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Importance Transgender and nonbinary youths have a higher incidence of a range of health conditions and may paradoxically face limited access to health care. Objective To describe the perspectives and needs of transgender youths in accessing health care. Evidence Review MEDLINE, Embase, PsycInfo, and the Cumulative Index to Nursing and Allied Health Literature were searched from inception to January 2021. Qualitative studies of transgender youths’ perspectives on accessing health care were selected. Results from primary studies were extracted. Data were analyzed using thematic synthesis. Findings Ninety-one studies involving 884 participants aged 9 to 24 years across 17 countries were included. We identified 6 themes: experiencing pervasive stigma and discrimination in health care, feeling vulnerable and uncertain in decision-making, traversing risks to overcome systemic barriers to transitioning, internalizing intense fear of consequences, experiencing prejudice undermining help-seeking efforts, and experiencing strengthened gender identity and finding allies. Each theme encapsulated multiple subthemes. Conclusions and Relevance This review found that transgender youths contend with feelings of gender incongruence, fear, and vulnerability in accessing health care, which are compounded by legal, economic, and social barriers. This can lead to disengagement from care and resorting to high-risk and unsafe interventions. Improving access to gender-affirming care services with a cultural humility lens and addressing sociolegal stressors may improve outcomes in transgender and nonbinary youths.

Kidney international 100(4), 753-779 (2021). doi:10.1016/j.kint.2021.05.015, Published by Elsevier, New York, NY

The Kidney Disease: Improving Global Outcomes (KDIGO) 2021 Clinical Practice Guideline for the Management of Blood Pressure in Chronic Kidney Disease for patients not receiving dialysis represents an update to the KDIGO 2012 guideline on this topic. Development of this guideline update followed a rigorous process of evidence review and appraisal. Guideline recommendations are based on systematic reviews of relevant studies and appraisal of the quality of the evidence. The strength of recommendations is based on the "Grading of Recommendations Assessment, Development and Evaluation" (GRADE) approach. The scope includes topics covered in the original guideline, such as optimal blood pressure targets, lifestyle interventions, antihypertensive medications, and specific management in kidney transplant recipients and children. Some aspects of general and cardiovascular health, such as lipid and smoking management, are excluded. This guideline also introduces a chapter dedicated to proper blood pressure measurement since all large randomized trials targeting blood pressure with pivotal outcomes used standardized preparation and measurement protocols adhered to by patients and clinicians. Based on previous and new evidence, in particular the Systolic Blood Pressure Intervention Trial (SPRINT) results, we propose a systolic blood pressure target of less than 120 mm Hg using standardized office reading for most people with chronic kidney disease (CKD) not receiving dialysis, the exception being children and kidney transplant recipients. The goal of this guideline is to provide clinicians and patients a useful resource with actionable recommendations supplemented with practice points. The burden of the recommendations on patients and resources, public policy implications, and limitations of the evidence are taken into consideration. Lastly, knowledge gaps and recommendations for future research are provided.

While patient-centered care is widely advocated in the management of rheumatic diseases, it can be challenging to implement, particularly for patients with complex systemic conditions. Patient-centered care involves identifying and integrating the patient's experiences, attitudes, and preferences in decision-making. Qualitative research is used to describe patient perspectives and priorities that may not always be expressed in clinical settings. Systematic reviews of qualitative studies can provide new and more comprehensive evidence of patients' beliefs and priorities across different populations and healthcare settings and are increasingly being reported across medical specialties, including rheumatology. In rheumatology, they have been used to examine topics including medication-taking and adherence, coping with systemic sclerosis and conservative management and exercise in osteoarthritis. By referencing recent examples of systematic qualitative reviews in the rheumatology literature, this article will outline the methodology and methods used, and provide an approach to guide the appraisal of reviews. We aim to give the reader a practical understanding of systematic reviews of qualitative literature and elucidate how knowledge gained from such reviews can be applied to improve the care of patients with rheumatic conditions.

The Kidney Disease: Improving Global Outcomes (KDIGO) Clinical Practice Guideline for Diabetes Management in Chronic Kidney Disease represents the first KDIGO guideline on this subject. The guideline comes at a time when advances in diabetes technology and therapeutics offer new options to manage the large population of patients with diabetes and chronic kidney disease (CKD) at high risk of poor health outcomes. An enlarging base of high-quality evidence from randomized clinical trials is available to evaluate important new treatments offering organ protection, such as sodium-glucose cotransporter-2 inhibitors and glucagon-like peptide-1 receptor agonists. The goal of the new guideline is to provide evidence-based recommendations to optimize the clinical care of people with diabetes and CKD by integrating new options with existing management strategies. In addition, the guideline contains practice points to facilitate implementation when insufficient data are available to make well-justified recommendations or when additional guidance may be useful for clinical application. The guideline covers comprehensive care of patients with diabetes and CKD, glycemic monitoring and targets, lifestyle interventions, antihyperglycemic therapies, and self-management and health systems approaches to management of patients with diabetes and CKD.

Shared care between rheumatologists and dermatologists is advocated for patients with psoriasis and psoriatic arthritis, but care provided by rheumatologists and dermatologists is often siloed, leading to inconsistencies in patient care and outcomes. This study aimed to describe rheumatologists’ and dermatologists’ perspectives on shared care. Face-to-face semi-structured interviews were conducted with 15 rheumatologists and 12 dermatologists across 27 centers in Australia. Transcripts were thematically analyzed. Five themes were identified: uncertainties in disciplinary tensions (lacking expertise to make diagnosis, hesitation managing outside of scope, doubting screening tools, defaulting to own disciplinary priorities, hampered by lack of evidence), working in fragmented care (frustration with working in silos, striving to coordinate with primary care, persevering despite inequities in access to care, overwhelmed by managing comorbidities, under-resourced for complexity), building trusting relationships (establishing a culture of collaboration, seeking reliable cross-specialty help, depending on unique skills), prioritizing efficiency (minimizing burden for patients, avoiding resource overuse, deferring to pragmatic decisions), and strengthened by integrated care models (improving the timeliness and accuracy of care, centering on patient goals and understanding, enhancing cross-specialty partnerships, providing opportunities for education and training). Rheumatologists and dermatologists endeavor to provide comprehensive care to their patients in disjointed healthcare settings but are hampered by a lack of training and a sense of feeling overburdened in the management of comorbidities. Interdisciplinary models are perceived to improve the care of patients but are limited by financial barriers to implementation and concerns about wasting health resources and improperly burdening patients.

