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BMC Pulmonary Medicine Mar 2024IgG4-related disease is a systemic fibroinflammatory disease that is mainly seen in older men, and involves multiple organs, such as the pancreas and lungs. However, 75%...
BACKGROUND
IgG4-related disease is a systemic fibroinflammatory disease that is mainly seen in older men, and involves multiple organs, such as the pancreas and lungs. However, 75% of patients with IgG4-related lung disease are asymptomatic (if they are symptomatic, they mainly complain of nasal congestion, rhinorrhoea, chest pain, and cough) and are incidentally diagnosed through chest computed tomograph. Although, nodules in the airway and bronchial wall thickening are criteria for diagnosis, it is important that nodules have been reported in peripheral airways in several cases and rarely in the central airway.
CASE PRESENTATION
A 74-year-old woman previously diagnosed with Mikulicz's disease presented with swelling of the eyelid margin on both sides and visual disturbances. Computed tomography revealed extensive multiple nodules and mucosal oedema of the trachea and both bronchi. On flexible bronchoscopy under local anaesthesia, extensive lesions were observed from the middle of the trachea to the carina, extending into both segmental bronchi. The nodules were continuous with the normal respiratory tract mucosa, and the surfaces were smooth with minimal neovascularisation. Due to the solid nature of the lesion, obtaining an adequate amount of specimen was challenging. Therefore, we used a 1.9 mm cryoprobe under intubation, resulting in minimal bleeding. Subsequently, the patient was diagnosed with IgG4-related lung disease.
CONCLUSIONS
The present case is very rare because of the presence of multiple nodules, severe mucosal edema of the central airway and the absence of mediastinal lymphadenopathy, ground glass nodules, and lung masses. Therefore, it is important to consider differential diagnoses. Thus, we emphasise the importance of endobronchial cryobiopsy for obtaining an adequate number of tissue specimens in such cases to establish a definitive pathological diagnosis.
Topics: Male; Female; Humans; Aged; Trachea; Immunoglobulin G4-Related Disease; Bronchi; Edema; Lung Diseases; Immunoglobulin G
PubMed: 38443819
DOI: 10.1186/s12890-024-02926-w -
Colorectal Disease : the Official... Apr 2024
Topics: Humans; Constriction, Pathologic; Crohn Disease; Digestive System Surgical Procedures; Ileal Diseases; Ileum; Intestinal Obstruction; Jejunal Diseases; Jejunum; Organ Sparing Treatments
PubMed: 38331585
DOI: 10.1111/codi.16911 -
Advances in Clinical and Experimental... Jan 2024Percutaneous treatment of calcified coronary lesions is still a challenge in modern interventional cardiology practice. Coronary angiography is limited to the precise... (Review)
Review
Percutaneous treatment of calcified coronary lesions is still a challenge in modern interventional cardiology practice. Coronary angiography is limited to the precise and quantitative assessment of calcium in coronary arteries. Intracoronary imaging (ICI) modalities, including optical coherence tomography (OCT) and intravascular ultrasound (IVUS), produce a very detailed image of calcifications and could help in proper percutaneous treatment. Intracoronary imaging indicates the need to use additional tools and improves the final effect of an intervention. Drawing on the already published literature, the authors focused on the qualification of patients to the procedure, conduct and result of interventional procedures involving calcified lesions supported by ICI. The article shows the advantages and disadvantages of both ICI methods in general and especially in calcified lesions. Currently available tools dedicated to dealing with coronary calcium and helping to meet optimal stent implantation criteria are also described. This article reviews the data on ICI implementation in daily clinical practice to improve the results of percutaneous interventions, and indicates further directions.
PubMed: 38235993
DOI: 10.17219/acem/175273 -
Journal of Clinical Rheumatology :... Mar 2024The 2019 American College of Rheumatology/European League Against Rheumatism Classification Criteria (2019 AECC) for IgG4-related disease (IgG4-RD) is considered a...
BACKGROUND/OBJECTIVE
The 2019 American College of Rheumatology/European League Against Rheumatism Classification Criteria (2019 AECC) for IgG4-related disease (IgG4-RD) is considered a significant advancement in the study of this condition. Most studies evaluating their performance have focused on White and Asian patients, leaving a knowledge gap regarding Latin American populations. Therefore, this study aimed to assess the performance of the 2019 AECC for IgG4-RD in a cohort of Latin American patients.
METHODS
A multicenter medical records review study was conducted, involving centers from Argentina, Chile, Mexico, Peru, and Uruguay. Data on IgG4-RD patients and mimicker conditions were collected through a standardized online form. The criterion standard for diagnosing IgG4-RD was based on the fulfillment of the Comprehensive Diagnostic Criteria for IgG4-RD and/or the Consensus Statement on Pathology. The 2019 AECC was retrospectively applied.
