Cushing's syndrome (CS) is a rare disorder, once exogenous causes have been excluded. However, when diagnosed, the majority of cases are adrenocorticotropic hormone (ACTH)-dependent, of which a substantial minority are due to a source outside of the pituitary, ectopic ACTH syndrome (EAS). Differentiating among pituitary-dependent CS, Cushing's disease (CD) and an ectopic source can be problematic. Because non-invasive tests in the evaluation of CS patients often lack adequate sensitivity and specificity, bilateral inferior petrosal sinus sampling (BIPSS), a minimally invasive procedure performed during the investigation of ACTH-dependent CS, can be extremely helpful. BIPSS is considered to be the gold standard for differentiating CD from the EAS. Furthermore, although such differentiation may indeed be challenging, BIPSS is itself a complex investigation, especially in recent times due to the widespread withdrawal of corticotrophin-releasing hormone and its replacement by desmopressin. We review current published data on this investigation and, in the light of this and our own experience, discuss its appropriate use in diagnostic algorithms.
一旦排除了外源性病因，库欣综合征（CS）是一种罕见的疾病。然而，一旦确诊，大多数病例都是促肾上腺皮质激素（ACTH）依赖型，其中相当一部分病例是由于垂体以外的原因引起的，即异位 ACTH 综合征（EAS）。区分垂体依赖性 CS、库欣病（CD）和异位源可能存在问题。由于评估 CS 患者的非侵入性检查往往缺乏足够的灵敏度和特异性，因此在检查 ACTH 依赖性 CS 时进行的微创手术--双侧下鼻底窦取样（BIPSS）会非常有帮助。BIPSS 被认为是区分 CD 和 EAS 的金标准。此外，尽管这种鉴别可能确实具有挑战性，但 BIPSS 本身也是一项复杂的检查，尤其是近来由于促肾上腺皮质激素释放激素的广泛停用并被去氨加压素所取代。我们回顾了目前已发表的有关该检查的数据，并根据这些数据和我们自己的经验，讨论了在诊断算法中使用该方法的适当性。
{"title":"Bilateral inferior petrosal sinus sampling in the differential diagnosis of ACTH-dependent Cushing's syndrome: A reappraisal","authors":"Majid Valizadeh, Behnaz Abiri, Farhad Hosseinpanah, Ashley Grossman","doi":"10.1111/joim.13789","DOIUrl":"10.1111/joim.13789","url":null,"abstract":"<p>Cushing's syndrome (CS) is a rare disorder, once exogenous causes have been excluded. However, when diagnosed, the majority of cases are adrenocorticotropic hormone (ACTH)-dependent, of which a substantial minority are due to a source outside of the pituitary, ectopic ACTH syndrome (EAS). Differentiating among pituitary-dependent CS, Cushing's disease (CD) and an ectopic source can be problematic. Because non-invasive tests in the evaluation of CS patients often lack adequate sensitivity and specificity, bilateral inferior petrosal sinus sampling (BIPSS), a minimally invasive procedure performed during the investigation of ACTH-dependent CS, can be extremely helpful. BIPSS is considered to be the gold standard for differentiating CD from the EAS. Furthermore, although such differentiation may indeed be challenging, BIPSS is itself a complex investigation, especially in recent times due to the widespread withdrawal of corticotrophin-releasing hormone and its replacement by desmopressin. We review current published data on this investigation and, in the light of this and our own experience, discuss its appropriate use in diagnostic algorithms.</p>","PeriodicalId":196,"journal":{"name":"Journal of Internal Medicine","volume":"296 1","pages":"2-23"},"PeriodicalIF":11.1,"publicationDate":"2024-04-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1111/joim.13789","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140595143","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
<p>In April 2023, the <i>New York Times</i> published an opinion piece by author and heart transplant patient, Amy Silverstein [<span>1</span>]. Ms. Silverstein's perspective provoked an array of responses, some of which were angry because of the perception that she lacked gratitude for the second and third chance at life she was given. However, as professionals in the transplant field, Ms. Silverstein's story resonated with us, particularly her description of what she called the “gratitude paradox” wherein solid-organ transplant patients are expected to be grateful for what they have—a new, functioning organ—and are either implicitly or explicitly discouraged from asking for more and better posttransplant treatment options [<span>1</span>]. While her observations were personal for us, we see parallels that are relevant for the entire healthcare community. Ms. Silverstein pointed to the conflicting emotions of her own gratitude for her two heart transplants in the wake of her terminal cancer diagnosis, a diagnosis she states likely resulted from long-term use of immunosuppression medications meant to preserve her transplanted organ, and her desire to have more life. She wasn't ungrateful in expressing that desire; she was simply being human. While the specifics of Ms. Silverstein's life are relevant to the field of transplantation, we believe the human desires she expressed should cause the entire healthcare community to pause and reflect about why we chose this calling and our inherent responsibilities.</p><p>The concept of the gratitude paradox is not new. The BBC correspondent Kate Morgan explored this issue in a 2021 piece examining the complexities of gratitude for being employed in the wake of the COVID-19 pandemic [<span>2</span>]. She discussed the dilemma many individuals experienced between being grateful to have a job during a time of rising unemployment and feeling underpaid, undervalued, and overburdened by employers [<span>2</span>]. Another, more historical example is the “separate but equal” laws, colloquially known as Jim Crow laws, that pervaded American life in the post-Civil War era through the Civil Rights movement of the 1960s. Under Jim Crow, Black Americans experienced and were expected to be grateful for (or as Davis [<span>3</span>] describes, “agreeable and non-challenging”), segregated conditions that proved to be anything but equal. There is a prevailing attitude that certain populations, in particular those who are vulnerable, such as patients with chronic medical conditions, racial and ethnic minority groups, or individuals from poorer socioeconomic backgrounds, should be thankful for whatever benefits of progress made in achieving a better life. They are viewed as troublemakers who lack gratitude whenever they suggest the bare minimum is not enough.</p><p>In our society, there is an expectation that disadvantaged and vulnerable populations should be grateful for having something that is one step above having nothin
