Maladies auto-immunes systémiques dans les Services de Médecine Interne et de Dermatologie du Centre Hospitalier Universitaire de Bouaké: profil de 50 patients / Systemic autoimmune diseases in the Internal Medicine and Dermatology Departments of the University Hospital of Bouake: profile of 50 patients

Introduction Les maladies auto-immunes systémiques (MAIS) sont peu connues malgré les progrès diagnostiques et thérapeutiques réalisés ces dernières années. L'objectif de ce travail était de décrire le profil épidémiologique, diagnostique et thérapeutique des MAIS dans les services de Médecine Interne et de Dermatologie du Centre Hospitalier Universitaire de Bouaké (CHU). Méthodes Il s'agissait d'une étude transversale descriptive réalisée dans les services de Médecine Interne et de Dermatologie du CHU de Bouaké, sur une période de 10ans (janvier 2009- décembre 2018). Résultats : Sur 30906 patients, 50 présentaient une MAIS soit une prévalence hospitalière de 0,16%. Les MAIS les plus fréquentes étaient le lupus érythémateux systémique (50%) et la sclérodermie systémique (42%). L'âge moyen était de 39,5ans ±15ans et le sex-ratio de 0,19. Le délai moyen de consultation était de 26,2 mois. Le tableau clinique était dominé par les signes généraux (98%), les manifestations cutanéomuqueuses (96%) et les manifestations articulaires (90%). Chez 37 patients ayant réalisé l'hémogramme, l'anémie représentait 51,3% des cas. Le syndrome inflammatoire était objectivé chez 67% des 12 patients possédant un bilan inflammatoire. Les auto-anticorps réalisés chez 05 patients étaient contributifs chez 03 patients. Les corticoïdes par voie générale étaient prescrits dans 56% des cas et les perdus de vue étaient observés dans 90% des cas. Conclusion : Les MAIS étaient rares dans notre étude, dominées par le lupus érythémateux systémique et la sclérodermie systémique. L'amélioration du plateau technique et l'accessibilité du bilan immunologique et la sensibilisation paraissent indispensables afin d'améliorer la prise en charge des patients.

Introduction: Systemic autoimmune diseases (SAID) are little known despite the diagnostic and therapeutic progress made in recent years. The objective of this work was to describe the epidemiological, diagnostic and therapeutic profile of SAID in Internal Medicine and Dermatology departments of the university hospital of Bouake. Methods: This was a cross-sectional study carried out in the Internal Medicine and Dermatology departments of the university hospital of Bouake, over a period of 10 years (January 2009-December 2018). Results: Of 30,906 patients, 50 presented SAID with a hospital prevalence of 0.16%. The most common SAID were systemic lupus erythematosus (50%) and systemic sclerosis (42%). The mean age was 39.5 ± 15 years, and the sex ratio was 0.19. The average consultation time was 26.2 months. The clinical picture was dominated by general signs (98%), mucocutaneous manifestations (96%) and articular manifestations (90%). In 25 patients who performed the blood count, anemia represented 76% of cases. The inflammatory syndrome was objectified in 67% of the 12 patients with an inflammatory profile. The auto-antibodies made in 05 patients were contributory in 03 patients. Systemic corticosteroids were prescribed in 56% of cases and patients were lost to follow-up in 90% of cases. Conclusion: SAID were rare in our study, dominated by systemic lupus erythematosus and systemic scleroderma. Improvement of the technical platform and accessibility of the immunological assessment appears essential in order to improve patient's care.

Oral complications of systemic sclerosis; a case report

Objective: Systemic Sclerosis is a connective tissue disorder with multi-system involvement. It is an autoimmune disease with multiple aetiological factors including; genetic, birth disorder, infectious and environmental factors. Sclerosis of the skin and systemic fibrotic manifestations are the hallmark of this condition. We report a case of a 30-year-old female, who presented to the Periodontology clinic of the University of Benin, Teaching Hospital, Benin City with complaints of breakage of her teeth and previous multiple extractions. Clinical examination revealed multiple carious lesions, retained root, mobile teeth with varying degrees of mobility, tooth wear lesions and gingival recession. Case Description: She also presented with extra-oral features which include sparse and fluffy hair, reduced lacrimation, progressively reducing mouth opening and thickening of skin of the forehead, cheeks, and extensor surfaces of the upper and lower limbs Conclusion: A multidisciplinary management approach, involving the periodontology's, oral surgeons, oral physicians, prosthodontist, rheumatologists, chest physicians and dermatologists, was instituted

