scholarly journals Granulomatosis with polyangiitis and cardio vascular co-morbidity in Denmark. A registry-based study of 21 years of follow-up

Author(s):  
Helle Laustrup ◽  
Anne Voss ◽  
Peter Enemark Lund
2021 ◽  
Vol 9 ◽  
pp. 205031212110291
Author(s):  
Targ Elgzyri ◽  
Jan Apelqvist ◽  
Eero Lindholm ◽  
Hedvig Örneholm ◽  
Magdalena Annersten Gershater

Background: Forefoot gangrene in patients with diabetes is a severe form of foot ulcers with risk of progress and major amputation. No large cohort studies have examined clinical characteristics and outcome of forefoot gangrene in patients with diabetes. The aim was to examine clinical characteristics and outcome of forefoot gangrene in patients with diabetes admitted to a diabetic foot centre. Methods: Patients with diabetes and foot ulcer consecutively presenting were included if they had forefoot gangrene (Wagner grade 4) at initial visit or developed forefoot gangrene during follow-up at diabetic foot centre. Patients were prospectively followed up until final outcome, either healing or death. The median follow-up period until healing was 41 (3–234) weeks. Results: Four hundred and seventy-six patients were included. The median age was 73 (35–95) years and 63% were males. Of the patients, 82% had cardiovascular disease and 16% had diabetic nephropathy. Vascular intervention was performed in 64%. Fifty-one patients (17% of surviving patients) healed after auto-amputation, 150 after minor amputation (48% of surviving patients), 103 had major amputation (33% of surviving patients) and 162 patients deceased unhealed. Ten patients were lost at follow-up. The median time to healing for all surviving patients was 41 (3–234) weeks; for auto-amputated, 48 (10–228) weeks; for minor amputated, 48 (6–234) weeks; and for major amputation, 32 (3–116) weeks. Conclusion: Healing without major amputation is possible in a large proportion of patients with diabetes and forefoot gangrene, despite these patients being elderly and with extensive co-morbidity.


2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 270.2-271
Author(s):  
J. Álvarez Troncoso ◽  
J. C. Santacruz Mancheno ◽  
A. Díez Vidal ◽  
S. Afonso Ramos ◽  
A. Noblejas Mozo ◽  
...  

