scholarly journals BPI-ANCA and Long-Term Prognosis among 46 Adult CF Patients: A Prospective 10-Year Follow-Up Study

2012 ◽  
Vol 2012 ◽  
pp. 1-8 ◽  
Author(s):  
Ulrika Lindberg ◽  
Malin Carlsson ◽  
Claes-Göran Löfdahl ◽  
Mårten Segelmark

Introduction. Anti-neutrophil cytoplasmic antibodies specific for bactericidal/permeability-increasing protein (BPI-ANCA) are frequent in CF patients and mainly develop in response to infection withPseudomonas aeruginosa. It is not known to what extent BPI-ANCA correlates to prognosis.Objectives. To evaluate the prognostic value of IgA-BPI-ANCA, measured at the beginning of the study, for transplantation-free survival.Methods. A cohort of 46 adult, nontransplanted CF patients was generated, 1995–1998, and characterized using Leeds criteria, lung function, and IgA-BPI-ANCA levels measured by ELISA. The cohort was followed until December 2009, using the combined endpoint of death or lung transplantation.Results. Lung function and IgA-BPI-ANCA, but not Leeds criteria, were significantly associated with adverse outcome. No patient with normal lung function at baseline reached endpoint. Within 10 years 8/11 with high BPI-ANCA reached an endpoint compared to 3/17 ANCA-negative patients. A similar result was seen within the Leeds I group where 7 out of 9 BPI-ANCA-positive patients reached endpoint, compared to none of the 5 patients without BPI-ANCA.Conclusions. IgA-BPI-ANCA is associated with adverse outcome amongPseudomonas aeruginosainfected CF patients, suggesting that BPI-ANCA is a biomarker of an unfavourable host-pathogen interaction.

Author(s):  
Christine Dalgård ◽  
Fang Wang ◽  
Ingrid Louise Titlestad ◽  
Kirsten Ohm Kyvik ◽  
Jørgen Vestbo ◽  
...  

Pulmonary surfactant protein D (SP-D) is an important component of the pulmonary innate immune system with the ability to dampen cigarette smoke-induced lung inflammation. However, cigarette smoking mediates translocation of SP-D from the lung to the blood, and serum SP-D (sSP-D) has therefore previously been suggested as marker for smoke-induced lung injury. In support of this notion, associations between high sSP-D and low lung function measurements have previously been demonstrated in smokers and in COPD. The present investigations employ a 12-year longitudinal Danish twin study to test the hypothesis that baseline sSP-D variation has the capacity to identify smokers with normal baseline lung function who are in high risk of significant future smoke-induced lung function decline. We find that sSP-D is significantly increased in those with normal lung function at baseline that develop lung function decline during follow up compared to those who stay lung healthy. Moreover, we demonstrate that it is the smoke-induced baseline sSP-D level, and not the constitutional level, which has capacity as biomarker, and which is linearly increased with the decline in lung function during follow up. In conclusion, we here present first observation of increased sSP-D for identification of high-risk smokers.


2020 ◽  
Vol 41 (Supplement_2) ◽  
Author(s):  
R Matsukawa ◽  
H Ikuta ◽  
A Okahara ◽  
S Kawai ◽  
M Tokutome ◽  
...  

