Deep hierarchical subtyping of multi-organ systemic sclerosis trajectories - a EUSTAR study.

Systemic sclerosis (SSc) is a chronic autoimmune disease with multi-organ involvement. Historically, SSc classification has focused on the type of skin involvement (limited versus diffuse); however, a growing evidence of organ-specific variability suggests the presence of more than two distinct subtypes. We propose a semi-supervised generative deep learning framework leveraging expert-driven definitions of organ-specific involvement and severity.

Unveiling the clinical spectrum of ACA-positive SSC-ILD: not as benign as expected.

Objective: Anticentromere antibody (ACA)-positive interstitial lung disease (ILD) in systemic sclerosis (SSc) is traditionally considered less aggressive than anti-topoisomerase I (ATA)-positive ILD. However, its clinical profile and prognosis remain poorly defined. We aimed to characterise ACA-associated SSc-ILD and compare it with other serological subsets.

Platelet bound B cells and their role in SSc: Implications for disease subtypes and clinical outcomes.

Objectives: Systemic sclerosis (SSc) is a complex autoimmune disease characterized by microvascular damage, immune dysregulation, and tissue fibrosis. While lymphocyte-platelet (PLT) complexes have been implicated in autoimmune diseases, their role in SSc is not well understood.

Methods: In a study of 21 predominantly female SSc patients, 66.

ANCA associated pulmonary fibrosis: vasculitis or not vasculitis.

Purpose Of Review: The intersection of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) and interstitial lung disease (ILD) represents a complex and increasingly recognized clinical challenge. This review aims to summarize current understanding, highlight diagnostic and therapeutic approaches, and identify key gaps in the literature regarding ANCA-associated ILD.

Recent Findings: ANCA positivity-particularly MPO-ANCA- is increasingly identified in patients with fibrotic ILD, even in the absence of systemic vasculitis.

A comparative analysis of risk stratification tools in SSc-associated pulmonary arterial hypertension: a EUSTAR analysis.

Objectives: The 2022 European Society of Cardiology and European Respiratory Society (ESC/ERS) guidelines for pulmonary arterial hypertension (PAH) recommend risk stratification to optimize management. However, the performance of generic PAH risk stratification tools in patients with SSc-associated PAH remains unclear. Our objective was to identify the most accurate approach for risk stratification at SSc-PAH diagnosis.

EULAR recommendations for the treatment of systemic sclerosis: 2023 update.

Objectives: To update the 2017 European Alliance of Associations for Rheumatology (EULAR) recommendations for treatment of systemic sclerosis (SSc), incorporating new evidence and therapies.

Methods: An international task force was convened in line with EULAR standard operating procedures. A nominal group technique exercise was performed in two rounds to define questions underpinning a subsequent systematic literature review.

Disease activity in patients with idiopathic inflammatory myopathy according to time since diagnosis and positivity to antisynthetase autoantibodies: data from the Myo-Spain registry.

Objective: To evaluate the main outcomes of disease activity and their association with other measures of activity, damage, and quality of life in patients with idiopathic inflammatory myopathy (IIM) according to time since diagnosis and positivity to antisynthetase autoantibodies (ASAs).

Methods: Cross-sectional multicenter study within the Spanish Myo-Spain registry. Cases were classified as incident (≤ 12 months since diagnosis) and prevalent.

Older Patients With Interstitial Lung Disease Feature a Distinct Clinical Profile.

Introduction: There are few studies investigating the clinical profile of older patients with interstitial lung disease (ILD), so this study investigated the characteristics of the older population diagnosed with ILD.

Material And Methods: Retrospective study in a population of new referrals at an ILD clinic from January 2013 to September 2017. Patients over 64 years were selected.

Nailfold videocapillaroscopy findings correlate with lung outcomes in idiopathic inflammatory myopathies-related interstitial lung disease.

Objectives: Idiopathic inflammatory myopathies (IIM) are a diverse group of muscle diseases often complicated by interstitial lung disease (ILD), which significantly impacts morbidity and mortality. Krebs von den Lungen-6 (sKL-6) has been proposed as a biomarker for ILD severity. Nailfold videocapillaroscopy (NVC) detects microvascular changes, but its diagnostic and prognostic value in IIM remains unclear.

Comparative Analysis of Classification Criteria in IgG4-Related Disease and Evaluating Diagnostic Accuracy from a Retrospective Cohort in Clinical Practice.

Introduction: We conducted a comprehensive comparative analysis of the Okazaki, Umehara, and American College of Rheumatology/European League Against Rheumatism (ACR/EULAR) classification criteria for diagnosing immunoglobulin G4-related disease (IgG4-RD).

Materials And Methods: A retrospective study was conducted in a single tertiary hospital, using expert clinical judgment as the gold standard. We compared the diagnostic accuracy of the Okazaki, Umehara, and ACR/EULAR criteria in a cohort of 41 patients with suspected IgG4-RD.

Clinical and Serological Profiles in Cryoglobulinemia: Analysis of Isotypes and Etiologies.

: Cryoglobulinemia (CG) is marked by abnormal immunoglobulins (Ig) in serum, precipitating at temperatures below 37 °C. Current classification categorizes CG into three subtypes (types I, II, and III) based on Ig clonality. The features distinguishing patients with CG based on their etiology remain unidentified.

Rituximab in the treatment of progressive interstitial lung disease associated with the antisynthetase syndrome.