{"title":"The gratitude paradox","authors":"Marie Chisholm-Burns, Richard N. Formica","doi":"10.1111/joim.13788","DOIUrl":"10.1111/joim.13788","url":null,"abstract":"<p>In April 2023, the <i>New York Times</i> published an opinion piece by author and heart transplant patient, Amy Silverstein [<span>1</span>]. Ms. Silverstein's perspective provoked an array of responses, some of which were angry because of the perception that she lacked gratitude for the second and third chance at life she was given. However, as professionals in the transplant field, Ms. Silverstein's story resonated with us, particularly her description of what she called the “gratitude paradox” wherein solid-organ transplant patients are expected to be grateful for what they have—a new, functioning organ—and are either implicitly or explicitly discouraged from asking for more and better posttransplant treatment options [<span>1</span>]. While her observations were personal for us, we see parallels that are relevant for the entire healthcare community. Ms. Silverstein pointed to the conflicting emotions of her own gratitude for her two heart transplants in the wake of her terminal cancer diagnosis, a diagnosis she states likely resulted from long-term use of immunosuppression medications meant to preserve her transplanted organ, and her desire to have more life. She wasn't ungrateful in expressing that desire; she was simply being human. While the specifics of Ms. Silverstein's life are relevant to the field of transplantation, we believe the human desires she expressed should cause the entire healthcare community to pause and reflect about why we chose this calling and our inherent responsibilities.</p><p>The concept of the gratitude paradox is not new. The BBC correspondent Kate Morgan explored this issue in a 2021 piece examining the complexities of gratitude for being employed in the wake of the COVID-19 pandemic [<span>2</span>]. She discussed the dilemma many individuals experienced between being grateful to have a job during a time of rising unemployment and feeling underpaid, undervalued, and overburdened by employers [<span>2</span>]. Another, more historical example is the “separate but equal” laws, colloquially known as Jim Crow laws, that pervaded American life in the post-Civil War era through the Civil Rights movement of the 1960s. Under Jim Crow, Black Americans experienced and were expected to be grateful for (or as Davis [<span>3</span>] describes, “agreeable and non-challenging”), segregated conditions that proved to be anything but equal. There is a prevailing attitude that certain populations, in particular those who are vulnerable, such as patients with chronic medical conditions, racial and ethnic minority groups, or individuals from poorer socioeconomic backgrounds, should be thankful for whatever benefits of progress made in achieving a better life. They are viewed as troublemakers who lack gratitude whenever they suggest the bare minimum is not enough.</p><p>In our society, there is an expectation that disadvantaged and vulnerable populations should be grateful for having something that is one step above having nothin","PeriodicalId":196,"journal":{"name":"Journal of Internal Medicine","volume":"295 6","pages":"712-714"},"PeriodicalIF":11.1,"publicationDate":"2024-04-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1111/joim.13788","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140595152","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Nutritional administration in acute pancreatitis (AP) management has sparked widespread discussion, yet contradictory mortality results across meta-analyses necessitate clarification. The optimal nutritional route in AP remains uncertain. Therefore, this study aimed to compare mortality among nutritional administration routes in patients with AP using consistency model.
Methods
This study searched four major databases for relevant randomized controlled trials (RCTs). Two authors independently extracted and checked data and quality. Network meta-analysis was conducted for estimating risk ratios (RRs) with 95% confidence interval (CI) based on random-effects model. Subgroup analyses accounted for AP severity and nutrition support initiation.
Results
A meticulous search yielded 1185 references, with 30 records meeting inclusion criteria from 27 RCTs (n = 1594). Pooled analyses showed the mortality risk reduction associated with nasogastric (NG) (RR = 0.34; 95%CI: 0.16–0.73) and nasojejunal (NJ) feeding (RR = 0.46; 95%CI: 0.25–0.84) in comparison to nil per os. Similarly, NG (RR = 0.45; 95%CI: 0.24–0.83) and NJ (RR = 0.60; 95%CI: 0.40–0.90) feeding also showed lower mortality risk than total parenteral nutrition. Subgroup analyses, stratified by severity, supported these findings. Notably, the timing of nutritional support initiation emerged as a significant factor, with NJ feeding demonstrating notable mortality reduction within 24 and 48 h, particularly in severe cases.
Conclusion
For severe AP, both NG and NJ feeding appear optimal, with variations in initiation timings. NG feeding does not appear to merit recommendation within the initial 24 h, whereas NJ feeding is advisable within the corresponding timeframe following admission. These findings offer valuable insights for optimizing nutritional interventions in AP.