Patient-centered care is widely advocated in rheumatology. This involves collaboration among patients, caregivers, and health professionals and is particularly important in chronic rheumatic conditions because the disease and treatment can impair patients’ health and well-being. Qualitative research can systematically generate insights about people’s experiences, beliefs, and attitudes, which patients may not always express in clinical settings. These insights can address complex and challenging areas in rheumatology, such as treatment adherence and transition to adult healthcare services. Despite this, qualitative research comprises 1% of studies published in top-tier rheumatology journals. A better understanding about the effect and role, methods, and rigor of qualitative research is needed. This overview highlights the recent contributions of qualitative research in rheumatology, summarizes the common approaches and methods used, and outlines the key principles to guide appraisal of qualitative studies.

Description The Kidney Disease: Improving Global Outcomes (KDIGO) 2021 clinical practice guideline for the management of blood pressure (BP) in patients with chronic kidney disease (CKD) not receiving dialysis is an update of the KDIGO 2012 guideline on the same topic and reflects new evidence on the risks and benefits of BP-lowering therapy among patients with CKD. It is intended to support shared decision making by health care professionals working with patients with CKD worldwide. This article is a synopsis of the full guideline. Methods The KDIGO leadership commissioned 2 co-chairs to convene an international Work Group of researchers and clinicians. After a Controversies Conference in September 2017, the Work Group defined the scope of the evidence review, which was undertaken by an evidence review team between October 2017 and April 2020. Evidence reviews were done according to the Cochrane Handbook. The GRADE (Grading of Recommendations Assessment, Development and Evaluation) approach was used to guide the development of the recommendations and rate the strength and quality of the evidence. Practice points were included to provide guidance when evidence was insufficient to make a graded recommendation. The guideline was revised after public consultation between January and March 2020. Recommendations The updated guideline comprises 11 recommendations and 20 practice points. This synopsis summarizes key recommendations pertinent to the diagnosis and management of high BP in adults with CKD, excluding those receiving kidney replacement therapy. In particular, the synopsis focuses on recommendations for standardized BP measurement and a target systolic BP of less than 120 mm Hg, because these recommendations differ from some other guidelines.

Clinical question What are the benefits and harms of sodium-glucose cotransporter 2 (SGLT-2) inhibitors and glucagon-like peptide 1 (GLP-1) receptor agonists when added to usual care (lifestyle interventions and/or other diabetes drugs) in adults with type 2 diabetes at different risk for cardiovascular and kidney outcomes? Current practice Clinical decisions about treatment of type 2 diabetes have been led by glycaemic control for decades. SGLT-2 inhibitors and GLP-1 receptor agonists are traditionally used in people with elevated glucose level after metformin treatment. This has changed through trials demonstrating atherosclerotic cardiovascular disease (CVD) and chronic kidney disease (CKD) benefits independent of medications’ glucose-lowering potential. Recommendations The guideline panel issued risk-stratified recommendations concerning the use of SGLT-2 inhibitors or GLP-1 receptor agonists in adults with type 2 diabetes • Three or fewer cardiovascular risk factors without established CVD or CKD: Weak recommendation against starting SGLT-2 inhibitors or GLP-1 receptor agonists. • More than three cardiovascular risk factors without established CVD or CKD: Weak recommendation for starting SGLT-2 inhibitors and weak against starting GLP-1 receptor agonists. • Established CVD or CKD: Weak recommendation for starting SGLT-2 inhibitors and GLP-1 receptor agonists. • Established CVD and CKD: Strong recommendation for starting SGLT-2 inhibitors and weak recommendation for starting GLP-1 receptor agonists. • For those committed to further reducing their risk for CVD and CKD outcomes: Weak recommendation for starting SGLT-2 inhibitors rather than GLP-1 receptor agonists. How this guideline was created An international panel including patients, clinicians, and methodologists created these recommendations following standards for trustworthy guidelines and using the GRADE approach. The panel applied an individual patient perspective. The evidence A linked systematic review and network meta-analysis (764 randomised trials included 421 346 participants) of benefits and harms found that SGLT-2 inhibitors and GLP-1 receptor agonists generally reduce overall death, and incidence of myocardial infarctions, and end-stage kidney disease or kidney failure (moderate to high certainty evidence). These medications exert different effects on stroke, hospitalisations for heart failure, and key adverse events in different subgroups. Absolute effects of benefit varied widely based on patients’ individual risk (for example, from five fewer deaths in the lowest risk to 48 fewer deaths in the highest risk, for 1000 patients treated over five years). A prognosis review identified 14 eligible risk prediction models, one of which (RECODe) informed most baseline risk estimates in evidence summaries to underpin the risk-stratified recommendations. Concerning patients’ values and preferences, the recommendations were supported by evidence from a systematic review of published literature, a patient focus group study, a practical issues summary, and a guideline panel survey. Understanding the recommendation We stratified the recommendations by the levels of risk for CVD and CKD and systematically considered the balance of benefits, harms, other considerations, and practical issues for each risk group. The strong recommendation for SGLT-2 inhibitors in patients with CVD and CKD reflects what the panel considered to be a clear benefit. For all other adults with type 2 diabetes, the weak recommendations reflect what the panel considered to be a finer balance between benefits, harms, and burdens of treatment options. Clinicians using the guideline can identify their patient’s individual risk for cardiovascular and kidney outcomes using credible risk calculators such as RECODe. Interactive evidence summaries and decision aids may support well informed treatment choices, including shared decision making.

