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Systemic sclerosis (SSc) is a connective tissue disease characterized by extensive fibrosis of the skin and internal organs, associated with vasculopathy and autoimmune features. Antinuclear antibodies (ANA) are found in almost all SSc patients and constitute strong diagnosis and prognosis biomarkers. However, it remains unclear whether ANA are simple bystanders or if they can have a role in the pathophysiology of the disease. One might think that the nuclear nature of their targets prevents any accessibility to autoantibodies. Nevertheless, recent data suggest that ANA could be pathogenic or at least contribute to the perennation of the disease. We review here first the indirect clues of the contribution of ANA to SSc: they are associated to the disease subtypes, they may precede disease onset, their titer correlates with disease activity and severity, there is an association between molecular subsets, and some patients can respond to B-cell targeting therapy. Then, we describe in a second part the mechanisms of ANA production in SSc from individual genetic background to post-transcriptional modifications of neoantigens. Finally, we elaborate on the potential mechanisms of pathogenicity: ANA could be pathogenic through immune-complex-mediated mechanisms; other processes potentially involve molecular mimicry and ANA penetration into the target cell, with a focus on anti-topoisomerase-I antibodies, which are the most probable candidate to play a role in the pathophysiology of SSc. Finally, we outline some technical and conceptual ways to improve our understanding in this field.

Objective Elevated inflammatory markers play a crucial role in the diagnosis and follow-up of patients with giant cell arteritis (GCA). This study aimed to describe the prevalence, characteristics, and outcomes of patients with low baseline (< 10 mg/L) C-reactive protein (CRP) in GCA. Methods A retrospective observational study was conducted at Lille University Hospital, involving all patients diagnosed with GCA between January 2000 and April 2023. Patients were categorized based on their CRP level at diagnosis. Baseline characteristics, clinical manifestations, laboratory findings, imaging results, and outcomes were compared between patients with baseline CRP < 10 mg/L (“low CRP”) and those with CRP ≥ 10 mg/L (“high CRP”). Results Of the 380 patients, 7.6% ( n  = 29) had baseline CRP < 10 mg/L at diagnosis. When compared to the high CRP group, the low CRP group exhibited a lower incidence of fever, and had a higher incidence of ocular involvement, particularly anterior ischemic optic neuropathy (28% vs. 13%, p  = 0.04), and limb claudication (24% vs. 8%, p  < 0.01). Plasma fibrinogen levels were elevated (> 4 g/L) in 77% of patients with low CRP. Despite differences in clinical presentation, relapse rates were equilibrated between the two groups. Conclusion GCA patients with low CRP are not rare and present with more ocular and peripheral vascular involvement and less constitutional symptoms in our study. Elevated fibrinogen in these patients suggests active inflammation despite low CRP. Clinicians should consider GCA even with a CRP < 10 mg/L, as these patients may present with severe complications.

Objective Describe the demographic data and clinical phenotype of cranial palsy induced by immune checkpoint inhibitors (CNP-ICI). Methods A systematic literature review of the literature was performed in Pubmed, Web of Science, and Embase, including 68 articles and 136 patients (PROSPERO no. CRD42024517262). Results Out of the 1205 articles screened, 68 articles were included after fulfilling the inclusion criteria, for a total of 136 patients. All articles were case reports and case series. In the cohort studied, 52% of patients were treated with anti PD-1/PDL-1 therapies, 14% with anti CTLA-4 therapies, and 34% with a combination of anti CTLA-4 and anti PD-1/PDL-1 therapies. The facial nerve was the most affected cranial nerve, involved in 38% of cases, followed by the optic nerve (35%), the cochleovestibular nerve (12%), and the abducens nerve (10%). The median time from the initial immune checkpoint inhibitor (ICI) injection to the onset CNP-ICI was 10 weeks (IQR 4–20). Magnetic resonance imaging demonstrated contrast enhancement or abnormal signal of the affected nerve in 43% of cases. Cerebrospinal fluid analysis indicated lymphocytic pleocytosis in 59% of cases. At the onset of immune-related adverse events, 89% of patients discontinued immunotherapy, and 92% received treatment for CNP-ICI. Treatment regimens included corticosteroids in 86% of cases, intravenous immunoglobulin in 21%, and plasma exchange in 5.1%. Among the whole population, 33% achieved recovery, 52% showed clinical improvement, 16% remained stable, and 3% experienced worsening of their condition. Rechallenge with immunotherapy was significantly associated with the emergence of new immune-related Adverse Events (irAEs). Conclusion ICI therapy may lead to cranial nerve involvement, particularly affecting the facial nerve, typically presenting around 10 weeks after treatment initiation. While corticosteroid therapy often resulted in patient improvement, rechallenging with ICIs were associated with new irAEs.

