Materiały źródłowe

• #1 Predictors of poor prognosis in ANCA-associated vasculitis (AAV): a single-center prospective study of inpatients in China

  https://pmc.ncbi.nlm.nih.gov/articles/PMC10390347/

  To identify potential predictors by assessing adverse outcomes in ANCA-associated vasculitis (AAV) patients. […] We determined risk factors of high-risk events, defined as developing tumors, renal replacement therapy (RRT), and death. […] Patients with lower serum potassium tended to develop tumors (P=0.033); with higher BVAS (HR=1.290, 95%CI 1.0751.549, P=0.006) and lower eGFR (HR=0.782, 95%CI 0.6800.901, P=0.001) were more likely to undergo RRT. […] Through multivariate COX analysis, age (HR=1.016, 95%CI 1.0161.105, P=0.006) and eGFR (HR=0.982, 95%CI 0.9680.997, P=0.018) predicted death in AAV, separately. […] The BVAS and eGFR could be a great prognosticator for RRT, while age and eGFR can independently predict the death. […] A meta-analysis on the prognosis of AAV proves that the risk of death is at least 2.7 times higher compared to the general population.

• #1 Vasculitis | Diagnosis & Disease Info – Rheumatology Advisor

  https://www.rheumatologyadvisor.com/ddi/vasculitis/

  Disease prognosis depends on the degree of end-organ involvement. The 5-year survival rate increased from 70% to 80% between the 1990s and 2000s, likely due to ANCA testing that led to earlier diagnosis and treatment. […] Factors that influence patient outcomes include: Age, Vasculitis damage index of at least 1, Birmingham vasculitis activity score, Serum creatinine, End-stage renal disease, First relapse within 1 year, Infection burden. […] Relapse incidence also helps determine AAV prognosis. In patients with GPA, the introduction of cyclophosphamide has led to a clinical response in 90% of patients. […] Early mortality spike within 1 to 2 years of diagnosis has been observed in patients with GCA. Main etiologies for mortality include cardiovascular diseases (39%), cerebrovascular diseases (14%), infections (13%), malignancies (13%), and gastrointestinal complications (4%). […] The 10-year overall survival for Takayasu arteritis is 97% and the 15-year overall survival is 86%.

• #1 Early predictors of one-year mortality in patients over 65 presenting with ANCA-associated renal vasculitis: a retrospective, multicentre study | BMC Nephrology | Full Text

  https://bmcnephrol.biomedcentral.com/articles/10.1186/s12882-018-1102-3

  The risk of early death is particularly high in patients over the age of 65 presenting with antineutrophil cytoplasmic antibody (ANCA)-associated renal vasculitis. […] The one-year mortality rate was 19.5%. […] In a multivariable analysis, the best predictors were a high FFS (relative risk (RR) [95% confidence interval (CI)]=2.57 [1.305.09]; p=0.006) and the occurrence of a severe infection during the first month (RR [95%CI]=2.74 [1.275.92]; p=0.01). […] When considering various disease severity markers in over-65 patients with ANCA-associated renal vasculitis, we found that an early, severe infection (which occurred in about a quarter of the patients) is a strong predictor of one-year mortality. […] Overall, 29 patients died during the first year of care, giving a one-year mortality rate of 19.5%.

• #1 Low levels of hemoglobin associate with critical illness and predict disease course in patients with ANCA-associated renal vasculitis | Scientific Reports

  https://www.nature.com/articles/s41598-022-23313-7

  Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a small vessel vasculitis often leading to critical illness by multi-organ failure. […] We here identified an association between low hemoglobin levels and requirement of ICU supportive care in patients with ANCA-associated renal vasculitis. Furthermore, levels of hemoglobin below 9.8 g/dL at admission independently predicted prolonged requirement of ICU supportive care in critically ill patients with ANCA-associated renal vasculitis. […] These findings confirm that low levels of hemoglobin negatively affect short-term outcome and could further improve our current understanding for the role of anemia in ANCA-associated renal vasculitis. […] Critical illness due to renal failure was associated with increased PEX and intravenous steroid pulse treatments in this subgroup.