RESULTS
We included 300 patients, with 180 (60%) having IgG4-RD and 120 (40%) having mimicker conditions. The 2019 AECC had a sensitivity of 66.7% and a specificity of 100%. Sensitivity increased to 73.3% when disease-specific autoantibody items were removed, without affecting specificity. The true-positive cases had more involved organs, a higher availability of biopsy results, and were more likely to belong to the Mikulicz/systemic and proliferative phenotypes.
CONCLUSIONS
The use of the 2019 AECC for IgG4-RD in a Latin American population confirms its high specificity in excluding those without the disease. The presence of concomitant autoimmune diseases and clinically nonsignificant disease-specific autoantibodies excludes a significant number of patients from fulfilling the criteria.
Topics: Humans; United States; Rheumatology; Immunoglobulin G4-Related Disease; Retrospective Studies; Latin America; Rheumatic Diseases; Autoantibodies
PubMed: 38206921
DOI: 10.1097/RHU.0000000000002052 -
The American Journal of Medicine Apr 2024
Topics: Humans; Mikulicz' Disease
PubMed: 38163535
DOI: 10.1016/j.amjmed.2023.12.023 -
Quantitative Imaging in Medicine and... Dec 2023
PubMed: 38106322
DOI: 10.21037/qims-23-563 -
Global Pediatric Health 2023Rhinoscleroma is a rare benign granulomatous disease in children, the pathogen of which is Klebsiella rhinoscleromatis. Rhinoscleroma almost always affects the...
Rhinoscleroma is a rare benign granulomatous disease in children, the pathogen of which is Klebsiella rhinoscleromatis. Rhinoscleroma almost always affects the respiratory tract, the nasal cavities are the most common site, but can also be found over the entire extent of the respiratory tree, clinically the patient often presents with nasal obstruction with other non-specific signs, Histology is the basis of the diagnosis of rhinoscleroma. Early and appropriate medical treatment improves the prognosis and prevents progression to disabling sequelae. We present the case of a 9-year-old child who presented to the pediatric emergency department with a 2-month history of nasal obstruction unresponsive to medical treatment (Oxymetazoline). This was accompanied by mild nasal swelling and intermittent episodes of epistaxis. A CT scan of the paranasal sinuses was performed, ruling out a malignant tumor in the nasal cavity due to the absence of bone lysis. A nasal cavity biopsy with histopathological examination was conducted, confirming the diagnosis of rhinoscleroma. This was supported by the presence of an infiltrate rich in histiocytes and plasma cells, along with the identification of Russell bodies during PAS (Periodic Acid Schiff) staining. The patient underwent a medical treatment regimen involving a sulfamide-based antibiotic therapy (trimethoprim/sulfamethoxazole) for 8 weeks, along with a short-term corticosteroid therapy at a dose of 1 mg/kg/24 hours. At the first follow-up after 1 month, there was a noticeable reduction in nasal swelling and an improvement in nasal obstruction. A second follow-up 3 months later showed favorable results, with complete disappearance of both the swelling and nasal obstruction. Considering the possibility of recurrence, an annual follow-up was decided upon.
PubMed: 38024464
DOI: 10.1177/2333794X231210673 -
Clinical and Experimental Rheumatology Dec 2023Recent studies have implicated cytotoxic CD4 and CD8 T cells in primary Sjögren's syndrome (pSS) and IgG4-related disease (IgG4-RD), but their association with immune...
OBJECTIVES
Recent studies have implicated cytotoxic CD4 and CD8 T cells in primary Sjögren's syndrome (pSS) and IgG4-related disease (IgG4-RD), but their association with immune aging and organ-specific clinical features remain unclear. CX3CR1 is expressed on cytotoxic CD4 and CD8 T cells. The aim of this study was to determine associations of peripheral CX3CR1+CD4 and CX3CR1+CD8 T cells with aging and clinical features.
METHODS
Whole blood samples were freshly obtained from consecutive patients with active, treatment-naïve pSS (n=57), IgG4-RD (n=54), and healthy individuals (n=40) and analysed by flow cytometry for CX3CR1+CD4 and CX3CR1+CD8 proportions. Associations of those T cells with aging and clinical features were determined.
