Post-traumatic stress disorder and post-traumatic stress symptoms in patients with systemic autoimmune diseases during the COVID-19 pandemic.

OBJECTIVES: The COVID-19 outbreak led to an increase in mental disorders, particularly post-traumatic stress disorder (PTSD), in the general population and especially in high-risk populations such as patients with rheumatologic conditions. Although these latters are considered vulnerable to developing PTSD, few specific data have been particularly reported in the framework of the pandemic. The aim of the present study was to investigate PTSD and post-traumatic stress symptoms (PTSS) in a sample of patients with systemic autoimmune disease (SAD), followed in the framework of a prospective observational study during the pandemic. METHODS: The PERMAS project is a prospective observational study including patients with SAD and involving the Rheumatology and the Psychiatric Clinics of the Azienda Ospedaliero Universitaria Pisana (AOUP, Pisa, Italy) and the Institute of Management of the Scuola Superiore Sant'Anna (Pisa, Italy). The assessments included: a data-sheet for sociodemographic and clinical characteristics; the Trauma and Loss Spectrum-Self Report (TALS-SR) and the Impact of Event Scale-Revised (IES-R), for PTSD and PTSS; the 36-item Short Form Survey (SF-36) to assess quality of life. RESULTS: The total sample consisted of 252 patients with SAD, including 131 with connective tissue disease, 101 with arthritis and 20 with systemic vasculitis. The diagnostic groups differed significantly in age (p<0.001), gender (p<0.001), prevalence of full-blown and partial PTSD (p=0.001), and other psychopathologic variables. Connective tissue disease and SF-36 were significantly associated with the TALS-SR scores in both univariate (p<0.001) and multivariate (p<0.025; p<0.001) analyses. CONCLUSIONS: Patients with SAD, and, in particular, patients with connective tissue diseases reported an increased risk of developing stress-related psychopathological symptoms, indicating the need for special psychological monitoring of this high-risk group.

One year in review 2020: economic and organisational aspects in rare and complex connective tissue diseases.

Rare and complex connective tissue diseases (rCTDs) encompass a considerable number of diseases and syndromes and their variability highly impacts on the clinical management, resulting in variable economic and organisational burden that might represent a challenge for healthcare systems. This paper is aimed at providing an overview of the most recent evidence regarding the economic and organisational impact of rCTDs. In particular, this work discusses the most relevant data on specific aspects related to health economics in rCTDs published in 2019.

Social determinants of male partner attendance in women's prevention-of mother-to-child transmission program in Malawi.

BACKGROUND: Male partners are rarely present during PMTCT (Prevention-Mother-To-Child-Transmission) services in Sub-Saharan Africa (SSA). Male involvement is increasingly recognised as an important element of women's access to care. This study aims to identify the socio-demographic characteristics, HIV-Knowledge, Attitude and Practice (KAP) among women accompanied and not accompanied by their male partners. METHODS: We included pregnant women enrolled in PMTCT programme between August 2018 and November 2019 in the Southern Region of Malawi. Eligible women were aged 18 years or older, living with a male partner, enrolled for the first time in one of the four selected facilities. We provided a KAP survey to women and their partners attending the facilities. Our primary objective was to assess and analyse the proportion of women who were accompanied by their partner at least once. We applied descriptive statistics and logistic regressions to study the association between being accompanied and explanatory variables. RESULTS: We enrolled 128 HIV-positive women: 82 (64.1%) were accompanied by their male partners and 46 (35.9%) were alone. In the multivariable model, women's unemployment and owning a means of transport are negatively associated with male attendance (respectively adjusted OR 0.32 [95% CI, 0.11-0.82] and 0.23 [95% CI, 0.07-0.77]), whereas, in the univariable model, high women's level of knowledge of HIV is positively associated with male attendance (OR 2.17 [95% CI, 1.03-4.58]). Level of attitude and practice toward HIV were not significantly associated to our study variable. CONCLUSIONS: Our study shows a high male attendance in Malawi compared to other studies performed in SSA. This study highlights that women's level of knowledge on HIV and their economic condition (employment and owning a means of transport) affects male attendance. Moreover, the study points out that gender power relationships and stringent gender norms play a crucial role thus they should be considered to enhance male involvement.

