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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]