Purpose of Review To summarize and explain the new guideline on blood pressure (BP) management in chronic kidney disease (CKD) published by Kidney Disease: Improving Global Outcomes (KDIGO), an independent global nonprofit organization which develops and implements evidence-based clinical practice guidelines in kidney disease. KDIGO issued its first clinical practice guideline for the Management of Blood Pressure (BP) in Chronic Kidney Disease (CKD) for patients not receiving dialysis in 2012 and now updated the guideline in 2021. Recent Findings Recommendations in this update were developed based on systematic literature reviews and appraisal of the quality of the evidence and strength of recommendation following the “Grading of Recommendations Assessment, Development and Evaluation” (GRADE) approach. The updated guideline includes five chapters covering BP measurement techniques, lifestyle interventions for lowering BP, and management of BP in three target populations, namely adults (with and without diabetes), kidney transplant recipients, and children. A dedicated chapter on BP measurement emphasizing standardized preparation and measurement protocols for office BP measurement is a new addition, following protocols used in large randomized trials of BP targets with pivotal clinical outcomes. Summary Based on the available evidence, and in particular in the CKD subgroup of the SPRINT trial, the 2021 guideline suggests a systolic BP target of

ObjectivesTo describe the experiences, priorities, and needs of patients with rheumatic disease and their parents during transition from paediatric to adult healthcare.SettingFace-to-face and telephone semistructured interviews were conducted from December 2018 to September 2019 recruited from five hospital centres in Australia.ParticipantsFourteen young people and 16 parents were interviewed. Young people were included if they were English speaking, aged 14–25 years, diagnosed with an inflammatory rheumatic disease (eg, juvenile idiopathic arthritis, juvenile dermatomyositis, systemic lupus erythematosus, panniculitis, familial Mediterranean fever) before 18 years of age. Young people were not included if they were diagnosed in the adult setting.ResultsWe identified four themes with respective subthemes: avoid repeat of past disruption (maintain disease stability, preserve adjusted personal goals, protect social inclusion); encounter a daunting adult environment (serious and sombre mood, discredited and isolated identity, fear of a rigid system); establish therapeutic alliances with adult rheumatology providers (relinquish a trusting relationship, seek person-focused care, redefine personal–professional boundaries, reassurance of alternative medical supports, transferred trust to adult doctor) and negotiate patient autonomy (confidence in formerly gained independence, alleviate burden on patients, mediate parental anxiety).ConclusionsDuring transition, patients want to maintain disease stability, develop a relationship with their adult provider centralised on personal goals and access support networks. Strategies to comprehensively communicate information between providers, support self-management, and negotiate individualised goals for independence during transition planning may improve satisfaction, and health and treatment outcomes.

Objective To evaluate sodium-glucose cotransporter-2 (SGLT-2) inhibitors and glucagon-like peptide-1 (GLP-1) receptor agonists in patients with type 2 diabetes at varying cardiovascular and renal risk. Design Network meta-analysis. Data sources Medline, Embase, and Cochrane CENTRAL up to 11 August 2020. Eligibility criteria for selecting studies Randomised controlled trials comparing SGLT-2 inhibitors or GLP-1 receptor agonists with placebo, standard care, or other glucose lowering treatment in adults with type 2 diabetes with follow up of 24 weeks or longer. Studies were screened independently by two reviewers for eligibility, extracted data, and assessed risk of bias. Main outcome measures Frequentist random effects network meta-analysis was carried out and GRADE (grading of recommendations assessment, development, and evaluation) used to assess evidence certainty. Results included estimated absolute effects of treatment per 1000 patients treated for five years for patients at very low risk (no cardiovascular risk factors), low risk (three or more cardiovascular risk factors), moderate risk (cardiovascular disease), high risk (chronic kidney disease), and very high risk (cardiovascular disease and kidney disease). A guideline panel provided oversight of the systematic review. Results 764 trials including 421 346 patients proved eligible. All results refer to the addition of SGLT-2 inhibitors and GLP-1 receptor agonists to existing diabetes treatment. Both classes of drugs lowered all cause mortality, cardiovascular mortality, non-fatal myocardial infarction, and kidney failure (high certainty evidence). Notable differences were found between the two agents: SGLT-2 inhibitors reduced admission to hospital for heart failure more than GLP-1 receptor agonists, and GLP-1 receptor agonists reduced non-fatal stroke more than SGLT-2 inhibitors (which appeared to have no effect). SGLT-2 inhibitors caused genital infection (high certainty), whereas GLP-1 receptor agonists might cause severe gastrointestinal events (low certainty). Low certainty evidence suggested that SGLT-2 inhibitors and GLP-1 receptor agonists might lower body weight. Little or no evidence was found for the effect of SGLT-2 inhibitors or GLP-1 receptor agonists on limb amputation, blindness, eye disease, neuropathic pain, or health related quality of life. The absolute benefits of these drugs vary substantially across patients from low to very high risk of cardiovascular and renal outcomes (eg, SGLT-2 inhibitors resulted in 3 to 40 fewer deaths in 1000 patients over five years; see interactive decision support tool ( https://magicevidence.org/match-it/200820dist/#!/ ) for all outcomes. Conclusions In patients with type 2 diabetes, SGLT-2 inhibitors and GLP-1 receptor agonists reduced cardiovascular and renal outcomes, with some differences in benefits and harms. Absolute benefits are determined by individual risk profiles of patients, with clear implications for clinical practice, as reflected in the BMJ Rapid Recommendations directly informed by this systematic review. Systematic review registration PROSPERO CRD42019153180.

Description: The Kidney Disease: Improving Global Outcomes (KDIGO) organization developed a clinical practice guideline in 2020 for the management of patients with diabetes and chronic kidney disease (CKD).Methods: The KDIGO Work Group (WG) was tasked with developing the guideline for diabetes management in CKD. It defined the scope of the guideline, gathered evidence, determined systematic review topics, and graded evidence that had been summarized by an evidence review team. The English-language literature searches, which were initially done through October 2018, were updated in February 2020. The WG used the GRADE (Grading of Recommendations Assessment, Development and Evaluation) approach to appraise evidence and rate the strength of the recommendations. Expert judgment was used to develop consensus practice points supplementary to the evidence-based graded recommendations. The guideline document underwent open public review. Comments from various stakeholders, subject matter experts, and industry and national organizations were considered before the document was finalized.Recommendations: The guideline includes 12 recommendations and 48 practice points for clinicians caring for patients with diabetes and CKD. This synopsis focuses on the key recommendations pertinent to the following issues: comprehensive care needs, glycemic monitoring and targets, lifestyle interventions, antihyperglycemic therapies, and educational and integrated care approaches.

