A PILOT TRIAL FOR SEVERE ,REFRACTORY SYSTEMIC AUTOIMMUNE DISEASE WITH STEM CELL TRANSPLANTATION

Objective To evaluate the feasibility, efficacy, and safety of high dose immunosuppressive therapy （HDIT） and autolognus hemopoietic stem cell transplantation （HSCT） with CD^34＋ cell selection in patients with severe, refractory autoim mune diseases. Methods Twenty-six patients with persistent systemic lupus erythematosus （SLE）, rheumatoid arthritis （RA）, primary Sjogren＇s syndrome （pSS）, or systemic sclerosis （SSc） who had been treated unsuccessfully with conventional treatment were enrolled in the trial in Peking Union Medical College Hospital from September 1999 to June 2004. The patients received HDIT with 200 mg/kg cyclophosphamide followed by an infusion of autologous stem cells that were CD34 selected. Disease acti- vity, adverse effect, hemopoietic and immune reconstitution, and time to recurrence of disease were monitored. Overall treatment related mortality was 7.7% （2/26） with 1 patient died of cytomegalovirus infection and an other of severe pneumonia. Relapse occurred in 3 SLE patients （17.6%） in 37, 26, and 19 months posttransplantation respectively, and 1 RA patient in 15 months posttransplantation. SLE Disease Activity Index （SLEDAI） scores of SLE survivors decreased significantly （P 〈 0.01）. RA patients recorded a drop of Disease Activity Score 28 （DAS 28）. The pSS patient remained symptoms free up to now, more than 50 months aider the transplantation. Conclusion HSCT can be performed relative safely in patients with severe autoimmune disease. Short-term effect of HSCT is promising. However treatment related mortality and relapse were observed in a subset of patients.

Panorama, Reasons for Seeking Care and Evolution of Systemic Autoimmune Diseases in Benin Hospitals in 2021

Introduction: Systemic autoimmune diseases have been poorly studied in sub-Saharan Africa and their frequency is not well known. This study provided an overview of the main reasons for the use of care and their evolution in the main hospitals in Benin. Methods: This was a multi-centric descriptive cross-sectional study conducted in the internal medicine, rheumatology, dermatology and nephrology departments of nine (09) public and private hospital centers in Benin over a 57-month period, from January 1st, 2017 to September 30th, 2021. It involved patients followed for a systemic autoimmune disease. The data was collected with a digital survey sheet and then processed and analyzed with the R software (version 3.4). Results: Two hundred and three (203) patients were recorded, which represents a hospital frequency of 0.13%. The mean age was 44 years and the sex ratio (M/F) was 0.29. Connective tissue disease accounted for 95.07% of systemic autoimmune diseases which were dominated by rheumatoid arthritis (40.9%) and systemic lupus (37.4%). Ten cases of vasculitis have been reported and dominated by Behçet’s disease (40%). The main reasons for seeking care were asthenia, weight loss and fever. Arthralgia and skin lesions are the main guiding signs. Six deaths (3.1%) were recorded among connective tissue disease and 1 death (10%) among vasculitis. Conclusion: In spite of being rare, systemic autoimmune diseases are a reality in Benin. A general population study would provide a better understanding of clinical characteristics and identify prognostic factors.

Difficulties in the Management of Systemic Autoimmune Diseases in Saint-Louis Du Senegal through the Analysis of a Series of 70 Observations

Introduction: Systemic Autoimmune Diseases (SAID) long considered very rare in Africa are increasingly the subject of publications. The objective of this work is to identify the difficulties in the management of these pathologies in an internal medicine department in northern Senegal by analyzing the epidemiological, clinical-biological, therapeutic and evolutionary aspects of SAID. Methods: This was a descriptive cross-sectional study carried out in the internal medicine department of the Saint-Louis University Hospital Center. Included were all the files of patients followed in outpatient and/or hospitalization for autoimmune diseases according to the criteria of the American College of Rheumatology, during the period from January 2017 to December 2020. The data were analyzed using SPSS software version 21.0. As the study was descriptive, no statistical test was performed. Results: Out of 3800 patients, 70 presented SAID, i.e. a hospital prevalence of 1.8%. Polyarthritis was the first reason for consultation in 97% followed by skin manifestations in 8%. The patients had positive anti-nuclear autoantibodies in 88% of cases. Rheumatoid arthritis was the predominant condition (71%) followed by systemic lupus erythematosus (SLE) (15%) and undifferentiated autoimmune diseases in 10%. Eleven percent (11%) of patients had an associated autoimmune disease. Corticosteroids were used in the treatment of these conditions in 97% of cases and methotrexate was the most prescribed immunosuppressant (54%). Thirty-two percent (32%) of patients are lost to follow-up. Conclusion: SAID are diverse and under diagnosed;they are characterized by diagnostic delay above all linked to access to specialists and sometimes to the high cost of paraclinical examinations, in particular immunology. Treatment remains based primarily on corticosteroid therapy and conventional immunosuppressants in the face of the unavailability of biotherapies.

