Granulomatosis with polyangiitis according to geographic origin and ethnicity: clinical-biological presentation and outcome in a French population.

Objectives: Granulomatosis with polyangiitis (GPA) mainly affects white Europeans, but rarely GPA may also affect non-Europeans. This study aimed to describe GPA clinical-biological presentation and outcome in black sub-Saharan Africans and Afro-Caribbeans and in North Africans. Methods: Among 914 GPA patients included in the French Vasculitis Study Group database, geographic origin and ethnicity were known for 760. Clinical-biological presentations and outcomes of white Europeans vs black sub-Saharans and Afro-Caribbeans and vs North Africans were analysed. Results: Among the 760 patients, 689 (91%) were white Europeans, 33 (4.3%) were North Africans and 22 (2.9%) were sub-Saharans (n = 8) or Afro-Caribbeans (French West Indies, n = 14). Black sub-Saharans and Afro-Caribbeans, compared with white Europeans, were significantly younger at GPA diagnosis (P = 0.003), had more frequent central nervous system involvement (P = 0.02), subglottic stenosis (P = 0.002) and pachymeningitis (P = 0.009), and tended to have more frequent chondritis and retroorbital tumour. Median serum creatinine levels and Birmingham Vasculitis Activity Score were significantly lower in sub-Saharans and Afro-Caribbeans (P = 0.002 and P = 0.003, respectively). In contrast, in comparison with white Europeans, North Africans had only less frequent arthralgias (P = 0.004). Time to relapse was shorter for black sub-Saharans and Afro-Caribbeans compared with white Europeans [adjusted HR = 1.96 (95% CI: 1.09, 3.51) (P = 0.02)], and did not differ for North Africans. In contrast, overall survival was not significantly different according to ethnicity. Conclusion: Our findings indicated different GPA clinical presentations in white Europeans and sub-Saharans and Afro-Caribbeans, with black patients having more frequent severe granulomatous manifestations. In addition, time to relapse was significantly shorter for black sub-Saharans and Afro-Caribbeans compared with white Europeans.

Lumbar Spinal Stenosis Treatment: Is Surgery Better than Non-Surgical Treatments in Afro-Descendant Populations?

(1) Background: Limited data are available on lumbar spine stenosis management in sub-Saharan African populations and Afro-descendant patients are underrepresented in European and US clinical trials. We aimed to compare the clinical response between decompressive surgery and conservative treatments in a population of self-reported Afro-Caribbean patients with lumbar spine stenosis over a 2-year follow-up period. (2) Methods: Prospective cohort of 137 self-reported Afro Caribbeans with lumbar spine stenosis based on clinical and radiological criteria. Patients were assigned to decompression surgery or to conservative treatments according to their outcome after a first course of steroid epidural injection and their preferences. The primary outcome was evolution of the Oswestry disability index at 3 months (3 M), 12 M, 18 M and 24 M follow-up. (3) Results: Decrease of ODI was significantly more important in the "decompression surgery" arm compared to "conservative treatment" arm at 3 M, 12 M and 18 M: −17.36 vs. 1.03 p < 10−4; −16.38 vs. −1.53 p = 0.0059 and −19.00 vs. −4.52 p = 0.021, respectively. No difference was reported at 24 M. (4) Conclusions: In this first comparative study between surgery and conservative treatments in an exclusively afro-descendant lumbar spine stenosis cohort, we report long term superiority of decompression surgery versus conservative treatments over an 18-month period.

Epidemiology and Characteristics of Spondyloarthritis in the Predominantly Afro-Descendant Population of Martinique, a French Caribbean Island.

(1) Background: The prevalence of Spondyloarthritis (SpA) varies significantly in different regions and ethnic groups due several factors such as heterogeneity in study populations, the diversity of classification criteria used in epidemiological studies, the prevalence variability of HLA-B27 or disparity in healthcare access. To our knowledge, there is no data on SpA in Martinique, a French region in the Caribbean with a predominantly Afro-descendant population and a high level of healthcare. (2) Methods: This was a retrospective study of all SpA patients treated at the Fort de France University Hospital between 1 January 1997 and 1 January 2008. (3) Results: In our cohort of 86 SpA patients, age at diagnosis was late (41 years old), ankylosing spondylitis (AS) was the most frequent sub-type (60.5%), inflammatory bowel disease was the most frequent extra articular feature (23.3%) and no one had personal familial history of the disease. Inflammatory syndrome concerned 55.6% of patients, no one was positive for HIV and HLA-B27 positivity was low (42.2%). However, HLA-B27 was statistically associated with AS. Out of 64 patients, 41 had sacroiliitis. (4) Conclusion: To our knowledge, this is the first comprehensive descriptive study of SpA subtypes in Martinique, a French region in the Caribbean. We report clinical and biological similarities in our SpA cohort with those of sub-Saharan Africa and with SpA subtypes reported in Afro-descendant populations.

Prevalence of rheumatoid arthritis in the French West Indies: Results of the EPPPRA study in Martinique.

