Thrombose coronaire dans le syndrome des anticorps antiphospholipides (à propos d'une série rétrospective de 25 cas personnels)

LYON1-BU Santé (693882101) / SudocPARIS-BIUM (751062103) / SudocSudocFranceF

Long-term visual and systemic prognoses of 83 cases of biopsy-proven sarcoid uveitis

International audienceAIMS: To determine the long-term visual and systemic outcomes of uveitis patients with biopsy-proven sarcoidosis. METHODS: A retrospective study of biopsy-proven sarcoid uveitis, with a 3-year minimum follow-up, seen at Lyon University Hospital, between April 2004 and January 2016. RESULTS: A total of 83 patients were included, with a median age at onset of 52 (37-62) years and an unbalanced gender ratio (women 77.1%). Thirty-one patients had original systemic sarcoidosis in addition to ocular localisation, whereas 52 initially presented with isolated sarcoid uveitis. Among the latter, 7.7% (n=4) developed an extraocular disease after a median follow-up duration of 60 (44-110) months. The systemic spread in these patients included cutaneous sarcoids (n=2), arthritis (n=1) and multiple mononeuritis (n=1). Complete visual recovery was obtained for 60.2% of all patients and 89.2% had retrieved best-corrected visual acuity (BCVA) \\textgreater20/50 in both eyes. A unilateral loss of BCVA of worse than 20/200 was documented in two patients in the isolated sarcoid uveitis group. No patient suffered from bilateral severe visual impairment or blindness. Factors linked to a poor visual prognosis, defined by BCV

Cardiac Sarcoidosis Is Uncommon in Patients with Isolated Sarcoid Uveitis: Outcome of 294 Cases

International audienceRecently, concerns have been raised about an increased risk of cardiac sarcoidosis in patients with sarcoid uveitis. While cardiac sarcoidosis has a high mortality burden, there is still a lack of precise data on this association. The objective of this study is to describe the frequency and type of cardiac complications associated with sarcoidosis of a large cohort of patients with sarcoid uveitis. We analyzed the cardiac outcomes of a monocentric retrospective cohort of consecutive adults with a diagnosis of sarcoid uveitis between January 2004 and March 2020 in a tertiary French university hospital. A total of 294 patients with a final diagnosis of sarcoid uveitis were included. At final follow-up, seven (2.4%) patients of the cohort had cardiac sarcoidosis. Cardiac sarcoidosis was more frequent among patients with previously reported systemic sarcoidosis (p = 0.008). The prevalence of cardiac sarcoidosis among patients with sarcoid uveitis is low, but patients with previously diagnosed sarcoidosis or those who develop systemic sarcoidosis during follow-up appear to be at increased risk

BTNL2 gene polymorphism and sarcoid uveitis

International audienceBackground Uveitis is a frequent and early feature of sarcoidosis. As BTNL2 (butyrophilin-like 2) gene polymorphism was found linked with the susceptibility to sarcoidosis, we investigated whether a specific genotype of BTNL2 gene G16071A (or rs2076530) single-nucleotide polymorphism (SNP) would be associated with the risk of sarcoid uveitis in all patient subgroups. Methods The study compared the genotype frequencies of SNP G16071A of 135 patients with sarcoid uveitis (Sa + Uv + ) with those of 196 patients with sarcoidosis without uveitis (Sa + Uv − ), 81 patients with uveitis without sarcoidosis (Sa − Uv + ), and 271 controls with no sarcoidosis nor uveitis (Sa − Uv − ). Three hypothetical subgroups of patients with sarcoid uveitis (Sa + Uv + cases) were considered: (1) subgroup I: patients aged 45 years; and (3) subgroup III: all other patients. Results A statistically significant difference in genotype frequencies was found between the groups Sa + Uv − and Sa − Uv − (p=3.2×10 −6 ) and between the groups Sa + Uv + and Sa + Uv − (p=7.1×10 −3 ). There was no difference between the three subgroups of Sa + Uv + patients. There was a statistically significant difference in genotype frequencies between Sa + Uv − and Sa + Uv + subgroup II (p=0.005) but no difference between Sa + Uv − and Sa + Uv + subgroup I. Conclusion No association was found between G16071A and the susceptibility to sarcoid uveitis. BTNL2 gene G16071A SNP seems to be a predisposing factor for sarcoidosis except in Caucasian postmenopausal women with sarcoid uveitis in whom the GG genotype prevails. These and future results will help in understanding differences between particular subgroups of patients with sarcoid uveitis

Factors Associated with Ocular and Extraocular Recovery in 143 Patients with Sarcoid Uveitis

International audienceBackground: Sarcoidosis is one of the leading causes of uveitis. To date, no studies have assessed the factors specifically related with recovery in ocular sarcoidosis. In this study, we aimed to determine factors associated with ocular and extraocular recovery in patients with sarcoid uveitis.Methods: A retrospective study of sarcoid uveitis, with a three-year minimum follow-up in Lyon University Hospital between December 2003 and December 2019. Patients presented biopsy-proven sarcoidosis or presumed sarcoid. Recovery was defined by a disease-free status, spontaneously or despite being off all treatments for three years or more.Results: 143 patients were included: 110 with biopsy-proven and 33 with presumed sarcoid uveitis. Seventy-one percent were women, the median age at presentation was 53 years, and 71% were Caucasian. Chronic uveitis was the main clinical presentation (75%), mostly panuveitis (48%) with bilateral involvement (82%). After a median follow-up of 83.5 months, recovery was reported in 26% of patients. In multivariable analysis, Caucasian ethnicity (p = 0.007) and anterior uveitis (p = 0.008) were significantly associated with recovery, while increased intraocular pressure was negatively associated (p = 0.039).Conclusion: In this large European cohort, one quarter of patients recovered. Caucasian ethnicity and anterior uveitis are associated with ocular and extraocular recovery

