disability. The vast majority of the world's children and their families live in less resourced countries (LRCs) and face significant socioeconomic and healthcare challenges. Current recommendations for standards of care and treatment for children with JIA do not consider children living in less resourced countries. In order to develop appropriate recommendations for the care of CYP with JIA in less resourced countries a meeting of experienced pediatric rheumatologists from less resourced countries was convened with additional input from a steering group of international pediatric rheumatologists with experience in developing recommendations and standards of care for JIA. Following a needs assessment survey of healthcare workers caring for CYP with JIA in LRC, a literature review was carried out and management recommendations formulated using Delphi technique and a final consensus conference. Responses from the needs assessment were received from 121/483 (25%) practitioners from 25/49 (51%) less resourced countries. From these responses, the initial 84 recommendations were refined and expanded through a series of 3 online Delphi rounds. A final list of 90 recommendations was proposed for evaluation. Evidence for each statement was reviewed, graded, and presented to the consensus group. The degree of consensus, level of agreement, and level of evidence for these recommendations are reported. Recommendations arrived at by consensus for CYP with JIA in less resourced countries cover 5 themes: (1) diagnosis, (2) referral and monitoring, (3) education and training, (4) advocacy and networks, and (5) research. Thirty-five statements were drafted. All but one statement achieved 100% consensus. The body of published evidence was small and the quality of evidence available for critical appraisal was low. Our recommendations offer novel insights and present consensus-based strategies for the management of JIA in less resourced countries. The emphasis on communicable and endemic diseases influencing the diagnosis and treatment of JIA serves as a valuable addition to existing JIA guidelines. With increasing globalization, these recommendations as a whole provide educational and clinical utility for clinicians worldwide. The low evidence base for our recommendations reflects a shortage of research specific to less resourced countries and serves as an impetus for further inquiry towards optimizing care for children with JIA around the world. [ABSTRACT FROM AUTHOR]

Background It is estimated that millions of children in Africa suffer from pediatric rheumatic diseases (PRD). Despite this, as of 2021, there were just 28 pediatric rheumatologists in only 10 of the 54 African countries. With ∼700 million children living in Africa, this number falls far short of the recommended minimum 1750 pediatric rheumatologists needed for ideal care. Rheumatic and musculoskeletal diseases are the second leading cause of disability globally. However, there is a paucity of data on PRD in low and middle-income countries (LMIC) due to limited diagnostic capacities, scarcity of pediatric rheumatologists, and limited awareness of PRD. We have much to learn about the prevalence of PRD as well as clinical manifestations, optimal treatments, and outcomes in African children with PRD. Juvenile dermatomyositis (JDM) can cause significant morbidity and irreversible damage when inadequately treated, resulting in medical, social, and economic burden on patients, families, and healthcare systems, particularly in LMIC. The few studies of JDM in Africa suggest high rates of severe manifestations, including calcinosis, vasculitis, and pulmonary involvement. The objective of this survey is to better understand the scope of JDM burden in Africa by obtaining an overview of JDM and comparing this to data in previously published work. Methods A survey was distributed to members of the African League of Associations for Rheumatology (AFLAR; n  = 233) and Paediatric Society of the African League Against Rheumatism (PAFLAR; n  = 130) via WhatsApp groups. Respondents were queried about the total number of JDM patients seen currently or within the last 10 years; morbidities and clinical outcomes, including calcinosis, interstitial lung disease (ILD), clinically inactive disease, remission, and death; and availability and accessibility of diagnostic tools and medications. Results A total of 43 (12%) individuals started the survey, with 4 (9%) partially and 16 (37%) fully completing it, describing 216 JDM patients. One general pediatrician participated; the remainder were adult (n  = 10; 50%), pediatric (n  = 7; 35%), or adult/pediatric (n  = 2; 10%) rheumatologists. Respondents represented the 5 regions of