Sodium-glucose cotransporter 2 inhibition in primary and secondary glomerulonephritis.

BACKGROUND: The role of sodium-glucose cotransporter 2 inhibitors (SGLT2i) in the management glomerular/systemic autoimmune diseases with proteinuria in real-world clinical settings is unclear. METHODS: This is a retrospective, observational, international cohort study. Adult patients with biopsy-proven glomerular diseases were included. The main outcome was the percentage reduction in 24-h proteinuria from SGLT2i initiation to 3, 6, 9 and 12 months. Secondary outcomes included percentage change in estimated glomerular filtration rate (eGFR), proteinuria reduction by type of disease and reduction of proteinuria ≥30% from SGLT2i initiation. RESULTS: Four-hundred and ninety-three patients with a median age of 55 years and background therapy with renin-angiotensin system blockers were included. Proteinuria from baseline changed by -35%, -41%, -45% and -48% at 3, 6, 9 and 12 months after SGLT2i initiation, while eGFR changed by -6%, -3%, -8% and -10.5% at 3, 6, 9 and 12 months, respectively. Results were similar irrespective of the underlying disease. A correlation was found between body mass index (BMI) and percentage proteinuria reduction at last follow-up. By mixed-effects logistic regression model, serum albumin at SGLT2i initiation emerged as a predictor of ≥30% proteinuria reduction (odds ratio for albumin <3.5 g/dL, 0.53; 95% CI 0.30-0.91; P = .02). A slower eGFR decline was observed in patients achieving a ≥30% proteinuria reduction: -3.7 versus -5.3 mL/min/1.73 m2/year (P = .001). The overall tolerance to SGLT2i was good. CONCLUSIONS: The use of SGLT2i was associated with a significant reduction of proteinuria. This percentage change is greater in patients with higher BMI. Higher serum albumin at SGLT2i onset is associated with higher probability of achieving a ≥30% proteinuria reduction.

Deprivation and chronic kidney disease-a review of the evidence.

The relationship between socioeconomic deprivation and health is inequitable. Chronic kidney disease (CKD) is an archetypal disease of inequality, being more common amongst those living in deprivation. The prevalence of CKD is rising driven by an increase in lifestyle-related conditions. This narrative review describes deprivation and its association with adverse outcomes in adults with non-dialysis-dependent CKD including disease progression, end-stage kidney disease, cardiovascular disease and all-cause mortality. We explore the social determinants of health and individual lifestyle factors to address whether patients with CKD who are socioeconomically deprived have poorer outcomes than those of higher socioeconomic status. We describe whether observed differences in outcomes are associated with income, employment, educational attainment, health literacy, access to healthcare, housing, air pollution, cigarette smoking, alcohol use or aerobic exercise. The impact of socioeconomic deprivation in adults with non-dialysis-dependent CKD is complex, multi-faceted and frequently under-explored within the literature. There is evidence that patients with CKD who are socioeconomically deprived have faster disease progression, higher risk of cardiovascular disease and premature mortality. This appears to be the result of both socioeconomic and individual lifestyle factors. However, there is a paucity of studies and methodological limitations. Extrapolation of findings to different societies and healthcare systems is challenging, however, the disproportionate effect of deprivation in patients with CKD necessitates a call to action. Further empirical study is warranted to establish the true cost of deprivation in CKD to patients and societies.

Management of antineutrophil cytoplasmic antibody-associated vasculitis with glomerulonephritis as proposed by the ACR 2021, EULAR 2022 and KDIGO 2021 guidelines/recommendations.

Updated guidelines on the management of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) were released in 2021 by the American College of Rheumatology jointly with the Vasculitis Foundation and, subsequently, in 2022 by the European Alliance of Associations for Rheumatology. In addition, in 2021, the Kidney Disease: Improving Global Outcomes had released updated recommendations on the treatment of AAV with glomerulonephritis (AAV-GN). Kidney involvement is particularly relevant in microscopic polyangiitis and granulomatosis with polyangiitis, but is less frequent in eosinophilic granulomatosis with polyangiitis. The management of AAV-GN has been a focus for drug development and change over the past 10 years. Avoidance of progression to end-stage kidney disease (ESKD) or kidney failure is one of the main unmet needs in the management of AAV, with ESKD having a major impact on morbidity, health costs and mortality risk. Relevant changes in AAV-GN management are related to remission-induction treatment of patients with severe kidney disease, the use of glucocorticoids and avacopan, and remission-maintenance treatment. All the documents provide guidance in accordance with the evidence-based standard of care available at the time of their release. With our work we aim to (i) show the progress made and identify the differences between guidelines and recommendations, (ii) discuss the supporting rationale for those, and (iii) identify gaps in knowledge that could benefit from additional research and should be revised in subsequent updates.

Recurrent croup is a good indicator of underlying paediatric airway issues: A 10-year retrospective cohort study of airway endoscopy.