Abstract Background/Introduction It has been reported that heart failure (HF) readmission has not declined even with current cardiology practice in the last 10 years. It has been also reported that HF readmission tends to occur shortly after discharge. This may be due to overwork and excessive salt intake after discharge. In other words, it is conceivable that patient factors are largely attributable, which should be intervene for better clinical outcomes. Purpose We hypothesized and investigated whether an early follow-up visit at outpatient care within 2 weeks after discharge affects the re-admission rate and prognosis in patients with decompensated HF. Methods We retrospectively investigated consecutive 407 hospitalized patients due to decompensated HF. After exclusion of 99 patients with in-hospital death, transfer to another hospital and readmission within 2 weeks after discharge, consecutive 308 out of 407 patients were investigated. Two-year clinical outcomes after discharge were collected and analyzed. An early follow-up was defined as an outpatient care visit within 2 weeks after discharge with the adjustment of drugs and/or the lifestyle guidance, if necessary. A setting of early follow-up in each patient was according to a physician's discretion. Results One hundred-twenty eight patients underwent early follow-up visits and other 180 patients were without it. An univariate analysis showed that the early follow-up was significantly associated with a lower HF readmission rate during 2 years (17.1% in the early follow-up group, 34.4% in the control group, p≤0.001, OR=0.397, 95% CI=0.230–0.685, Figure) and a 2-year composite adverse outcome (all cause death and HF readmission; 18.7% vs. 40.5%, p<0.001, OR=0.332, 95% CI=0.196–0.563, Figure). To exclude possible relationships of other co-variable factors, we performed a multivariate analysis about the association with HF readmission rate and the 2-year composite adverse outcome (co-variate factors as follows are included; factors of which p-value was less than 0.1 and general confounding factors). The multivariate analysis showed that the early follow-up was independently associated with HF readmission during 2 years (p=0.002, OR=0.376, 95% CI=0.197–0.716) and the 2-year composite outcome (p<0.001, OR=0.343, 95% CI=0.182–0.648). Finally, we characterized the practical interventions at outpatient care after discharge. Lifestyle guidance was done in all patients. However, medication adjustments were done in only 36.7% patients. Interestingly, whether or not a medication adjustment was done at the early follow-up visit was not associated with the HF readmissions (p=0.781). Conclusions The present study suggests that an early follow-up approach after discharge in decompensated HF patients may improve the long-term prognosis. These results were not dependent on whether a medication adjustment was performed or not. An early follow-up may help improve patient factors of HF worsening. Main results Funding Acknowledgement Type of funding source: None


2021 ◽  
Vol 39 (15_suppl) ◽  
pp. e12545-e12545
Author(s):  
Hans-Christian Kolberg ◽  
Alexandra Edimiris ◽  
Oliver Hoffmann ◽  
Sarah Wetzig ◽  
Mohamed Shaheen ◽  
...  

e12545 Background: C-reactive protein (CRP) is an acute phase reactant influenced by inflammation and tissue damage. It has been demonstrated that elevated CRP levels are associated with poor outcome of cancer including metastatic breast cancer. However, evidence regarding an impact of CRP levels on outcome in early breast cancer (EBC) are missing. Methods: Patients after neoadjuvant chemotherapy (NACT) for EBC and with available data regarding CRP levels before therapy, pathologic complete remission (pCR) and follow-up were included. The association between CRP at baseline and outcome parameters was analyzed. Results: 156 women were included in this analysis, median follow up was 5.8 years. No association between CRP at baseline and pCR rates could be detected. 6.4% of the patients developed a local recurrence, 10.3% developed a distant recurrence and 5.1% died from breast cancer. A negative correlation (Spearman-Rho) between CRP at baseline and overall survival (OS) (Correlation coefficient (CC) -0.255; p = 0.45), disease free survival (DFS)(CC -0.348; p = 0.075), local recurrence free survival (LRFS)(CC -0.245; p = 0.327) and distant disease free survival (DDFS)(CC -0.422; p = 0.057) was not statistically significant, although especially in DFS and DDFS a strong trend was detected. The probability of death from breast cancer was 2% if the CRP was < 0.08 mg/dl and 40% if the CRP was > 2.08 m/dl, this association was highly statistically significant (Chi Square; p < 0.001). These results were independent from age, estrogen and progesterone receptor status, HER2 status and grading. Conclusions: CRP at baseline is not predictive for pCR in EBC after NACT in our patient dataset. However, an association of parameters of long-term prognosis with CRP could be demonstrated. Although the correlations of higher CRP at baseline and shorter OS, DFS, LRFS and DDFS were not significant, a strong trend could be detected that was reproduced in the analysis of different cut-offs for CRP and the probability of breast cancer mortality. Higher CRP-levels are indicating a worse prognosis in early breast cancer after NACT in this retrospective analysis. These results justify further investigation of CRP as a biomarker of long-term prognosis in early breast cancer in prospective trials.