RESULTS
CX3CR1+CD4 and CX3CR1+CD8 T cells selectively expressed perforin and granzyme B. Proportions of CX3CR1+CD4 and CX3CR1+CD8 T cells were significantly higher in pSS and IgG4-RD than in healthy individuals. Higher proportions of CX3CR1+CD8 T cells were associated with aging in pSS and IgG4-RD but not in healthy individuals. Sex differences were not associated with proportions of CX3CR1+CD8 T cells. Furthermore, patients with pSS with interstitial lung disease showed higher proportions of CX3CR1+CD8 T cells than those without interstitial lung disease. IgG4-RD patients with retroperitoneal fibrosis and/or aortitis exhibited higher proportions of CX3CR1+CD8 T cells compared with those with Mikulicz's disease. Moreover, proportions of CX3CR1+CD8 T cells were decreased following glucocorticoid treatment in paralleled with clinical improvements in IgG4-RD.
CONCLUSIONS
CX3CR1+CD8 T cells might be involved in immune aging and distinct clinical phenotypes of patients with pSS or IgG4-RD.
Topics: Humans; Male; Female; Sjogren's Syndrome; Immunoglobulin G4-Related Disease; CD8-Positive T-Lymphocytes; Aging; Lung Diseases, Interstitial; CX3C Chemokine Receptor 1
PubMed: 37812481
DOI: 10.55563/clinexprheumatol/kfsd65 -
Cells Sep 2023Prolactin-inducible protein (PIP), also referred to as gross cystic disease fluid protein 15 (GCDFP-15), has been a trending topic in recent years due to its potential... (Review)
Review
Prolactin-inducible protein (PIP), also referred to as gross cystic disease fluid protein 15 (GCDFP-15), has been a trending topic in recent years due to its potential role as a specific marker in breast cancer. PIP binds to aquaporin-5 (AQP5), CD4, actin, fibrinogen, β-tubulin, serum albumin, hydroxyapatite, zinc α2-glycoprotein, and the Fc fragment of IgGs, and the expression of PIP has been demonstrated to be modulated by various cytokines, including IL4/13, IL1, and IL6. PIP gene expression has been extensively studied due to its captivating nature. It is influenced by various factors, with androgens, progesterone, glucocorticosteroids, prolactin, and growth hormone enhancing its expression while estrogens suppress it. The regulatory mechanisms involve important proteins such as STAT5A, STAT5B, Runx2, and androgen receptor, which collaborate to enhance PIP gene transcription and protein production. The expression level of PIP in breast cancer is dependent on the tumor stage and subtype. Higher expression is observed in early-stage tumors of the luminal A subtype, while lower expression is associated with luminal B, basal-like, and triple-negative subtypes, which have a poorer prognosis. PIP expression is also correlated with apocrine differentiation, hormone receptor positivity, and longer metastasis-free survival. PIP plays a role in supporting the immune system's antitumor response during the early stages of breast cancer development. However, as cancer progresses, the protective role of PIP may become less effective or diminished. In this work, we summarized the clinical significance of the PIP molecule in breast cancer and its potential role as a new candidate for cell-based therapies.
PubMed: 37759471
DOI: 10.3390/cells12182252 -
The British Journal of Ophthalmology Jun 2024Accurate risk stratification of uveal melanoma (UM) patients is important for determining the interval and frequency of surveillance. Loss of BAP1 expression has been...
BACKGROUND
Accurate risk stratification of uveal melanoma (UM) patients is important for determining the interval and frequency of surveillance. Loss of BAP1 expression has been shown to be strongly associated with UM-related death and metastasis.
METHODS
In this study of 164 enucleated UMs, we assessed the prognostic role of preferentially expressed antigen in melanoma (PRAME) expression and Ki67 proliferation index measured by digital quantitation using QuPath programme in patients with BAP1-positive and BAP1-loss UMs.
RESULTS
In univariate analyses with log-rank tests and Kaplan-Meier curves, PRAME further stratified only overall survival (OS) in BAP1-positive and BAP1-loss tumour groups. However, Ki67 further stratified both OS and disease-free survival (DFS) in BAP1-positive and BAP1-loss tumour groups. In multivariate analyses, Ki67 percentage and BAP1 were independent survival predictors for both OS and DFS, whereas PRAME was not a significant covariate. In model comparisons, combined Ki67 and BAP1 performed better than combined PRAME and BAP1 in risk-stratifying patients for both OS and DFS. Ki67 was better than PRAME in risk stratification of BAP1-positive UMs. Low Ki67 index correlated with significantly prolonged DFS in BAP1-loss UMs.
CONCLUSION
A panel of Ki67 and BAP1 could be a helpful risk stratification strategy for UM.
Topics: Humans; Ubiquitin Thiolesterase; Tumor Suppressor Proteins; Uveal Neoplasms; Melanoma; Ki-67 Antigen; Male; Female; Middle Aged; Biomarkers, Tumor; Antigens, Neoplasm; Aged; Risk Assessment; Adult; Prognosis; Retrospective Studies; Aged, 80 and over; Survival Rate
PubMed: 37734766
DOI: 10.1136/bjo-2023-323816