Africa, primarily Northern Africa (n  = 12; 60%). Mortality was reported at 14% and 29% in Kenya and Zambia, respectively (Table 1). Many respondents identified limited availability of medications (56%), unfamiliarity with JDM by caregivers (69%) and other medical providers (44%), and delayed presentation to care (75%). Access and/or cost limited use of diagnostic tools and medications. Of the 27 queried diagnostic tools and medications, 13 were reported to be accessible to only 50% or fewer of respondents, the majority (69%) of whom were from Northern or Southern African countries. Despite the lack of cost or other apparent access barriers, disease assessment tools are used by only 53% of respondents. E33 Table 1. Clinical outcomes in children with juvenile dermatomyositis in Africa among survey respondents (n  = 20) Country (Number of Respondents).  Total Patients (n = 216) c.  Clinically Inactive Disease a.  Remission b.  Calcinosis.  Interstitial Lung Disease.  Deaths.  Northern Africa (12) Egypt (6)   68 c   28  13  36  5  2  Libya (3)   55 c   18 e   39 e   12  5 e   3  Morocco (1)   19  6 (31.6%)  4 (21.1%)  7 (36.8%)  6 (31.6%)  0 (0%)  Tunisia (2)   3 c   6  6  0  4  0 e   Eastern Africa (3) Kenya (2)   42  15 (35.7%)  12 (28.6%)  13 (31.0%)  8 (19.0%)  6 (14.3%)  Zambia (1)   7  5 (71.4%)  0 (0%)  3 (42.9%)  1 (14.3%)  2 (28.6%)  Central Africa (3) Cameroon (2) d   11  6 (54.5%)  5 (45.5%)  3 (27.3%)  2 (18.2%)  0 (0%)  Democratic Republic of the Congo (1) d   3  3 (100%)  1 (33.3%)  3 (100%)  0 (0%)  e   Western Africa (1) Nigeria (1)   4  2 (50.0%)  1 (25.0%)  0 (0%)  1 (25.0%)  0 (0%)  Southern Africa (1) South Africa (1)   4  1 (25.0%)  1 (25.0%)  2 (50.0%)  0 (0%)  0 (0%)  Country (Number of Respondents).  Total Patients (n = 216) c.  Clinically Inactive Disease a.  Remission b.  Calcinosis.  Interstitial Lung Disease.  Deaths.  Northern Africa (12) Egypt (6)   68 c   28  13  36  5  2  Libya (3)   55 c   18 e   39 e   12  5 e   3  Morocco (1)   19  6 (31.6%)  4 (21.1%)  7 (36.8%)  6 (31.6%)  0 (0%)  Tunisia (2)   3 c   6  6  0  4  0 e   Eastern Africa (3) Kenya (2)   42  15 (35.7%)  12 (28.6%)  13 (31.0%)  8 (19.0%)  6 (14.3%)  Zambia (1)   7  5 (71.4%)  0 (0%)  3 (42.9%)  1 (14.3%)  2 (28.6%)  Central Africa (3) Cameroon (2) d   11  6 (54.5%)  5 (45.5%)  3 (27.3%)  2 (18.2%)  0 (0%)  Democratic Republic of the Congo (1) d   3  3 (100%)  1 (33.3%)  3 (100%)  0 (0%)  e   Western Africa (1) Nigeria (1)   4  2 (50.0%)  1 (25.0%)  0 (0%)  1 (25.0%)  0 (0%)  Southern Africa (1) South Africa (1)   4  1 (25.0%)  1 (25.0%)  2 (50.0%)  0 (0%)  0 (0%)  Number of patients reported as n; (%) also reported when total patient's known. a Defined as lack of evidence of myositis disease activity as assessed by global and extra-muscular assessments, stable muscle strength and function, and normal muscle enzyme levels, per the International Myositis Assessment & Clinical Studies (IMACS) criteria for lack of evidence of active myositis. b Defined according to the IMACS 2005 definition: clinically inactive disease while not receiving any drug therapy for a 6-month continuous period. c At least 1 respondent unsure of total number of patients, therefore number provided is an underestimation and percentages were unable to be calculated; Egypt = 3 of 6 respondents unsure about prior patients, Libya = 1 of 3 respondents unsure about total current and total prior patients, Tunisia = 2 of 2 respondents unsure about total current patients and 1 of 2 unsure about prior patients. d Respondents answered questions only about current, not prior, patients (Cameroo n  = 1 respondent, Democratic Republic of Congo = 1 respondent). e Response missing from 1 respondent. Open in new tab E33 Table 1. Clinical outcomes in children with juvenile dermatomyositis in Africa among survey respondents (n  = 20) Country (Number of Respondents).  Total Patients (n = 216) c.  Clinically Inactive Disease a.  Remission b.  Calcinosis.  Interstitial Lung Disease.  Deaths.  Northern Africa (12) Egypt (6)   68 c   28  13  36  5  2  Libya (3)   55 c   18 e   39 e   12  5 e   3  Morocco (1)   19  6 (31.6%)  4 (21.1%)  7 (36.8%)  6 (31.6%)  0 (0%)  Tunisia (2)   3 c   6  6  0  4  0 e   Eastern Africa (3) Kenya (2)   42  15 (35.7%)  12 (28.6%)  13 (31.0%)  8 (19.0%)  6 (14.3%)  Zambia (1)   7  5 (71.4%)  0 (0%)  3 (42.9%)  1 (14.3%)  2 (28.6%)  Central Africa (3) Cameroon (2) d   11  6 (54.5%)  5 (45.5%)  3 (27.3%)  2 (18.2%)  0 (0%)  Democratic Republic of the Congo (1) d   3  3 (100%)  1 (33.3%)  3 (100%)  0 (0%)  e   Western Africa (1) Nigeria (1)   4  2 (50.0%)  1 (25.0%)  0 (0%)  1 (25.0%)  0 (0%)  Southern Africa (1) South Africa (1)   4  1 (25.0%)  1 (25.0%)  2 (50.0%)  0 (0%)  0 (0%)  Country (Number of Respondents).  