Para médicos · reumatologia
Resumos de artigos, podcasts e newsletters sobre Vasculite por IgA, para atualização médica.
A newsletter destaca que adolescentes com vasculite por IgA apresentam maior risco de púrpura persistente e proteinúria, exigindo vigilância renal redobrada. Na espondiloartrite axial, o uso de upadacitinibe mostrou-se superior à troca por outro anti-TNF ou anti-IL-17 após falha terapêutica inicial. O conteúdo também aborda o diagnóstico de eritema ab igne e a identificação de dano miocárdico subclínico no lúpus através de técnicas avançadas de imagem.
This study aimed to develop and validate a risk stratification scale for unfavourable outcomes in adult patients with IgA vasculitis nephritis (IgAVN). The derivation cohort in this study was constructed using the existing prognosis data from adult IgAVN cohorts. We extracted the risk factors and their hazard ratios. Only statistically significant risk factors were included in our final risk stratification scale. Then this study validated the risk stratification scale in an external cohort of Chinese patients. The performance of the risk stratification scale was evaluated by the receiver operating characteristic (ROC), calibration, decision, and Kaplan-Meier curves. Ten cohorts involving 1,814 adult patients with IgAVN were included in this meta-analysis. Serum albumin (ALB), estimated glomerular filtration rate (eGFR), endocapillary hypercellularity (E1), and tubular atrophy/interstitial fibrosis (T1/2) were included in the risk stratification and scored according to their weightings (maximum score: 6.5). An external cohort comprising 133 patients was used to validate the risk stratification scale. The area under the curve (AUC) value of the scoring scale was 0.88 (95%CI: 0.78-0.99), with a sensitivity of 0.79 (95%CI: 0.49-0.95) and specificity of 0.89 (95%CI: 0.82-0.94), at a cut-off value of 3. The calibration, decision, and Kaplan-Meier curves further confirmed the robust performance of the risk stratification scale. In this study, we established a simple and practical tool to identify adult IgAVN patients at high risk of unfavourable outcomes. Reasonable use of the risk stratification scale can help make early clinical decisions and facilitate the development of precision medicine.
Polyarteritis Nodosa is a rare necrotizing vasculitis with a broad and variable clinical presentation, driven by its ability to affect multiple organs and systems. This heterogeneity influences disease severity, relapse risk, and mortality, highlighting the prognostic importance of its diverse manifestations. This systematic review and meta-analysis synthesize the available evidence to define the clinical profile of Polyarteritis Nodosa and highlight key prognostic factors. A systematic search of electronic databases was conducted to identify studies reporting clinical manifestations and outcomes of patients diagnosed with Polyarteritis Nodosa. Pooled prevalence summary estimates were calculated using R software, including subgroup analyses by age group. Case Fatality Rate was calculated to determine the clinical severity of the manifestations. Across adult and pediatric groups, the most common manifestations were fever (~ 52%), myalgia (~ 53%), and cutaneous involvement (~ 56%). Age significantly moderated the prevalence of fever, arthralgia, hypertension, and peripheral neuropathy. Several symptoms were frequently observed among patients who experienced relapse, particularly cutaneous involvement (~ 66%) and myalgia (~ 64%). While cardiac involvement was associated with higher case fatality, gastrointestinal manifestations accounted for a greater proportion of reported deaths overall. Total mortality was approximately 13%, relapse occurred in about 27% of patients, while roughly 65% of patients achieved remission. This systematic review with meta-analysis provides crucial information to clinical physicians, regarding the clinical profile and the prognostic factors of Polyarteritis Nodosa. Thus, these insights could guide management strategies and increase the survival and remission rate of these patients. PROSPERO: https://www.crd.york.ac.uk/PROSPERO/view/CRD420251249274 .