OBJECTIVES: Studies suggest that rheumatoid arthritis (RA) is less frequent in African populations. However, no recent precise data exists for Afro-Caribbeans. The EPPPRA project is a prospective epidemiological survey to describe prevalence and clinical aspects of RA in the French West Indies (Martinique, Guadeloupe, French Guiana). METHODS: EPPPRA involved all rheumatologists from the French West Indies who included all patients with a known clinical diagnosis of RA, during a one-year period. We outline here results for Martinique. RESULTS: EPPPRA estimated an overall world age-standardized prevalence of RA at 0.10% [95% CI 0.09% to 0.11%] in Martinique, with a high female predominance (88.1%) and 93.1% of self-reported Afro-Caribbeans. Mean age at diagnosis was 49.6±16.0 years. A majority of subjects presented at least 4 criteria points from the 1987 American College of Rheumatology (ACR) classification (94.4%) and at least 6 points (78.2%) from the 2010 ACR/European League Against Rheumatism (EULAR) classification. A high immune seropositivity rate was highlighted (84.2%). Despite functional impact observed in 40.5% of patients, 71.4% presented a low disease activity level. Methotrexate was the most common ongoing treatment (73%), followed by biotherapies (24.4%). Numerous patients (68.6%) received a steroid regimen. Cardiovascular risk factors were very frequent, contrasting with a very low tobacco use (8.7%), CONCLUSION: This work outlines low standardized prevalence of RA in a French Afro-Caribbean population with specific characteristics (high female predominance, high immune seropositivity, low tobacco use). Despite easy access to care and biotherapies, approximately half of RA patients still present destructive disease with functional impact.

Epidemiology and Characteristics of Kikuchi-Fujimoto Disease in the African-Descent Population of Martinique, French West Indies.

OBJECTIVE: To provide an epidemiologic description of Kikuchi-Fujimoto disease (KFD), and to describe its relationship with systemic lupus erythematosus (SLE) in a population of sub-Saharan origin. METHODS: Patients were retrospectively included on the basis of lymph node histology compatible with KFD reported in Martinique from 1991 until 2013. In order to describe the characteristics of the disease in a larger cohort, we subsequently included more patients of Afro-Caribbean origin from Guadeloupe and French Guiana. RESULTS: In Martinique, mean annual incidence between 1991 and 2013 was 2.78 cases for 1 million inhabitants (95% confidence interval 1.73-3.93). A total of 36 Afro-Caribbean patients from the 3 French American regions were included. Mean age was 30.5 years (range 5-59 years) and the female:male ratio was 3:1. The main characteristics were cervical adenopathies (88.8%), fever (83.3%), asthenia (73.0%), weight loss (64.4%), and recurrence in 33.3%. KFD was associated with lupus (n = 9 for SLE, n = 2 for cutaneous lupus) in 36.6% (11 of 30). CONCLUSION: We report the first epidemiologic description of KFD in a population of sub-Saharan origin. According to our data, this disease is present in the black African diaspora and is strongly associated with autoimmune diseases, particularly lupus.

Brief Report: Management of Chronic Post-Chikungunya Rheumatic Disease: The Martinican Experience.

OBJECTIVE: To describe chronic chikungunya manifestations seen during the outbreak in the Caribbean from December 2013 to January 2015. METHODS: Patients were seen at our center, the only rheumatology department in Martinique Island, and were examined by a senior rheumatologist using a standard care report form. Chikungunya was diagnosed collectively based on consensus among all clinicians. The median time from onset of acute chikungunya to the first rheumatology consultation was calculated, severity was evaluated based on clinical scales and the degree of joint destruction, and each patient's treatment was recorded. RESULTS: For the 147 patients analyzed, the median time between onset of acute chikungunya and the first rheumatology consultation was 8 months. After review of each patient's medical record, 19 (12.9%) were diagnosed as having epidemic-influenced chikungunya. Four distinct rheumatologic patterns were observed in the remaining patients (those with compatible history and positive serologic findings): 47 patients (32%) had reactivation of painful chronic mechanical manifestations, 9 patients (6.1%) had fibromyalgia, 45 patients (30.6%) met criteria for spondyloarthritis (as evaluated before the chikungunya virus infection in all patients) and experienced a flare, and 27 patients (18.4%), with no history of joint disease, developed de novo bilateral symmetric chronic inflammatory joint disease in response to chikungunya virus infection. For inflammatory arthritis, most patients were treated with methotrexate (up to 25 mg/week), with good response and tolerance. Thirteen patients were treated with conventional doses of anti-tumor necrosis factor agents, with good tolerance and efficacy as expected. CONCLUSION: The term "chronic chikungunya syndrome" covers multiple etiologies. Compliance with the French Society of Rheumatology recommendations, careful recording of patient histories, and serologic verification help prevent errors inherent to the epidemic context and ensure early therapeutic intervention for these patients. To avoid late initiation of treatment, patients should receive rheumatologic consultation as early as possible.

Increased proviral load in HTLV-1-infected patients with rheumatoid arthritis or connective tissue disease.