• #1

  https://link.springer.com/article/10.1007/s10067-016-3195-z

  On multivariate models, renal replacement therapy (RRT) requirement (HR 8.07, CI 1.7537.4, p=0.008) and proteinuria (HR 1.49, CI 1.032.14, p=0.034) at presentation predicted renal survival, while age (HR 1.10, CI 1.011.21, p=0.041) and infective events during the induction phase (HR 4.72, 1.0122.1, p=0.049) negatively influenced patient survival. […] At present, ANCA-based serological classification may predict AAV relapses, but neither clinical nor serological categories predict renal or patient survival. […] Age, renal function and proteinuria at presentation, histopathology, and infectious complications constitute the main outcome predictors and should be considered for individualized management.

• #1

  https://link.springer.com/article/10.1007/s10067-016-3195-z

  Several classification schemes have been developed for anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV), with actual debate focusing on their clinical and prognostic performance. […] Clinical presentation parameters were compared at baseline between classification groups, and the predictive value of different classification categories for disease and renal remission, relapse, renal, and patient survival was analyzed. […] Serological classification predicted relapse rate (PR3-ANCA hazard ratio for relapse 2.93, 1.207.17, p=0.019). […] There were no differences in disease or renal remission, renal, or patient survival between clinical and serological categories. […] Histopathological classification predicted response to therapy, with a poorer renal remission rate for sclerosing group and those with less than 25 % normal glomeruli; in addition, it adequately delimited 24-month glomerular filtration rate (eGFR) evolution, but it did not predict renal nor patient survival.

• #1 Development and internal validation of a model to predict long-term survival of ANCA associated vasculitis

  https://www.degruyter.com/document/doi/10.2478/rir-2023-0005/html?lang=en

  Risk stratification and prognosis prediction are critical for appropriate management of anti-neutrophil cytoplasmic antibody (ANCA) associated vasculitis (AAV). Herein, we aim to develop and internally validate a prediction model specifically for long-term survival of patients with AAV. […] Our model performs well in predicting outcomes of AAV patients. Patients with moderate-to-high probability of death should be followed closely and personalized monitoring plan should be scheduled. […] In this study, we developed a new model to predict long-term survival of patients with AAV (PRESAAV) and internally validate the performance of the model by the bootstrap resampling method. […] The optimism-corrected C-index and integrated Brier score of our prediction model were 0.728 and 0.109. The calibration plots showed fine agreement between observed and predicted probability of all-cause death.

• #1 Development and internal validation of a model to predict long-term survival of ANCA associated vasculitis

  https://www.degruyterbrill.com/document/doi/10.2478/rir-2023-0005/html?lang=en&srsltid=AfmBOoqZp75agMuwn-gbEIdqT2C_PZpKa4J8TFMJN9_eEKcS2p49uJlE

  Risk stratification and prognosis prediction are critical for appropriate management of anti-neutrophil cytoplasmic antibody (ANCA) associated vasculitis (AAV). Herein, we aim to develop and internally validate a prediction model specifically for long-term survival of patients with AAV. […] Our model performs well in predicting outcomes of AAV patients. Patients with moderate-to-high probability of death should be followed closely and personalized monitoring plan should be scheduled. […] In this study, we developed a new model to predict long-term survival of patients with AAV (PRESAAV) and internally validate the performance of the model by the bootstrap resampling method. […] The optimism-corrected C-index and integrated Brier score of our prediction model were 0.728 and 0.109. The calibration plots showed fine agreement between observed and predicted probability of all-cause death.

• #1 AAV prognosis predicted with good accuracy using new statistical model | ANCA Vasculitis NewsEnvelope icon

  https://ancavasculitisnews.com/news/aav-prognosis-predicted-with-good-accuracy-using-new-statistical-model/

  Results showed the 5-year C-index for the model was about 0.75, suggesting a fairly good predictive ability. Its C-index scores were generally higher than those of two currently used AAV prognostic systems — the revised five factor score (rFFS) and the Birmingham vasculitis activity score (BVAS) system. […] “Compared with the rFFS and the BVAS system, the current prediction model had higher predictive power for overall survival,” the researchers wrote, adding that “in a quite wide range of threshold probabilities, our prediction model had higher net benefits, implying probable better clinical values.” […] “In addition to the factors included in the widely used rFFS system, our prediction model showed that lung involvement, hemoglobin levels, and AAV subtypes” can help predict survival, they wrote. […] While the new model may be useful for predicting AAV prognosis, the researchers acknowledged their analysis was limited by the use of retrospective data from a single specialty center in China and that more research is needed to test whether their model performs well for a broader population.

