Tolerance and efficacy of targeted therapies prescribed for off-label indications in refractory systemic autoimmune diseases: data of the first 100 patients enrolled in the TATA registry (TArgeted Therapy in Autoimmune Diseases).

OBJECTIVES: To assess the tolerance and efficacy of targeted therapies prescribed off-label in refractory low-prevalence autoimmune and inflammatory systemic diseases. METHODS: The TATA registry (TArgeted Therapy in Autoimmune Diseases) is a prospective, observational, national and independent cohort follow-up. The inclusion criteria in the registry are as follows: age >18 years; low-prevalence autoimmune and inflammatory systemic disease treated with off-label drugs started after 1 January 2019. RESULTS: Hundred (100) patients (79 women) were enrolled. The median age was 52.5 years (95% CI 49 to 56) and the median disease duration before enrolment was 5 years (3 to 7). The targeted therapies at enrolment were as follows: Janus kinase/signal transducers and activators of transcription inhibitors (44%), anti-interleukin (IL)-6R (22%), anti-IL-12/23, anti-IL-23 and anti-IL-17 (9%), anti-B cell activating factor of the tumour necrosis factor family (5%), abatacept (5%), other targeted treatments (9%) and combination of targeted treatments (6%). 73% of patients were receiving corticosteroid therapy at enrolment (median dose 10 mg/day). The current median follow-up time is 9 months (8 to 10).Safety: 11 serious infections (incidence rate of 14.8/100 patient-years) and 1 cancer (1.3 cancers/100 patient-years) were observed. Two patients died from severe COVID-19 (2.7 deaths/100 patient-years).Efficacy: the targeted treatment was considered effective by the clinician in 56% of patients and allowed, in responders, a median reduction of oral corticosteroids of 15 (9 to 21) mg/day, below 7.5 mg/day in 76% of patients, while 28% discontinued. CONCLUSION: These initial results of the TATA registry confirm the diversity of targeted treatments prescribed off-label in refractory autoimmune diseases and their corticosteroid-sparing effect when effective. Tolerance was acceptable in these refractory patients with a long history of treatment with immunosuppressive drugs.

Utilization of preventive services in a systemic lupus erythematosus population-based cohort: a Lupus Midwest Network (LUMEN) study.

BACKGROUND: Systemic lupus erythematosus (SLE) is a disease that can lead to damage of multiple organs and, along with certain treatments, increase the risk of developing cancer, cardiovascular disease, diabetes, osteoporosis, and infections. Preventive services are particularly important in patients with SLE to mitigate the aforementioned risks. We aimed to evaluate the trends of preventive services utilization in patients with systemic lupus erythematosus, compared with non-SLE population. METHODS: All ≥19-year-old patients in the Lupus Midwest Network (LUMEN) registry, a population-based cohort, with SLE on January 1, 2015, were included and matched (1:1) by sex, age, race, and county to non-SLE comparators. Among both groups, we compared the rates of screenings for breast and cervical cancer, hypertension, hyperlipidemia, diabetes mellitus, and osteoporosis as well as immunizations. RESULTS: We included 440 SLE patients and 430 non-SLE comparators. The probability of breast cancer screening among women with SLE was similar to comparators (hazard ratio [HR] 1.09, 95% CI 0.85-1.39), while cervical cancer screening was lower (HR 0.75, 95% CI 0.58-0.96). Hypertension screening was higher among patients with SLE (HR 1.35, 95% CI 1.13-1.62); however, hyperlipidemia screening was similar to comparators (HR 1.16, 95% CI 0.96-1.41). Diabetes and osteoporosis screenings were more likely to be performed for SLE patients than for comparators (HR 2.46, 95% CI 2.11-2.87; and HR 3.19, 95% CI 2.31-4.41; respectively). Influenza and pneumococcal immunizations were higher among SLE patients (HR 1.31, 95% CI 1.12-1.54; and HR 2.06, 95% CI 1.38-3.09; respectively), while zoster vaccination was similar (HR 1.17, 95% CI 0.81-1.69). CONCLUSIONS: The trends of utilization of preventive services by SLE patients vary according to screening or vaccine compared with the general population. Considering these differences, we demonstrate an opportunity for improvement, particularly in cervical cancer, hyperlipidemia, and osteoporosis screenings and vaccinations.

Épidémiologie de l'atteinte dermatologique dans le syndrome de Sjögren : données provenant de trois populations françaises de Syndrome de Sjogren primitif (TEARS, ASSESS, diapSS)

• L'atteinte cutanée est habituelle dans le syndrome de Sjögren primitif (SSp), mais la prévalence et les caractéristiques sont difficiles à établir. • L'atteinte cutanée la plus courante du SSp est la sécheresse cutanée (évaluée par l'EVA). • D'autres signes dermatologiques du SSp sont rares, mais sont associés à l'activité de l'ESSDAI dans d'autres domaines. Déterminer la prévalence et les caractéristiques de l'atteinte dermatologique dans le syndrome de Sjögren primitif (SSp). Nous avons utilisé 2 cohortes françaises de SSp (ASSESS, qui a évalué la prévalence des atteintes cutanées chez 395 patients atteints de SSp ;et diapSS, dans laquelle 76 sur 139 patients atteints de SSp ont été examinés par un dermatologue) et les données de base de l'essai randomisé TEARS (110 patients atteints de SSp récents ou actifs traités avec du rituximab ou un placebo et évalués pour la sécheresse de la peau à l'aide d'une échelle visuelle analogique (EVA) sur 100). Les manifestations cutanées incluses dans l'indice d'activité EULAR du syndrome de Sjögren (ESSDAI) étaient rares dans la cohorte ASSESS (n = 16/395, 4,1 %, principalement des purpuras ;seuls 3 avaient une activité élevée), mais elles étaient associées à une activité dans les autres domaines de l'ESSDAI (système neurologique périphérique (p < 0,001), musculaire (p < 0,01), hématologique (p < 0,05), biologique (p < 0,05), antécédents d'arthrite (p < 0,01), splénomégalie (p < 0,05) et taux de gammaglobulines plus élevés (p < 0,01)). Dans la cohorte diapSS, comparés aux patients SSp ne recevant pas de consultation dermatologique, les patients SSp qui ont eu une consultation dermatologique avaient significativement plus d'atteintes dermatologiques en dehors du score ESSDAI (38,2 % (29/76) contre 15,9 % (10/63) ;p < 0,01). L'étude TEARS a montré une forte prévalence de la sécheresse cutanée (EVA≥50 ;48, 2 %) et a constaté que les patients ayant la peau sèche présentaient des scores EVA de douleur et de sécheresse globale plus élevés (p < 0,01 et p < 0,001 respectivement). Les signes dermatologiques inclus dans le score ESSDAI sont rares. Ces atteintes cutanées sont associées à l'activité de l'ESSDAI dans divers domaines notamment à l'hypergammaglobulinémie et à l'activité globale de l'ESSDAI. L'examen dermatologique systématique est instructif pour les lésions cutanées non-spécifiques. L'atteinte cutanée la plus fréquente est la sécheresse cutanée, qui est associée à une douleur plus forte et à une sécheresse subjective globale plus importante. [ FROM AUTHOR] Copyright of Revue du Rhumatisme is the property of Elsevier B.V. and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full . (Copyright applies to all s.)