Background:Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAV) include granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), and eosinophilic granulomatosis with polyangiitis (EPGA). Renal involvement is frequent in AAV and is an important factor for morbidity and mortality.Objectives:The main objective of this study was to analyze the demographic, clinical, histological and therapeutic characteristics of renal involvement in patients with AAV and the risk of renal replacement therapy (RRT) or death.Methods:Retrospective observational study of 56 patients with AAV fulfilling classificatory criteria and renal involvement diagnosed between 1995 and 2020 from a Spanish tertiary centre. We studied the histological involvement (according to the 2010 classification in focal, crescentic, mixed or sclerotic), immunofluorescence (IF) and the treatment received with the risk of RRT or death.Results:We included 56 patients diagnosed with AAV and renal involvement. The mean age was 61.08±4.05 years; 58.9% were women. The mean follow-up time of these patients was 16.14± 8.80 years. Only 57.1% of patients presented systemic involvement.Most frequent non-renal AAV manifestations were lung involvement (39.3%), central nervous system (30.4%), otorhinolaryngology (ORL) (14.3%), skin (8.9%) and cardiac involvement (8.9%). Main immunological findings were ANCA-MPO+ (69.6%), ANCA-PR3+ (23.2%), ANCA-negative (5.4%). Low C3 was found in 19.6% patients. Histologic classification (HC) and need of RRT is described in table 1. Main HC in renal AAV was crescentic, mixed, focal and sclerotic respectively. Eight patients had not biopsy performed. IF was positive for C3 deposits in 20 patients (35.7%). Half of the patients presented <50% normal glomeruli.The treatment of renal involvement in AAV in our cohort was as follows: 83.9% (47) corticosteroids (CS) and cyclophosphamide (of which 40 received intravenous and 7 oral cyclophosphamide; and 12 patients associated plasma exchange (PE) with this treatment), 5.36% CS alone, 2 patients received CS and mycophenolate; 1 CS and rituximab, 1 CS and PE, and 2 patients received no treatment. A total of 13 patients received PE and 18 RRT. The mean time to RRT was 65.44±32.72 months. Relapses were not uncommon, 33.93% of the patients presented ≥1 relapse and 10.71% presented ≥2.Infections were very frequent since they were present in 91.07% of the patients. Other frequent non-immunological complications observed in the follow-up of these patients were thrombosis in 31.14%, cardiovascular events in 28.57% and cancer in 19.64%.Patients with ANCA-PR3+ were younger at diagnosis (p<0.001), were more likely to present cardiac (p=0.045) and ORL involvement (p<0.001). However, neither ANCA-PR3+ nor ANCA-MPO+ were specifically associated with the need of RRT or higher risk of death in our cohort. Use of CS alone for the treatment of renal AAV was associated with higher mortality (p=0.006) but CS and cyclophosphamide with lower mortality (p=0.044). ANCA-negative patients were more likely to receive no treatment. Having <50% normal glomeruli and C3 deposits on IF were associated with an increased need for RRT. Presenting focal disease on HC was protective for the need of RRT. Older age at diagnosis, systemic involvement of AAV and need of RRT was associated with higher mortality.Conclusion:AAVs are complex vasculitides with frequent renal involvement. Increased C3 deposition, non-focal histological forms, and <50% normal glomeruli were related to the need for RRT. In turn, the need for RRT, a later age at diagnosis, and systemic involvement were associated with higher mortality. Holistic and multidisciplinary early management of AAVs in experience centers can help improve renal prognosis and decrease mortality.References:[1]Binda et al. ANCA-associated vasculitis with renal involvement. J Nephrol. 2018 Apr;31(2):197-208.[2]Kronbichler et al. Clinical associations of renal involvement in ANCA-associated vasculitis. Autoimmun Rev. 2020 Apr;19(4):102495.Disclosure of Interests:None declared


2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 1076.1-1077
Author(s):  
L. Moroni ◽  
L. Giudice ◽  
G. A. Ramirez ◽  
S. Sartorelli ◽  
A. Cariddi ◽  
...  