Total Patients (n = 216) c.  Clinically Inactive Disease a.  Remission b.  Calcinosis.  Interstitial Lung Disease.  Deaths.  Northern Africa (12) Egypt (6)   68 c   28  13  36  5  2  Libya (3)   55 c   18 e   39 e   12  5 e   3  Morocco (1)   19  6 (31.6%)  4 (21.1%)  7 (36.8%)  6 (31.6%)  0 (0%)  Tunisia (2)   3 c   6  6  0  4  0 e   Eastern Africa (3) Kenya (2)   42  15 (35.7%)  12 (28.6%)  13 (31.0%)  8 (19.0%)  6 (14.3%)  Zambia (1)   7  5 (71.4%)  0 (0%)  3 (42.9%)  1 (14.3%)  2 (28.6%)  Central Africa (3) Cameroon (2) d   11  6 (54.5%)  5 (45.5%)  3 (27.3%)  2 (18.2%)  0 (0%)  Democratic Republic of the Congo (1) d   3  3 (100%)  1 (33.3%)  3 (100%)  0 (0%)  e   Western Africa (1) Nigeria (1)   4  2 (50.0%)  1 (25.0%)  0 (0%)  1 (25.0%)  0 (0%)  Southern Africa (1) South Africa (1)   4  1 (25.0%)  1 (25.0%)  2 (50.0%)  0 (0%)  0 (0%)  Number of patients reported as n; (%) also reported when total patient's known. a Defined as lack of evidence of myositis disease activity as assessed by global and extra-muscular assessments, stable muscle strength and function, and normal muscle enzyme levels, per the International Myositis Assessment & Clinical Studies (IMACS) criteria for lack of evidence of active myositis. b Defined according to the IMACS 2005 definition: clinically inactive disease while not receiving any drug therapy for a 6-month continuous period. c At least 1 respondent unsure of total number of patients, therefore number provided is an underestimation and percentages were unable to be calculated; Egypt = 3 of 6 respondents unsure about prior patients, Libya = 1 of 3 respondents unsure about total current and total prior patients, Tunisia = 2 of 2 respondents unsure about total current patients and 1 of 2 unsure about prior patients. d Respondents answered questions only about current, not prior, patients (Cameroo n  = 1 respondent, Democratic Republic of Congo = 1 respondent). e Response missing from 1 respondent. Open in new tab Conclusion This is the first study to explore JDM on a broad scale across the African continent. We identified 216 African children with JDM within the past 10 years, exceeding the previously published reports of 196 JDM patients seen in the last 25 years and likely still underestimating prevalence. Our results suggest substantially higher rates of severe disease, with mortality rates in some countries vastly different from the 1–3% reported in high-income countries. Many respondents were limited in their capacity to diagnose and treat JDM, but there was a wide range across African regions that seemed to parallel known disparities in healthcare resource availability in Africa. This survey highlighted important possible differences in disease manifestations and outcomes that warrant further study. Future collaboration with our African colleagues is critical to raise awareness of JDM to ultimately improve disease severity and outcomes. Ethics Institutional review board approval was obtained with waiver of informed consent. [ABSTRACT FROM AUTHOR]

Background: Paediatric rheumatic diseases are a leading cause of acquired disability in Southeast Asia and Asia-Pacific Countries (SE ASIA/ASIAPAC). The aims of this study were to identify and describe the challenges to the delivery of patient care and identify solutions to raise awareness about paediatric rheumatic diseases. Methods: The anonymised online survey included 27 items about paediatric rheumatology (PR) clinical care and training programmes. The survey was piloted and then distributed via Survey-Monkey™ between March and July 2019. It was sent to existing group lists of physicians and allied health professionals (AHPs), who were involved in the care pathways and management of children with rheumatic diseases in SE ASIA/ASIAPAC. Results: Of 340 participants from 14 countries, 261 participants had been involved in PR care. The majority of the participants were general paediatricians. The main reported barriers to providing specialised multidisciplinary service were the absence or inadequacy of the provision of specialists and AHPs in addition to financial issues. Access to medicines was variable and financial constraints cited as the major obstacle to accessing biological drugs within clinical settings. The lack of a critical mass of specialist paediatric rheumatologists was the main perceived barrier to PR training. Conclusions: There are multiple challenges to PR services in SE ASIA/ASIAPAC countries. There is need for more specialist multidisciplinary services and greater access to medicines and biological therapies. The lack of specialist paediatric rheumatologists is the main barrier for greater access to PR training. [ABSTRACT FROM AUTHOR]