ObjectivePatients with chronic kidney disease (CKD) requiring kidney replacement therapy (KRT) in rural communities encounter many barriers in accessing equitable care and have worse outcomes compared with patients in urban areas. This study aims to describe the perspectives of patients and caregivers on access to KRT in rural communities to inform strategies to maximise access to quality care, and thereby reduce disadvantage, inequity and improve health outcomes.Setting18 studies (n=593 participants) conducted across eight countries (Australia, Canada, the UK, New Zealand, Ghana, the USA, Tanzania and India).ResultsWe identified five themes: uncertainty in navigating healthcare services (with subthemes of struggling to absorb information, without familiarity and exposure to options, grieving former roles and yearning for cultural safety); fearing separation from family and home (anguish of homesickness, unable to fulfil family roles and preserving sense of belonging in community); intense burden of travel and cost (poverty of time, exposure to risks and hazards, and taking a financial toll); making life-changing sacrifices; guilt and worry in receiving care (shame in taking resources from others, harbouring concerns for living donor, and coping and managing in isolation).ConclusionPatients with CKD in rural areas face profound and inequitable challenges of displacement, financial burden and separation from family in accessing KRT, which can have severe consequences on their well-being and outcomes. Strategies are needed to improve access and reduce the burden of obtaining appropriate KRT in rural communities.

Background Intravenous (IV) cyclophosphamide has been first-line treatment for inducing disease remission in lupus nephritis. The comparative efficacy and toxicity of newer agents such as mycophenolate mofetil (MMF) and calcineurin inhibitors are uncertain. Study Design Network meta-analysis. Setting & Population Patients with proliferative lupus nephritis. Selection Criteria for Studies Randomized trials of immunosuppression to induce or maintain disease remission. Interventions IV cyclophosphamide, oral cyclophosphamide, MMF, calcineurin inhibitor, plasma exchange, rituximab, or azathioprine, alone or in combination. Outcomes Complete remission, end-stage kidney disease, all-cause mortality, doubling of serum creatinine level, relapse, and adverse events. Results 53 studies involving 4,222 participants were eligible. Induction and maintenance treatments were administered for 12 (IQR, 6-84) and 25 (IQR, 12-48) months, respectively. There was no evidence of different effects between therapies on all-cause mortality, doubling of serum creatinine level, or end-stage kidney disease. Compared to IV cyclophosphamide, the most effective treatments to induce remission in moderate- to high-quality evidence were combined MMF and calcineurin inhibitor therapy, calcineurin inhibitors, and MMF (ORs were 2.69 [95% CI, 1.74-4.16], 1.86 [95% CI, 1.05-3.30], and 1.54 [95% CI, 1.04-2.30], respectively). MMF was significantly less likely than IV cyclophosphamide to cause alopecia (OR, 0.21; 95% CI, 0.12-0.36), and MMF combined with calcineurin inhibitor therapy was less likely to cause ovarian failure (OR, 0.25; 95% CI, 0.07-0.93). Regimens generally had similar odds of major infection. MMF was the most effective strategy to maintain remission. Limitations Outcome definitions not standardized, short duration of follow-up, and possible confounding by previous or subsequent therapy. Conclusions Evidence for induction therapy for lupus nephritis is inconclusive based on treatment effects on all-cause mortality, doubling of serum creatinine level, and end-stage kidney disease. MMF, calcineurin inhibitors, or their combination were most effective for inducing remission compared to IV cyclophosphamide, while conferring similar or lower treatment toxicity. MMF was the most effective maintenance therapy.

Objective.Managing juvenile-onset systemic lupus erythematosus (SLE) is particularly challenging. The disease may be severe, adolescent patients have complex medical and psychosocial needs, and patients must navigate the transition to adult services. To inform patient-centered care, we aimed to identify the healthcare and research priorities of young patients with SLE and describe the reasons underpinning their priorities.Methods.Face-to-face, semistructured interviews and focus groups were conducted with patients with SLE, aged from 14 to 26 years, from 5 centers in Australia. For each of the 5 allocation exercises, participants allocated 10 votes to (1) research topics; research questions on (2) medical management, (3) prevention and diagnosis, (4) lifestyle and psychosocial; and (5) healthcare specialties, and discussed the reasons for their choices. Descriptive statistics were calculated for votes and qualitative data were analyzed thematically.Results.The 26 participants prioritized research that alleviated the psychological burden of SLE. They allocated their votes toward medical and mental health specialties in the management of SLE, while fewer votes were given to physiotherapy/occupational therapy and dietetics. The following 7 themes underpinned the participants’ priorities: improving service shortfalls, strengthening well-being, ensuring cost efficiency, minimizing family/community burden, severity of comorbidity or complications, reducing lifestyle disruption, and fulfilling future goals.Conclusion.Young patients with SLE value comprehensive care with greater coordination among specialties. They prioritized research focused on alleviating poor psychological outcomes. The healthcare and research agenda for patients with SLE should include everyone involved, to ensure that the agenda aligns with patient priorities, needs, and values.

Objectives We aimed to describe patients’ attitudes and experiences of transition from paediatric to adult healthcare in rheumatology to inform patient-centred transitional care programmes. Methods We searched MEDLINE, EMBASE, PsycINFO and CINAHL to August 2019 and used thematic synthesis to analyse the findings. Results From 26 studies involving 451 people with juvenile-onset rheumatic conditions we identified six themes: a sense of belonging (comfort in familiarity, connectedness in shared experiences, reassurance in being with others of a similar age, desire for normality and acceptance); preparedness for sudden changes (confidence through guided introductions to the adult environment, rapport from continuity of care, security in a reliable point of contact, minimizing lifestyle disruptions); abandonment and fear of the unknown (abrupt and forced independence, ill-equipped to hand over medical information, shocked by meeting adults with visible damage and disability, vulnerability in the loss of privacy); anonymous and dismissed in adult care (deprived of human focus, sterile and uninviting environment, disregard of debilitating pain and fatigue); quest for autonomy (controlled and patronized in the paediatric environment, liberated from the authority of others, freedom to communicate openly); and tensions in parental involvement (overshadowed by parental presence, guilt of excluding parents, reluctant withdrawal of parental support). Conclusion Young people feel dismissed, abandoned, ill-prepared and out of control during transition. However, successful transition can be supported by preparing for changes, creating a sense of belonging and negotiating parental involvement and autonomy. Incorporating patient-identified priorities into transitional services may improve satisfaction and outcomes in young people with juvenile-onset rheumatic conditions.