{"title":"Impact of nutritional support routes on mortality in acute pancreatitis: A network meta-analysis of randomized controlled trials","authors":"Ping-Han Hsieh, Tsung-Chieh Yang, Enoch Yi-No Kang, Pei-Chang Lee, Jiing-Chyuan Luo, Yi-Hsiang Huang, Ming-Chih Hou, Shih-Ping Huang","doi":"10.1111/joim.13782","DOIUrl":"10.1111/joim.13782","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Background</h3>\u0000 \u0000 <p>Nutritional administration in acute pancreatitis (AP) management has sparked widespread discussion, yet contradictory mortality results across meta-analyses necessitate clarification. The optimal nutritional route in AP remains uncertain. Therefore, this study aimed to compare mortality among nutritional administration routes in patients with AP using consistency model.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>This study searched four major databases for relevant randomized controlled trials (RCTs). Two authors independently extracted and checked data and quality. Network meta-analysis was conducted for estimating risk ratios (RRs) with 95% confidence interval (CI) based on random-effects model. Subgroup analyses accounted for AP severity and nutrition support initiation.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>A meticulous search yielded 1185 references, with 30 records meeting inclusion criteria from 27 RCTs (<i>n</i> = 1594). Pooled analyses showed the mortality risk reduction associated with nasogastric (NG) (RR = 0.34; 95%CI: 0.16–0.73) and nasojejunal (NJ) feeding (RR = 0.46; 95%CI: 0.25–0.84) in comparison to nil per os. Similarly, NG (RR = 0.45; 95%CI: 0.24–0.83) and NJ (RR = 0.60; 95%CI: 0.40–0.90) feeding also showed lower mortality risk than total parenteral nutrition. Subgroup analyses, stratified by severity, supported these findings. Notably, the timing of nutritional support initiation emerged as a significant factor, with NJ feeding demonstrating notable mortality reduction within 24 and 48 h, particularly in severe cases.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Conclusion</h3>\u0000 \u0000 <p>For severe AP, both NG and NJ feeding appear optimal, with variations in initiation timings. NG feeding does not appear to merit recommendation within the initial 24 h, whereas NJ feeding is advisable within the corresponding timeframe following admission. These findings offer valuable insights for optimizing nutritional interventions in AP.</p>\u0000 </section>\u0000 </div>","PeriodicalId":196,"journal":{"name":"Journal of Internal Medicine","volume":"295 6","pages":"759-773"},"PeriodicalIF":11.1,"publicationDate":"2024-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140334066","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Andreas Barratt-Due, Kristin Pettersen, Tuva Børresdatter-Dahl, Jan Cato Holter, Renathe H. Grønli, Anne Ma Dyrhol-Riise, Tøri Vigeland Lerum, Aleksander Rygh Holten, Kristian Tonby, Marius Trøseid, Ole H. Skjønsberg, Beathe Kiland Granerud, Lars Heggelund, Anders Benjamin Kildal, Camilla Schjalm, Trond Mogens Aaløkken, Pål Aukrust, Thor Ueland, Tom Eirik Mollnes, Bente Halvorsen, NOR-Solidarity study groupThe Norwegian SARS-CoV-2 study group
Background
The complement system, an upstream recognition system of innate immunity, is activated upon SARS-CoV-2 infection. To gain a deeper understanding of the extent and duration of this activation, we investigated complement activation profiles during the acute phase of COVID-19, its persistence post-recovery and dynamic changes in relation to disease severity.
Methods
Serial blood samples were obtained from two cohorts of hospitalized COVID-19 patients (n = 457). Systemic complement activation products reflecting classical/lectin (C4d), alternative (C3bBbP), common (C3bc) and terminal pathway (TCC and C5a) were measured during hospitalization (admission, days 3–5 and days 7–10), at 3 months and after 1 year. Levels of activation and temporal profiles during hospitalization were related to disease severity defined as respiratory failure (PO2/FiO2 ratio <26.6 kPa) and/or admission to intensive care unit, 60-day total mortality and pulmonary pathology after 3 months.
Findings
During hospitalization, TCC, C4d, C3bc, C3bBbP and C5a were significantly elevated compared to healthy controls. Severely ill patients had significantly higher levels of TCC and C4d (p < 0.001), compared to patients with moderate COVID-19. Escalated levels of TCC and C4d during hospitalization were associated with a higher risk of 60-day mortality (p < 0.001), and C4d levels were additionally associated with chest CT changes at 3 months (p < 0.001). At 3 months and 1 year, we observed consistently elevated levels of most complement activation products compared to controls.
Conclusion
Hospitalized COVID-19 patients display prominent and long-lasting systemic complement activation. Optimal targeting of the system may be achieved through enhanced risk stratification and closer monitoring of in-hospital changes of complement activation products.
{"title":"Escalated complement activation during hospitalization is associated with higher risk of 60-day mortality in SARS-CoV-2-infected patients","authors":"Andreas Barratt-Due, Kristin Pettersen, Tuva Børresdatter-Dahl, Jan Cato Holter, Renathe H. Grønli, Anne Ma Dyrhol-Riise, Tøri Vigeland Lerum, Aleksander Rygh Holten, Kristian Tonby, Marius Trøseid, Ole H. Skjønsberg, Beathe Kiland Granerud, Lars Heggelund, Anders Benjamin Kildal, Camilla Schjalm, Trond Mogens Aaløkken, Pål Aukrust, Thor Ueland, Tom Eirik Mollnes, Bente Halvorsen, NOR-Solidarity study groupThe Norwegian SARS-CoV-2 study group","doi":"10.1111/joim.13783","DOIUrl":"10.1111/joim.13783","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Background</h3>\u0000 \u0000 <p>The complement system, an upstream recognition system of innate immunity, is activated upon SARS-CoV-2 infection. To gain a deeper understanding of the extent and duration of this activation, we investigated complement activation profiles during the acute phase of COVID-19, its persistence post-recovery and dynamic changes in relation to disease severity.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>Serial blood samples were obtained from two cohorts of hospitalized COVID-19 patients (<i>n</i> = 457). Systemic complement activation products reflecting classical/lectin (C4d), alternative (C3bBbP), common (C3bc) and terminal pathway (TCC and C5a) were measured during hospitalization (admission, days 3–5 and days 7–10), at 3 months and after 1 year. Levels of activation and temporal profiles during hospitalization were related to disease severity defined as respiratory failure (PO<sub>2</sub>/FiO<sub>2</sub> ratio <26.6 kPa) and/or admission to intensive care unit, 60-day total mortality and pulmonary pathology after 3 months.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Findings</h3>\u0000 \u0000 <p>During hospitalization, TCC, C4d, C3bc, C3bBbP and C5a were significantly elevated compared to healthy controls. Severely ill patients had significantly higher levels of TCC and C4d (<i>p </i>< 0.001), compared to patients with moderate COVID-19. Escalated levels of TCC and C4d during hospitalization were associated with a higher risk of 60-day mortality (<i>p </i>< 0.001), and C4d levels were additionally associated with chest CT changes at 3 months (<i>p </i>< 0.001). At 3 months and 1 year, we observed consistently elevated levels of most complement activation products compared to controls.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Conclusion</h3>\u0000 \u0000 <p>Hospitalized COVID-19 patients display prominent and long-lasting systemic complement activation. Optimal targeting of the system may be achieved through enhanced risk stratification and closer monitoring of in-hospital changes of complement activation products.</p>\u0000 </section>\u0000 </div>","PeriodicalId":196,"journal":{"name":"Journal of Internal Medicine","volume":"296 1","pages":"80-92"},"PeriodicalIF":11.1,"publicationDate":"2024-03-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1111/joim.13783","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140304208","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Jiazhen Zheng, Can Ni, S. W. Ricky Lee, Fu-Rong Li, Jinghan Huang, Rui Zhou, Yining Huang, Gregory Y. H. Lip, Xianbo Wu, Shaojun Tang
Background
The association of a broad spectrum of infectious diseases with cardiovascular outcomes remains unclear.
