Outcomes after per oral pyloromyotomy based on gastroparesis etiology.

Background: Per oral pyloromyotomy (POP) has been shown to be effective in patients with gastroparesis. The three most common etiologies of gastroparesis are diabetic, postsurgical, and idiopathic. Our aim was to compare outcomes after POP based on the etiology of gastroparesis.

Fundoplication at the time of paraesophageal hernia repair does not decrease the rate of hernia recurrence or postoperative reflux.

Background: Fundoplication at the time of paraesophageal hernia (PEH) repair is thought to help prevent the development or persistence of postoperative gastroesophageal reflux (GERD) and might also prevent hernia recurrence. However, the published data is not strong enough to definitively recommend this approach. This study was designed to evaluate the effectiveness and complications of a fundoplication at the time of paraesophageal hernia repair.

The effects of age and frailty on the risks of end-stage renal disease, death, and severe infection in older adults with antineutrophil cytoplasmic antibody-associated vasculitis: a retrospective cohort study.

Background: Frailty, a measure of biological age, might predict poor outcomes in older adults better than chronological age. We aimed to compare the effect of age and frailty on end-stage renal disease, death, and severe infection within 2 years of diagnosis in older adults with incident antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis.

Methods: This retrospective cohort study included individuals aged 65 years or older from the Mass General Brigham ANCA-associated vasculitis cohort in the USA who were treated between Jan 1, 2002, and Dec 31, 2019.

Elevated preoperative lower esophageal sphincter pressure predicts improved clinical outcomes after per oral endoscopic myotomy (POEM).

Background: Although per oral endoscopic myotomy (POEM) has shown to be beneficial for the treatment of achalasia, it can be difficult to predict who will have a robust and long-lasting response. Historically, it has been shown that higher lower esophageal sphincter pressures have been associated with poorer responses to alternative endoscopic therapies such as Botox therapy and pneumatic dilation. This study was designed to evaluate if modern preoperative manometric data could similarly predict response to therapy after POEM.

Initial experience and outcomes of per oral pyloromyotomy for the treatment of refractory gastroparesis.

Background: Gastroparesis can be a debilitating disease process for which durable treatment options are lacking. While dietary changes and pharmacotherapy have some efficacy, symptoms frequently recur and some patients progress to needing supplemental enteral feeding access. Per oral pyloromyotomy (POP) has been shown to be a durable minimally invasive treatment option for refractory gastroparesis with a low side effect profile, and therefore has been performed at this institution for the past 6 years.

Does practice make perfect? Studying the relationship between surgeon experience and patient outcomes for paraesophageal hernia repairs.

Background: It has been reported that higher surgeon experience leads to better patient outcomes. In this study, we look at surgeon experience and its association with postoperative outcomes and variation among the practice of surgeons performing paraesophageal hernia repairs (PEH).

Method: This was a retrospective study of 1155 patients who underwent PEH repair at a single institution (2010-2023).

Multiorgan involvement and circulating IgG1 predict hypocomplementaemia in IgG4-related disease.

Objectives: Hypocomplementaemia is common in patients with IgG4-related disease (IgG4-RD). We aimed to determine the IgG4-RD features associated with hypocomplementaemia and investigate mechanisms of complement activation in this disease.

Methods: We performed a single-centre cross-sectional study of 279 patients who fulfilled the IgG4-RD classification criteria, using unadjusted and multivariable-adjusted logistic regression to identify factors associated with hypocomplementaemia.

Does preoperative symptom duration affect outcomes following per oral endoscopic myotomy (POEM)?

Background: Per oral endoscopic myotomy (POEM) is a safe therapy for the treatment of achalasia. Long-term effects of untreated achalasia include worsening dysmotility and disruptions in esophageal anatomy, i.e.

Sex as a predictor of clinical phenotype and determinant of immune response in IgG4-related disease: a retrospective study of patients fulfilling the American College of Rheumatology-European League Against Rheumatism classification criteria.

Background: IgG4-related disease is a multiorgan fibroinflammatory disease considered to have an autoimmune origin. Case series describing individual organ involvement have suggested differences in phenotypic expression between males and females. We aimed to characterise differences in IgG4-related disease manifestations between male and female patients in a large single-centre cohort.

Current and future advances in practice: IgG4-related disease.

IgG4-related disease (IgG4-RD) is an increasingly recognized cause of fibroinflammatory lesions in patients of diverse racial and ethnic backgrounds and is associated with an increased risk of death. The aetiology of IgG4-RD is incompletely understood, but evidence to date suggests that B and T cells are important players in pathogenesis, both of which are key targets of ongoing drug development programmes. The diagnosis of IgG4-RD requires clinicopathological correlation because there is no highly specific or sensitive test.

Proliferative features of IgG4-related disease.

IgG4-related disease is an immune-mediated disease that can lead to substantial morbidity and organ damage. Capable of affecting nearly any organ system or anatomic site, and showing considerable overlap in clinical presentation with various other diseases, IgG4-related disease often poses a diagnostic challenge for clinicians. Furthermore, there are no diagnostic biomarkers with high specificity for IgG4-related disease, and histopathological examination is nuanced and requires clinical correlation for accurate diagnosis.