BACKGROUND: Human T-lymphotropic virus type 1 (HTLV-1) proviral load is related to the development of HTLV-1-associated myelopathy/tropical spastic paraparesis (HAM/TSP) and has also been shown to be elevated in the peripheral blood in HTLV-1-infected patients with uveitis or alveolitis. Increased proliferation of HTLV-1-infected cells in, or migration of such cells into, the central nervous system is also seen in HAM/TSP. In the present study, we evaluated the proviral load in a cohort of HTLV-1-infected patients with arthritic conditions. RESULTS: HTLV-1 proviral load in the peripheral blood from 12 patients with RA and 6 patients with connective tissue disease was significantly higher than that in matched asymptomatic HTLV-1 carriers, but similar to that in matched HAM/TSP controls. HAM/TSP was seen in one-third of the HTLV-1-infected patients with RA or connective tissue disease, but did not account for the higher proviral load compared to the asymptomatic carrier group. The proviral load was increased in the synovial fluid and tissue from an HTLV-1-infected patient with RA, the values suggesting that the majority of infiltrated cells were HTLV-1-infected. In the peripheral blood from HTLV-1-infected patients with RA or connective tissue disease, HTLV-1 proviral load correlated with the percentages of memory CD4+ T cells and activated T cells, and these percentages were shown to be markedly higher in the synovial fluid than in the peripheral blood in an HTLV-1-infected patient with RA. CONCLUSIONS: These biological findings are consistent with a role of the retrovirus in the development of arthritis in HTLV-1-infected patients. A high level of HTLV-1-infected lymphocytes in the peripheral blood and their accumulation in situ might play a central role in the pathogenesis of HTLV-1-associated inflammatory disorders. Alternatively, the autoimmune arthritis, its etiological factors or treatments might secondarily enhance HTLV-1 proviral load.

[Peripheral facial paralysis in a patient infected with HTLV-1 virus. 2 case reports]. / Paralysie faciale périphérique chez des patients infectés par le virus HTLV-1. Deux observations.

INTRODUCTION: Facial nerve palsy is a frequent situation of varying etiology and the former concept of frozen facial nerves is progressively being abandoned. The HTLV-1 virus, endemic to some tropical areas, is one of the most recent causes discovered. OBSERVATIONS: We present two HTLV-1 seropositive patients of Caribbean origin who developed facial palsy and several complications related to this retrovirus. COMMENTS: When confronted with facial palsy, the presence of risk factors for HTLV-1 infection (particularly when native in high seroprevalence areas) must lead to serological examinations in the search for this retrovirus.

Dengue fever in patients under biologics.

Dengue fever (DF) is an epidemic viral mosquito-borne infection limited to tropical and subtropical countries. Biological therapies have been frequently used for the last 15 years in the treatment of inflammatory rheumatic conditions like rheumatoid arthritis. However, no data is available regarding the characteristics of this infection in patients on biological therapy. Yet, numerous patients on biotherapy have holidays in countries where DF exists. Moreover, the mosquitoes Aedes albopictus, vector of this viral disease, is now found in some developed countries such as southern Europe and the USA, allowing the possibility of a DF outbreak. We conducted a survey of individuals on biotherapy and described a case series of the patients experiencing DF. Our 8 patients on biotherapy (anti-TNF, n=6; rituximab, n=2) for a rheumatic condition did not experience severe DF.

Incidence of chronic cutaneous lupus erythematosus in French Guiana: a retrospective population-based study.

OBJECTIVE: To retrospectively study the incidence of chronic cutaneous lupus erythematosus (CCLE) in French Guiana (FG), South America, during the period 1995-1999. METHODS: Private and public physicians specializing in dermatology, rheumatology, and internal medicine were asked during the year 2000 about lupus cases. We reviewed hospitals' files in data-processing departments. RESULTS: Twenty new cases of CCLE, mostly discoid form, were identified during this 5-year period in this population of predominantly African descent. The average annual incidence of the disease was 2.59 per 100,000 inhabitants (95% confidence interval 1.5-4). However, our methodology could introduce underestimation of the incidence of the disease. CONCLUSION: The average annual incidence of CCLE in FG appears to be low in this retrospective study, but is very similar to the only previously published data in the US.

New onset of myasthenia gravis after treatment of systemic sclerosis by autologous hematopoietic stem cell transplantation: sustained autoimmunity or inadequate reset of tolerance?

Autologous hematopoietic stem-cell transplantation (HSCT) showed promising results for the treatment of primary severe autoimmune diseases (ADs). In this context, development of secondary AD after HSCT has exceptionally been observed, further questioning the roles of patient propensity for AD and of the HSCT procedure. Herein, we report new onset of myasthenia gravis 3 years after successful HSCT in a patient with severe systemic sclerosis, while in complete remission from her first AD. The de novo occurrence of secondary AD (myasthenia gravis) after HSCT was accompanied by the appearance of clonal T-cell expansions measured by the "immunoscope" technique in the context of an ongoing T-cell immune reconstitution. Secondary ADs are increasingly recognized after HSCT for AD. In our case, development of myasthenia followed clonal T-cell expansion. Detailed T-cell repertoire analysis may shed light on autoreactivity mechanisms after HSCT and may help to identify patients at risk.