Objectives To investigate lung perfusion in systemic sclerosis (SSc). Methods The study population included 101 patients who underwent dual-energy CT (DECT) in the follow-up of SSc with pulmonary function tests obtained within 2 months. Fifteen patients had right heart catheterization–proven PH. Results Thirty-seven patients had no SSc-related lung involvement (Group A), 56 patients had SSc-related interstitial lung disease (Group B) of variable extent (Group B mild: ≤ 10% of lung parenchyma involved: n = 17; Group B moderate: between 11 and 50%: n = 31; Group B severe: > 50%: n = 8), and 8 patients had PVOD/PCH (Group C). Lung perfusion was abnormal in 8 patients in Group A (21.6%), 14 patients in Group B (25%), and 7 patients in Group C (87.5%). In Group A and Group B mild ( n = 54), (a) patients with abnormal lung perfusion ( n = 14; 26%) had a higher proportion of NYHA III/IV scores of dyspnea (7 [50%] vs 7 [17.5%]; p = 0.031) and a shorter mean walking distance at the 6MWT (397.0 [291.0; 466.0] vs 495.0 [381.0; 549.0]; p = 0.042) but no evidence of difference in the DLCO% predicted (61.0 [53.0; 67.0] vs 68.0 [61.0; 78.0]; p = 0.055) when compared to patients with normal lung perfusion ( n = 40; 74%); (b) a negative correlation was found between the iodine concentration in both lungs and the DLCO% predicted but it did not reach statistical significance ( r = −0.27; p = 0.059) and no correlation was found with the PAPs ( r = 0.16; p = 0.29) and walking distance during the 6MWT ( r = −0.029; p = 0.84). Conclusions DECT lung perfusion provides complementary information to standard HRCT scans, depicting perfusion changes in SSc patients with normal or minimally infiltrated lung parenchyma. Key Points • In a retrospective observational study of 101 consecutive patients with SSc, dual-energy CT pulmonary angiography was obtained to evaluate lung perfusion. • Lung perfusion was abnormal in 14 out of 54 patients (26%) with no or mild SSc-related lung infiltration. • Patients with abnormal perfusion and no or mild SSc-related lung infiltration had more severe scores of dyspnea and shorter walking distance than patients with similar lung findings and normal perfusion, suggesting the presence of small vessel vasculopathy.

ELAD USSRY \"Art of Two Germanys\" (Los Angeles County Museum of Art) Aside from this show's sociopolitical importance, and how carefully It travels through the complicated and layered history of twentieth-century Germany, what really won me over was seeing many of my favorite contemporary artists - Genzken, Trockel, Schütte, the Bechers, Palermo, Farocki, and Polke - under one roof and contextualized by their historical antecedents in a single exhibition. Los Angeles Lying on the carpet in the back corner of a dark screening room, hiding from other museum visitors and absorbing the shot of Black Flag's Henry Rollins writhing onstage at the beginning of Rock My Religion, 1982-84, 1 thought: FRED WILSON Nick Cave, \"Recent Soundsults\" (Jack Shainman Gallery, New York) Cave's otherworldly costumed characters - some festooned with toys, ceramic birds, and beaded fabric, some covered with human hair in vibrant colors - made an oddly comfortable contrast with his sculptures incorporating lawn jockeys, that genre of antique racist American statuary which is also now classified blandly (and without a hint of irony) under the rubric \"black collectibles.\"