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Clinical and Experimental Rheumatology Jun 2024To evaluate whether the grade of IgG4+ plasma cell infiltration in biopsies is associated with clinical or serologic outcomes in IgG4-RD.
OBJECTIVES
To evaluate whether the grade of IgG4+ plasma cell infiltration in biopsies is associated with clinical or serologic outcomes in IgG4-RD.
METHODS
We included 57 patients with biopsy proven IgG4-RD according to the Comprehensive Diagnostic Criteria and/or the 2019 ACR/EULAR Classification Criteria. We collected histological, clinical (disease duration, phenotype, remission and relapses) and serological variables.
RESULTS
29 (50.9%) patients were men, mean age 49.9 years, with a median disease duration of 22 months. The distribution among clinical phenotypes were 14% pancreato-hepato-biliary, 12.3% retroperitoneal/aortic, 29.8% head and neck-limited, 29.8% Mikulicz/systemic and 14% undefined. Thirty-nine patients had a proliferative and 18 a fibrotic phenotype. Most biopsies were from lacrimal gland, lymph node, pancreas, orbit, kidney, retroperitoneum and thyroid gland. Thirty-nine (68.4%) patients had <100 IgG4+ plasma cells/HPF and 18 (31.6%) ≥100 IgG4+ plasma cells/HPF. Patients with ≥100 IgG4+ plasma cells/HPF were more likely to belong to the pancreato-hepato-biliary and the proliferative phenotypes, had fewer relapses and a higher remission rate. On multivariate analysis, the OR for remission at last follow-up was 6.7, 95% CI 1.1-4.42, p=0.03. The log-rank test showed a difference in relapse-free survival between the two groups (HR 2.6, 95% CI 1.2-5.6, p=0.01). According to the ROC analysis, patients with more than 61 IgG4+ plasma cells were less likely to relapse.
CONCLUSIONS
A count of ≥100 IgG4+ plasma cells/HPF may identify patients with a proliferative phenotype, fewer relapses and a higher remission rate.
PubMed: 38910564
DOI: 10.55563/clinexprheumatol/cniut0 -
BMC Geriatrics Jun 2024IgG4-related diseases are very uncommon, and its diagnosis and treatment are complicated as it encompasses multiple disciplines.
BACKGROUND
IgG4-related diseases are very uncommon, and its diagnosis and treatment are complicated as it encompasses multiple disciplines.
CASE PRESENTATION
A 77-year-old woman was admitted with a jaw mass and nausea and vomiting. Laboratory tests showed elevated serum IgG4, pituitary MRI suggested thickening of the pituitary stalk, and head and neck CT suggested orbital and mandibular masses. Patients with mandibular mass were diagnosed with Mikulicz's disease with IgG4-related hypophysitis. We found no other evidence of causing thickening of the pituitary stalk. She was given oral prednisolone 30 mg daily, and her nausea and vomiting improved significantly, and the mandibular and ocular masses decreased in size.
CONCLUSION
Mikulicz's disease combined with IgG4-related hypophysitis is a rare case of IgG4-RD in elderly women. IgG4-RD is one of the causes of head and neck exocrine gland mass and pituitary stalk thickening in the elderly.
Topics: Humans; Aged; Female; Mikulicz' Disease; Immunoglobulin G4-Related Disease; Autoimmune Hypophysitis; Immunoglobulin G; Prednisolone; Magnetic Resonance Imaging
PubMed: 38880897
DOI: 10.1186/s12877-024-05142-7 -
World Journal of Orthopedics May 2024Unicompartmental knee arthroplasty (UKA) and high tibial osteotomy (HTO) are well-established operative interventions in the treatment of knee osteoarthritis. However,...
BACKGROUND
Unicompartmental knee arthroplasty (UKA) and high tibial osteotomy (HTO) are well-established operative interventions in the treatment of knee osteoarthritis. However, which intervention is more beneficial to patients with knee osteoarthritis remains unknown and a topic of much debate. Simultaneously, there is a paucity of research assessing the relationship between radiographic parameters of knee joint alignment and patient-reported clinical outcomes, preoperatively and following HTO or UKA.
AIM
To compare UKAs and HTOs as interventions for medial-compartment knee osteoarthritis: Examining differences in clinical outcome and investigating the relationship of joint alignment with respect to this.
METHODS
This longitudinal observational study assessed a total of 42 patients that had undergone UKA ( = 23) and HTO ( = 19) to treat medial compartment knee osteoarthritis. Patient-reported outcome measures (PROMs) were collected to evaluate clinical outcome. These included two disease-specific (Knee Injury and Osteoarthritis Outcome Score, Oxford Knee Score) and two generic (EQ-5D-5L, Short Form-12) PROMs. The radiographic parameters of knee alignment assessed were the: Hip-knee-ankle angle, mechanical axis deviation and angle of Mikulicz line.
