Heart involvement is the leading cause of death of patients with eosinophilic granulomatosis with polyangiitis (EGPA; formerly Churg-Strauss syndrome) and is more frequent in anti-neutrophil ...cytoplasm antibody (ANCA)-negative patients. Post-transplant outcome has only been reported once.
We conducted a retrospective international multicenter study. Patients satisfying the criteria of the American College of Rheumatology and/or revised Chapel Hill Consensus Conference Nomenclature were identified by collaborating vasculitis and transplant specialists, and the help of the Churg-Strauss Syndrome Association.
Nine ANCA(-) patients who received transplants between October 1987 and December 2009 were identified. The vasculitis and cardiomyopathy diagnoses were concomitant for 5 patients and separated by 12 to 288 months for the remaining 4 patients. Despite ongoing immunosuppression, histologic examination of 7 (78%) patients' explanted hearts showed histologic patterns suggestive of active vasculitis. The overall 5-year survival rate was low (57%), but rose to 80% when considering only the 6 patients transplanted during the last decade. After survival lasting 3 to 60 months, 4 (44%) patients died sudden deaths.
The search for EGPA-related cardiomyopathy is mandatory early in the course of this type of vasculitis. Indeed, prompt treatment with corticosteroids and cyclophosphamide may achieve restore cardiac function. Most patients in this series were undertreated. For patients with refractory EGPA, heart transplantation should be performed, which carries a fair prognosis. No optimal immunosuppressive strategy has yet been identified.
After a systematic review of all publications on recurrent pericarditis from 1966 to 2006, we identified 8 major clinical series including a total of 230 patients with idiopathic recurrent ...pericarditis (mean age 46 years, men/women ratio: 0.9). After a mean follow-up of 61 months, the complication rate was 3.5% cardiac tamponade and 0% constrictive pericarditis and left ventricular dysfunction. The overall life prognosis is excellent in idiopathic recurrent pericarditis and complications are uncommon. In conclusion constrictive pericarditis was never reported despite numerous recurrences, and the risk is lower than in idiopathic acute pericarditis (approximately 1%). Thus, it is important to reassure patients on their prognosis, explaining the nature of the disease, and the likely course. Therapeutic choices should take into account of the overall good outcome of these patients, including less toxic agents.
Neonatal Lupus (NL) is a rare syndrome caused by placental transfer of maternal anti-SSA/Ro and anti-La/SSB autoantibodies to the fetus. The rarity of this condition requires the establishment of ...multidisciplinary registries in order to improve our knowledge.
Inclusion criteria in this retrospective study were the maternal confirmed positivity for anti-SSA/Ro and/or anti-SSB/La antibodies, and the presence of II or III degree congenital heart block (CHB)
or neonatal period (up to 27 days after birth).
Eighty-nine cases of CHB were observed in 85 women with 88 pregnancies that occurred between 1969 and 2017. CHB was mostly detected
(84 cases, 94.2%), while five cases were observed in the neonatal period. A permanent pacemaker was implanted in 51 of 73 children born alive (69.8), whereas global mortality rate was 25.8% (23 cases): 16
, five perinatal, and two during childhood. By univariate analysis, factors associated with fetal death were pleural effusion (
= 0.005, OR > 100; CI 95% 2.88->100 and hydrops (
= 0.003,
= 14.09; CI 95% 2.01-122). Fluorinated steroids (FS) were administered in 71.4% pregnancies, and its use was not associated with better survival. Some centers treated all cases with fluorinated steroids and some centers did not treat any case. CHB was initially incomplete in 24 fetuses, and of them five cases of II degree block reverted to a lower degree block after treatments. Recurrence rate in subsequent pregnancies was 17.6% (3 out of 17). A prophylactic treatment was introduced in 10 of these 16 subsequent (58.8%) pregnancies, mostly with FS or high dose intravenous immunoglobulins.
This is the first report from the Italian Registry of neonatal lupus/CHB. The live birth rate was nearly 80%, with nearly two thirds of the children requiring the implantation of a pacemaker. The management of fetuses diagnosed with CHB was heterogeneous across Italian Centers. The registry at present is mainly rheumatological, but involvement of pediatric cardiologists and gynecologists is planned.