Background:Subglottic stenosis (SGS) is defined as airway narrowing below the vocal cords and is a common and potentially life-threatening manifestation of Granulomatosis with Polyangiitis (GPA), with an estimated prevalence of 16-23% (1). Balloon catheter dilation is effective in GPA-related SGS, but relapses are frequent. Little is known about the role of immunosuppression in this setting.Objectives:to analyse the clinical characteristics of a monocentric GPA cohort, describe phenotype differences among patients with and without SGS and investigate the role of surgical and medical treatments on relapse risk and general outcome.Methods:Biopsy-proven patients with SGS were identified by review of medical charts among a cohort of patients with GPA, classified according to the algorithm of the European Medicine Agency (2). The clinical characteristics of patients with SGS were retrospectively collected over a median follow-up time of 15.9 years and compared to those of patients without SGS.Results:Fourteen patients with SGS-GPA were identified, with a female to male ratio of 1:1 and a prevalence of 29.2% among the cohort. The mean ± SD age at GPA onset was 30.8 ± 14.4 years, with a mean time from GPA diagnosis to SGS onset of 4.7 ± 4.2 years. ANCA were positive in 78.6% (54.0% anti-PR3, 18.1% anti-MPO and 27.9% IFI only). The mean Birmingham Vasculitis Activity Score (BVAS) at onset was 10.0 ± 5.6. The main clinical manifestations associated with SGS were crusty rhinitis (100%), sinusitis (78%), pulmonary disease (72.7%), otitis/mastoiditis (50%), glomerulonephritis (42.9%), orbital pseudotumor (28.6%). Six patients (42.9%) received medical treatment only, other six (42.9%) had one to three balloon dilations and two (14.2%) underwent four or more procedures. Eight patients had no SGS relapse (maximum one dilation) and they all received immunosuppression with rituximab (RTX), cyclophosphamide (CYC) or azathioprine (AZA). All patients who received no immunosuppression, methotrexate (MTX) or mycophenolate (MMF) had at least one relapse. Patients treated with MTX or MMF had a mean relapse-free survival of 13.1 months, which was comparable to the one of patients not receiving medical treatment (40.2 months; p=NS) and shorter than the one of patients receiving CYC or RTX (153.2 months; p=0.032). CYC use also inversely correlated with the number of surgical procedures (r=-0.691, p=0.006). Compared to patients without SGS (31 consecutive patients with at least 4 years of follow-up), patients with SGS-GPA had an earlier disease onset (mean age 30.8 vs 50.4 years; p<0.001), but with lower BVAS (mean 10.0 vs 15.3; p=0.013) and showed a higher prevalence of crusty rhinitis (100% vs 67.7%; p=0.019). No difference was observed in damage accrual over time between the two groups.Conclusion:Subglottic stenosis is highly prevalent in patients with GPA and may define a milder disease subset occurring more frequently in younger patients. MTX and MMF might be insufficient to prevent SGS relapses requiring balloon dilation. Aggressive immunosuppression (CYC or RTX) might have a non-redundant role in this setting and reduce the risk of relapses.References:[1]Quinn KA, et al. Subglottic stenosis and endobronchial disease in granulomatosis with polyangiitis. Rheumatology 2019; 58 (12), 2203-2211.[2]Watts R, et al. Development and validation of a consensus methodology for the classification of the ANCA associated vasculitides and polyarteritis nodosa for epidemiological studies. Ann Rheum Dis 2007; 66: 222-7.Disclosure of Interests:Luca Moroni: None declared, Laura Giudice: None declared, Giuseppe Alvise Ramirez: None declared, Silvia Sartorelli: None declared, adriana cariddi: None declared, Angelo Carretta: None declared, Enrica Bozzolo: None declared, Lorenzo Dagna Grant/research support from: The Unit of Immunology, Rheumatology, Allergy and Rare Diseases (UnIRAR) received unresctricted research/educational grants from Abbvie, Bristol-Myers Squibb, Celgene, Janssen, Merk Sharp & Dohme, Mundipharma Pharmaceuticals, Novartis, Pfizer, Roche, Sanofi-Genzyme, and SOBI., Consultant of: Prof Lorenzo Dagna received consultation honoraria from Abbvie, Amgen, Biogen, Bristol-Myers Squibb, Celltrion, Novartis, Pfizer, Roche, Sanofi-Genzyme, and SOBI.


2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 1563.3-1563
Author(s):  
H. Tamaki ◽  
S. Fukui ◽  
T. Nakai ◽  
G. Kidoguchi ◽  
S. Kawaai ◽  
...  