The effectiveness of interventions to involve men living with HIV positive pregnant women in low-income countries: a systematic review of the literature.

BACKGROUND: Male involvement (MI) along the continuum of HIV healthcare services has been promoted as a critical intervention in low-income countries and represents one of the reasons for dropout and low retention of women along the cascade of care. The present review aims to identify interventions adopted to improve MI across Antenatal Clinics (ANCs). METHODS: For this systematic review, we searched electronic databases, including Scopus, PubMed, Web of Science (from 2008 to 2018) in English language. We included all interventions explicitly aimed at involving partners in pregnant women's HIV continuum of care and we excluded studies performed in developed countries, not involving pregnant women. We followed the PRISMA checklist. RESULTS: We identified a total of 1694 records and excluded 1651 after duplicates were removed and abstract eligibility assessments were performed. Forty-three full-text articles were screened, but only 12 studies were included. Recurrent intermediate outcomes were antenatal partner attendance rate and male HIV testing. We subdivided articles according to the type of intervention: single intervention (7) and multiple interventions (5). Among single interventions, two studies evaluated the use of an invitation letter sent via women to encourage male attendance to the ANC. Four Randomized Controlled Trials (RCTs) compared the invitation card (standard of care, SC) to word of mouth, information letter, home visit and invitation card plus partner tracing. The partner attendance rate was lower in SC than in the intervention arm in three RCTs: information letter (14.2% vs 16.2%), home-visit (39% vs 87%) and invitation card plus partner tracing (52% vs 74%). Home visit strategies seemed the most effective. One study evaluated words of encouragement adopted to trigger women to invite their partners. Among multiple interventions, the most effective strategies in terms of male attendance included health promotion through education and healthcare worker development. These interventions were more likely to be effective in promoting MI than single interventions. CONCLUSIONS: From the review emerges the importance of male involvement in HIV cascade for pregnant women in countries with a significant HIV incidence and the need to define more precise indicators for measuring MI.

Comparative health technology assessment of robotic-assisted, direct manual laparoscopic and open surgery: a prospective study.

BACKGROUND: Despite many publications reporting on the increased hospital cost of robotic-assisted surgery (RAS) compared to direct manual laparoscopic surgery (DMLS) and open surgery (OS), the reported health economic studies lack details on clinical outcome, precluding valid health technology assessment (HTA). METHODS: The present prospective study reports total cost analysis on 699 patients undergoing general surgical, gynecological and thoracic operations between 2011 and 2014 in the Italian Public Health Service, during which period eight major teaching hospitals treated the patients. The study compared total healthcare costs of RAS, DMLS and OS based on prospectively collected data on patient outcome in addition to healthcare costs incurred by the three approaches. RESULTS: The cost of RAS operations was significantly higher than that of OS and DMLS for both gynecological and thoracic operations (p < 0.001). The study showed no significant difference in total costs between OS and DMLS. Total costs of general surgery RAS were significantly higher than those of OS (p < 0.001), but not against DMLS general surgery. Indirect costs were significantly lower in RAS compared to both DMLS general surgery and OS gynecological surgery due to the shorter length of hospital stay of RAS approach (p < 0.001). Additionally, in all specialties compared to OS, patients treated by RAS experienced a quicker recovery and significantly less pain during the hospitalization and after discharge. CONCLUSIONS: The present HTA while confirming higher total healthcare costs for RAS operations identified significant clinical benefits which may justify the increased expenditure incurred by this approach.

A cost-of-illness study of Behçet syndrome in Italy.

