Diagnostic Implications and Correlates of Plasma Adenosine Deaminase 2 Activity and ADA2 Variants
Arthritis &Rheumatology, Volume 78, Issue 3, Page 734-742, March 2026.
Para médicos · reumatologia
Poliarterite Nodosa | Lumien
Resumos de artigos, podcasts e newsletters sobre Poliarterite Nodosa, para atualização médica.
TRT 88
A newsletter aborda as atualizações do ACR 2025 sobre a investigação de vasculites cutâneas, enfatizando a diferenciação entre quadros limitados à pele e manifestações sistêmicas. Destaca também que a doença pulmonar intersticial é uma complicação frequente e grave na esclerose sistêmica limitada, especialmente em pacientes com anticorpo anti-topoisomerase I positivo.
Trends in Cardiovascular Mortality Associated With Systemic Connective Tissue Disorders in the United States: A 22-Year Population-Based National Analysis (1999-2020).
To examine national trends and disparities in cardiovascular mortality associated with systemic connective tissue disorders (CTDs) in the United States from 1999 to 2020. We analyzed mortality data from the CDC WONDER database. Deaths were included where CTD (ICD-10: M05, M06, M30-M35) was the underlying cause and cardiovascular disease was a contributing cause. Age-adjusted mortality rates (AAMRs) per 1 000 000 were calculated using the 2000 US Standard Population. Joinpoint regression identified annual and average annual percentage changes. Analyses were stratified by sex, race/ethnicity, census region, and urbanization. Disease subgroup and state-level analyses were performed. Between 1999 and 2020, 47 752 cardiovascular deaths occurred among individuals with systemic CTDs. The national AAMR declined from 14.4 to 8.2 per 1 000 000 (AAPC: -2.68%, 95% CI: -2.89 to -2.47, p < 0.001). Females had consistently higher mortality than males (average AAMR: 13.5 vs. 5.9 per 1 000 000; p < 0.001). Non-Hispanic Black individuals had the highest rates (average AAMR: 14.9 per 1 000 000), with widening disparities after 2008. Rural areas had higher mortality than urban areas (average AAMR: 11.4 vs. 9.9 per 1 000 000; p = 0.01). Subgroup analyses revealed heterogeneous trends across CTD subtypes, with SLE showing the slowest improvement (AAPC: -1.87%) and dermatomyositis the steepest decline (AAPC: -4.98%). State-level AAMRs ranged 2.2-fold, from 6.3 (District of Columbia) to 13.6 (Montana) per 1 000 000. Cardiovascular mortality associated with systemic CTDs has declined significantly over two decades; however, persistent racial disparities, urban-rural differences, heterogeneous disease-specific trends, and substantial geographic variation underscore the need for targeted, equitable interventions in this high-risk population.
IgA Vasculitis with necrotizing arteritis: a multicenter retrospective study from the French Vasculitis Study Group and systematic review of the literature.
IgA vasculitis (IgAV) primarily affects small vessels, but rare cases with necrotizing arteritis (NA) raise questions about overlap with polyarteritis nodosa (PAN). To characterize IgAV with necrotizing arteritis (IgAV-NA) and compare its phenotype with classical IgAV and PAN. We performed a multicenter retrospective study combined with a systematic literature review (1990-2025). Patients fulfilled EULAR/PRINTO/PRES IgAV criteria, had pathological or imaging evidence of NA in small or medium arteries, and were ANCA-negative. Thirty patients were included (7 from databases, 23 from the literature). NA was confirmed by biopsy (n = 16) or vascular imaging (n = 14). Clinical features, treatments, remission, and mortality were compared with 257 adult IgAV and 196 PAN patients. Median age was 54.5 years. IgAV-NA was characterized by severe manifestations, including gastrointestinal bleeding, perforation, surgical abdomen, neuropathy, pancreatitis, and livedo. Compared with classical IgAV, IgAV-NA showed significantly higher rates of multi-organ involvement and mortality. Compared with PAN, IgAV-NA shared vascular complications but had less fever and neuropathy. Despite arterial involvement, patients did not fulfil PAN criteria. IgAV-NA represents a rare, severe IgAV phenotype with life-threatening complications rather than an IgAV-PAN overlap. Severe or atypical IgAV presentations should prompt vascular imaging and intensified immunosuppression.