Author(s):  
Sun-Wung Hsieh ◽  
Da-Wei Wu ◽  
Chih-Wen Wang ◽  
Szu-Chia Chen ◽  
Chih-Hsing Hung ◽  
...  

Previous studies have reported an association between the impairment of cognitive performance and lung diseases. However, whether obstructive or restrictive lung diseases have an impact on cognitive function is still inconclusive. We aimed to investigate the association between cognitive function and obstructive or restrictive lung diseases in Taiwanese adults using the Mini-Mental State Examination (MMSE). In this study, we used data from the Taiwan Biobank. Cognitive function was evaluated using the MMSE. Spirometry measurements of forced expiratory volume in 1 s (FEV1) and forced vital capacity (FVC) were obtained to assess lung function. Participants were classified into three groups according to lung function, namely, normal, restrictive, and obstructive lung function. In total, 683 patients enrolled, of whom 357 participants had normal lung function (52.3%), 95 had restrictive lung function (13.9%), and 231 had obstructive lung function (33.8%). Compared to the normal lung function group, the obstructive lung function group was associated with a higher percentage of cognitive impairment (MMSE < 24). In multivariable analysis, a low MMSE score was significantly associated with low FVC, low FEV1, and low FEV1/FVC. Furthermore, a low MMSE score was significantly associated with low FEV1 in the participants with FEV1/FVC < 70%, whereas MMSE was not significantly associated with FVC in the participants with FEV1/FVC ≥ 70%. Our results showed that a low MMSE score was associated with low FEV1, low FVC and low FEV1/FVC. Furthermore, a low MMSE score was associated with obstructive lung diseases but not with restrictive lung diseases.


2021 ◽  
Vol 11 (1) ◽  
Author(s):  
Jeong-Seon Lee ◽  
Joong-Gon Kim ◽  
Soyoung Lee

AbstractChildhood-onset polyarteritis nodosa (PAN) is a rare and systemic necrotising vasculitis in children affecting small- to medium-sized arteries. To date, there have been only a few reports because of its rarity. Thus, we aimed to investigate the clinical manifestations, laboratory findings, treatment, and long-term outcomes in patients with childhood-onset PAN and to evaluate the usefulness of the paediatric vasculitis activity score (PVAS). We retrospectively analysed the data of nine patients with childhood-onset PAN from March 2003 to February 2020. The median ages at symptom onset, diagnosis, and follow-up duration were 7.6 (3–17.5), 7.7 (3.5–17.6), and 7.0 (1.6–16.3) years, respectively. All patients had constitutional symptoms and skin manifestations, while five exhibited Raynaud’s phenomenon. Organ involvement was observed in one patient. The median PVAS at diagnosis was 7 (range: 2–32). Prednisolone was initially used for induction in all patients, and other drugs were added in cases refractory to prednisolone. All patients survived, but three patients with high PVAS at diagnosis experienced irreversible sequelae, including intracranial haemorrhage and digital amputation. In conclusion, early diagnosis and treatment may minimise sequelae in patients with childhood-onset PAN. This study suggests that high PVAS score at diagnosis may be associated with poor prognosis.