OBJECTIVE: This study aims at evaluating the cost-of-illness (COI) of patients diagnosed with Behcet's syndrome (BS) in Italy, trying to depict the impact of different costs' components to the overall economic burden and analysing the variability of costs according to years since diagnosis and age at first symptoms. METHODS: With a cross-sectional evaluation, we surveyed a large sample of BS patients in Italy assessing several dimensions related to BS, also including fact related to the use of health resources utilization, formal and informal care, and productivity losses. Overall costs, direct health, direct non-health, and indirect costs were thus estimated per patient/year considering a Societal perspective and the impact of years since diagnosis, age at first symptoms on costs was evaluated using generalized linear model (GLM) and a two-part model, adjusting for age and distinguishing among employed and non-employed responders. RESULTS: A total of 207 patients were considered in the present study. From the perspective of the Society, mean overall costs for BS patient were estimated to be 21,624 € (0;193,617) per patient/year. Direct non-health expenses were the main costs component accounting for 58% of the overall costs, followed direct health costs, 36%, while indirect costs because of productivity losses represented 6% of the overall costs. Being employed resulted in significantly lower overall costs (p = 0.006). Results from the multivariate regression analyses suggested that the probability of incurring in overall costs equal to zero decreased as time from BS diagnosis is 1 year or more as compared to newly diagnosed patients (p < 0.001); while among those incurring in expenses, costs decreased for those experiencing first symptoms between 21 and 30 years (p = 0.027) or later (p = 0.032) as compared to those having symptoms earlier. Similar findings emerged among the subgroups of patients declaring themselves as workers, while no impact of years since diagnosis or age of first symptoms was found among non-workers. CONCLUSIONS: The present study offers a comprehensive overview of the economic consequences imposed by BS in a societal perspective, providing insights into the distribution of the different costs component related to BS, thus helping the development of targeted policies.

Patient Care Pathways for Pregnancy in Rare and Complex Rheumatic Diseases: Results From an International Survey.

OBJECTIVE: To map existing organizational care pathways in clinical centers of expertise that care for pregnant women affected by rare and complex connective tissue diseases (rcCTDs). METHODS: An international working group composed of experts in the field of pregnancy in rcCTDs co-designed a survey focused on organizational aspects related to the patient's pathway before, during, and after pregnancy. The survey was distributed to subject experts through referral sampling. RESULTS: Answers were collected from 69 centers in 21 countries. Patients with systemic lupus erythematosus and/or antiphospholipid syndrome were followed by more than 90% of centers, whereas those with disorders such as IgG4-related diseases were rarely covered. In the majority of centers, a multidisciplinary team was involved, including an obstetrician/gynecologist in 91.3% of cases and other healthcare professionals less frequently. Respondents indicated that 96% of the centers provided routine pre-pregnancy care, whereas the number of patient visits during pregnancy varied across centers. A formalized care pathway was described in 49.2% of centers, and 20.3% of centers had a predefined protocol for the monitoring of pregnant patients. Access to therapies during pregnancy also was heterogeneous among different centers. CONCLUSION: In international referral centers, a high level of care is provided to patients with rcCTDs before, during, and after pregnancy. No significant discrepancies were found between European and non-European countries. However, this work highlights a potential benefit to streamlining the care approaches across countries to optimize pregnancy and perinatal outcomes among patients with rcCTDs.

Systemic lupus erythematosus and the economic perspective: a systematic literature review and points to consider.

Systemic lupus erythematosus (SLE) is a chronic, disabling, progressive disease, with many associated comorbidities, affecting patients during prime working years resulting in a high economic burden on society, producing high direct, indirect and intangible costs. In this article, our goals are two-fold. First, we review and discuss studies published in the period 2002-2012 concerning costs of SLE and point out gaps in the published literature. Second, we propose further research studies to advance our understanding of the economic perspective in SLE in the current area of new and emerging therapies. The literature evaluating disease costs in SLE remains limited and to date has only included a small number of countries. Despite these limitations, available studies indicate that SLE has significant socio-economic ramifications. Future studies are needed, especially to assess novel biologic therapies which have been made available or currently under investigation for SLE. An interesting approach in these new economic evaluations in SLE may be represented by the selection of the targets of the treatment to include in the cost-effectiveness and cost-utility analyses. Future treat-to-target strategies will likely include evaluation of their pharmacoeconomic implications.

