Primary Immunodeficiencies in Africa: Primary Immunodeficiencies in Africa: a preliminary ideaa preliminary idea

Bousfiha AA, Esser M, Ailal F, Jeddane L, Boukari R, El Marsafi A, Eley B, Barbouche R, Bejaoui M

From African Society for ImmunoDeficiencies, website: www.asid.ma

There is renewed interest in Africa for Primary ImmunoDeficiencies, especially since the creation of the African Society for ImmunoDeficiencies (ASID; Casablanca, 2008). So national teams have doubled their efforts and an extraordinary collaboration has been established between the countries where care and research have made some progress. Sensitization sessions and training schools were held in several countries, including Mauritania and South-Africa. The second ASID congress is expected in March 2011 in Tunisia. This work’s purpose is to estimate the number of PIDs in countries who have series, including publications from the works presented during the first ASID congress in 2008.

Population (2009) PIDs. * PID patients / year. **

Register of current patients.

World 6 829 360 438 254 052 703 424 ?Africa 1 009 892 844 37 568 104 019 1049

Europe 732 758 546 27 258 75 474 10 279

Table 1- Number of PID patients and Number of PID patients per year estimated in the world, Africa and Europe. * Calculated on the basis of a prevalence of 3.72 per 100 000 (B. Gathmann, 2009). ** Calculated on the basis of an incidence of 10.3 per 100,000 persons per year (AY JOSHI, 2009). [1,2]

DIP MAROC TUNISIE ALGERIE EGYPTE

AF . DU SUD

ASID ESID

N=236 N=397 N=61 N=204 N=195 N=1093 N=7099Incidence*              

A. Combined T and B cell immunodeficiency (CID)

21,6% 23,4% 18% 25,4% 13,84% 21.4% 9.2%

SCID 49% 31.2%   61.5%   36.7% 35.2%OMMEN SD 2% 8.6%   9.6%   6%  

MHC CLASS II deficiency 27.5% 36.6%   7.7%   22.2% 8.5% B. Predominant antibody

deficiencies 25,8% 24,7% 55,7% 24,1% 49,73% 31% 56.8%

X-Linked Agamma (Bruton) 19.7% 21.4%       9.7% 10.8%

CVID 18% 30.6%   28.6%   16.2% 38.2%Hyper IgM 14.8% 22.4%   4.1%   9.7% 2.3%

SUBCLASS IgG DEFICIENCY 9.8% 1%   4.1%   2,7% 12.6%

SELECTIVE IgA DEFICIENCY 29.5% 16.3%   30.6%   14.5% 13.5%

C. Other well-defined immunodeficiency syndromes

32,2% 23,2% 9,8% 16,66%   19% 17.1%

WAS 2.6% 8.7%   14.7%   7.2% 23.8%

ATAXIA TELANGIECTASIA 35.5% 70.7% 100% 41.1%   53.8% 33.3%

DIGEORGE ANOMALY 13.2% 4.3%   8.8%   8.2% 20.3%

HYPER IgE SD 39.5% 15.2%   26.4%   25.5% 9.9%

D. Diseases of immune dysregulation

2,1% 2%   2%   1,6% 3.6%

CHEDIACK HIGASHI SD 40% 62.5%       41.1% 5.8%GRISCELLI SD 60%     50%   29.4% 6.2%

FAMILIAL HEMOPHAGOCYTIC LYMPHOHISTIOCYTOSIS SD

  37.5%       17.6% 34.4%

E. Congenital defects of phagocytes

8,9% 23,2% 16,4% 9,3% 7,69% 14.4% 9.8%

SEVERE CONGENITAL NEUTROPENIA

19%   30%     4.5%  

KOSTMANN DISEASE 9.5%         1.3% 11.2%LAD 4.8% 18.5%   15.8%   13.4% 4.7%CGD 28.6% 44.6% 70% 68.4%   42.7% 29.6%

MSMD 14.3% 21.7%       14.6% 4.1%

F. Defects in innate immunity 0.4%         0,1% 0.2%

G. Autoinflammatory disorders 4.2%         0,9% 1.1%

H.Complement deficiencies 4.7% 1%     20% 4,9% 2.1%Table 2- PID distribution in several African countries and in Europe [3]

National efforts and African cooperation are ongoing, supported by ESID but also national associations, the Jeffrey Modell Foundation and IPOPI. In this framework, an African registry project is in progress with the support of the ESID.

References: 1.Gathmann B, France, 2009.2.Joshi A.Y, USA, 2009. 3.Bousfiha AA et al, Epidémiologie et classification des déficits immunitaires primitifs, Rev Mar Mal Enf 2008, vol 18