2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 661.1-661
Author(s):  
Y. Sun ◽  
L. Ma ◽  
H. Chen ◽  
C. Rongyi ◽  
L. Jiang

Background:Hypertension occurred in 30-80% of TAK patients around the world. The occurrence of hypertension might severely worsen TAK prognosis. Nevertheless, data describing the specific imaging features in hypertensive TAK patients and the associations between hypertensive severity, blood pressure control status and long-term outcome were still lacking.Objectives:To investigate the characteristics and associations of hypertensive characteristics with adverse events-free survival in Takayasu arteritis (TAK) patients with hypertension.Methods:This research was based on a prospectively on-going observational cohort-East China Takayasu Arteritis (ECTA) cohort. In all, 618 TAK patients, who registered in the ECTA cohort up to December 2019, were enrolled. The main outcome was the adverse-events-free survival among hypertensive TAK patients during the follow-up ended on August 2020.Results:Totally, 204 (33.0%) patients suffered from hypertension, with 48 (23.5%), 62 (30.4%), and 94 (46.1%) mild, moderate, and severe hypertension, respectively. Cluster analysis indicated three imaging phenotypes for hypertensive TAK patients: Cluster 1: involvement of the abdominal aorta and/or renal artery (n=56, 27.5%); Cluster 2: involvement of the ascending aorta, thoracic aorta, and the aortic arch and its branches (n=38, 18.6%); Cluster 3: combined involvement of Cluster 1 and Cluster 2 (n=111, 54.4%). By the end of the follow-up, the blood pressure control rate was 50.8%, while the adverse-events-free survival was 67.9% in the entire hypertensive population. Multivariate Cox regression analysis indicated that well-controlled blood pressure (HR=2.13, 95%CI 1.32–3.78, p=0.047), co-existence of severe aortic valve regurgitation (HR=0.87, 95%CI 0.64–0.95, p=0.043), Cluster 1 (HR=0.69, 95%CI 0.48–0.92, p=0.017) and Cluster 3 (HR=0.72, 95%CI 0.43–0.94, p=0.048) imaging phenotype was associated with the adverse-events-free survival.Conclusion:Patients with controlled hypertension showed better adverse-events-free survival, while those with the Cluster 1 imaging phenotype were more likely to suffer from worse adverse-events-free survival. Hypertension occurred in 30-80% of TAK patients around the world. The occurrence of hypertension might severely worsen TAK prognosis.References:[1]Johnston SL, Lock RJ, Gompels MM. Takayasu arteritis: a review. J Clin Pathol 2002; 55:481–6.[2]Watanabe Y, Miyata T, Tanemoto K. Current clinical features of new patients with Takayasu arteritis observed from a cross-country research in Japan: age and sex specificity. Circulation 2015; 132:1701–9.[3]Yilmaz N, Can M, Oner FA, et al. Impaired quality of life, disability and mental health in Takayasu’s arteritis. Rheumatol. (Oxford) 2013; 52:1898–904.[4]Laurent A, Julien H, Nicolas L, et al. Takayasu arteritis in France: a single-center retrospective study of 82 cases comparing white, North African, and black patients. Medicine 2010; 89:1–17.[5]Mwipatayi BP, Jeffery PC, Beningfield SJ, et al. Takayasu arteritis: clinical features and management: report of 272 cases. ANZ J Surg 2005; 75:110–7.Disclosure of Interests:None declared


2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 1552.3-1552
Author(s):  
A. Mekinian ◽  
D. Saadoun ◽  
J. C. N. F. [email protected] ◽  
I. Q. M. F. [email protected] ◽  
P. Jégo ◽  
...  