RheumMadness Over Two Years: Engaging Participants in Active Learning and Connecting Early Trainees to the Rheumatology Community.

Objective: RheumMadness is an online learning collaborative that seeks to actively engage the rheumatology community. The objective of this manuscript is to analyze the educational experience of RheumMadness over two years.

Methods: Direct measures of participant engagement were obtained using web-based analytics.

Enhancing Faculty Development Through Compiled Verbal Feedback on Clinical Teaching From Trainees.

Objective: Feedback from fellows-in-training (FITs) is important for faculty development and to enrich clinical teaching. We sought to evaluate the effectiveness of traditional online evaluations and a novel compiled verbal feedback mechanism.

Methods: An annual feedback system was implemented in our rheumatology division in which FITs provided verbal feedback on all faculty to a facilitator who compiled, deidentified, and shared the feedback with individual faculty members.

Assessment of patient-reported symptoms and distress in IgG4-related disease (IgG4-RD): Development, clinical validation, and content validation of the IgG4-RD Symptom Severity Index.

Objective: IgG4-related disease (IgG4-RD), a multi-organ autoimmune disease, causes diverse manifestations that can lead to symptoms and distress. We developed and validated the Symptom Severity Index (SSI) to assess symptom burden.

Methods: A pilot SSI was tested in n = 5; several gaps were identified.

IgG4-related disease as a variable-vessel vasculitis: A case series of 13 patients with medium-sized coronary artery involvement.

Introduction: IgG4-related disease (IgG4-RD) is a systemic autoimmune fibroinflammatory disease that can affect multiple organ systems. Although large-vessel vasculitis is a well-recognized manifestation of IgG4-RD, this condition is generally not regarded as a vasculitis. We aimed to describe coronary artery involvement (CAI), a vascular distribution about which little is known in IgG4-RD.

RheumMadness: Creating an Online Community of Inquiry in Rheumatology.

Objective: To evaluate the educational impact of RheumMadness, an online tournament of rheumatology concepts grounded in social constructivist theory, as viewed through the community of inquiry (CoI) framework.

Methods: The curricular scaffold of RheumMadness was a bracket of 16 rheumatology concepts competing as "teams" in a tournament. Participants could create and review "scouting reports" about each team, listen to a RheumMadness podcast, discuss on social media, and submit a bracket predicting tournament outcomes according to the perceived importance of each team.

Environmental Triggers for Vasculitis.

Systemic vasculitides are autoimmune diseases characterized by vascular inflammation. Most types of vasculitis are thought to result from antigen exposure in genetically susceptible individuals, suggesting a likely role for environmental triggers in these conditions. Seasonal and geographic variations in incidence provide insight into the potential role of environmental exposures in these diseases.

Proteomic signature for detection of high-grade ovarian cancer in germline BRCA mutation carriers.

No current screening methods for high-grade ovarian cancer (HGOC) guarantee effective early detection for high-risk women such as germline BRCA mutation carriers. Therefore, the standard-of-care remains risk-reducing salpingo-oophorectomy (RRSO) around age 40. Proximal liquid biopsy is a promising source of biomarkers, but sensitivity has not yet qualified for clinical implementation.

The value of SOX2 in the differential diagnosis of SMARCA4 (BRG1)-deficient uterine neoplasms.

SWI/SNF-related, matrix-associated, actin-dependent regulator of chromatin, subfamily A, member 4 (SMARCA4/BRG1)-deficient undifferentiated uterine sarcoma (SDUS) is a recently described uterine sarcoma. It is characterized by predominantly rhabdoid or large epithelioid cells with abundant cytoplasm and varying components of small and spindle cells, resembling the 'large cell variant' of small cell carcinoma of the ovary, hypercalcemic type (SCCOHT). In addition, SMARCA4-inactivating mutations have been described as the driver mutations in SDUS.

Association between depression, anxiety, chronic pain, or opioid use and tumor necrosis factor inhibitor persistence in inflammatory arthritis.

Introduction: Depression, anxiety, and chronic pain are common comorbidities in patients with rheumatoid arthritis (RA), psoriatic arthritis (PsA), and ankylosing spondylitis (AS) and may substantially impact patient outcomes. We aimed to determine whether these comorbidities were associated with earlier TNF-inhibitor (TNFi) discontinuation.

Methods: This retrospective cohort study using Optum's de-identified Clinformatics® Data Mart Database 2000-2014 identified patients with RA, PsA, and AS initiating a first TNFi.

Clinical Perspectives on IgG4-Related Disease and Its Classification.

Immunoglobulin G4-related disease (IgG4-RD) is a systemic immune-mediated fibroinflammatory disease. Since its discovery nearly two decades ago, our understanding of its pathophysiology and clinical manifestations has grown substantially. Early diagnosis and treatment of this elusive disease can prevent substantial organ damage from end-stage fibrosis, emphasizing the need for prompt recognition and accurate characterization of IgG4-RD.