Systemic sclerosis (Scleroderma) among adults attending the rheumatology clinic of a tertiary institution

Background: Systemic sclerosis is a multi-systemic autoimmune disorder characterised by the generation of autoantibodies, massive deposition of collagen and other matrix substances in the connective tissues and alterations of the microvasculature. Methods: This was a prospective study of all the cases of scleroderma seen between January 2012 and June 2015 at the Rheumatology Clinic of the Olabisi Onabanjo University Teaching Hospital. All the patients with the diagnosis of scleroderma were included. Excluded from the study were patients with other skin lesions not typical of scleroderma. Results: Six hundred and six patients with rheumatologic disorders were seen over the study period but eight of them had scleroderma. All the eight cases of scleroderma were females. The age range was 36-52 years with a mean age of 45 years (SD ± 11.4 years). All the cases had arthritis and diffuse skin lesions. Other features included sclerodactyly, microstomia and salt and pepper skin appearance in all the eight cases while Raynaud's phenomenon and leg ulcers were not frequent. Inflammatory marker (erythrocyte sedimentation rate) was significantly elevated in all the cases. Renal and cardiovascular complications were the leading causes of death.Conclusion: Diffuse systemic sclerosis is a more serious disease than the limited form. Early referral of patients with skin lesions suggestive of scleroderma to a specialist centre is recommended as symptomatic treatment helps to improve the quality of life

Connective Tissue Diseases: Challenges of management among Nigerians

Contrary to previous assumptions, Connective Tissue Diseases (CTD) are increasingly being reported among Africans, Nigerians inclusive. The clinical presentations are mostly similar to patterns described in other parts of the world, though with few differences. The management is patterned, as universally done, but drugs such as the biologics are infrequently used because of the prohibitive cost. The challenges of management arise mainly from a poor understanding of the spectrum, presentation and management of CTD as well as the non-affordability of the required drugs. The superstitious belief that these poorly-understood conditions are due to 'spiritual attacks' further compounds the problem of late presentation and sometimes accounts for the high morbidity and mortality. Continuing training of rheumatologists will enhance the understanding and management of rheumatological diseases

Etude rétrospective de 35 cas de sclérodermie systémique à Bamako Mali

A partir d'une étude rétrospective; sur dossiers de 35 cas de sclérodermie systémique observes dans l'Unité de Dermatologie de l'Institut Marchoux; les auteurs tentent de rapporter les particularités épidémiologiques; cliniques; évolutives et thérapeutiques de cette affection au Mali. L'affection semble rare au Mali; 35 cas en 10 ans. Les 35 dossiers de cas ont été colliges en 1985 a 1994. La définition du cas a été faite selon les critères de l'Association Américaine de Rhumatologie (ARA). L'âge moyen des patients était de 34;4 ans (10-60). La prédominance féminine était notable : 6 femmes/1 homme. Les cas ont évolué pendant 1 àplus de 2 ans avant de consulter en dermatologie. 3 cas ont été initialement diagnostiques lèpre; 5 cas étaient pris en charge en néphrologie pour des œdèmes et 1 cas en Cardiologie pour des troubles du rythme et douleurs thoraciques. Les troubles pigmentaires; notamment l'hypochromie mouchetee; dominaient le tableau dermatologique (31/35). Le syndrome de Raynaud était absent chez la moitié des patients. L'atteinte des extrémités comportaient des ulcérations pulpaires (12 fois); des déformations en griffe des doigts (20 fois) et l'amputation des dernières phalanges (25 fois); articulaires (19 fois); pulmonaires (16 fois) et digestives (35 fois) étaient observes chez les malades; Parmi les médicaments utilises; seule la D-penicillamine a donné de bons résultats (4/4) sur les symptômes cutanées et systématiques. La rareté de la sclérodermie systémique au Mali semble être compensée par la sévérité du tableau clinique et le pronostic sombre a plus ou moins longue échéance