• #1 Development and internal validation of a model to predict long-term survival of ANCA associated vasculitis

  https://www.degruyterbrill.com/document/doi/10.2478/rir-2023-0005/html?lang=en&srsltid=AfmBOoqZp75agMuwn-gbEIdqT2C_PZpKa4J8TFMJN9_eEKcS2p49uJlE

  The three risk groups were defined as low risk (PI 2.294), moderate risk (2.294 PI 3.479) and high risk (PI 3.479), respectively. The five-year survival probability in low risk, moderate risk and high risk were 90.04%, 70.96% and 70.96% according to the Formula 1, respectively. […] The C-index of rFFS for survival prediction was 0.74 in Spanish patients with AAV implying modest discriminative ability. In our patients, the C-index of rFFS for 5-year survival prediction was 0.735, which is comparable to the Spanish patients. Meanwhile, in our prediction model, the optimism corrected C-index for 5-year survival prediction was 0.748, which indicates that the discrimination of our model is comparable to rFFS. […] We developed and internally validated a model specifically for AAV to predict long term survival of patients with this disease. In addition to the factors included in the widely used rFFS system, our prediction model showed that lung involvement, hemoglobin levels and AAV subtypes are also predictive factors for the survival of AAV patients. And the long-term survival prediction model developed in this study performs well.

• #1 Neutrophil to lymphocyte ratio at diagnosis can estimate vasculitis activity and poor prognosis in patients with ANCA-associated vasculitis: a retrospective study | BMC Nephrology | Full Text

  https://bmcnephrol.biomedcentral.com/articles/10.1186/s12882-018-0992-4

  Neutrophil to lymphocyte ratio (NLR) was introduced to predict poor prognosis in various diseases, but not all variants of ANCA-associated vasculitis (AAV). In this study, we aimed to investigate whether NLR at diagnosis can estimate vasculitis activity at diagnosis and poor prognosis during follow-up in patients with AAV. […] NLR at diagnosis can estimate vasculitis activity at diagnosis and predict relapse during follow-up in patients with AAV. […] NLR5.9 at diagnosis is a predictor of relapse of AAV, but not death, during follow-up. Therefore, we believe that NLR at diagnosis is a useful marker to estimate vasculitis activity at diagnosis and poor prognosis during follow-up in AAV patients. […] We first demonstrated that NLR5.9 at diagnosis can predict relapse of AAV during follow-up. […] With these results, we suggest that physicians may calculate NLR at diagnosis to predict poor prognosis of AAV during follow-up.

• #1 Urinary T cells identify renal antineutrophil cytoplasmic antibody-associated vasculitis and predict prognosis: a proof of concept study – MDC Repository

  https://edoc.mdc-berlin.de/id/eprint/23285/

  Urinary T cells identify renal antineutrophil cytoplasmic antibody-associated vasculitis and predict prognosis: a proof of concept study […] Patients whose kidney biopsies had been classified as „crescentic” according to Berden classification showed higher urinary T cell counts. […] Furthermore, urinary T(reg) and T helper cells (T(H)17) patterns were associated with clinical response and risk of renal relapse. […] Their promising potential as noninvasive diagnostic and prognostic biomarkers deserves further exploitation.

• #1 AAV prognosis predicted with good accuracy using new statistical model | ANCA Vasculitis NewsEnvelope icon

  https://ancavasculitisnews.com/news/aav-prognosis-predicted-with-good-accuracy-using-new-statistical-model/

  A new statistical model that considers age, disease type, patterns of organ involvement, and lab findings may help predict long-term survival in ANCA-associated vasculitis (AAV) better than available prognostic systems, a study suggests. […] While “risk stratification and prognosis prediction are critical for appropriate management of” AAV, a model for prognosis prediction is still in need, wrote researchers in China who analyzed clinical data from 653 people with AAV treated at a single center in the country between 1999-2019 to create one. […] In the final model, the prognosis was generally poorer for those who were older, had lung and/or heart involvement, had high blood levels of creatinine, or low hemoglobin levels. It was also poorest for MPA patients, followed by those with GPA and then EGPA.

