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Objectives: To investigate factors associated with dermatomyositis (DM) complete clinical response and overall survival with a focus on the use of immunosuppressive therapies in patients with cancer-associated DM., Methods: We performed a multicentre, retrospective cohort study. Multivariable survival analyses used a Cox model with time-dependent covariates and adjustments with inverse probability censoring weighting., Results: We included 73 patients with cancer-associated DM. Median follow-up was 3.92 years. Overall, 40 (54.8%) patients achieved cancer remission, with DM complete clinical response in 28/40 (70.0%). DM complete clinical response was associated with cancer remission (hazard ratio [HR] 2.46, 95%CI [1.13-5.32]) and younger age (HR 0.68, 95%CI [0.49-0.95]). Risk of mortality was associated with sustained cancer activity (HR 12.93, 95% CI [2.42-69.25]), male sex (HR 2.82, 95% CI [1.19-6.70]), and older age (HR 1.86, 95% CI [1.26-2.79]) but not sustained DM activity (HR 0.40, 95%CI [0.13-1.26]). Oral corticosteroids use was a protective factor only on univariate analysis (HR 0.18, 95% CI [0.08-0.42])., Conclusion: This study provides strong evidence of a significant association between the evolutions of DM and cancer, both in terms of overall survival and DM complete clinical response. Immunosuppressive treatments for DM were not significantly associated with mortality., Trial Registration: ClinicalTrials.gov, NCT04637672., (© The Author(s) 2024. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For permissions, please email: journals.permissions@oup.com.)

Objectives: To describe the clinical and pathological features of biopsy-proven cutaneous vasculitis (CV) associated with SLE, focusing on diagnosis classification and impact on overall SLE activity., Methods: Retrospective multicentric cohort study including SLE patients with biopsy-proven CV identified by (i) data from pathology departments of three university hospitals and (ii) a national call for cases. SLE was defined according to 1997 revised ACR and/or 2019 ACR/EULAR criteria. CV diagnosis was confirmed histologically and classified by using the dermatological addendum of the Chapel Hill classification. SLE activity and flare severity at the time of CV diagnosis were assessed independently of vasculitis items with the SELENA-SLEDAI and SELENA-SLEDAI Flare Index., Results: Overall, 39 patients were included; 35 (90%) were female. Cutaneous manifestations included mostly palpable purpura (n = 21; 54%) and urticarial lesions (n = 18; 46%); lower limbs were the most common location (n = 33; 85%). Eleven (28%) patients exhibited extracutaneous vasculitis. A higher prevalence of Sjögren's syndrome (51%) was found compared with SLE patients without CV from the French referral centre group (12%, P < 0.0001) and the Swiss SLE Cohort (11%, P < 0.0001). CV was mostly classified as urticarial vasculitis (n = 14, 36%) and cryoglobulinaemia (n = 13, 33%). Only 2 (5%) patients had no other cause than SLE to explain the CV. Sixty-one percent of patients had inactive SLE., Conclusion: SLE-related vasculitis seems very rare and other causes of vasculitis should be ruled out before considering this diagnosis. Moreover, in more than half of patients, CV was not associated with another sign of active SLE., (© The Author(s) 2022. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For permissions, please email: journals.permissions@oup.com.)

Objective: We recently recorded a high prevalence of inclusion body myositis (IBM) in patients with Sjögren's syndrome (SS). Whether myositis patients with SS differ from myositis patients without SS in terms of the characteristics of the myositis is currently unknown. Anti-cytosolic 5'-nucleotidase 1 A (cN1A) has recently been proposed as a biomarker for IBM but is also frequent in SS. Whether anti-cN1A is independently associated with IBM is still an open question. We aimed to assess the significance of SS and anti-cN1A in myositis patients., Methods: Cumulative data on all myositis patients (EULAR/ACR 2017 criteria) screened for SS (ACR/EULAR 2016 criteria) in a single centre were analysed. Ninety-nine patients were included, covering the whole spectrum of EULAR/ACR 2017 myositis subgroups and with a median follow-up of 6 years (range 1.0-37.5). The 34 myositis patients with SS (myositis/SS+) were compared with the 65 myositis patients without SS (myositis/SS-)., Results: . IBM was present in 24% of the myositis/SS+ patients vs 6% of the myositis/SS- group (P = 0.020). None of the IBM patients responded to treatment, whether they had SS or not. Anti-cN1A was more frequent in myositis/SS+ patients (38% vs 6%, P = 0.0005), independently of the higher prevalence of IBM in this group (multivariate P value: 0.02). Anti-cN1A antibody specificity for IBM was 0.96 (95% CI: 0.87, 0.99) in the myositis/SS- group but dropped to 0.70 (95% CI: 0.48, 0.85) in the myositis/SS+ group., Interpretation: In myositis patients, SS is associated with IBM and with anti-cN1A antibodies, independently of the IBM diagnosis. As a consequence, anti-cN1A has limited specificity for IBM in myositis patients with SS., (© The Author(s) 2021. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For permissions, please email: journals.permissions@oup.com.)

Background: Only about 30 cases of borrelial lymphocytoma (BL) with identification of the causative species of Borrelia have been published to date, mainly from Eastern or Central European countries., Objectives: To identify the species of B. burgdorferi complex responsible for BL in France., Methods: Nine patients with BL acquired in France and for whom skin samples were sent to the national reference centre laboratory between 1994 and 2007 were included in this retrospective study. Direct detection of Borrelia in skin samples was made by polymerase chain reaction targeting the fla gene. Culture was performed when technically possible, and identification of each species was made by hybridization of a fragment of the fla gene with a panel of species-specific oligonucleotides., Results: Borrelia afzelii was identified in three cases, B. garinii and B. burgdorferi sensu stricto in one case each. Culture was positive in only one case (B. garinii)., Conclusions: Borrelia afzelii seems to be the predominant species of Borrelia responsible for BL in France, as already reported in other European countries.

From August 1991 to May 1993, 100 unselected consecutive patients in whom an aortic bioprosthesis was indicated underwent aortic valve replacement with the O'Brien-Angell stentless porcine xenograft (Bravo Cardiovascular Model 300). The indication was calcified aortic stenosis (AS) in 62 cases (isolated in 44, with associated cardiac lesions in 18), aortic insufficiency in 24 cases (isolated in 13 with associated cardiac lesions in 11), and redo operations in 14 cases. Forty-four percent of the patients were over 70 years of age. With the recommended supra-annular single running suture technique, the aortic cross-clamp times in isolated procedures ranged from 32 to 70 min, mean 39 min. Valve function was studied by echocardiography in 92 patients. Early post-operative transvalvular gradients were usually in the low range, below 15 mmHg in 68% of the cases. Isolated central valvular regurgitation was absent in 60.8%, "microscopic to trivial in 31.5% and mild to moderate in 1.2%. Perivalvular regurgitation was absent in 65.2%, microscopic to trivial in 26.2% and mild to moderate in 3.2%. Thirty-five of 40 patients with follow-ups exceeding 6 months had non-invasive controls. Transvalvular gradients showed a 30 to 40% reduction when compared to the immediate postoperative values. There were no new isolated central regurgitations but in two cases previously mild-to-moderate perivalvular leaks evolved to moderately severe leaks. Two valves were explanted, one for rupture of a Prolene 4/0 and one due to a slack Prolene 4/0 suture. We consider that the O'Brien-Angell stentless valve was easy to handle in all situations encountered.(ABSTRACT TRUNCATED AT 250 WORDS)