A systematic review of patient-reported outcome measures in patients with anti-neutrophil cytoplasmic antibody associated vasculitis.

Objectives: ANCA-associated vasculitis (AAV) is associated with significant morbidity, fatigue, pain and poor health-related quality of life (HRQoL). This review aims to assess the comprehensiveness of existing patient reported outcome measures (PROMs) used in AAV and identify associations with poorer HRQoL outcomes.

Methods: A literature review of studies using PROMs, including those labelled HRQoL in people with AAV as a primary or secondary study outcome were screened and reviewed up to July 2023.

The Improved Kidney Risk Score in ANCA-Associated Vasculitis for Clinical Practice and Trials.

Significance Statement: Reliable prediction tools are needed to personalize treatment in ANCA-associated GN. More than 1500 patients were collated in an international longitudinal study to revise the ANCA kidney risk score. The score showed satisfactory performance, mimicking the original study (Harrell's C=0.

Recrudescence of Severe Carbamazepine- Induced DRESS Syndrome after Initiation of Levetiracetam.

Drug reaction with eosinophilia and systemic symptoms (DRESS) syndrome is a potentially fatal cutaneous hypersensitivity reaction commonly precipitated by antiepileptic drugs (AEDs). Cross-reactivity among aromatic AEDs is well-documented, but between aromatic and nonaromatic AEDs. We report a patient with severe DRESS syndrome precipitated by aromatic AED carbamazepine with recrudescence approximately 2 weeks after substitution with nonaromatic AED levetiracetam.

Low-Dose Intravenous Methylprednisolone in Remission Induction Therapy for ANCA-Associated Vasculitis.

Key Points: The contribution of IV methylprednisolone to glucocorticoid toxicity is often overlooked with limited evidence supporting its use. Markedly reduced cumulative glucocorticoid dosing for remission induction therapy in AAV is safe and effective. Reduced IV methylprednisolone and radical steroid avoidance strategies have not been shown to have any significant adverse effect on outcomes.

Rituximab in the treatment of primary FSGS: time for its use in routine clinical practice?

Focal segmental glomerulosclerosis (FSGS) is a common cause of nephrotic syndrome and whilst advances have been made in the pathophysiology, diagnostics and management of other podocytopathies, primary FSGS remains the most elusive. It has been assumed for a long time that a circulatory permeability factor exists that mediates podocyte injury, and the potential for autoantibody-mediated disease therefore raises the question as to whether patients may benefit from targeted B-cell therapy with rituximab. The prospective case series of seven patients by Roccatello adds to the limited but growing evidence suggesting that B-cell depletion therapy can be safe and effective in the treatment of primary FSGS.

COVID-19 Infection and Vaccination and Its Relation to Amyloidosis: What Do We Know Currently?

Amyloidosis is a complex disorder characterized by deposited insoluble fibrillar proteins which misfold into β-pleated sheets. The pathogenesis of amyloidosis can vary but can be the result of immune dysregulation that occurs from sustained high inflammatory states, often known as AA amyloidosis. Multi-organ involvement including hepatic, gastrointestinal, renal, cardiac and immunological pathological manifestations has been observed amongst individuals presenting with amyloidosis.

Perceived neighborhood disorder as a moderator of the relationship between marijuana use and disinhibition in a sample of emerging adult African American females.

Disinhibition is associated with myriad risk-taking behaviors and adverse outcomes. Both marijuana use and poor neighborhood conditions have been associated with disinhibition. However, the extent to which neighborhood disorder interacts with marijuana use to influence disinhibition has not been studied, extensively.

The Limitations of Frailty Assessment Tools in ANCA-Associated Vasculitis.

Anti-neutrophil cytoplasmic antibody (ANCA) associated vasculitis (AAV) can be associated with a high burden of morbidity and mortality in an ageing population. It is increasingly recognised that individualised management is needed. Few studies have looked specifically at frailty related outcomes in AAV and a gap remains in understanding the application of frailty assessment tools in these patients.