Vasculitis often poses a significant burden on individuals, their families and the health care system. Understanding its epidemiology can aid in facilitating timely interventions. We present a province-wide population study comparing the 1) incidence of vasculitis amongst farmers, rural-non farmers and urban residents, 2) the use of health services and 3) all-cause mortality rates across the three cohorts. The groups were randomly selected on the basis of provincial health data. Criteria for ascertaining vasculitis cases included either one hospital admission, two physician visits within a 2-year interval, or two ambulatory care visits within 2 years related to the vasculitis diagnosis. Descriptive statistics were used to compare the incidence rates. A total sample size of 302,089, with 5437 vasculitis cases. Farmers had the highest incidence of all types of vasculitis (109.8/100,000 person-years (PY)), followed by rural non-farmers (93.1/100,000 PY) and urbanites (71.7/100,000 PY). Age at diagnosis was higher among farmers (66.2 years) compared to rural non-farmers (64.5 years) and urbanites (63.9 years). Polymyalgia rheumatica (PMR) accounted for 47% of cases, followed by Arteritis Unspecified (15%) and small-vessel vasculitis (14%). Within the farming population, a higher percentage of males had positive cases of vasculitis compared to other populations. Rural non-farmer population had the highest use of health care services and unadjusted non-injury mortality rate (31.8/100,000 PY), followed by rural farmers (25.4/100,000 PY) and urban residents (23.8/100,000 PY). Our province-wide study revealed that farmers face the highest incidence rates of vasculitis as well as the second highest burden of disease in terms of healthcare service needs and mortality. Key PointsAQ • There is a notable variation in the incidence rates of vasculitis among farmers, rural and urban residents with the farming population showing the highest incidence rate across most
Immunoglobulin A vasculitis (IgAV), also known as Henoch- Schönlein purpura (HSP), is the most common systemic vasculitis in childhood. Potential differences in demographic characteristics, clinical presentation, laboratory findings, and treatments approaches across age groups, remain poorly explored. To the best of our knowledge, no multicenter study in Latin America has systematically addressed these features. We aimed to assess demographic, clinical and laboratory features, and treatments in children versus adolescents with (IgAV)/ (HSP) in a large multicenter study. A multicenter study involving four tertiary centers evaluated 687 children and adolescents (≤ 18 years-old) with IgAV/HSP (EULAR/PRINTO/PRES classification criteria) at first 3 months after diagnosis. The charts were retrospectively assessed for demographic data, initial clinical manifestations, laboratory tests and treatments. Data were compared between children (< 10 years-old) and adolescents (≥ 10 years-old), according to WHO definition. IgAV/HSP was diagnosed in 599/687(87%) children [5.33(0.88–9.91) years-old] and 88/687(13%) adolescents [11.33(10-17.5) years-old]. The median duration of purpura/petechiae was significantly lower in children compared to adolescents [14(1-120) vs. 15(2–90) days, p = 0.04]. The frequency of persistent purpura/petechiae (≥ 6 weeks of duration) was significantly reduced in the former group (7.2% vs. 19.5%, p = 0.002), likewise the frequency of gastrointestinal bleeding (17% vs. 34.1%, p = 0.01) and proteinuria (49.7% vs. 84%, p = 0.002). In contrast, the frequencies of arthritis/arthralgia (82.7% vs. 73%, p = 0.03) and orchitis(16.6% vs. 4.8%, p = 0.04) were significantly higher in children. Further analysis of laboratory tests showed that the median value of serum IgA was significantly lower in children than in adolescents [179.1(40-1002.0) vs. 279.0(104.0-488.0) mg/dL, p = 0.01], whereas thrombocytosis was higher (40.1% vs. 23%, p = 0.007). Logistic regression demonstrated that persistent purpura/petechiae after
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.
To evaluate the utility of magnetic resonance imaging (MRI)-guided muscle biopsy (MB) in diagnosing small- to medium-vessel vasculitis. We retrospectively included patients with antineutrophil cytoplasmic antibody-associated vasculitis (AAV) or polyarteritis nodosa (PAN) between April 2020 and March 2025 who underwent MRI and MB. The primary outcome was the diagnostic sensitivity of MRI-guided MB for AAV and PAN. The secondary outcome was the diagnostic sensitivity for PAN alone. MB was considered positive when it demonstrated either necrotizing vasculitis or non-necrotizing vasculitis. Eighteen patients who underwent MRI and MB were included: 11 patients had AAV and 7 had PAN. The median interval between MRI and MB was 3.5 days. The mean Birmingham Vasculitis Activity Score was 13.6. Muscle pain was observed in 11 patients; however, none of the patients exhibited elevated creatine kinase levels. The sensitivity of MB for diagnosing AAV and PAN was 83.3% (15/18; 95% confidence intervals [CI] 58.6-96.4%), whereas that for PAN alone was 85.7% (6/7; 95% CI 42.1-96.3%). No biopsy-related complications were observed. There were no apparent differences in clinical characteristics between the MB-positive and MB-negative groups. MRI-guided MB may represent a diagnostic option for small- to medium-vessel vasculitis, even in patients without muscle pain or when other suitable biopsy sites are unavailable. Although this was a small exploratory retrospective single-center study, these findings should be validated in larger multicenter prospective studies.