Aim There is no national consensus on infection control in haemodialysis units in Australia and New Zealand. The primary aim of this guideline was to provide recommendations on screening for blood-borne viruses and multi-resistant organisms for dialysis units based on the available evidence. Methods The Kidney Health Australia Caring for Australasians with Renal Impairment guidelines, overall approach to guideline development follows the GRADE framework. A facilitated workshop was conducted to ensure that patient and caregiver concerns were considered. The evidence from relevant medical databases on the impact of screening on detection and transmission rates, hospitalization, mortality and psychosocial care, was reviewed and critically appraised. The guideline group made recommendations from the evidence available. Results The main guideline recommendations are: Dialysis units adopt a comprehensive approach that encompasses standard infection control precautions. Conduct routine surveillance for key blood-borne viruses and methicillin-resistant Staphylococcus aureus. Conduct routine surveillance of individual levels of protection against hepatitis B for patients on haemodialysis. Use dedicated dialysis machines for HBV-infected patients. The evidence in totality was not found to support routine surveillance of vancomycin-resistant Enterococci . Enhanced surveillance in light of the local risk of transmittable infectious agents should be considered by dialysis units. Very few studies have reported on the potential adverse effects of screening and associated practices. Conclusions Future research should focus on the potential benefits and adverse effects of screening and associated practices on clinical outcomes including infections prevented and health service delivery, and psychosocial domains for patients. Given the results of trials in the critical setting, the effectiveness of methicillin-resistant Staphylococcus aureus decolonization in people receiving dialysis therapy warrants further research.

This guideline addresses issues relevant to the diagnosis, management and extra-renal complications of autosomal-dominant polycystic kidney disease (ADPKD). Also included in this summary is a screening algorithm for ADPKD in at-risk individuals (Fig. 1) and an algorithm for screening intracranial aneurysms (ICA) (Fig. 2).

Introduction: The integration of patient and caregiver input into guideline development can help to ensure that clinical care addresses patient expectations, priorities, and needs. We aimed to identify topics and outcomes salient to patients and caregivers for inclusion in the Kidney Health Australia Caring for Australasians with Renal Impairment (KHA-CARI) clinical practice guideline on the screening and management of infectious microorganisms in hemodialysis units. Methods: A facilitated workshop was conducted with 11 participants (patients [n = 8], caregivers [n = 3]). Participants identified and discussed potential topics for inclusion in the guidelines, which were compared to those developed by the guideline working group. The workshop transcript was thematically analyzed to identify and describe the reasons underpinning their priorities. Findings: Patients and caregivers identified a range of topics already covered by the scope of the proposed guidelines and also suggested additional topics: privacy and confidentiality, psychosocial care during/after disease notification, quality of transportation, psychosocial treatment of patients in isolation, patient/caregiver education and engagement, and patient advocacy. Five themes characterized discussion and underpinned their choices: shock and vulnerability, burden of isolation, fear of infection, respect for privacy and confidentiality, and confusion over procedural inconsistencies. Discussion: Patients and caregivers emphasized the need for guidelines to address patient education and engagement, and the psychosocial implications of communication and provision of care in the context of infectious microorganisms in hemodialysis units. Integrating patient and caregiver perspectives can help to improve the relevance of guidelines to enhance quality of care, patient experiences, and health and psychosocial outcomes.

Objective.Systemic sclerosis (SSc) is a chronic, progressive autoimmune disease with major end-organ involvement. Much attention has been focused on the management of physical and clinical manifestations; however, the effect of the disease and treatment on the patient’s identity, relationships, functioning, and mental well-being are less known. We aimed to describe the patients’ perspectives and experiences of living with SSc.Methods.Electronic databases were searched to October 2014. Thematic synthesis was used to analyze the findings.Results.We included 26 studies involving 463 patients. Six key themes were identified: distressing appearance transformation (disturbing facial changes, stigmatizing sickness, unrecognizable self), palpable physical limitations (bodily restrictions, frustrating mind-body disconnect, pervasive fatigue, disabling pain), social impairment (breaking intimacy, struggling to fulfill family responsibilities, maintaining work, losing independence), navigating uncertainty (diagnostic ambiguity, medically fending for oneself, unpredictable course of illness), alone and misunderstood (fearful avoidance of fellow patients, invisible suffering), and gradual acceptance and relative optimism (adapting to change and accepting limitations, taking a positive spin, cautious hoping, empowering relationships, valuing medical support).Conclusion.SSc is a rare and unpredictable illness that undermines patients’ sense of certainty and control and impairs their self-image, identity, and daily functioning. Patient-centered care that encompasses strategies to promote self-esteem, resilience, and self-efficacy may help to improve treatment satisfaction and health and quality of life outcomes for patients with SSc.

Objective To describe the range and depth of perspectives and experiences of patients with psoriasis and psoriatic arthritis to inform gaps in patient-centered care. Methods We searched MEDLINE, Embase, PsycINFO, and CINAHL to April 2018. Thematic synthesis was used to analyze the findings. Results We included 56 studies involving 1,484 adult patients with psoriasis (n = 1,147) and psoriatic arthritis (n = 337). Six themes (and subthemes) were identified: suffering uncontrollable and ongoing upheaval (dictating life choices and course, disrupting family and social roles, limited by debilitating symptoms, unstoppable and far-reaching fatigue), weighed down by mental load (anxiety provoked by the volatility of symptoms, dreading deterioration, struggling with unrecognized distress, helpless and nihilistic), harboring shame and judgement (marked as unhygienic and contagious, rejected and isolated, hiding away and resenting own appearance, pain and embarrassment in intimacy), demoralized by inadequacies and burden of therapy (disappointed by unmet expectations of treatment benefit, daily drudgery, deterred by unpalatable or inconvenient treatments, disempowered by lack of personalized care), gaining control (making sense of the condition, accepting a new health status, regaining independence and normality, attuning to the body), and making confident treatment decisions (trading off perceptible benefits against safety and convenience, relying on family input, seeking empowering and reassuring relationships). Conclusion Patients with psoriasis and psoriatic arthritis contend with disruption in their functioning, roles, and life course and have unmet expectations about treatment. Enhanced therapeutic relationships, addressing treatment expectations and supporting psychosocial needs may improve satisfaction and outcomes.