Using Oncotype DX breast recurrence score® assay to define the role of neoadjuvant endocrine therapy in early-stage hormone receptor-positive breast cancer.

Purpose: The role of neoadjuvant endocrine therapy in the treatment of patients with early-stage, hormone receptor-positive (HR +) breast cancer is not well defined. Tools to better determine which patients may benefit from neoadjuvant endocrine therapy versus chemotherapy or upfront surgery remain an unmet need.

Methods: We assessed the rate of clinical and pathologic complete response (cCR, pCR) among a pooled cohort of patients with early-stage HR + breast cancer who had been randomized to neoadjuvant endocrine therapy or neoadjuvant chemotherapy in two earlier studies to understand better how outcomes varied by Oncotype DX Breast Recurrence Score® assay.

A Meta-Analysis and Cohort Study of Histopathologic and Clinical Outcomes in ANCA-Negative versus -Positive Vasculitis.

Background: ANCA-negative pauci-immune glomerulonephritis (PIGN) represents a rare and often under-studied subgroup of the vasculitides. This study aims to investigate differences in the clinical phenotype, renal histological features, and clinical outcomes of patients with PIGN, with and without serum ANCA positivity.

Methods: A cohort of biopsy-proven PIGN with and without detectable circulating ANCA was constructed from a single center between 2006 and 2016.

Risk Stratification to Predict Renal Survival in Anti-Glomerular Basement Membrane Disease.

Significance Statement: Most patients with anti-glomerular basement membrane (GBM) disease present with rapidly progressive glomerulonephritis, and more than half develop ESKD. Currently, no tools are available to aid in the prognostication or management of this rare disease. In one of the largest assembled cohorts of patients with anti-GBM disease (with 174 patients included in the final analysis), the authors demonstrated that the renal risk score for ANCA-associated vasculitis is transferable to anti-GBM disease and the renal histology is strongly predictive of renal survival and recovery.

Monoclonal gammopathy of undetermined significance causing large vessel vasculitis.

A man in his late 50s presented with unilateral pain and discolouration of his fourth and fifth toes suggestive of digital ischaemia. He had a medical history of two unprovoked venous thromboembolisms in the preceding 18 months and a history of monoclonal gammopathy of undetermined significance (MGUS). A CT scan showed evidence of large vessels vasculitis in the absence of circulating antineutrophil cytoplasmic antibodies.

Glucocorticoid Therapy in ANCA Vasculitis: Using the Glucocorticoid Toxicity Index as an Outcome Measure.

Background: ANCA-associated vasculitis (AAV) is an autoimmune disease. Induction remission and maintenance treatment typically includes high-dose, tapering glucocorticoids (GC), in addition to other immunosuppressive medication. The use of theGlucocorticoid Toxicity Index (GTI) provides a global, quantifiable assessment tool in which clinicians can assess GC-associated morbidity.

Cardiovascular disease and ANCA-associated vasculitis: are we missing a beat?

The association between cardiovascular (CV) disease and anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is well documented. The recent work by Massicotte-Azarniouch . confirms the risk and adds to the existing evidence by describing the highest risk in the first 3 months after diagnosis.

IL-27 Derived From Macrophages Facilitates IL-15 Production and T Cell Maintenance Following Allergic Hypersensitivity Responses.

Crosstalk between T cells, dendritic cells, and macrophages in temporal leukocyte clusters within barrier tissues provides a new concept for T cell activation in the skin. Activated T cells from these leukocyte clusters play critical roles in the efferent phase of allergic contact hypersensitivity (CHS). However, the cytokines driving maintenance and survival of pathogenic T cells during and following CHS remain mostly unknown.

Teledermatology: How It Is Impacting the Underserved.

The rapid mobilization of telemedicine during the COVID-19 pandemic has brought about renewed concern about the digital divide. Understanding the benefits and limitations of remote medical care is an ongoing process. Access to telemedicine appointments, as well as the experience of the virtual appointment itself, varies across demographic groups.