The economic impact of gout: a systematic literature review.

This article performs a systematic literature review of the last decade studies assessing the economic impact of gout. The literature review confirms the fact that gout reduces productivity and increases annual total healthcare costs, since care of gout absorbs relevant amounts of healthcare resources. One important aspect to be considered is represented by prevention and monitoring of the disease after the diagnosis, as gout is sometimes underestimated by patients and this leads to a reduced adherence to follow up and to treatment with consequences on the disease course and outcome. In fact, the lack of prevention and the scarce adherence to monitoring increase the number and costs of hospitalisation. Prevention, monitoring the level of sUA and using a urate-lowering therapy appear to have a central role for controlling gout and reducing hospitalisation, with positive advantages in terms of healthcare costs and healthcare utilisation. One limitation on the analysis of gout related costs, however, resides in the fact that the majority of the retrieved studies are retrospective and the definition of the economic impact of the disease is made difficult by differences in inclusion criteria, costs assessment, use of gout-related healthcare resources.

Systemic vasculitis: how little we know about their societal and economic burden.

This article attempts to perform an evaluation of the state of the art of the economic and societal burden of systemic vasculitis (VAs). Due to the rarity of these diseases and their variable clinical picture, few data are available in the literature on their health economic issues, and only some papers have been published that marginally examine the problem. Since VAs are severe conditions with a high medical and societal impact and determine high healthcare resource consumption, studies able to define societal, quality of life and economic burden of these pathologies are needed. Policy makers, private and public organisations involved in the care of VAs need data to programme future investment or make cost-effectiveness analysis for introducing new drugs or protocols.

A systematic literature review of the economic impact of ankylosing spondylitis.

This article reviews the last decade studies on the economic impact of ankylosing spondylitis (AS). Interestingly, a common observation is that in AS indirect costs are higher than the use of direct healthcare resources. Country, age, gender, and severity of the diseases impact on per patient annual costs AS related. Different payment and reimbursement regimes may impact on the amount and distribution of indirect costs. The differences observed among countries on absolute and relative (compared with direct costs) amounts of indirect costs can be explained with the capability of a country of actually measure productivity losses and indirect costs. Low indirect costs without other indicators should not be considered as a sign of efficiency in AS care, but may be due to an underestimation of AS-related costs; as a consequence, indirect costs may be a net loss for patients that nobody can repay. A private insurance reimbursement regime has the highest capability of inducing players to define, select and actually identify indirect costs better than in different reimbursement regimes. Therefore indirect costs may become very high in case of private insurance regimes because of their more detailed identification.

Economic evaluation of da Vinci-assisted robotic surgery: a systematic review.

BACKGROUND: Health technology assessment (HTA) is frequently used when a new and expensive technology is being introduced into clinical practice. This certainly is the case with the da Vinci surgical robot, with costs ranging from $1 to $2.5 million for each unit. This systematic review documents major variability in the reported cost evaluation studies of da Vinci robot-assisted operations compared with those performed by the direct manual laparoscopic approach. METHODS: Published studies in the English language related to the period 2000-2010 were searched using economic and clinical electronic databases. RESULTS: All 11 reports included some form of cost analysis, which made it possible for the authors to extract information on certain specific economic outcomes: operating room time, hospital stay, and total costs. With the exception of two studies, the reported operating room time was higher with the robotic approach than with manual laparoscopic surgery, and the hospital stay was the same for the two techniques. Robotic surgery is significantly more expensive if the purchase and maintenance costs of the robot system are included in the total costs. Only 3 of the 11 publications included these costs. CONCLUSIONS: The disadvantage of robotic surgery is its higher costs related to purchase and maintenance of technology and its longer operating room time. However, emerging evidence shows that operating room time decreases with experience using the robot. From the HTA viewpoint, the result of this review is that the jury still is out on the HTA of da Vinci-assisted robotic surgery.

