Objective: Anti-SS-B antibodies are often associated with anti-SS-A in Sjögren’s disease. Compared to anti-SS-A antibody positivity, the significance of the immunological profile anti-SS-B positive/anti-SS-A negative remains controversial. We aimed to evaluate the prevalence and diagnostic significance of isolated anti-SS-B antibodies. Methods: We conducted a retrospective study across three hospitals of the Assistance Publique—Hôpitaux de Paris. Patients with anti-SS-B positivity were identified using ELISA, addressable laser beam immunoassay (ALBIA) and immunodot assays. They were retained if anti-SS-B was positive in two techniques and anti-SS-A was absent. Clinical, biological and immunological data were extracted and presented in a descriptive analysis. Results: A total of 80 540 requests for anti-SS-B antibody testing were carried out over a period of 7.9 years. Anti-SS-B positivity was found in 1693 patients. Among them, 335 (19.8%) patients had isolated anti-SS-B in ELISA/ALBIA. Immunodot was performed in 186 of them and confirmed anti-SS-B positivity in 61 patients (3.6% of anti-SS-B positivity). 24 patients (39.3%) presented with a history of various autoimmune or autoinflammatory diseases and only 6 were diagnosed with a new connective tissue disease. After a median follow-up of 26 months, only two new diagnoses were made. Conclusion: Anti-SS-B without anti-SS-A is exceedingly rare when accurately identified by a rigorous immunological approach. The initial anti-SS-B positivity does not correlate with a specific condition, both at the time of initial identification and after a 26-month follow-up period. This supports the fact that isolated anti-SS-B has no diagnostic or prognostic value.

Objectives: Polyarteritis nodosa (PAN) is a rare necrotizing vasculitis occurring alone or associated with other conditions, including VEXAS (vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic) syndrome and chronic myelomonocytic leukaemia (CMML). We aimed to compare the presentation and outcomes of VEXAS- or CMML-associated PAN (VEXAS-PAN and CMML-PAN) with those of primary PAN. Methods: We conducted a retrospective, multicentre study of patients diagnosed with primary PAN, VEXAS-PAN or CMML-PAN. Each VEXAS-PAN or CMML-PAN case was matched to three primary PAN cases by age and gender. We analysed baseline and therapeutic characteristics, along with survival and relapse rates. Results: Twenty-three patients were included (12 with VEXAS-PAN, 11 with CMML-PAN). Secondary PAN occurred at older ages: 73 years for VEXAS-PAN, 70 years for CMML-PAN and 54 years for primary PAN (P < 0.01). VEXAS-PAN was associated with a higher frequency of skin manifestations (100%), orchitis (67%) and ocular manifestations (58%) than primary PAN. By contrast, the clinical features of CMML-PAN closely resembled those of primary PAN. VEXAS-PAN was less likely than primary PAN to achieve remission [adjusted odds ratio (aOR) 0.15; 95% CI 0.02–1.00] and more likely to experience relapses [adjusted hazard ratio (aHR) 3.24; P = 0.043]. Similar trends were observed for CMML-PAN regarding remission (aOR 0.12; 95% CI 0.02–0.73) and relapses (aHR 2.23; P = 0.145). Mortality was higher in VEXAS-PAN (50%) than in CMML-PAN (15%) or primary PAN (17%). Conclusion: VEXAS- and CMML-PAN are distinct clinical entities with poorer prognosis and unique therapeutic challenges. Recognizing these conditions early and providing individualized management are crucial to improving outcomes in these patients.

Background/Objectives : Our aim was to evaluate the performance of the reticulocyte hemoglobin content (CHr) assay to diagnose iron deficiency anemia in a context of inflammation in an elderly population, by comparison with the gold standard. Methods: Patients over 18 years old, with anemia and biological inflammatory syndrome, were included consecutively. The accuracy of CHr was compared with that of the gold standard, soluble transferrin receptor/log ferritin. Analyses were performed according to age (subjects over and under 75 years of age) and included specificity, sensitivity, positive predictive value (PPV), negative predictive value (NPV), and the area under the ROC curve (AUC). Results: A total of 174 patients were included, with respectively 84 and 89 patients aged under or 75 years and over, the mean age being 72.5 years (SD 16.9). In the older population, the mean age was 84.6 years (SD 5.2), and 40% were female; the mean hemoglobin was 9.6 (SD 1.4) g/dl and mean CRP 89.7 (SD 72.9) mg/L. The optimal threshold value, determined by maximizing the Youden index, was 25.2pg, at which sensitivity and specificity reached 67% and 94%, respectively. The corresponding PPV and NPV were 62% and 95%. The AUC of the ROC curve was 0.81 with 95% CI: [0.65-0.97], suggesting good diagnostic accuracy. Conclusions: In an elderly population with anemia in a context of inflammation, assessing iron deficiency by including CHr is simple, inexpensive, rapid and practical, as it can be measured as a by-product of a routine complete blood count at little incremental cost, and good NPV can exclude a diagnosis of IDA.