Objectives
We aim to provide the cardiovascular risk profiles associated with a wide range of infectious diseases and explore the extent to which infections reduce life expectancy.
Methods
We ascertained exposure to 900+ infectious diseases before cardiovascular disease (CVD) onset in 453,102 participants from the UK Biobank study. Time-varying Cox proportional hazard models were used. Life table was used to estimate the life expectancy of individuals aged ≥50 with different levels of infection burden (defined as the number of infection episodes over time and the number of co-occurring infections).
Results
Infectious diseases were associated with a greater risk of CVD events (adjusted HR [aHR] 1.79 [95% confidence interval {CI} 1.74–1.83]). For type-specific analysis, bacterial infection with sepsis had the strongest risk of CVD events [aHR 4.76 (4.35–5.20)]. For site-specific analysis, heart and circulation infections posed the greatest risk of CVD events [aHR 4.95 (95% CI 3.77–6.50)], whereas noncardiac infections also showed excess risk [1.77 (1.72–1.81)]. Synergistic interactions were observed between infections and genetic risk score. A dose–response relationship was found between infection burden and CVD risks (p-trend <0.001). Infection burden >1 led to a CVD-related life loss at age 50 by 9.3 years [95% CI 8.6–10.3]) for men and 6.6 years [5.5–7.8] for women.
Conclusions
The magnitude of the infection-CVD association showed specificity in sex, pathogen type, infection burden, and infection site. High genetic risk and infection synergistically increased the CVD risk.
{"title":"Association of hospital-treated infectious diseases and infection burden with cardiovascular diseases and life expectancy","authors":"Jiazhen Zheng, Can Ni, S. W. Ricky Lee, Fu-Rong Li, Jinghan Huang, Rui Zhou, Yining Huang, Gregory Y. H. Lip, Xianbo Wu, Shaojun Tang","doi":"10.1111/joim.13780","DOIUrl":"10.1111/joim.13780","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Background</h3>\u0000 \u0000 <p>The association of a broad spectrum of infectious diseases with cardiovascular outcomes remains unclear.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Objectives</h3>\u0000 \u0000 <p>We aim to provide the cardiovascular risk profiles associated with a wide range of infectious diseases and explore the extent to which infections reduce life expectancy.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>We ascertained exposure to 900+ infectious diseases before cardiovascular disease (CVD) onset in 453,102 participants from the UK Biobank study. Time-varying Cox proportional hazard models were used. Life table was used to estimate the life expectancy of individuals aged ≥50 with different levels of infection burden (defined as the number of infection episodes over time and the number of co-occurring infections).</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>Infectious diseases were associated with a greater risk of CVD events (adjusted HR [aHR] 1.79 [95% confidence interval {CI} 1.74–1.83]). For type-specific analysis, bacterial infection with sepsis had the strongest risk of CVD events [aHR 4.76 (4.35–5.20)]. For site-specific analysis, heart and circulation infections posed the greatest risk of CVD events [aHR 4.95 (95% CI 3.77–6.50)], whereas noncardiac infections also showed excess risk [1.77 (1.72–1.81)]. Synergistic interactions were observed between infections and genetic risk score. A dose–response relationship was found between infection burden and CVD risks (<i>p</i>-trend <0.001). Infection burden >1 led to a CVD-related life loss at age 50 by 9.3 years [95% CI 8.6–10.3]) for men and 6.6 years [5.5–7.8] for women.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Conclusions</h3>\u0000 \u0000 <p>The magnitude of the infection-CVD association showed specificity in sex, pathogen type, infection burden, and infection site. High genetic risk and infection synergistically increased the CVD risk.</p>\u0000 </section>\u0000 </div>","PeriodicalId":196,"journal":{"name":"Journal of Internal Medicine","volume":"295 5","pages":"679-694"},"PeriodicalIF":11.1,"publicationDate":"2024-03-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140287819","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Amarens van der Vaart, Coby Eelderink, Harry van Goor, Jan-Luuk Hillebrands, Charlotte A. te Velde-Keyzer, Stephan J.L. Bakker, Andreas Pasch, Peter R. van Dijk, Gozewijn D. Laverman, Martin H. de Borst
Background and aims
Individuals with type 2 diabetes (T2D) have a higher risk of cardiovascular disease, compared with those without T2D. The serum T50 test captures the transformation time of calciprotein particles in serum. We aimed to assess whether serum T50 predicts cardiovascular mortality in T2D patients, independent of traditional risk factors.
Methods
We analyzed 621 individuals with T2D in this prospective cohort study. Cox regression models were performed to test the association between serum T50 and cardiovascular and all-cause mortality. Causes of death were categorized according to ICD-10 codes. Risk prediction improvement was assessed by comparing Harrell's C for models without and with T50.
Results
The mean age was 64.2 ± 9.8 years, and 61% were male. The average serum T50 time was 323 ± 63 min. Higher age, alcohol use, high-sensitive C-reactive protein, and plasma phosphate were associated with lower serum T50 levels. Higher plasma triglycerides, venous bicarbonate, sodium, magnesium, and alanine aminotransferase were associated with higher serum T50 levels. After a follow-up of 7.5[5.4–10.7] years, each 60 min decrease in serum T50 was associated with an increased risk of cardiovascular (fully adjusted HR 1.32, 95% CI 1.08–1.50, and p = 0.01) and all-cause mortality (HR 1.15, 95%CI 1.00–1.38, and p = 0.04). Results were consistent in sensitivity analyses after exclusion of individuals with estimated glomerular filtration rate <45 or <60 mL/min/1.73 m2 and higher plasma phosphate levels.