Background Cyclophosphamide, in combination with corticosteroids, has been first-line treatment for inducing disease remission for proliferative lupus nephritis, reducing death at five years from over 50% in the 1950s and 1960s to less than 10% in recent years. Several treatment strategies designed to improve remission rates and minimise toxicity have become available. Treatments, including mycophenolate mofetil (MMF) and calcineurin inhibitors, alone and in combination, may have equivalent or improved rates of remission, lower toxicity (less alopecia and ovarian failure) and uncertain effects on death, end-stage kidney disease (ESKD) and infection. This is an update of a Cochrane review first published in 2004 and updated in 2012. Objectives Our objective was to assess the evidence and evaluate the benefits and harms of different immunosuppressive treatments in people with biopsy-proven lupus nephritis. The following questions relating to management of proliferative lupus nephritis were addressed: 1) Are new immunosuppressive agents superior to or as effective as cyclophosphamide plus corticosteroids? 2) Which agents, dosages, routes of administration and duration of therapy should be used? 3) Which toxicities occur with the different treatment regimens? Search methods We searched the Cochrane Kidney and Transplant Specialised Register up to 2 March 2018 with support from the Cochrane Information Specialist using search terms relevant to this review. Studies in the Specialised Register are identified through searches of CENTRAL, MEDLINE, and EMBASE, conference proceedings, the International Clinical Trials Register (ICTRP) Search Portal and ClinicalTrials.gov. Selection criteria Randomised controlled trials (RCTs) and quasi-RCTs comparing any immunosuppressive treatment for biopsy-proven class III, IV, V+III and V+VI lupus nephritis in adult or paediatric patients were included. Data collection and analysis Data were abstracted and the risks of bias were assessed independently by two authors. Dichotomous outcomes were calculated as risk ratio (RR) and measures on continuous scales calculated as mean differences (MD) with 95% confidence intervals (CI). The primary outcomes were death (all causes) and complete disease remission for induction therapy and disease relapse for maintenance therapy. Evidence certainty was determined using GRADE. Main results In this review update, 26 new studies were identified, to include 74 studies involving 5175 participants overall. Twenty-nine studies included children under the age of 18 years with lupus nephritis, however only two studies exclusively examined the treatment of lupus nephritis in patients less than 18 years of age. Induction therapy Sixty-seven studies (4791 participants; median 12 months duration (range 2.5 to 48 months)) reported induction therapy. The effects of all treatment strategies on death (all causes) and ESKD were uncertain (very low certainty evidence) as this outcome occurred very infrequently. Compared with intravenous (IV) cyclophosphamide, MMF may have increased complete disease remission (RR 1.17, 95% CI 0.97 to 1.42; low certainty evidence), although the range of effects includes the possibility of little or no difference. Compared to IV cyclophosphamide, MMF is probably associated with decreased alopecia (RR 0.29, 95% CI 0.19 to 0.46; 170 less (129 less to 194 less) per 1000 people) (moderate certainty evidence), increased diarrhoea (RR 2.42, 95% CI 1.64 to 3.58; 142 more (64 more to 257 more) per 1000 people) (moderate certainty evidence) and may have made little or no difference to major infection (RR 1.02, 95% CI 0.67 to 1.54; 2 less (38 less to 62 more) per 1000 people) (low certainty evidence). It is uncertain if MMF decreased ovarian failure compared to IV cyclophosphamide because the certainty of the evidence was very low (RR 0.36, 95% CI 0.06 to 2.18; 26 less (39 less to 49 more) per 1000 people). Studies were not generally designed to measure ESKD. MMF combined with tacrolimus may have increased complete disease remission (RR 2.38, 95% CI 1.07 to 5.30; 336 more (17 to 1048 more) per 1000 people (low certainty evidence) compared with IV cyclophosphamide, however the effects on alopecia, diarrhoea, ovarian failure, and major infection remain uncertain. Compared to standard of care, the effects of biologics on most outcomes were uncertain because of low to very low certainty of evidence. Maintenance therapy Nine studies (767 participants; median 30 months duration (range 6 to 63 months)) reported maintenance therapy. In maintenance therapy, disease relapse is probably increased with azathioprine compared with MMF (RR 1.75, 95% CI 1.20 to 2.55; 114 more (30 to 236 more) per 1000 people (moderate certainty evidence). Multiple other interventions were compared as maintenance therapy, but patient-outcome data were sparse leading to imprecise estimates. Authors' conclusions In this review update, studies assessing treatment for proliferative lupus nephritis were not designed to assess death (all causes) or ESKD. MMF may lead to increased complete disease remission compared with IV cyclophosphamide, with an acceptable adverse event profile, although evidence certainty was low and included the possibility of no difference. Calcineurin combined with lower dose MMF may improve induction of disease remission compared with IV cyclophosphamide, but the comparative safety profile of these therapies is uncertain. Azathioprine may increase disease relapse as maintenance therapy compared with MMF.

Background Patients with psoriasis and psoriatic arthritis have a lower health related quality of life than the normal population and experience high rates of treatment dissatisfaction1. The complexity of unmet needs in diagnosis and treatment2 necessitate a deep understanding of the experience of people with both conditions to guide development of outcomes important to patients and improve patient centred care. Objectives To describe the perspectives and experiences of patients with psoriasis and psoriatic arthritis. Methods Databases (MEDLINE, Embase, PsycINFO, CINAHL) were searched to October 2016. Thematic synthesis was used to analyse the findings. Results From 46 studies (n=37 psoriasis and n=9 psoriatic arthritis) involving 1290 adult patients with psoriasis (n=1105) and psoriatic arthritis (n=185) we identified six themes (with subthemes): suffering uncontrollable and ongoing upheaval (dictating life choices and course, disrupting role functioning, limited by debilitating symptoms, unstoppable and far reaching fatigue); weighed down by mental load (struggling with unrecognised distress, anxiety provoked by the volatility and constancy of symptoms, depleting motivation and pleasure); harbouring shame and judgement (marked as unhygienic and contagious, rejected and isolated, resenting own appearance, pain and embarrassment in intimacy); demoralised by inadequacies and burden of therapy (disappointed by unmet expectations of treatment benefit, daily drudgery, deterred by unpalatable or inconvenient treatments, disempowered by lack of personalised care, fearing long term side effects); gaining control (making sense of the condition, shutting the disease out, accepting a new health status, attuning to the body); and making confident treatment decisions (trading off perceptible benefits against safety and convenience, relying on family input, reassured by clinician acknowledgement of fears, seeking empowering relationships with clinicians). Conclusions Patients with psoriasis and psoriatic arthritis contend with profound disruption in their functioning, roles and life course; fear deterioration of their health; and have unmet expectations about their treatment and care. Patients with psoriasis feel marked by their disease, stigmatised and rejected by others while patients with psoriatic arthritis experience social withdrawal and depleted motivation due to fatigue, joint impairment and pain. Establishing therapeutic relationships, addressing treatment expectations, and supporting psychosocial needs may help to improve satisfaction and outcomes in patients with psoriasis and psoriatic arthritis. References [1] Katherine M. Mercy, Kenneth B. Gordon, Amy S. Paller. Patient Satisfaction and Quality of Life in Psoriasis and Psoriatic Arthritis. JAMA. 2014;312(24):2676–2677 [2] Helliwell, et al. Qualifying Unmet Needs and Improving Standards of Care in Psoriatic Arthritis. Arthritis Care & Research Vol. 66, No. 12, December 2014, pp 1759–1766 Disclosure of Interest None declared