A two-dose regimen of Pfizer-BioNTech COVID-19 vaccination confers 95% protection against COronaVIrus Disease 19 (COVID-19) and the safety profile is adequate. To the submission date, there were no ...reports in literature of acute pericarditis after BNT162b2 vaccination. However, pericarditis has been reported as a rare event associated with COVID-19 infection, which could be due to the pro-inflammatory effects of the spike protein. Recent evidence of post-vaccine myocarditis has been published. Herein we describe the case of a middle-aged healthy women who developed symptoms and signs of acute pericarditis 7-10 days after the second dose of Pfizer-BioNTech COVID-19 vaccination. Although a direct effect cannot be stated, it is important to report a potential adverse vaccine reaction effect that could be associated with the expression of SARS-CoV-2 spike protein induced from the mRNA of the vaccine.
ObjectiveThe aim of this work is to explore patient’ unmet needs of rare and complex rheumatic tissue diseases (rCTDs) patients during pregnancy and its planning by means of the narrative-based ...medicine (NBM) approach.MethodsA panel of nine rCTDs patients’ representatives was identified to codesign a survey aimed at collecting the stories of rCTD patients who had one or more pregnancies/miscarriages. The results of the survey and the stories collected were analysed and discussed with a panel of patients’ representatives to identify unmet needs, challenges and possible strategies to improve the care of rCTD patients.Results129 replies were collected, and 112 stories were analysed. Several unmet needs in the management of pregnancy in rCTDs were identified, such as fragmentation of care among different centres, lack of education and awareness on rCTD pregnancies among midwifes, obstetricians and gynaecologists. The lack of receiving appropriate information and education on rCTDs pregnancy was also highlighted by patients and their families. The need for a holistic approach and the availability specialised pregnancy clinics with a multidisciplinary organisation as well as the provision of psychological support during all the phases around pregnancy was considered also a priority.ConclusionThe adoption of the NBM approach enabled a direct identification of unmet needs, and a list of possible actions was elaborated to improve the care of rCTD patients and their families in future initiatives.
Inflammation has been associated with an increased risk for cancer development, while innate immune system activation could counteract the risk for malignancies. Familial Mediterranean fever (FMF) is ...a severe systemic inflammatory condition and also represents the archetype of innate immunity deregulation. Therefore, the aim of this study is to investigate the risk for cancer development in FMF.
The risk ratio (RR) for malignancies was separately compared between FMF patients and fibromyalgia subjects, Still's disease patients and Behçet's disease patients. Clinical variables associated with cancer development in FMF patients were searched through binary logistic regression.
580 FMF patients and 102 fibromyalgia subjects, 1012 Behçet's disease patients and 497 Still's disease patients were enrolled. The RR for the occurrence of malignant neoplasms was 0.26 (95% Confidence Interval CI. 0.10-0.73, p=0.006) in patients with FMF compared to fibromyalgia subjects; the RR for the occurrence of malignant cancer was 0.51 (95% CI. 0.23-1.16,
=0.10) in FMF compared to Still's disease and 0.60 (95% CI. 0.29-1.28,
=0.18) in FMF compared to Behçet's disease. At logistic regression, the risk of occurrence of malignant neoplasms in FMF patients was associated with the age at disease onset (β1 = 0.039, 95% CI. 0.001-0.071,
=0.02), the age at the diagnosis (β1 = 0.048, 95% CI. 0.039-0.085,
=0.006), the age at the enrolment (β1 = 0.05, 95% CI. 0.007-0.068,
=0.01), the number of attacks per year (β1 = 0.011, 95% CI. 0.001- 0.019,
=0.008), the use of biotechnological agents (β1 = 1.77, 95% CI. 0.43-3.19,
=0.009), the use of anti-IL-1 agents (β1 = 2.089, 95% CI. 0.7-3.5,
=0.002).
The risk for cancer is reduced in Caucasic FMF patients; however, when malignant neoplasms occur, this is more frequent in FMF cases suffering from a severe disease phenotype and presenting a colchicine-resistant disease.
Background No preventive pharmacologic strategies have been proven efficacious for the prevention of postoperative effusions after cardiac surgery. Colchicine is safe and efficacious for the ...prevention of pericarditis. On this basis, we realized a substudy of the COPPS trial to assess the efficacy and safety of colchicine for the prevention of postoperative pericardial and pleural effusions. Methods The COPPS is a multicenter, double-blind, randomized trial, where 360 consecutive patients (mean age 65.7 ± 12.3 years, 66% men), 180 in each treatment arm, were randomized on the third postoperative day to receive placebo or colchicine for 1 month (1.0 mg twice daily for the first day, followed by a maintenance dose of 0.5 mg twice daily in patients ≥70 kg, and halved doses for patients <70 kg). The incidence of postoperative effusions was evaluated in each study group. Results Despite similar baseline features, colchicine significantly reduced the incidence of postoperative pericardial (12.8% vs 22.8%, P = .019, relative risk reduction 43.9%, no. of patients needed to treat 10) and pleural effusions (12.2% vs 25.6%, P = .002, relative risk reduction 52.3%, no. of patients needed to treat 8). The rate of side effects (only gastrointestinal intolerance) and drug withdrawal was similar in the study groups with a trend toward an increased rate of both events for colchicine. In multivariable analysis, female gender (hazard ratio 1.76, 95% CI 1.03-3.03, P = .040) and pleura incision (hazard ratio 2.58, 95% CI 1.53-4.53, P < .001) were risk factors for postoperative effusions. Conclusions Colchicine is safe and efficacious for the primary prevention of postoperative effusions after cardiac surgery.