Applications of Next-generation Sequencing in Systemic Autoimmune Diseases

Systemic autoimmune diseases are a genetic and environmental factors. Although group of heterogeneous disorders caused by both numerous causal genes have been identified by genome-wide association studies （GWAS）, these susceptibility genes are correlated to a relatively low disease risk, indicating that environmental factors also play an important role in the pathogen- esis of disease. The intestinal microbiome, as the main symbiotic ecosystem between the host and host-associated microorganisms, has been demonstrated to regulate the development of the body＇s immune system and is likely related to genetic mutations in systemic autoimmune diseases. Next-generation sequencing （NGS） technology, with high-throughput capacity and accuracy, provides a powerful tool to discover genomic mutations, abnormal transcription and intestinal microbiome identification for autoimmune diseases. In this review, we briefly outlined the applications of NGS in systemic autoimmune diseases. This review may provide a reference for future studies in the pathogenesis of systemic autoimmune diseases.

Epidemiological Profile of Autoimmune Diseases in Thiès, Senegal: About a Descriptive Observational Study over 11 Years in 2 Internal Medicine Departments and a Dermatology Department

Introduction: Autoimmune diseases are characterized by a very large clinical polymorphism that can lead to a diagnostic wandering. So, we aimed to determine their epidemiological profile outside the context of Dakar (capital of Senegal) where the technical plateau is more elevated. Methodology: We conducted a retrospective descriptive and observational study from January 1, 2007 to December 31, 2017. All patients admitted or followed in outpatient in the Internal Medicine departments of the Saint Jean de Dieu and Regional Hospitals of Thiès as well as in the dermatology department of the CHRT (Regional Hospital Center of Thiès), and who met the MAI criteria (autoimmune diseases) have been included. The data were collected on a standardised sheet and analysed by EPI INFO version 7.2. Results: A total of 121 patients were included out of 25951 records i.e a prevalence of 0.46% in internal medicine departments. In dermatology, out of 31973 patients, 95 had MAIS (systemic autoimmune diseases): 0.29% as hospital prevalence. The average age was 40.7 years in internal medicine departments compared to 37.66 years 14.8 years in the dermatology department. Patients aged 30 to 59 years represented 57.89% of the study population. The sex ratio (H/F) was 0.3 in the internal medicine departments compared to 0.17 in the dermatology department. Circumstances of discovery were incidental in 16.52% and clinical in 3.30%. Biermer disease accounted for 29.75% of organ-specific MAI. Concerning systemic presentations, rheumatoid arthritis (RA) was present in 23.14%. Lupus was more representative in dermatology (65.2%) as well as systemic scleroderma (21%), dermatomyositis (6.3%). Cytopenia was found in 105 patients, showing in detail anemia (42.9%);leukopenia (14.8%);thrombocytopenia (2.4%). Autoantibodies were tested in 58 patients (47.9%). Skin histology was contributory in all cases of systemic scleroderma and in 5 cases of lupus. The main therapy prescribed was corticosteroid therapy alone or in combination with an immunosuppressant. Conclusion: In addition of infectious diseases, Subsaharan Africa is under the era of changing face of its epidemiology, and cardiovascular diseases shows signs of emergence, like auto-immune presentations. However, the difficult apprehension of these so subtle last diseases suggests that they are few reported. Technical tools in regions should be enhanced associated to a non-binding capacity building system targeting such diseases with an emphasis on good record keeping.