Conclusions
Serum T50 improves prediction of cardiovascular and all-cause mortality risk in individuals with T2D. Serum T50 may be useful for risk stratification and to guide therapeutic strategies aiming to reduce cardiovascular mortality in T2D.
{"title":"Serum T50 predicts cardiovascular mortality in individuals with type 2 diabetes: A prospective cohort study","authors":"Amarens van der Vaart, Coby Eelderink, Harry van Goor, Jan-Luuk Hillebrands, Charlotte A. te Velde-Keyzer, Stephan J.L. Bakker, Andreas Pasch, Peter R. van Dijk, Gozewijn D. Laverman, Martin H. de Borst","doi":"10.1111/joim.13781","DOIUrl":"10.1111/joim.13781","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Background and aims</h3>\u0000 \u0000 <p>Individuals with type 2 diabetes (T2D) have a higher risk of cardiovascular disease, compared with those without T2D. The serum T50 test captures the transformation time of calciprotein particles in serum. We aimed to assess whether serum T50 predicts cardiovascular mortality in T2D patients, independent of traditional risk factors.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>We analyzed 621 individuals with T2D in this prospective cohort study. Cox regression models were performed to test the association between serum T50 and cardiovascular and all-cause mortality. Causes of death were categorized according to ICD-10 codes. Risk prediction improvement was assessed by comparing Harrell's C for models without and with T<sub>50.</sub></p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>The mean age was 64.2 ± 9.8 years, and 61% were male. The average serum T50 time was 323 ± 63 min. Higher age, alcohol use, high-sensitive C-reactive protein, and plasma phosphate were associated with lower serum T<sub>50</sub> levels. Higher plasma triglycerides, venous bicarbonate, sodium, magnesium, and alanine aminotransferase were associated with higher serum T50 levels. After a follow-up of 7.5[5.4–10.7] years, each 60 min decrease in serum T50 was associated with an increased risk of cardiovascular (fully adjusted HR 1.32, 95% CI 1.08–1.50, and <i>p</i> = 0.01) and all-cause mortality (HR 1.15, 95%CI 1.00–1.38, and <i>p</i> = 0.04). Results were consistent in sensitivity analyses after exclusion of individuals with estimated glomerular filtration rate <45 or <60 mL/min/1.73 m<sup>2</sup> and higher plasma phosphate levels.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Conclusions</h3>\u0000 \u0000 <p>Serum T50 improves prediction of cardiovascular and all-cause mortality risk in individuals with T2D. Serum T50 may be useful for risk stratification and to guide therapeutic strategies aiming to reduce cardiovascular mortality in T2D.</p>\u0000 </section>\u0000 </div>","PeriodicalId":196,"journal":{"name":"Journal of Internal Medicine","volume":"295 6","pages":"748-758"},"PeriodicalIF":11.1,"publicationDate":"2024-03-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1111/joim.13781","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140287820","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
<p>At present, the dominating modality of assisted reproductive technology (ART) is in vitro fertilization (IVF). This treatment was introduced in 1978 with the birth of Louise Joy Brown in the United Kingdom [<span>1</span>]. The field of ART has ever since expanded, and today, more than 10 million children have been born as a result of IVF [<span>2</span>].</p><p>About a third of embryo transfers after IVF result in a clinical pregnancy, and a fourth in a live-born child [<span>3</span>]. This results in an annual increase of half a million children born after IVF as the result of 2 million annual embryo transfers. The remaining embryos are, in most cases, cryopreserved and available for future embryo transfers [<span>4</span>]. Use of frozen and thawed embryo transfers (FET) was previously considered to lead to fewer successful pregnancies as compared to fresh embryo transfers, but technical advances, such as innovative freezing techniques, vitrification and visual embryo selection of embryos or blastocysts, have improved the success rate of FET to a level on par with that of fresh embryo transfers [<span>5</span>].</p><p>The risk of venous thromboembolism (VTE), including pulmonary embolism (PE), is increased in women during pregnancy [<span>6</span>]. The incidence is most pronounced during the third trimester of pregnancy and in the immediate postpartum period. The mortality rate in pregnant women afflicted by VTE has been estimated to be between 0.8 and 1.5 per 100,000 pregnancies, with more than 90% of fatal VTEs being due to PE.</p><p>IVF results in a more than eightfold increase in both VTEs and PEs during the first trimester of fresh embryo transfer pregnancies [<span>7</span>]. There was no such increase in the incidence of VTEs and PEs after FET/thawed embryo transfer pregnancies [<span>8</span>]. This indicates that ovarian stimulation, with its oestrogen surge, seems to be a necessary prerequisite to trigger the increase in VTEs and PEs.</p><p>Furthermore, gestational hypertension and pre-eclampsia have been reported to increase during IVF pregnancies [<span>9</span>]. A recent interesting observation suggests that this could be related to the absence of a corpus luteum in some pregnancies.</p><p>Concerning cardiovascular disease associated with unsuccessful IVF treatment – the majority of embryo transfers – there are conflicting reports. One study showed an increased incidence of PE after unsuccessful IVF, but on the contrary, another study showed a lower incidence of VTE after failed ART.</p><p>Furthermore, many women will experience multiple subsequent IVF cycles due to the fact that only a quarter of the embryo transfers result in a live-born child. The cardiovascular effect of multiple subsequent IVF cycles has not yet been studied.</p><p>There has been a paucity of studies concerning the long-term effects of IVF on cardiovascular health. The heterogeneity of the few pre-existing studies precluded any final conclusions, but a