Objectives:To assess long term efficacy of tocilizumab in treatment-naive patients with Takayasu arteritis (TAK).Methods:In this multicenter, prospective, open-labelled trial, we aim to evaluate the benefit of adding tocilizumab to steroids in treatment-naïve patients with TAK, on discontinuation of steroids after 6 months of tocilizumab treatment, and to assess relapse-free survival following tocilizumab discontinuation.Results:Thirteen patients with TAK were included, with a median age of 32 years [19-45] and 12 (92%) females. Six (54%) patients met the primary end-point. Among 11 (85%) patients which achieved remission at 6 months, 6 (54%) have reached primary endpoint.. Among the 5 remaining patients which continued steroids, 3 had a prednisone-equivalent dosage < 5mg/day. A significant decrease of disease activity was observed after 6 months of tocilizumab therapy: decrease of median NIH scale (3 [3-4] at baseline, versus 1 [0-2] after 6 months; p <0.001), ITAS-2010 score (5 [2-7] versus 3 [0-8]; p = 0.002), and ITAS-A score (7 [4-10] versus 4 [1-15]; p = 0.0001)]. All patients discontinued tocilizumab after 7 infusions, and no other immunosuppressive drugs was introduced, except for 1 patient which received methotrexate. After 9 and 12 months, respectively 7 (54%) and 6 (50%) patients achieved remission with less than 7.5 mg/day of prednisone, and 9 (69%) and 9 (75%) with doses <10 mg/day. During the 12 months follow-up after tocilizumab discontinuation, a relapse occurred among 5 patients (45%) out of 11 in which achieved remission after 6 months of tocilizumab.No severe AEs were considered related to study treatment and none required tocilizumab interruption or dose reduction. No deaths have occurred during the study period.Conclusion:Tocilizumab seems an effective steroid sparing therapy in TAK but its effect appears to be suspensive.Disclosure of Interests:Arsene Mekinian: None declared, david Saadoun: None declared, [email protected] [email protected]: None declared, [email protected] [email protected]: None declared, Patrick Jégo: None declared, [email protected] [email protected]: None declared, wxv wxv: None declared, Jacques-Eric Gottenberg Grant/research support from: BMS, Pfizer, Consultant of: BMS, Sanofi-Genzyme, UCB, Speakers bureau: Abbvie, Eli Lilly and Co., Roche, Sanofi-Genzyme, UCB, Mathieu Vautier: None declared, [email protected]>; [email protected]>;: None declared, Patrice cacoub: None declared, olivier fain: None declared


2021 ◽  
Vol 10 (2) ◽  
pp. 180
Author(s):  
Frédéric Bouisset ◽  
Jean-Bernard Ruidavets ◽  
Jean Dallongeville ◽  
Marie Moitry ◽  
Michele Montaye ◽  
...  

Background: Available data comparing long-term prognosis according to the type of acute coronary syndrome (ACS) are scarce, contradictory, and outdated. Our aim was to compare short- and long-term mortality in ST-elevated (STEMI) and non-ST-elevated myocardial infarction (non-STEMI) ACS patients. Methods: Patients presenting with an inaugural ACS during the year 2006 and living in one of the three areas in France covered by the Monitoring of Trends and Determinants in Cardiovascular Disease (MONICA) registry were included. Results: A total of 1822 patients with a first ACS—1121 (61.5%) STEMI and 701 (38.5%) non-STEMI—were included in the study. At the 28-day follow-up, the mortality rates were 6.7% and 4.7% (p = 0.09) for STEMI and non-STEMI patients, respectively, and after adjustment of potential confounding factors, the 28-day probability of death was significantly lower for non-STEMI ACS patients (Odds Ratio = 0.58 (0.36–0.94), p = 0.03). At the 10-year follow-up, the death rates were 19.6% and 22.8% (p = 0.11) for STEMI and non-STEMI patients, respectively, and after adjustment of potential confounding factors, the 10-year probability of death did not significantly differ between non-STEMI and STEMI events (OR = 1.07 (0.83–1.38), p = 0.59). Over the first year, the mortality rate was 7.2%; it then decreased and stabilized at 1.7% per year between the 2nd and 10th year following ACS. Conclusion: STEMI patients have a worse vital prognosis than non-STEMI patients within 28 days following ACS. However, at the 10-year follow-up, STEMI and non-STEMI patients have a similar vital prognosis. From the 2nd year onwards following the occurrence of a first ACS, the patients become stable coronary artery disease patients with an annual mortality rate in the 2% range, regardless of the type of ACS they initially present with.


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