Objective: To assess the real-world, long-term effectiveness of rituximab (RTX) as a rescue therapy in patients with antisynthetase syndrome and progressive interstitial lung disease (ASS-ILD).

Methods: Multicentre observational retrospective longitudinal study of a cohort of patients with ASS-ILD that started treatment with RTX due to recurrent or ongoing progressive ILD despite therapy with glucocorticoids and immunosuppressants.

Results: Twenty-eight patients were analyzed.

Diagnostic Value of Krebs von den Lungen (KL-6) for Interstitial Lung Disease: A European Prospective Cohort.

Introduction: Krebs von den Lungen 6 (KL-6) is a mucin-1 glycoprotein produced by type II pneumocytes. High levels of KL-6 in blood may be found in patients with lung fibrosis. In Asia this biomarker is used for diagnosis and prognosis in interstitial lung diseases (ILD).

Prognostic significance of lymphocytic foci composition in minor salivary gland biopsies for severe disease flare and severity in Sjögren's syndrome: a 3-year follow-up cohort study.

Introduction: This was an ambispective cohort study evaluating the prognostic significance of lymphocytic foci and its lymphoid composition in minor salivary gland biopsy (MSGB) for short-term disease flare and severity in Sjögren's syndrome (SS).

Methods: The inclusion criteria comprised individuals meeting the ACR/EULAR 2016 criteria who underwent MSGB with an infiltration of more than 50 lymphocytes and received clinical diagnosis between September 2017 and December 2018. Patients with inadequate biopsy samples were excluded.

Systematic Review of Systemic Corticosteroids for Treatment of Organizing Pneumonia.

Introduction: Regardless corticosteroids are recommended for the treatment of organizing pneumonia there is limited evidence supporting this practice. Thus, we performed a systematic review of the literature on systemic corticosteroid treatment for organizing pneumonia.

Methods: A search was implemented in the PubMed database (Medline) for articles published in the last 20 years.

Impaired Regulation by IL-35 in Systemic Sclerosis.

This study investigated the role of IL-35 in systemic sclerosis (SSc) patients, focusing on CD4+ T cell response and immunomodulatory cytokine production. By comparing the cytokine levels in healthy donors (HD) and SSc patients using ELISAs, we found a significantly lower plasma IL-35 concentration in the SSc patients (52.1 ± 5.

SER-SEPAR recommendations for the management of rheumatoid arthritis-related interstitial lung disease. Part 1: Epidemiology, risk factors and prognosis.

Objective: To develop multidisciplinary recommendations to improve the management of rheumatoid arthritis-related interstitial lung disease (RA-ILD).

Methods: Clinical research questions relevant to the objective of the document were identified by a panel of rheumatologists and pneumologists selected based on their experience in the field. Systematic reviews of the available evidence were conducted, and evidence was graded according to the Scottish Intercollegiate Guidelines Network (SIGN) criteria.

SER-SEPAR recommendations for the management of rheumatoid arthritis-related interstitial lung disease. Part 2: Treatment.

Objective: To develop multidisciplinary recommendations to improve the management of rheumatoid arthritis-related interstitial lung disease (RA-ILD).

Methods: Clinical research questions relevant to the objective of the document were identified by a panel of rheumatologists and pneumologists selected based on their experience in the field. Systematic reviews of the available evidence were conducted, and evidence was graded according to the Scottish Intercollegiate Guidelines Network (SIGN) criteria.

Krebs von den Lungen-6 glycoprotein circulating levels are not useful as prognostic marker in COVID-19 pneumonia: A large prospective cohort study.

Coronavirus disease 2019 (COVID-19) has rapidly expanded worldwide. Currently, there are no biomarkers to predict respiratory worsening in patients with mild to moderate COVID-19 pneumonia. Small studies explored the use of Krebs von de Lungen-6 circulating serum levels (sKL-6) as a prognostic biomarker of the worsening of COVID-19 pneumonia.

Management of progressive pulmonary fibrosis associated with connective tissue disease.

Introduction: Fibrotic interstitial lung disease (ILD) is a frequent and severe complication of connective tissue disease (CTD).

Areas Covered: In this narrative review, we update the most relevant differential characteristics of fibrotic ILD associated with CTD (CTD-ILD) and propose a diagnostic and therapeutic approach based on a review of the articles published between 2002 and 2022 through PubMed.

Expert Opinion: The subset of ILD, mainly the radiological/histological pattern and the degree of fibrotic component, usually determines the prognosis and therapeutic strategy for these patients.

A multi-criteria decision analysis on the value of nintedanib for interstitial lung diseases.

Objectives: Our aim was to assess the value of nintedanib for non-idiopathic progressive fibrosing interstitial lung disease (non-IPF PF-ILD) and systemic sclerosis-associated ILD (SSc-ILD) in the Spanish context, using a multi-criteria decision analysis (MCDA).

Methods: Following an adaptation of the Evidence and Value: Impact on DEcision Making (EVIDEM) MCDA methodology, the estimated value of nintedanib was obtained by means of an additive linear model that combined individual weights (100-points distribution) of criteria with the individual scoring of nintedanib in each criterion for every indication, assigned by a multidisciplinary committee of twelve clinicians, patients, pharmacists, and decision-makers. To assess the reproducibility, an alternative weighting method was applied, as well as a re-test of weights and scores at a different moment of time.