目前，辅助生殖技术（ART）的主要方式是体外受精（IVF）。1978 年，随着路易丝-乔伊-布朗在英国的诞生，这种治疗方法被引入[1]。从那时起，ART 领域不断扩大，如今，已有超过 1000 万名婴儿通过体外受精出生[2]。因此，在每年 200 万次胚胎移植的基础上，体外受精后出生的婴儿每年增加 50 万。在大多数情况下，剩余的胚胎被冷冻保存起来，供将来的胚胎移植使用 [4]。与新鲜胚胎移植相比，冷冻和解冻胚胎移植（FET）以前被认为会导致较少的成功妊娠，但创新的冷冻技术、玻璃化和胚胎或囊胚的可视化胚胎选择等技术进步已将 FET 的成功率提高到与新鲜胚胎移植相当的水平[5]。妊娠期妇女发生静脉血栓栓塞（VTE），包括肺栓塞（PE）的风险增加[6]。据估计，受 VTE 影响的孕妇死亡率为每 10 万次妊娠中 0.8 至 1.5 例，其中 90% 以上的致命 VTE 是由 PE 引起的。在新鲜胚胎移植妊娠的前三个月，IVF 会导致 VTE 和 PE 的发生率增加 8 倍以上[7]。而在 FET/解冻胚胎移植妊娠后，VTE 和 PE 的发生率并没有增加[8]。这表明卵巢刺激及其雌激素激增似乎是引发 VTE 和 PE 增加的必要先决条件。最近一项有趣的观察表明，这可能与某些妊娠缺乏黄体有关。关于与试管婴儿治疗不成功（大多数胚胎移植）相关的心血管疾病，有相互矛盾的报道。一项研究显示，试管婴儿失败后 PE 的发病率增加，但相反，另一项研究显示，ART 失败后 VTE 的发病率较低。此外，由于只有四分之一的胚胎移植结果是活产，许多妇女会经历多次后续试管婴儿周期。关于试管婴儿对心血管健康的长期影响的研究还很少。现有的几项研究存在异质性，因此无法得出最终结论，但可能存在中风发病率增高的趋势[10, 11]。然而，在本期《内科学杂志》上，Yamada 等人[12] 报道了 ART 与产后因心脏病住院的风险增加有关（HR 1.99，CI：1.80-2.20）。这是规模最大的回顾性队列研究，包括 31,339,991 例分娩，其中 287,813 例接受过不孕症治疗。高血压疾病的风险最大（HR 2.16，CI：1.92-2.42）。随访时间为 1 年，风险增加在分娩后 30 天就已显现。这与之前指出产后高血压风险增加的研究结果一致[10, 11]。作者最近报告了抗逆转录病毒疗法后第一年因中风住院的发生率增加[13]。然而，最近一项来自北欧国家的大型研究报告显示，抗逆转录病毒疗法后分娩的妇女患心血管疾病的风险并没有增加[14]。这项研究涵盖了 2496 441 名准妈妈，其中 97 474 人是在抗逆转录病毒疗法后分娩的。然而，可以注意到的是，北欧研究没有报告高血压疾病的发病率，而且他们还指出，随访是从产后 2 年开始的。这意味着这两项研究无法就产后第一年的心血管疾病进行比较。在这种情况下，还可以注意到之前的一项研究也报告称，产后头几年高血压疾病的发病率显著增加[11]。然而，缺血性心脏病主要是一种高龄疾病，而且没有一项研究有足够长的随访时间。北欧研究中，随访结束时妇女的中位年龄为 41 岁。 据报道，不孕症妇女的心血管风险因素较低。此外，多囊卵巢综合征是导致女性不孕的常见原因，而患有多囊卵巢综合征的女性往往具有多种心血管代谢风险因素。卵巢刺激过程中雌激素水平升高可能是鲜胚移植试管婴儿术后早期血栓栓塞症的诱因或始作俑者，这是因为卵巢刺激过程中雌激素水平升高后，与自然妊娠相比，雌激素水平在这三个月的前三分之二会显著升高。这意味着雌激素水平的升高不会在产后持续。然而，人们可能会推测，雌激素可能是妊娠高血压紊乱（HDP）的诱因，并可能在产后继续存在。需要填补的一个重要知识空白是，有关试管婴儿失败后心血管疾病风险的结果相互矛盾。必须在全国范围的登记册中对这一问题进行检验。这一点至关重要，因为如前所述，不成功的试管婴儿占试管婴儿的大多数。即使是不涉及试管婴儿的其他类型的 ART 治疗，如宫腔内人工授精，也会使用卵巢刺激方案来增加成熟卵母细胞的数量。这意味着雌激素水平会增加。此外，最近关于在 FET 和卵母细胞捐献过程中是否存在黄体的影响的有趣发现也应进一步探讨。试管婴儿四十多年来满足了许多妇女在生育能力下降的情况下怀孕的愿望。然而，对于这些妇女来说，妊娠期血管内血栓形成是一种可能危及生命的疾病。产后至少在第一年内，罹患高血压疾病和中风的风险似乎会增加。然而，心血管疾病的长期风险必须等待大型队列研究的长期跟踪：作者声明无利益冲突。
{"title":"Infertility treatment and cardiovascular disease: What do we know?","authors":"Peter Henriksson","doi":"10.1111/joim.13779","DOIUrl":"10.1111/joim.13779","url":null,"abstract":"<p>At present, the dominating modality of assisted reproductive technology (ART) is in vitro fertilization (IVF). This treatment was introduced in 1978 with the birth of Louise Joy Brown in the United Kingdom [<span>1</span>]. The field of ART has ever since expanded, and today, more than 10 million children have been born as a result of IVF [<span>2</span>].</p><p>About a third of embryo transfers after IVF result in a clinical pregnancy, and a fourth in a live-born child [<span>3</span>]. This results in an annual increase of half a million children born after IVF as the result of 2 million annual embryo transfers. The remaining embryos are, in most cases, cryopreserved and available for future embryo transfers [<span>4</span>]. Use of frozen and thawed embryo transfers (FET) was previously considered to lead to fewer successful pregnancies as compared to fresh embryo transfers, but technical advances, such as innovative freezing techniques, vitrification and visual embryo selection of embryos or blastocysts, have improved the success rate of FET to a level on par with that of fresh embryo transfers [<span>5</span>].</p><p>The risk of venous thromboembolism (VTE), including pulmonary embolism (PE), is increased in women during pregnancy [<span>6</span>]. The incidence is most pronounced during the third trimester of pregnancy and in the immediate postpartum period. The mortality rate in pregnant women afflicted by VTE has been estimated to be between 0.8 and 1.5 per 100,000 pregnancies, with more than 90% of fatal VTEs being due to PE.</p><p>IVF results in a more than eightfold increase in both VTEs and PEs during the first trimester of fresh embryo transfer pregnancies [<span>7</span>]. There was no such increase in the incidence of VTEs and PEs after FET/thawed embryo transfer pregnancies [<span>8</span>]. This indicates that ovarian stimulation, with its oestrogen surge, seems to be a necessary prerequisite to trigger the increase in VTEs and PEs.</p><p>Furthermore, gestational hypertension and pre-eclampsia have been reported to increase during IVF pregnancies [<span>9</span>]. A recent interesting observation suggests that this could be related to the absence of a corpus luteum in some pregnancies.</p><p>Concerning cardiovascular disease associated with unsuccessful IVF treatment – the majority of embryo transfers – there are conflicting reports. One study showed an increased incidence of PE after unsuccessful IVF, but on the contrary, another study showed a lower incidence of VTE after failed ART.</p><p>Furthermore, many women will experience multiple subsequent IVF cycles due to the fact that only a quarter of the embryo transfers result in a live-born child. The cardiovascular effect of multiple subsequent IVF cycles has not yet been studied.