Background:Currently it is hypothesized that many systemic autoimmune diseases occur due to environmental risk factors in addition to genetic risk factors. Anti-Neutrophil Cytoplasmic Antibody (ANCA) is mainly associated with three systemic autoimmune disease including granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), eosinophilic granulomatosis with polyangiitis (EGPA). It is known that ANCA can be positive before clinical symptoms in patients with known diagnosis of GPA and ANCA titers rise before clinical manifestations appear. However, prevalence of ANCA among general population is not well known. It has not been described as well how many of people with positive ANCA eventually develop clinical manifestations of ANCA associated Vasculitis.Objectives:This study aims to estimate prevalence of ANCA in general population without ANCA associated Vasculitis. It also describes natural disease course of people with positive ANCA without ANCA associated Vasculitis. Risk factors for positive ANCA are also analyzed.Methods:This is a single center retrospective study at Center for Preventive Medicine of St. Luke’s International Hospital in Tokyo. ANCA was checked among the patients who wished to between 2018 and 2019. St. Luke’s Health Check-up Database (SLHCD) was utilized to collect the data. The patients whose serum was measured for ANCA were identified. The data for basic demographics, social habits, dietary habits and laboratory data were extracted. The charts of the patients with positive ANCA were reviewed.Results:Sera of total 1204 people were checked for ANCA. Of these 1204 people, 587 (48.8%) are male and the mean age was 55.8 years (32.6 to 79). There were total 11 patients with positive ANCA. Myeloperoxidase ANCA (MPO-ANCA) was positive for 3 patients and proteinase 3 ANCA (PR3-ANCA) was positive for 8 patients. Of these 11 patients, 5 were male (45.5%) and the mean age was 54.6 years. Two patients had history of autoimmune disease (primary biliary cirrhosis and ulcerative colitis). Five patients were evaluated by rheumatologists with the median follow-up period of 274 days. None of them developed clinical signs and symptoms of ANCA associated Vasculitis. Four out of five patients had ANCA checked later, two of which turned negative. The prevalence of ANCA in this cohort was 0.9% (95% confidence interval [95% CI]: 0.5% to 1.6%). Univariate analysis was performed to identify risk factors of positive ANCA. The variables analyzed include age, gender, body mass index (BMI), smoking habits, alcohol intake, dietary habits (fruits, fish, red meat), hypertension, dyslipidemia, and laboratory data. None of these variables demonstrated statistically significant differences except for positive rheumatoid factor (ANCA positive group: 33 % vs ANCA negative group: 9.1%, p value = 0.044).Conclusion:The prevalence of ANCA in this cohort was 0.9% (95% CI: 0.5% to 1.6%). None of them who had a follow-up developed ANCA associated Vasculitis during the follow-up period. Longer follow-up and more patients are necessary to determine natural course of people with positive ANCA.Disclosure of Interests:None declared


2021 ◽  
Vol 108 (Supplement_7) ◽  
Author(s):  
Jenna Delaney ◽  
Joshua Templeman ◽  
Keith Hussey

Abstract Introduction A systematic review published in 2017 concluded that ‘kissing’ iliac stents to treat occlusive disease of the aortic bifurcation was associated with high technical procedural success and good medium-term patency. We have explored this in a regional vascular unit. Methods Retrospective review of patients managed with endovascular treatment of aorto-iliac segment (March 2015 to September 2020). The median follow-up was 2-years. Key outcome variables included the patency of the reconstruction and requirement for secondary intervention. Results There were 1,348 iliac endovascular procedures performed for occlusive atherosclerotic arterial disease of the aorto-iliac segment of which 56 were ‘kissing’ iliac stents. Intervention was performed for chronic limb threatening ischaemia in 29 cases. In 37 cases patients had unilateral symptoms only and in 41 cases patients had either TASC A or B lesions. Technical success was achieved in 55 cases and the median duration of admission was 1-day. At follow-up there was clinical patency in 54 cases, although there were 3 major extremity amputations performed (all on the index admission). Conclusion As with much of the work on ‘kissing’ iliac stents the number of patients is small and the group of patients variable in terms of medical co-morbidity, presenting clinical features and morphology of iliac disease. The procedure appears to have high technical success and can be achieved with little in the way of major morbidity. Medium term durability is good.


2006 ◽  
Vol 134 (11-12) ◽  
pp. 503-508
Author(s):  
Natasa Jovanovic ◽  
Mirjana Lausevic ◽  
Biljana Stojimirovic