Aim Percutaneous renal biopsy is often essential for providing reliable diagnostic and prognostic information for people with suspected kidney disease, however the procedure can lead to complications and concerns among patients. This study aims to identify and integrate patient priorities and perspectives into the Kidney Health Australia - Caring for Australasians with Renal Impairment clinical practice guidelines for renal biopsy, to ensure patient-relevance. Methods We convened a workshop, consisting of three simultaneous focus groups and a plenary session, with 10 patients who had undergone a renal biopsy and seven caregivers. Topics and outcomes prioritized by patients and their caregivers were compared to those identified by the guideline working group, which was comprised of seven nephrologists. Transcripts and flipcharts were analyzed thematically to identify the reasons for participants' choices. Results In total, 34 topics/outcomes were identified, 14 of which were common to the list of 28 previously identified by the guideline working group. Most of the new topics identified by patients/caregivers were related to communication and education, psychosocial support, and self-management. We identified five themes underpinning the reasons for topic and outcome selection: alleviating anxiety and unnecessary distress, minimizing discomfort and disruption, supporting family and caregivers, enabling self-management, and protecting their kidney. A new topic on patient care and education was added to the guideline as a result. Conclusions Patient and caregiver involvement in developing guidelines on renal biopsy ensured that their concerns and needs for education, psychosocial support, and self-management were explicitly addressed; enabling a patient-centred approach to renal biopsies.

Aim: This study aimed to identify consumer perspectives on topics and outcomes to integrate in the Kidney Health Australia Caring for Australasians with Renal Impairment (KHA-CARI) clinical practice guidelines on autosomal-dominant polycystic kidney disease (ADPKD). Methods: A workshop involving three concurrent focus groups with 18 consumers (patients with ADPKD (n = 15), caregivers (n = 3)) was convened. Guideline topics, interventions and outcomes were identified, and integrated into guideline development. Thematic analysis was used to analyse the reasons for their choices. Results: Twenty-two priority topics were identified, with most focussed on non-pharmacological management (diet, fluid intake, physical activity, complementary medicine), pain management and psychosocial care (mental health, counselling, cognitive and behavioural training, education, support groups). They also identified 26 outcomes including quality of life (QoL), progression of kidney disease, kidney function, cyst growth and nephrotoxity. Almost all topics and outcomes suggested were identified by health professionals with the exception of five topics/outcomes. Six themes reflected reasons for their choices: clarifying ambiguities, resolving debilitating pain, concern for family, preparedness for the future, taking control and significance of impact. Conclusion: Although there was considerable concordance between the priority topics and outcomes of health professionals and consumers for guidelines of ADPKD, there was also important discordance with consumers focused on fewer issues, but particularly on lifestyle, psychosocial support, pain, and QoL and renal outcomes. Active consumer engagement in guidelines development can help to ensure the inclusion of patient-centred recommendations, which may lead to better management of disease progression, symptoms, complications, and psychosocial impact.

Background. Autosomal dominant polycystic kidney disease (ADPKD) is a life-threatening genetic disorder and has multiple complications including, infection, pain, intracranial aneurysm and kidney failure leading to significantly impaired quality of life and reduced survival. These outcomes are well described, but patient perspectives and experiences of living with ADPKD are under-recognized. Methods. MEDLINE, Embase, PsycINFO and CINAHL were searched to August 2014. Studies were analyzed using thematic synthesis. Results. From 21 studies (n = 247), we derived five themes: unvalidated pain (medical trivialization, inadequacy of pain management); persisting uncertainties and ambiguities (lacking diagnostic clarity, disempowerment in self-care, unpredictable daily disruptions, inability to plan ahead, financial discrimination); genetic guilt and resentment (blaming parents, selfblame, constant burden of guilt); precariousness in pursuing parenthood (prognostic uncertainty, owning the decision, needing directive counselling); and defining parental responsibility for genetic testing and disclosure (preserving normality, doubting necessity of genetic testing, respecting the child’s autonomy and hope in future technologies, facilitating preparedness). Conclusions. The erratic onset of pain contributes to the substantial unpredictability of daily living and prevents patients from establishing long-term life goals. Decisions about family planning, genetic testing of children and disclosure involves making profoundly difficult judgments about ethical parental responsibility. Patient engagement in pain management, strategies for self-care, counselling to reduce the burden of ‘genetic guilt’ and specific family planning decision support tools may be priorities for care to improve patient-centred outcomes in ADPKD.

Objectives Systemic lupus erythematosus (SLE) is a complex autoimmune disease that can affect multiple organ systems, with specialists from many disciplines often involved, which may lead to inconsistent care. We aimed to describe the attitudes and perspectives of specialists from different medical disciplines on the management of people with SLE. Methods Face-to-face, semi-structured interviews were conducted with rheumatologists (n=16), nephrologists (n=16), and immunologists (n=11) providing care to adults with SLE from 19 centers across Australia in 2015. All interviews were transcribed and analyzed thematically. Results Five themes were identified: uncertainties in judgments (hampered by unknown and unclear etiology, inapplicable evidence, comprehending information dispersion); reflexive responses (anchoring to specialty training, anticipating outcomes, avoiding disaster, empathy for the vulnerable); overarching duty to patients (achieving patient priorities, maximizing adherence, controlling the disease, providing legitimate information, having adequate and relevant expertise); safeguarding professional opportunities (diversifying clinical skills, protecting colleagues’ interests); and optimizing access to treatment (capitalizing on multidisciplinary care, acquiring breakthrough therapies). Conclusions Specialists strive to deliver evidence-informed patient-centered care, but recognize they are anchored by their training. To overcome uncertainties in clinical management due to lack of high-quality evidence and specialty silo structures, specialists translated evidence from other disease settings and collaborated with other specialists in routine care. Developing robust evidence, tools to support evidence informed decisions, and multidisciplinary shared-care pathways may improve the management of people with this complex disease. This article is protected by copyright. All rights reserved.