</p><p>There has been a paucity of studies concerning the long-term effects of IVF on cardiovascular health. The heterogeneity of the few pre-existing studies precluded any final conclusions, but a ","PeriodicalId":196,"journal":{"name":"Journal of Internal Medicine","volume":"295 5","pages":"580-582"},"PeriodicalIF":11.1,"publicationDate":"2024-03-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1111/joim.13779","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140157184","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"18th Key Symposium: Longevity and Healthy Ageing: What can we learn from Blue Zones?","authors":"","doi":"10.1111/joim.13730","DOIUrl":"10.1111/joim.13730","url":null,"abstract":"","PeriodicalId":196,"journal":{"name":"Journal of Internal Medicine","volume":"295 4","pages":"386"},"PeriodicalIF":11.1,"publicationDate":"2024-03-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140130300","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Both ovarian and testicular germ cell tumors (GCTs) arise from the primordial germ cell and share many similarities. Both malignancies affect mainly young patients, show remarkable responsiveness to cisplatin-based therapy, and have an excellent prognosis, which also highlights the importance of minimizing long-term side effects. However, certain differences can be noted: The spreading of the disease differs, and the staging system and treatment recommendations are dissimilar. Moreover, the prognosis for ovarian GCTs is significantly inferior to that for testicular cancer, as exemplified in this review comparing the survival in Swedish patients diagnosed with testicular (1995–2022) and ovarian (1990–2018) GCTs. The 5-year overall survival in ovarian GCTs was 85.2%, versus 98.2% for testicular GCTs. How can this be explained? One reason may be the difference in knowledge, experience, and evidence because the incidence rate of testicular cancer is more than 15 times that of ovarian GCTs. Given the rarity of the disease in women and the lack of established guidelines, a comprehensive understanding of the disease and treatment decisions is challenging. The main objective of this review is to derive insights from testicular GCTs (seminoma and non-seminoma) by reviewing etiological, tumor biological, and clinical knowledge, and to thereafter suggest actions for ovarian GCTs based on this. We hypothesize that by adopting specific treatment strategies from testicular GCTs—including de-escalating adjuvant chemotherapy for low-risk patients and implementing more standardized and intensive treatment protocols in cases of relapse—we can improve the prognosis and minimize long-term side effects in ovarian GCT patients.
{"title":"Malignant ovarian and testicular germ cell tumors: Common characteristics but different prognoses","authors":"Camilla Sköld, Anna K Jansson, Ingrid Glimelius","doi":"10.1111/joim.13778","DOIUrl":"10.1111/joim.13778","url":null,"abstract":"<p>Both ovarian and testicular germ cell tumors (GCTs) arise from the primordial germ cell and share many similarities. Both malignancies affect mainly young patients, show remarkable responsiveness to cisplatin-based therapy, and have an excellent prognosis, which also highlights the importance of minimizing long-term side effects. However, certain differences can be noted: The spreading of the disease differs, and the staging system and treatment recommendations are dissimilar. Moreover, the prognosis for ovarian GCTs is significantly inferior to that for testicular cancer, as exemplified in this review comparing the survival in Swedish patients diagnosed with testicular (1995–2022) and ovarian (1990–2018) GCTs. The 5-year overall survival in ovarian GCTs was 85.2%, versus 98.2% for testicular GCTs. How can this be explained? One reason may be the difference in knowledge, experience, and evidence because the incidence rate of testicular cancer is more than 15 times that of ovarian GCTs. Given the rarity of the disease in women and the lack of established guidelines, a comprehensive understanding of the disease and treatment decisions is challenging. The main objective of this review is to derive insights from testicular GCTs (seminoma and non-seminoma) by reviewing etiological, tumor biological, and clinical knowledge, and to thereafter suggest actions for ovarian GCTs based on this. We hypothesize that by adopting specific treatment strategies from testicular GCTs—including de-escalating adjuvant chemotherapy for low-risk patients and implementing more standardized and intensive treatment protocols in cases of relapse—we can improve the prognosis and minimize long-term side effects in ovarian GCT patients.</p>","PeriodicalId":196,"journal":{"name":"Journal of Internal Medicine","volume":"295 6","pages":"715-734"},"PeriodicalIF":11.1,"publicationDate":"2024-03-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1111/joim.13778","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140100579","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
João Fernandes-Serodio, Sergio Prieto-González, Georgina Espígol-Frigolé, Roberto Ríos-Garcés, Verónica Gómez-Caverzaschi, Olga Araújo, Gerard Espinosa, Raül Jordà-Sánchez, Marco A. Alba, Luis Quintana, Miquel Blasco, Elena Guillen, Odette Viñas, Estíbaliz Ruiz-Ortiz, Laura Pelegrín, Maite Sainz de la Maza, Bernardo Sánchez-Dalmau, Adriana García-Herrera, Manel Solé, Paola Castillo, Iban Aldecoa, María D. Cano, Jacobo Sellarés, Fernanda Hernández-González, Carlos Agustí, Carmen M. Lucena, Antonio López-Rueda, Marcelo Sánchez, Mariana Benegas, Sebastián Capurro, Raimon Sanmartí, Josep M. Grau, Isabel Vilaseca, Isam Alobid, Maria C. Cid, José Hernández-Rodríguez
Background
Microscopic polyangiitis (MPA) and granulomatosis with polyangiitis (GPA) are the two major antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV).
