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Review
. 2017 Jun 30:27:162.
doi: 10.11604/pamj.2017.27.162.12572. eCollection 2017.

[Mixed connective tissue disease: prevalence and clinical characteristics in African black, study of 7 cases in Gabon and review of the literature]

[Article in French]
Landry Missounga  1 , Josaphat Iba Ba  1 , Ingrid Rosalie Nseng Nseng Ondo  1 , Maria Ines Carine Nziengui Madjinou  1 , Doris Malekou  1 , Emeline Gracia Mouendou Mouloungui  1 , Emmanuel Ecke Nzengue  2 , Jean Bruno Boguikouma  1 , Moussavou Kombila  1
Affiliations
Review

[Mixed connective tissue disease: prevalence and clinical characteristics in African black, study of 7 cases in Gabon and review of the literature]

[Article in French]
Landry Missounga et al. Pan Afr Med J. .

Abstract
in English, French

The literature reports that mixed connective tissue disease seems more frequent in the black population and among Asians. This study aims to determine the prevalence of mixed connective tissue disease (MCTD) among connective tissue disorders and all rheumatologic pathologies in a hospital population in Gabon as well as to describe the clinical features of this disease. We conducted a retrospective study by reviewing the medical records of patients treated for mixed connective tissue disease (Kasukawa criteria) and other entities of connective tissue disorders (ACR criteria) in the Division of Rheumatology at the University Hospital in Libreville between January 2010 and December 2015. For each case of MCTD the parameters studied were articular and extra-articular manifestations, anti-U1RNP antibodies levels, patient's evolution. Over a period of 6 years, data were collected by medical records of 7 patients out of 6050 patients and 67 cases of connective tissue disorders, reflecting a prevalence of 0.11% and 10.44% respectively. the 7 patients were women (100%), with an average age of 39.5 years. Articular manifestations included: polyarthritis, myalgias, chubby fingers and Raynaud's phenomenon in 87.5%, 87.5%, 28.6% and 14% respectively. The 7 patients had high anti-U1RNP antibodies levels, ranging between 5 and 35N (N≤ 7 IU). A case of death due to pulmonary arterial hypertension (PAH) was certified. This is the largest case series of MCTD reported in Black Africa. The disease seems to be rare among the black Africans; the reason could be genetic. The demographic and clinical aspects appear similar to those in Caucasians, Asians and Blacks except for a low frequency of Raynaud?s phenomenon among Blacks.

La littérature rapporte que la connectivite mixte semble plus fréquente dans la population noire et chez les asiatiques. Le but de l'étude était de déterminer la prévalence de la connectivite mixte (CM) parmi les connectivites et l'ensemble des pathologies rhumatologiques dans une population hospitalière au Gabon; de décrire ensuite les caractéristiques cliniques de la maladie. Il s'agissait d'une étude rétrospective des dossiers de patients suivis pour connectivite mixte (critères de Kasukawa) et les autres entités de connectivites (critères ACR) en rhumatologie au CHU de Libreville entre janvier 2010 et décembre 2015. Pour chaque cas de CM, les manifestations articulaires et extra-articulaires, le taux d'anticorps anti-U1RNP, l'évolution, étaient les paramètres étudiés. Sept cas ont été colligés en 6 ans parmi 6050 patients et 67 cas de connectivites soit une prévalence de 0,11% et de 10,44% respectivement. Il s'agissait de 7 femmes (100%), d'âge moyen de 39,5 ans. Les signes articulaires comprenaient: polyarthrite, myalgies, doits boudinés et phénomène de Raynaud dans 87,5%, 87,5%, 28,6% et 14% respectivement. Les 7 patients avaient un taux d'anti-U1RNP élevé entre 5 et 35N (N≤7 UI). Un cas de décès par HTAP était constaté. Il s'agit de la série de CM la plus importante rapportée en Afrique noire. La maladie semble rare chez le noir africain, la raison pourrait être génétique. Les aspects démographiques et cliniques paraissent similaires chez les caucasiens, les asiatiques et les noirs hormis une faible fréquence du phénomène de Raynaud chez les noirs.

Keywords: Gabon; Mixed connective tissue disease; black African; prevalence.

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References

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