Background:
Rosai-Dorfman disease (RDD) is rare a sinus histiocytosis typically causing lymphadenopathy. Heart involvement is anecdotal, and <30 cases of cardiac RDD (cRDD) have been reported so far.
...Case Presentation:
A 46-year old woman with positive clinical history for RDD was admitted to our cardiology department with transthoracic echocardiography diagnosis of severe pericardial effusion and right atrial masses. Pericardiocentesis with catheter insertion was performed 3 days after the admission due to clinical evidence of cardiac tamponade. After 10 weeks of maximal medical therapy for inflammatory pericarditis, including non-steroidal anti-inflammatory drugs (NSAIDs), colchicine, steroids, and anakinra, at least 100 ml of pericardial citric liquid has been daily drained suggesting no clinical improvement. Pericardial liquid analysis demonstrated no malignant cells, but immunohistochemical analysis resulted positive for AE1–AE3, D2–40, S100, and CD68 consistent with an RDD diagnosis. Surgical management was judged clinically indicated, and 2 months after admission, the patient underwent pericardiectomy and debulking of atrial mass with freezing of remaining atrial neoformation. Regular clinical and echocardiography evaluation was performed without pericardial effusion recurrence after 2 years of follow-up.
Conclusions:
This is the first case ever reported of cRDD who survived after 2 years of follow-up. Pericardiectomy could be feasible and effective for recurrent pericardial effusion in cRDD. Close follow-up and a multidisciplinary environment is needed to take care of cRDD patients.
The progressive cutting of hospital beds in some health systems, together with the increased needs related to the aging population, has led to the phenomenon of patients hospitalized outside the ...appropriate ward (outliers). This is particularly relevant in the context of Internal Medicine. Despite its relevance in daily clinical practice, available evidence for the potential impact of this phenomenon is limited. The aim of this study is to evaluate the effects of this situation on patients’ outcomes and possibly identify organizational and managerial aspects related to the presence of outliers. The multicenter, observational, prospective Study Protocol of Safety Issues and SurvIval For Medical Outliers (SISIFO) was promoted by the Italian Federation of Associations of Hospital Doctors on Internal Medicine (FADOI). The primary study endpoint is the evaluation of in-hospital mortality in outliers versus controls. A sample size of 2400 patients has been estimated by assuming a mortality rate of 12% and 8% in outliers and controls, respectively. By virtue of the multicentric dimension, the expected number of patients, and the controlled design, the FADOI-SISIFO study might provide interesting and useful findings to better manage the phenomenon of outliers.
•Preventive treatment of asymptomatic non-severe hyperuricemia is not recommended.•Allopurinol may be considered in AH in three conditions (Table 5).•Long-term data on cardiovascular and chronic ...kidney disease prevention are needed.
While there is consensus on starting urate-lowering therapy (ULT) in cases of symptomatic hyperuricemia, the frequent condition of asymptomatic hyperuricemia (AH) remains a challenge due to differences in the findings of studies that have addressed the issue. Uric acid has anti-oxidant properties, but high levels predispose to gout and may play a role in metabolic syndrome. We systematically evaluated randomized controlled trials (RCTs) addressing ULT in patients with AH, to assess the current evidence. We found broad heterogeneity among the studies (13 RCTs), in terms of study design and population, making findings challenging to interpret and generalize; hard end-points were not assessed. Allopurinol is often prescribed for AH despite the fact that its use is not backed by conclusive evidence from prospective RCTs, nor is it recommended by the guidelines. Its potential benefits, in terms of absolute risk reduction, must be weighed against its potential for harm since it can trigger severe adverse hypersensitivity reactions, sometimes even fatal. RCTs with hard end-points are needed to assess the risk/benefit of lowering uric acid in subjects with AH, particularly as secondary prevention for cardiovascular risk and in patients with different degrees of renal disease. To date, particularly after the result from the CARES trial, preventive treatment of asymptomatic and non-severe hyperuricemia is not recommended.
PDF