• #1 IgA Vasculitis (Henoch-Schonlein Purpura): Practice Essentials, Background, Pathophysiology

  https://emedicine.medscape.com/article/984105-overview

  IgAV is the most common vasculitis in childhood. The incidence decreases with age, but in adults the severity increases with age. […] Acute involvement of the GI tract impacts the short-term prognosis of the disease, while long-term prognosis depends on the severity of the kidney disease. Progression to chronic kidney failure may occur more than a decade after the onset of IgAV. […] IgAV is generally a benign disease with an excellent prognosis. Spontaneous resolution is usual: Most patients experience complete resolution of symptoms within 8 weeks, and probably fewer than 5% experience chronic symptoms. However, initial attacks of IgAV can last several months, and relapses are possible. […] A clinical course with complete resolution of the disease usually occurs in patients with the following: Mild kidney involvement, No neurologic complications, Disease that lasts less than 4-6 weeks initially.

• #1 IgA Vasculitis (Henoch-Schonlein Purpura): Practice Essentials, Background, Pathophysiology

  https://emedicine.medscape.com/article/984105-overview

  Recurrences occur in as many as 50% of patients within 6 weeks but can happen as late as 7 years after the initial disease. […] Although IgAV generally resolves without permanent consequences, serious GI and renal complications may occur, and the higher the number of recurrences, the higher the likelihood of permanent kidney damage. […] Kidney damage related to IgAV is the primary cause of morbidity and mortality. As many as 15% of patients may have long-term kidney insufficiency, but no more than 1-2% will have ESKD. […] Predictors of serious nephropathy or end-stage kidney disease (ESKD) include bloody stools and persistent rash.

• #1 Imaging in diagnosis, monitoring and outcome prediction of large vessel vasculitis: a systematic literature review and meta-analysis informing the 2023 update of the EULAR recommendations | RMD Open

  https://rmdopen.bmj.com/content/9/3/e003379

  Objectives To update the evidence on imaging for diagnosis, monitoring and outcome prediction in large vessel vasculitis (LVV) to inform the 2023 update of the European Alliance of Associations for Rheumatology recommendations on imaging in LVV. […] No evidence was found to determine whether imaging severity might predict worse clinical outcomes. […] Overall, imaging did not predict response to treatment, occurrence of relapses or ischaemic complications. The presence of FDG uptake at baseline, however, was linked with the subsequent development of angiographic changes (stenosis, occlusion or aneurysm) in both GCA and TAK.

• #1 Imaging in diagnosis, outcome prediction and monitoring of large vessel vasculitis: a systematic literature review and meta-analysis informing the EULAR recommendations | RMD Open

  https://rmdopen.bmj.com/content/4/1/e000612

  Objectives To perform a systematic literature review on imaging techniques for diagnosis, outcome prediction and disease monitoring in large vessel vasculitis (LVV) informing the European League Against Rheumatism recommendations for imaging in LVV. […] Studies on outcome prediction and monitoring disease activity/damage were limited and mainly descriptive. […] More data on the role of imaging for diagnosis of extracranial large vessel GCA and TAK, as well as for outcome prediction and monitoring in LVV are warranted. […] Studies on the role of imaging techniques for outcome prediction and monitoring of disease activity and damage of LVV are limited and mainly non-informative because of heterogeneous study design and of being mostly descriptive, not enabling reaching an inferential conclusion.

• #1 Cryptococcosis in Non-HIV Immunocompromised Hosts: Epidemiology, Pathogenesis, and Management | IntechOpen

  https://www.intechopen.com/online-first/1213803

  In cryptococcosis, serum CrAg is now a well-accepted diagnostic biomarker for detecting or confirming cryptococcal infection; like in HIV patients with cryptococcus infection, high titers herald a poor prognosis. […] Regarding cryptococcus meningitis in non-HIV patients, over the years there have been described significant prognostic factors, including low CSF glucose, high CSF lactate, high CSF cryptococcal antigen titer ( or = 1:1024), initial level of consciousness, the presence of seizure, hydrocephalus, and central nervous system vasculitis. […] Non-HIV immunosuppressed hosts have been noted to have a poorer prognosis. […] In a retrospective study that included 302 patients diagnosed with cryptococcosis between 1996 and 2010, mortality was highest in the NHNT group (27%) vs. SOT (14%) and HIV (20%).