State of the art and future directions in assessing the quality of life in rare and complex connective tissue and musculoskeletal diseases.

Background: As chronic conditions, rare and complex connective tissue and musculoskeletal diseases (rCTDs) significantly affect the quality of life generating an impact on the physical, psychological, social, and economic dimensions of the patients' lives, having implications on the family, changing the lifestyle and interpersonal relationships. Traditionally, generic and disease-specific measures for Quality of Life (QoL) provide valuable information to clinicians since QoL affects healthcare services utilization, predicts morbidities and mortalities, workability, etc. Moreover, the assessment of unmet clinical needs, satisfaction, the experience with the treatment and the care, the psychological dimensions, and the effects of the diseases, such as fatigue, could represent valuable dimensions to be considered in the QoL impact assessment. It is also necessary to measure the impact of rCTDs by considering the perspectives of family members/informal caregivers, for instance considering values, beliefs, experiences, life circumstances, psychological aspects, family relationships, economic issues, changes in social activities, etc. Objective: The aim of this scoping review is to better understand the status of QoL metrics used in clinical and economic research for the assessment of the individual's perspective on living with rCTDs. Research question: What are the main challenges in QoL measures (and/or) measurement/assessment in rCTDs? Materials and methods: Scoping review of the literature referring to QoL measures in rCTDs. Database: PUBMED, ISI-Web of Science; last date: 21/09/2021. Results: Anxiety and depression, body image satisfaction, daily activity, fatigue, illness perception, pain, personality, QoL, resilience, satisfaction with the relationship, self-management, sexual QoL, sleep quality, social support, stress, uncertainty, and work productivity are the observed dimensions covered by the included studies. However, "more shadows than lights" can summarize the review's outcome in terms of Patient Reported Outcome Measures (PROMs) domains covered for each of the rCTDs. Also, for those diseases characterized by a relatively high prevalence and incidence, such as Systemic Lupus Erythematosus, Sjögren's Syndrome, and Systemic Sclerosis, the analysis of patients' resilience, satisfaction with the quality of the relationship, personality, and stress are still missing dimensions. It has been observed how reducing items, increasing the number of domains, and disease-specific questionnaires characterize the "technological trajectory," such as the evolution of questionnaires' characteristics for assessing QoL and QoL-related dimensions and the burden of rCTDs. Conclusion: The scoping review presents an overview of studies focused on questionnaires used to evaluate the different dimensions of quality of life in terms of general instruments and disease-specific questionnaires. Future research should include the co-design with patients, caregivers, and patient representatives to create questionnaires focused on the unmet needs of people living with rCTDs.

Understanding the meanings of male partner support in the adherence to therapy among HIV-positive women: a gender analysis.

BACKGROUND: Previous literature reports that low male partner support is a barrier to women's adherence and retention in HIV care programs. OBJECTIVE: This qualitative study explored the relationships between partners to understand what is meant by male partner support in adherence of HIV-positive women in four healthcare facilities in Southern Malawi. METHODS: We conducted 8 semi-structured focus group discussions (FGDs) with 73 participants (40 men and 33 women) and 10 in-depth interviews (IDIs) between August 2018 to December 2019. Participants were HIV-positive patients, healthcare workers (HCWs), expert patients (EPs), and couples attending the clinic. All data were digitally recorded, transcribed verbatim, and analysed using a gender-responsive grounded theory approach. RESULTS: This study confirms previous literature, which suggests male partner support is expressed by providing access to transport to the clinic and accompaniment to appointments. However, we found that men can also control access to resources and decision-making. Support is more complex than previous literature reported and, in some cases, gender norms significantly limit women's capacity to engage in care independently of male support since women need male partner permission to access the resources to attend clinics. CONCLUSIONS: This paper suggests that restrictive male-partner gender norms limit women's power to engage in care. Most importantly, the gender analysis reveals that what previous literature describes as male partner support can sometimes hide male partner control in permitting access to resources to attend health facilities. For this reason, policies enhancing male support should consider the gender power relationship between partners to avoid reinforcing gender inequality.