OBJECTIVE: Children with a history of recurrent croup alert the ENT clinician to the potential for underlying laryngotracheal pathology. There is equipoise about the likelihood of identifying any underlying structural issues or subglottic stenosis in those children who undergo airway assessment. METHODS: A retrospective cohort study in a tertiary UK paediatric hospital of a decade of children with recurrent croup who underwent a rigid laryngo-tracheo-bronchoscopy (airway endoscopy). MAIN OUTCOME(S): airway pathology seen on endoscopy and need for further airway surgery. RESULTS: In ten years, 139 children underwent airway endoscopy for recurrent croup. Operative findings were abnormal in 62 (45 %) cases. Twelve cases (9%) had subglottic stenosis. Although recurrent croup was more common in males (78% of cases), this was not found to predispose them to operative findings. Children with previous intubations had >2 times the risk of abnormal findings and children born prematurely (<37 wks) had a trend towards abnormal operative findings versus children with no airway findings in our cohort. Even in those patients with abnormal findings, none necessitated further airway surgery. CONCLUSIONS: Surgeons and parents can be reassured that rigid airway endoscopy for children with recurrent croup demonstrated high diagnostic utility but will rarely lead to further surgical intervention. Greater understanding about recurrent croup may require consensus clarification about definitions of recurrent croup and/or a universal adoption of a minimum standard operative record or grading system after rigid endoscopy for recurrent croup.

The management of lupus nephritis as proposed by EULAR/ERA 2019 versus KDIGO 2021.

In 2019 and 2021, the European League for Rheumatism (EULAR) jointly with the European Renal Association (ERA) and the Kidney Disease: Improving Global Outcomes (KDIGO), respectively, released updated guidelines on the management of lupus nephritis (LN). The Immunology Working Group of the ERA reviewed and compared both updates. Recommendations were either consistent or differences were of negligible clinical relevance for: indication for kidney biopsy, kidney biopsy interpretation, treatment targets, hydroxychloroquine dosing, first-line initial immunosuppressive therapy for active class III, IV (±V) LN, pregnancy in LN, LN in paediatric patients and LN patients with kidney failure. Relevant differences in the recommended management relate to the recognition of lupus podocytopathies, uncertainties in steroid dosing, drug preferences in specific populations and maintenance therapy, treatment of pure class V LN, therapy of recurrent LN, evolving alternative drug options and diagnostic work-up of thrombotic microangiopathy. Altogether, both documents provide an excellent guidance to the growing complexity of LN management. This article endeavours to prevent confusion by identifying differences and clarifying discrepancies.

A pilot study to evaluate the safety and efficacy of treprostinil in the treatment of calcinosis in systemic sclerosis.

OBJECTIVES: We evaluated the safety and efficacy of oral treprostinil in preventing progression of SSc-associated calcinosis. METHODS: This prospective open-label study enrolled 12 SSc patients meeting 2013 ACR/EULAR classification criteria with confirmed clinical and radiographic evidence of one or more calcinosis deposit in the hands. Patients received oral treprostinil for 1 year. Primary endpoints were safety/tolerability and percentage of patients without radiographic progression of calcinosis at 1 year (<25% increase in Scleroderma Clinical Trials Consortium radiographic score). Secondary endpoints included 1-year changes in Scleroderma HAQ (SHAQ), Cochin Hand Functional Scale, Medical Outcomes Survey Short Form 36 (SF-36), Raynaud Condition Score and patient/physician assessment of calcinosis severity. RESULTS: Twelve female patients were enrolled, half with diffuse cutaneous disease; median age was 55 years (range 35-68 years). Five patients completed the study. Seven patients withdrew due to intolerable adverse effects (n = 3), intercurrent unrelated illness (n = 2, cirrhosis, cancer), progressive SSc (n = 1) and personal reasons (n = 1). Most patients developed headaches and gastrointestinal adverse effects. Four of 11 (36%) patients with 1-year follow-up hand radiographs experienced progression of calcinosis. Of five who completed treatment, calcinosis was stable in four (80%) with progression in one. Based on SF-36 Physical and Mental Component and Domain scores, transition question and SF-6D utility score, all patients who finished the trial reported overall improvement or no change compared with baseline. CONCLUSION: Oral treprostinil was poorly tolerated in SSc patients with calcinosis. Of five patients who completed treatment, most (80%) had documented stability of calcinosis on hand radiographs at 1 year. CLINICALTRIALS.GOV IDENTIFIER: NCT02663895.

Renal association clinical practice guideline in post-operative care in the kidney transplant recipient.

These guidelines cover the care of patients from the period following kidney transplantation until the transplant is no longer working or the patient dies. During the early phase prevention of acute rejection and infection are the priority. After around 3-6 months, the priorities change to preservation of transplant function and avoiding the long-term complications of immunosuppressive medication (the medication used to suppress the immune system to prevent rejection). The topics discussed include organization of outpatient follow up, immunosuppressive medication, treatment of acute and chronic rejection, and prevention of complications. The potential complications discussed include heart disease, infection, cancer, bone disease and blood disorders. There is also a section on contraception and reproductive issues.Immediately after the introduction there is a statement of all the recommendations. These recommendations are written in a language that we think should be understandable by many patients, relatives, carers and other interested people. Consequently we have not reworded or restated them in this lay summary. They are graded 1 or 2 depending on the strength of the recommendation by the authors, and AD depending on the quality of the evidence that the recommendation is based on.