• #1 Cryptococcosis in Non-HIV Immunocompromised Hosts: Epidemiology, Pathogenesis, and Management | IntechOpen

  https://www.intechopen.com/online-first/1213803

  On univariate analyses, prognostic factors positively associated with 90-day mortality included cancer (P = 0.018), fever (P = 0.031), altered mental status (P = 0.001), positive blood cultures (P 0.001), and high (1:512) serum CrAg (P = 0.021). […] In multivariable logistic regression analyses, cryptococcemia [odds ratio (OR) 5.09, 95% confidence interval (CI) 2.5410.22; P 0.001], baseline opening pressure of 25 cm H2O (OR 2.93, 95% CI 1.256.88; P = 0.013), and altered mental status (OR 1.96, 95% CI 0.983.91; P = 0.057) were associated with increased odds of mortality. […] Another retrospective study included 28 confirmed cryptococcal infections between 2005 and 2017. […] The case fatality rate was highest among patients with underlying hematologic malignancies (63%), followed by SOT (50%) and HIV patients (20%).

• #1 Cryptococcosis in Non-HIV Immunocompromised Hosts: Epidemiology, Pathogenesis, and Management | IntechOpen

  https://www.intechopen.com/online-first/1213803

  A 12-year review at a tertiary care center (University of Kentucky HealthCare system) included 114 patients with cryptococcosis; 23 (20.2%) had HIV infection, 11 (9.6%) had transplants (nine patients had SOT and two had stem cell transplants), and 80 (70.2%) were NHNT patients. […] Overall, mortality was 28.1% (32/114), and relapse occurred in 10.5% (12/114) of patients. […] The mortality trend was higher (OR = 2.346, p = 0.287) in the transplant group (45.5%, 5/11) than in the HIV (26.1%, 6/23) and NHNT groups (26.3%, 21/80). […] Chronic liver disease had a large and statistically significant association with mortality on multivariate analysis (OR = 3.583, p = 0.013), which was more pronounced than the HIV or transplant groups.

• #1 A Risk Score for Predicting Short-Term Incidence of Death or Relapse in Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis – ACR Meeting Abstracts

  https://acrabstracts.org/abstract/a-risk-score-for-predicting-short-term-incidence-of-death-or-relapse-in-anti-neutrophil-cytoplasmic-antibody-associated-vasculitis/

  A Risk Score for Predicting Short-Term Incidence of Death or Relapse in Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis […] Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV), combining granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA), is associated with a substantial risk of relapse or death. […] Accurate prediction of short-term prognosis is crucial to identify patients at highest risk of an adverse event. […] We aimed to develop a risk score to predict the 1-year risk of death or relapse in newly diagnosed AAV. […] This integrative AAV-risk prediction score may be useful in predicting a patients risk of death or relapse on short-term follow-up and may contribute to better risk-stratified characterization and management of AAV. Further validation in other AAV populations is required.

• #2 Development and internal validation of a model to predict long-term survival of ANCA associated vasculitis

  https://www.degruyter.com/document/doi/10.2478/rir-2023-0005/html?lang=en

  Risk stratification and prognosis prediction are critical for appropriate management of anti-neutrophil cytoplasmic antibody (ANCA) associated vasculitis (AAV). Herein, we aim to develop and internally validate a prediction model specifically for long-term survival of patients with AAV. […] Our model performs well in predicting outcomes of AAV patients. Patients with moderate-to-high probability of death should be followed closely and personalized monitoring plan should be scheduled. […] In this study, we developed a new model to predict long-term survival of patients with AAV (PRESAAV) and internally validate the performance of the model by the bootstrap resampling method. […] The optimism-corrected C-index and integrated Brier score of our prediction model were 0.728 and 0.109. The calibration plots showed fine agreement between observed and predicted probability of all-cause death.