Improving Male Partner Involvement in HIV-Positive Women's Care Through Behavioral Change Interventions in Malawi (WeMen Study): A Prospective, Controlled Before-and-After Study.

Several strategies and interventions have been implemented to improve male partner involvement (MI) in Sub-Saharan Africa, but evidence on successful interventions is scarce. This controlled before-and-after intervention study aims to evaluate the impact of three interventions on male partners' involvement in HIV+ women's care in Malawi. We piloted these three interventions: the organization of a special day for men, the deployment of male champions in communities to increase awareness on MI, and the delivery of an incentive (food package) for couples attending the facility. We observed a significant increase in the number of women accompanied by their partners (from 48.5 to 81.4%) and the number of women feeling safe at home (from 63.5 to 95.2%) after the special day intervention. This outcome increased after the deployment of male champions in communities (from 44.0 to 75.0%). No significant improvement was observed in the site where we delivered the incentive to couples. Our findings showed that the special day for men and the use of male champions might effectively increase the male involvement in the health of their female partners.

An Opportunity to Harmonise the Approach to Patients' Care Pathways for Rare and Complex Diseases: RarERN Path™.

As a matter of fact, organisation always matters when discussing about healthcare, since it is fundamental in order to ensure the delivery of the most appropriate care to patients in the most appropriate way. Unfortunately, the pandemic brought by the severe acute respiratory syndrome-coronavirus 2 (SARS-CoV-2) imposed a huge reorganisation of the healthcare systems, with several repercussions on the care of several chronic conditions, that were in many cases discontinued. This was the case of rare diseases (RDs), conditions that even under normal circumstances can experience diagnostic delays and difficulties in receiving appropriate care. The context of the European Reference Networks (ERNs) represents one of the most appropriate settings for the creation of organisational reference models for patient care pathways (PCP). As a matter of fact, the main mission of ERNs is to improve the care of patients with RDs in Europe through a patient-centred approach, thanks to real multistakeholder involvement. For this reason, in the last years, an extensive effort has been made towards the creation of a methodological approach aimed at providing organisational reference models for PCP in RDs across the different Member States. In fact, in order to develop the reference model, a structured methodology was created to enable the design of the PCP based on a deep sharing of expertise on high-quality care and characterised by a strong patient-centred approach: RarERN Path™. Among the different stakeholders that need to be involved in planning strategic actions to ensure care also during an emergency, patients' representatives, healthcare professionals, hospital managers, and experts in healthcare organisations play a crucial role.

Exploring patient's experience and unmet needs on pregnancy and family planning in rare and complex connective tissue diseases: a narrative medicine approach.

OBJECTIVE: The 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. METHODS: A 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. RESULTS: 129 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. CONCLUSION: The 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.

An overlook on the current registries for rare and complex connective tissue diseases and the future scenario of TogethERN ReCONNET.

Background: Patient registries play a crucial role in supporting clinical practice, healthcare planning and medical research, offering a real-world picture on rare and complex connective tissue diseases (rCTDs). ERN ReCONNET launched the first European Registry Infrastructure with the aim to plan, upgrade and link registries for rCTDs, with the final goal to promote a harmonized data collection approach all over Europe for rCTDs. Methods: An online survey addressed to healthcare professionals and patients' representatives active in the field of rCTDs was integrated by an extensive database search in order to build a mapping of existing registries for rCTDs. Findings: A total of 140 registries were found, 38 of which include multiple diseases. No disease-specific registry was identified for relapsing polychondritis, mixed connective tissue disease and undifferentiated connective tissue disease. Discussion: This overview on the existing registries for rCTDs provides a useful starting point to identify the gaps and the strengths of registries on the coverage of rCTDs, and to develop a common data set and data collection approach for the establishment of the TogethERN ReCONNET Infrastructure.