Introduction:Most of patients with chronic renal failure are affected by normochromic, normocytic anemia caused by different etiological factors. Anemia causes a series of symptoms in chronic renal failure, which can hardly be recognized from the uremic signs. Anemia adds to morbidity and mortality rates in patients affected by advanced chronic renal failure. Blood count partially improves during the first months after starting the chronic renal replacement therapy, in correlation with the quality of depuration program, with extension of erythrocyte lifetime and with hemoconcentration due to reduction of plasma volume. Recent trials found that higher residual renal function (RRF) significantly reduced co-morbidity, the rate and duration of hospitalization and risk of treatment failure. Objective: The aim of the study was to follow blood count parameters in 32 patients on chronic continuous ambulatory peritoneal dialysis (CAPD) during the first six months of treatment, to evaluate the influence of demographic and clinical factors on blood count and RRF, and to examine the correlation between RRF and blood count parameters. Method: A total of 32 patients affected by end-stage renal disease of different major cause during the first six months of CADP treatment were studied. RRF and blood count were evaluated as well as their relationship during the follow-up. Results: Blood count significantly improved in our patients during the first six months of CAPD treatment even if Hb and HTC failed to reach normal values. Iron serum level slightly decreased because of more abundant erythropoiesis and iron utilization during the first six months of treatment. RRF slightly decreased. After six months of CAPD treatment, the patients with higher RRF had significantly higher Hb, HTC and erythrocyte number and a lot of positive correlations between RRF and anemia markers were observed. Conclusion: After 6-month follow-up period, the patients with higher RRF had significantly higher blood count parameters, and several positive correlations between RRF and blood count markers were confirmed.


PLoS ONE ◽  
2021 ◽  
Vol 16 (5) ◽  
pp. e0250769
Author(s):  
Dimitria Doi ◽  
Romulo Ribeiro do Vale ◽  
Jean Michell Correia Monteiro ◽  
Glauco Cabral Marinho Plens ◽  
Mario Ferreira Junior ◽  
...  

The demand for high value health care uncovered a steady trend in laboratory tests ordering and inappropriate testing practices. Residents’ training in laboratory ordering practice provides an opportunity for quality improvement. We collected information on demographics, the main reason for the appointment, preexisting medical conditions and presence of co-morbidities from first-visit patients to the internal medicine outpatient service of our university general hospital. We also collected information on all laboratory tests ordered by the attending medical residents. At a follow-up visit, we recorded residents’ subjective perception on the usefulness of each ordered laboratory test for the purposes of diagnosis, prognosis, treatment or screening. We observed that 17.3% of all ordered tests had no perceived utility by the attending resident. Tests were usually ordered to exclude differential diagnoses (26.7%) and to help prognosis estimation (19.1%). Age and co-morbidity influenced the chosen category to legitimate usefulness of tests ordering. This study suggests that clinical objectives (diagnosis, prognosis, treatment or prevention) as well as personalization to age and previous health conditions should be considered before test ordering to allow a more appropriate laboratory tests ordering, but further studies are necessary to examine this framework beyond this medical training scenario.


2021 ◽  
Vol 18 (2) ◽  
pp. 79-84
Author(s):  
Olusola Ayodele Sogebi ◽  
Emmanuel Abayomi Oyewole

Background: Nasal septum collections (hematoma and abscess) can lead to structural and functional abnormalities. Our objective was to assess the clinical characteristics, management and complications of nasal septal collections, and document factors associated withtheir complications. Methods: This was a retrospective study of patients managed for nasal septal collections. Socio-demographic and clinical information was recorded, and the main investigations and results noted. Follow-up and complications of septal collections were documented and the clinical factors associated with the complications explored. Results: Twenty-four patients records were studied: male: female ratio=2:1, mean age 40.1±13.1years,62.5% presented with complaints of nasal obstruction, 66.7% had antecedent nasal trauma, presentation was from 2 to 13 days,25%had co-morbid disease(s). All patients had incision and drainage of the septal collection within 1–7 h; 41.2% of the aspirated collections cultured microorganisms, 20.8% developed complications. Increased age above 45 years, co-morbidity, delayed presentation, culture-positive aspirate was all significantly associated with development of complications. Conclusion: Nasal septal collections were more common in adult males with antecedent nasal trauma; 20% developedcomplications associated with the presence of culturepositive abscesses, increased age, and duration of septal collection. Keywords: Nasal trauma, Septal hematoma, Septal abscess, Complications


2018 ◽  
Author(s):  
Louise Thornton ◽  
Frances Kay-Lambkin ◽  
Bree Tebbutt ◽  
Tanya L Hanstock ◽  
Amanda L Baker