• #2 Predictors of poor prognosis in ANCA-associated vasculitis (AAV): a single-center prospective study of inpatients in China

  https://pmc.ncbi.nlm.nih.gov/articles/PMC10390347/

  Some prove CVD, malignancy, and renal death can be risk factors for premature death from AAV. […] Other have suggested that renal function, disease activity state and age are important predictors of prognosis in AAV. […] The important thing about this study is that only age at diagnosis and eGFR level independently predict death in AAV patients. […] The leading causes of death include vasculitis activity and infection, as well as cardiovascular events. Therefore, early screening of cardio and renal function in AAV patients should be performed to improve prognosis. […] In conclusion, most MPA patients with MPO positive are associated with renal damage at diagnosis, and the long-term prognosis remains poor. Decreased eGFR and C3 levels may be predictors of poor prognosis in AAV. The combination of BVAS and eGFR is a great prognosticator for RRT outcome. Age at diagnosis and eGFR can independently predict the death.

• #2 Early predictors of one-year mortality in patients over 65 presenting with ANCA-associated renal vasculitis: a retrospective, multicentre study | BMC Nephrology | Full Text

  https://bmcnephrol.biomedcentral.com/articles/10.1186/s12882-018-1102-3

  In a univariate analysis, age, the CCI, a performance status of 3 or 4, a lack of CTZ prophylaxis, and disease activity parameters (such as albuminaemia, haemoglobin level, peak SCr level, and dialysis status) were significantly associated with one-year mortality. […] Our present results highlighted the impact of early infections on mortality. […] This finding should be taken into account during the initial steps in patient management. A reduction in immunosuppression, the early detection of infections, and systematic CTZ prophylaxis might help to reduce mortality in this population.

• #2 Low levels of hemoglobin associate with critical illness and predict disease course in patients with ANCA-associated renal vasculitis | Scientific Reports

  https://www.nature.com/articles/s41598-022-23313-7

  Our observation that low levels of hemoglobin at admission correlated with prolonged requirement of ICU supportive care in this subgroup confirms that severe anemia also affects short-term disease course in critically ill patients with biopsy-proven ANCA-associated renal vasculitis. […] Our finding that low levels of hemoglobin independently predict short-term disease course in critically ill patients with biopsy-proven ANCA-associated renal vasculitis is of relevance.

• #2 Development and internal validation of a model to predict long-term survival of ANCA associated vasculitis

  https://www.degruyter.com/document/doi/10.2478/rir-2023-0005/html?lang=en

  The long-term survival of patients with AAV has been improving in the past several decades. […] In the present retrospective cohort study, we developed and internally validated a new prognostic model to predict the probability of long-term survival in patients with AAV. […] The C-index, the integrated Brier score and the calibration plots demonstrated that our prediction model performed well. […] Different from the rFFS, which only classified patients as low-, medium- and high-risk of death at the fifth year, our prediction model could obtain individual prediction of the probability of five-year survival via the Formula or the Nomogram. […] The limitations of this study include the following aspects. […] We developed and internally validated a model specifically for AAV to predict long term survival of patients with this disease. In addition to the factors included in the widely used rFFS system, our prediction model showed that lung involvement, hemoglobin levels and AAV subtypes are also predictive factors for the survival of AAV patients. And the long-term survival prediction model developed in this study performs well.

• #2 IgA Vasculitis (Henoch-Schonlein Purpura): Practice Essentials, Background, Pathophysiology

  https://emedicine.medscape.com/article/984105-overview

  Recurrences occur in as many as 50% of patients within 6 weeks but can happen as late as 7 years after the initial disease. […] Although IgAV generally resolves without permanent consequences, serious GI and renal complications may occur, and the higher the number of recurrences, the higher the likelihood of permanent kidney damage. […] Kidney damage related to IgAV is the primary cause of morbidity and mortality. As many as 15% of patients may have long-term kidney insufficiency, but no more than 1-2% will have ESKD. […] Predictors of serious nephropathy or end-stage kidney disease (ESKD) include bloody stools and persistent rash.

• #2 Imaging in diagnosis, outcome prediction and monitoring of large vessel vasculitis: a systematic literature review and meta-analysis informing the EULAR recommendations | RMD Open

  https://rmdopen.bmj.com/content/4/1/e000612

  Current evidence on the role for imaging modalities for outcome prediction, as well as for monitoring disease activity and damage in LVV is limited. […] The development of standard tools for monitoring disease activity and damage in LVV is urgently needed and might facilitate further research on the evaluation of the role of imaging for disease monitoring and outcome prediction.

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