Background and aims Pharmacological treatments have improved survival in lupus nephritis. However, intravenous cyclophosphamide as first-line therapy has considerable toxicity and lacks evidence of efficacy to prevent end-stage kidney disease. The comparative efficacy of newer strategies compared with intravenous cyclophosphamide remains unclear. Methods We updated a random-effects meta-analysis of randomised controlled trials on induction and maintenance therapy for proliferative lupus nephritis. Evidence quality was assessed using GRADE. Results 59 trials (4465 participants) were eligible, including nine new trials. Compared with intravenous cyclophosphamide, mycophenolate mofetil (MMF) incurred similar risks of complete remission, mortality, or major infection, while risks of alopecia and ovarian failure were lower (Table 1) (evidence quality=moderate). There was no evidence combined MMF and tacrolimus had different effects on complete remission or major infection than intravenous cyclophosphamide (Table 1) (evidence quality=low-very low). In maintenance therapy (Table 2), MMF decreased risks of disease relapse compared to azathioprine (evidence quality=moderate), although there was no evidence of different effects between maintenance therapies on mortality, end-stage kidney disease, or major infection (evidence quality = very low –low). Conclusions MMF is as effective as intravenous cyclophosphamide in inducing remission in patients with proliferative lupus nephritis, with lower risks of alopecia and ovarian failure, although comparative effects of treatment on end-stage kidney disease and mortality remain uncertain. MMF is the most effective maintenance treatment to prevent relapse.

Background and aims Clinicians from different medical specialists are involved in the management of patients with systemic lupus erythematosus (SLE), however, unwarranted variation in practice remains largely unexplained. This study aims to describe specialists’ attitudes and perspectives on the management of patients with SLE. Methods Face-to-face, semi-structured interviews were conducted with rheumatologists, nephrologists, and immunologists providing care to adult patients with SLE from 19 centres across Australia. All interviews were transcribed and analysed thematically. Results The 43 participants (Table 1) identified five themes and subthemes: uncertainties in judgements (hampered by unknown and unclear aetiology, inapplicable evidence, comprehending information dispersion); reflexive responses (anchoring to speciality training, anticipating outcomes, avoiding disaster, empathy for the vulnerable); overarching duty to patients (achieving patient priorities, maximising adherence, controlling the disease, legitimate educator, having adequate and relevant expertise); safeguarding professional opportunities (diversifying clinical skills, protecting colleagues’ interests); and optimising access to treatment (capitalising on multidisciplinary care, acquiring breakthrough therapies). Illustrative quotations are provided in Table 2, and patterns and relationships among all themes are shown in Figure 1. Conclusions Specialists endeavour to achieve optimal outcomes for patients with SLE but uncertainties in clinical decisions arise due to the ill-defined aetiology of SLE, lack of robust, consistent and implementable evidence, and speciality silo structures. Developing tools to support evidence-informed decisions, generating robust evidence to address clinical priorities, and establishing collaborative and multidisciplinary care pathways may support clinical decision making and management of a complex and heterogeneous disease, and help to minimise unwarranted variation in practice

550 a. We recommend that adult patients diagnosed with autosomal dominant polycystic kidney disease are referred to their regional genetics service for genetic counseling if they are interested in and would like to discuss (2B) the following: i. Inheritance pattern and clarifying/communicating disease risk to family members ii. Molecular genetic testing (role, indication, and interpretation) iii. Family planning and prenatal testing options (including preimplantation genetic diagnosis) b. We recommend adults and children at risk of autosomal dominant polycystic kidney disease are referred to their regional genetics service for genetic counseling if they are interested in and would like to discuss (2A) the following: i. Inheritance pattern and their risk of disease ii. Predictive testing (via renal imaging and/or molecular genetic testing) and associated issues iii. Family planning and prenatal testing options (including preimplantation genetic diagnosis)

Autosomal dominant polycystic kidney disease (ADPKD) is the commonest inherited cause of renal failure in adults, and is due to loss-of-function mutations in either the PKD1 or PKD2 genes, which encode polycystin-1 and polycystin-2, respectively. These proteins have an essential role in maintaining the geometric structure of the distal collecting duct in the kidney in adult life, and their dysfunction predisposes to renal cyst formation. The typical renal phenotype of ADPKD is the insidious development of hundreds of renal cysts, which form in childhood and grow progressively through life, causing end-stage kidney failure in the fifth decade in about half affected by the mutation. Over the past 2 decades, major advances in genetics and disease pathogenesis have led to well-conducted randomized controlled trials, and observational studies that have resulted in an accumulation of evidence-based data, and raise hope that the lifetime risk of kidney failure due to ADPKD will be progressively curtailed during this century. This review will provide a contemporary summary of the current state of the field in disease pathogenesis and therapeutics, and also briefly highlights the importance of clinical practice guidelines, patient perspectives, patient-reported outcomes, uniform trial reporting, and health-economics in ADPKD.

a. We recommend screening for polycystic liver disease in all patients diagnosed with autosomal dominant polycystic kidney disease using abdominal ultrasound (1C). b. We recommend that all female patients with autosomal dominant polycystic kidney disease and liver cysts undergo counseling regarding the risks of pregnancy and exogenous estrogen exposure in worsening liver cyst growth (1C). c. We recommend that females at risk of symptoms from hepatic cysts avoid estrogen supplements (1D). d. We recommend that a multidisciplinary team (hepatologist, hepatobiliary surgeon, interventional radiologist, and nephrologist) care for patients with severe polycystic liver disease associated with autosomal dominant polycystic kidney disease (1D).

Semi a. We recommend that clinicians should include the evaluation of pain in patients with autosomal dominant polycystic kidney disease during clinic visits (1D). b. We recommend that patients be involved in the management of their pain, non-pharmacological treatments be emphasized in the first instance (1D). c. We suggest that surgical interventions for cyst decortication may be warranted in individuals with