BACKGROUND Background: People with mental health disorders live on average 20 years less than those without, often due to poor physical health including cardio-vascular disease (CVD). Evidence-based interventions are required to reduce this lifespan gap. OBJECTIVE Objective: This study aimed to develop, trial, and evaluate a mobile-based lifestyle program (MyHealthPA) to help people with mental health problems improve key health risk behaviors and reduce their risk of CVD. METHODS Methods: The development of MyHealthPA occurred in three stages: (1) a review of the literature; (2) a scoping survey (n=251) among people with and without experience of mental health problems; and (3) program development informed by stages (1) and (2). A small pilot trial among young people with and without mental health (MH) disorders was also conducted. Participants completed a baseline assessment and given access to the MyHealthPA program for a period of eight weeks. They were then asked to complete an end-of-treatment assessment and a follow-up assessment one month later. RESULTS Results: Twenty-eight young people aged 19 to 25 years were recruited to the pilot trial. Of these, 12 (43%) had been previously diagnosed with a MI. Twelve participants (43%) completed the end-of-treatment assessment and six (21%) completed the follow-up assessment. Small improvements in fruit and vegetable consumption, level of physical activity, alcohol use, and mood were found between baseline and end-of-treatment and follow-up, particularly among people with experience of MH issues. Most participants (57-60%) reported the program had above average usability, however only 29-40% of participants reported that they would like to use the program frequently and would recommend it to other young people. Participants also identified a number of ways in which the program could be improved. CONCLUSIONS Conclusions: This article describes the formative research and process of planning that formed the development of MyHealthPA and the evidence base underpinning the approach. The MyHealthPA program represents an innovative approach to CVD risk reduction among people with mental health problems. MyHealthPA appears to be an acceptable, easy to use, and potentially effective mHealth intervention to assist young people with mental illness to monitor risk factors for CVD. However, ways in which the program could be improved for future testing and dissemination were identified and are discussed.


Author(s):  
S Sunil Kumar ◽  
Oliver Joel Gona ◽  
Nagaraj Desai ◽  
B Shyam Prasad Shetty ◽  
KS Poornima ◽  
...  

Introduction: Vitamin K Antagonists (VKAs) have been in use for more than 50 years. They have remained as mainstay therapy in the prevention of thromboembolic events in atrial fibrillation, mechanical heart valves and venous thromboembolism. Despite many years of clinical experience with VKAs, the quality of anticoagulation achieved in routine clinical practice is suboptimal. Aim: To study the effects of structured Anticoagulation Clinic (ACC) interventions on patient centred outcomes in subjects taking VKAs. Materials and Methods: A retrospective study was conducted among patients taking VKAs enrolled in ACC. A total of 169 patients receiving VKAs for at least six months with 4 INR (International Normalised Ratio) values and completed 12 months of follow-up were analysed. Anticoagulation related quality measures like Time in the Therapeutic Range (TTR), Percentage of International Normalised Ratios in the therapeutic Range (PINRR) and clinical outcomes like stroke, systemic embolic events and bleeding was analysed at the time of enrolment and compared with those during ACC care. Results: Among 352 patients enrolled in ACC, 169 patients were eligible for analysis. The mean age of the study population was 55.62±13.77 years. Atrial fibrillation (59%) was the most common indication for VKA therapy. Hypertension (66.3%) was the most common co-morbidity. Mean TTRs were significantly higher in the ACC care when compared with the pre-ACC care at 12 months follow-up (77.58±8.85% vs 51.01±16.7%, p<0.0001). There was a significant improvement in TTRs as early as three months of ACC intervention (73.18±13.56%). At the time of enrolment, 21.9% of patients had individual TTRs (i-TTR) >70% which increased to 70.4% at 12 months of follow-up. INR testing was done more frequently in ACC care. Adverse clinical events were higher in pre-ACC care than ACC care (4.7% vs 2.4%, p>0.05). Major bleeding and thromboembolic events were higher in pre-ACC care than ACC care (1.8% vs. 0.6% and 2.4% vs. 0.6% respectively). Conclusion: ACC services helps in achieving better quality of anticoagulation control as measured by time in therapeutic range translating into better clinical outcomes.


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