Quality of life in patients with uveitis: data from the ULISSE study (Uveitis: cLInical and medico-economic evaluation of a Standardised Strategy for the Etiological diagnosis)

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Infection Risk in Patients with Dermatomyositis Associated with Anti-MDA5 Antibodies: A Historical Cohort Study

International audienceObjective: Dermatomyositis associated with anti-MDA5 autoantibodies (DM-MDA5+) is a rare autoimmune disease usually characterized by skin involvement, often-severe lung involvement, and general features. Several reports of infections have been described, sometimes early after the introduction of immunosuppressive therapy. We studied the infection risk in a DM-MDA5+ population. Methods: A retrospective cohort study comparing the number and type of infections during the follow-up of 19 patients with DM-MDA5+ and 37 patients with another type of inflammatory myopathy was analyzed. Patients in both groups were matched on initial immunosuppressive therapy. We described and compared significant infectious complications (SIC) in each group. Results: Patients DM-MDA5+ had more SIC: 27 events in the DM-MDA5+ group versus 6 in the controls (HR 7.08, 95% CI 2.50–20.04, p < 0.0001). The number of SIC per patient was higher in DM-MDA5+ (1.4 ± 1.57 vs. 0.16 ± 0.44, p < 0.001). These were mainly lung (n = 13, 48%) and skin infections (n = 6, 22%), more often infections of an undetermined infectious agent (n = 11, 41%) or of bacterial origin (n = 9, 33%). A few cases of opportunistic infections were reported. The median duration of follow-up without SIC event in the DM-MDA5+ cohort was 3.5 months. Conclusion: Patients with DM-MDA5+ have an increased infection risk compared to others inflammatory myopathies irrespective of immunosuppressive therapy exposure. These results highlight the importance of monitoring for infection during patient follow-up

Place of a new radiological index in predicting pulp exposure before intervention for deep carious lesions

International audienceBackground During interventions for deep caries lesions without severe symptoms, preserving pulpal vitality is important to ensure treatment success, improve organ prognosis, and decrease cost-effectiveness. Current pre-operative radiographs allow visual estimation but not accurate measurement of lesion depth. Purpose Investigate the ability of ratio 'remaining/total dentin thickness' (RDT/TDT, as determined on pre-operative radiographs) to predict pulp exposure during excavation. Methods This retrospective study (January 2018-June 2020) analyzed data on 360 patients. Four independent raters examined standard pre-operative radiographs and their contrasted versions. Lines put at the dentino-enamel junction, the floor of the carious lesion, and the pulp chamber wall allowed deriving RDT/TDT. Inter-rater agreements and concordance were assessed. A logistic regression accounting for measurement errors provided odds ratios that estimated the ability of the RDT/TDT to predict pulp exposure. Results The median RDT/TDT ratio ranges were 16.8-26.5% on standard and 16.2-24.6% on contrasted radiographs. Inter-rater agreements on RDT/TDT were rather poor and inter-rater reliability was low and similar in standard and contrasted radiographs: the concordance correlation coefficients (95% CIs) were estimated at 0.46 (0.40; 0.51) and 0.46 (0.40; 0.52), respectively. The risk of pulp exposure increased by 2.5 times [odds ratio (95% CI) 2.57 (2.06; 3.20)] per 10-point decrease of the ratio on standard radiographs vs. 4.15 (3.15; 5.46) on contrasted radiographs. Conclusion RDT/TDT ratio is potentially helpful in predicting pulp exposure. However, the measurement errors on RDT and TDT being non-negligible and the interrater agreements poor, there is still place for advances through development of an automated process that will improve reliability and reproducibility of pulp exposure risk assessment

Comment on: Inaugural dropped head syndrome and camptocormia in inflammatory myopathies: a retrospective study: Reply

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Inaugural dropped head syndrome and camptocormia in inflammatory myopathies: a retrospective study

International audienceObjectives: Inaugural axial muscle involvement, defined as dropped head syndrome (DHS) and/or camptocormia (CC), is poorly described in inflammatory myopathies (IM). This study aimed to further characterize IM patients with inaugural DHS/CC, their outcome and care management.Methods: This retrospective study included IM patients diagnosed between 2000 and 2021. The main inclusion criterion was IM revealed by axial muscle deficit (DHS/CC).Results: Twenty-seven patients were included; median (IQR) age at first symptoms was 66.0 years (55.5–75.0); 21 were female (77.8%). There were nine IBM, 33.3%, nine overlap myositis (OM, 33.3%), five DM, 18.5%, two immune checkpoint inhibitor-related myositis (7.4%), one focal myositis (3.7%) and one myositis with anti-Hu antibodies (3.7%). Age at first symptoms was ≤70 years in 16 patients (59.3%), including all DM patients and 8/9 OM patients (88.9%). In this group, partial remission of the disease was obtained in 9/16 (56.3%) and complete remission in 1/16 patients (6.3%); regression of DHS/CC was achieved in 3/16 patients (18.8%). Conversely, in the group of 11 patients aged >70 years at first symptoms, there were eight IBM (72.7%). Partial remission was obtained in 5/11 patients (45.5%), the disease was stable in 6/11 patients (54.5%); no complete remission was obtained nor regression of DHS/CC.Conclusion: The analysis of IM patients with inaugural DHS/CC delineates two groups of patients according to the age at first symptoms in terms of clinical and outcome specificities, and proposes an adapted diagnostic and care management approach to prevent long-term complications