Objectives
To characterize a homogenous AAV cohort and to assess the impact of clinicopathological profiles and ANCA serotypes on clinical presentation and prognosis. Clinical differences in GPA patients according to ANCA serotype and the diagnostic yield for vasculitis of biopsies in different territories were also investigated.
Results
This retrospective study (2000–2021) included 152 patients with AAV (77 MPA/75 GPA). MPA patients (96.1% myeloperoxidase [MPO]–ANCA and 2.6% proteinase 3 [PR3]–ANCA) presented more often with weight loss, myalgia, renal involvement, interstitial lung disease (ILD), cutaneous purpura, and peripheral nerve involvement. Patients with GPA (44% PR3–ANCA, 33.3% MPO, and 22.7% negative/atypical ANCA) presented more commonly with ear, nose, and throat and eye/orbital manifestations, more relapses, and higher survival than patients with MPA. GPA was the only independent risk factor for relapse. Poor survival predictors were older age at diagnosis and peripheral nerve involvement. ANCA serotypes differentiated clinical features in a lesser degree than clinical phenotypes. A mean of 1.5 biopsies were performed in 93.4% of patients in different territories. Overall, vasculitis was identified in 80.3% (97.3% in MPA and 61.8% in GPA) of patients.
Conclusions
The identification of GPA presentations associated with MPO–ANCA and awareness of risk factors for relapse and mortality are important to guide proper therapeutic strategies in AAV patients. Biopsies of different affected territories should be pursued in difficult-to-diagnose patients based on their significant diagnostic yield.
{"title":"Significance of clinical-immunological patterns and diagnostic yield of biopsies in microscopic polyangiitis and granulomatosis with polyangiitis","authors":"João Fernandes-Serodio, Sergio Prieto-González, Georgina Espígol-Frigolé, Roberto Ríos-Garcés, Verónica Gómez-Caverzaschi, Olga Araújo, Gerard Espinosa, Raül Jordà-Sánchez, Marco A. Alba, Luis Quintana, Miquel Blasco, Elena Guillen, Odette Viñas, Estíbaliz Ruiz-Ortiz, Laura Pelegrín, Maite Sainz de la Maza, Bernardo Sánchez-Dalmau, Adriana García-Herrera, Manel Solé, Paola Castillo, Iban Aldecoa, María D. Cano, Jacobo Sellarés, Fernanda Hernández-González, Carlos Agustí, Carmen M. Lucena, Antonio López-Rueda, Marcelo Sánchez, Mariana Benegas, Sebastián Capurro, Raimon Sanmartí, Josep M. Grau, Isabel Vilaseca, Isam Alobid, Maria C. Cid, José Hernández-Rodríguez","doi":"10.1111/joim.13777","DOIUrl":"10.1111/joim.13777","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Background</h3>\u0000 \u0000 <p>Microscopic polyangiitis (MPA) and granulomatosis with polyangiitis (GPA) are the two major antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV).</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Objectives</h3>\u0000 \u0000 <p>To characterize a homogenous AAV cohort and to assess the impact of clinicopathological profiles and ANCA serotypes on clinical presentation and prognosis. Clinical differences in GPA patients according to ANCA serotype and the diagnostic yield for vasculitis of biopsies in different territories were also investigated.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>This retrospective study (2000–2021) included 152 patients with AAV (77 MPA/75 GPA). MPA patients (96.1% myeloperoxidase [MPO]–ANCA and 2.6% proteinase 3 [PR3]–ANCA) presented more often with weight loss, myalgia, renal involvement, interstitial lung disease (ILD), cutaneous purpura, and peripheral nerve involvement. Patients with GPA (44% PR3–ANCA, 33.3% MPO, and 22.7% negative/atypical ANCA) presented more commonly with ear, nose, and throat and eye/orbital manifestations, more relapses, and higher survival than patients with MPA. GPA was the only independent risk factor for relapse. Poor survival predictors were older age at diagnosis and peripheral nerve involvement. ANCA serotypes differentiated clinical features in a lesser degree than clinical phenotypes. A mean of 1.5 biopsies were performed in 93.4% of patients in different territories. Overall, vasculitis was identified in 80.3% (97.3% in MPA and 61.8% in GPA) of patients.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Conclusions</h3>\u0000 \u0000 <p>The identification of GPA presentations associated with MPO–ANCA and awareness of risk factors for relapse and mortality are important to guide proper therapeutic strategies in AAV patients. Biopsies of different affected territories should be pursued in difficult-to-diagnose patients based on their significant diagnostic yield.</p>\u0000 </section>\u0000 </div>","PeriodicalId":196,"journal":{"name":"Journal of Internal Medicine","volume":"295 5","pages":"651-667"},"PeriodicalIF":11.1,"publicationDate":"2024-03-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140093107","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
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