RESULTS
Statistical analyses demonstrated significant ( < 0.001), preoperative to postoperative, improvements in the PROM scores of both groups. There were, however, no significant inter-group differences in the postoperative PROM scores of the UKA and HTO group. Several significant correlations associated a more distolaterally angled Mikulicz line with worse knee function and overall health preoperatively ( < 0.05). Postoperatively, two clusters of significant correlations were observed between the disease-specific PROM scores and knee joint alignment parameters (hip-knee-ankle angle, mechanical axis deviation) within the HTO group; yet no such associations were observed within the UKA group.
CONCLUSION
UKAs and HTOs are both efficacious operations that provide a comparable degree of clinical benefit to patients with medial compartment knee osteoarthritis. Clinical outcome has a limited association with radiographic parameters of knee joint alignment postoperatively; however, a more distolaterally angled Mikulicz line appears associated with worse knee function/health-related quality of life preoperatively.
PubMed: 38835690
DOI: 10.5312/wjo.v15.i5.444 -
Clinical Case Reports Jun 2024This case highlights the importance of a definite diagnosis of an IgG4-related chronic sclerosing duodenitis based on histological and radiological findings to rule out...
KEY CLINICAL MESSAGE
This case highlights the importance of a definite diagnosis of an IgG4-related chronic sclerosing duodenitis based on histological and radiological findings to rule out any malignancy in the mass. While dealing with patients having concentric duodenal thickening resulting in stricture formation, one should think of inflammatory etiology as well. IgG4-related disease is one of these inflammatory disorders where we see soft tissue thickening without a large mass or any associated lymphadenopathy as in our case.
ABSTRACT
Immunoglobulin G4-related disease (IgG4-RD) is distinguished as an infiltration of IgG-4-positive plasmacytes involving inflammatory lesions across multiple organs which is accompanied by raised IgG4 levels in the serum. Several inflammatory disorders are recognized as part of the IgG4-RD family based on shared histopathological features, which include Mikulicz's disease, chronic sclerosing sialadenitis, or Riedel's thyroiditis. Our case highlights a distinctive presentation of IgG4-related diseases; a 58-year-old man presenting with duodenal stricture highly suspicious of a duodenal mass/ampullary mass later found to be due to IgG4-related sclerosing duodenitis with negative malignancy on biopsy. We present the diagnostic challenges faced and relevant findings noted.
PubMed: 38808195
DOI: 10.1002/ccr3.8980 -
Malaysian Orthopaedic Journal Mar 2024The present inquiry seeks to investigate whether the current regimens of QSEs (Quadriceps-Strengthening Exercises) aggravate the disease while mitigating symptoms.
Is Quadriceps-Strengthening Exercises (QSE) in Medial-Compartment Knee Osteoarthritis with Neutral and Varus Malalignment a Paradox? - A Risk-Appraisal of Strength-Training on Disease Progression.
INTRODUCTION
The present inquiry seeks to investigate whether the current regimens of QSEs (Quadriceps-Strengthening Exercises) aggravate the disease while mitigating symptoms.
MATERIALS AND METHODS
A comparative study was conducted on 32 patients with medial compartment osteoarthritis of knees. While the neutral group of 16 patients was constituted of those with an anatomical-lateral-femoro-tibial-angle (aFTA) 176-180º, varus group comprised an equal number of patients with an aFTA >180º. A home-based 12-week strength-training program involving weekly visits to hospital for supervised sessions was administered. The outcome measures were visual-analog-scale (VAS), medial patello-femoral joint tenderness (MPFJT), time-up-and-go-test (TUGT), stair-climb test, step test, WOMAC, IKDC scores, aFTA, hip-knee-ankle (HKA) angle, lateral-tibio-femoral-joint-separation (LTFJS), and horizontal-distance-from-centre-of-knee-to-Mikulicz-line.
RESULTS
There was a significant increase in quadriceps strength (p<0.01) in both groups. Values for neutral group with VAS score (p=0.01), MPFJT (p=0.01), TUGT (p=0.01), timing of the stair climb test (p=0.01), WOMAC (p<0.01), and IKDC (p=0.03) were better compared to varus group with VAS score (p=0.13), MPFJT (p=0.03), TUGT (p=0.90), timing of stair climb test (p=0.68), WOMAC (p<0.02), and IKDC (p=0.05). Varus group also showed an increase in aFTA and LTFJS in 12 patients, increase in HKA in 11, and increase in horizontal distance from the centre of knee to the Mikulicz line in 7 patients.
CONCLUSION
The present study brings to the fore the paradoxical role played by QSEs in management of medial knee OA. While there is a radiological progression of the disease in both neutral and varus mal-aligned knees more so in the latter than the former.
PubMed: 38638654
DOI: 10.5704/MOJ.2403.010 -
Zeitschrift Fur Rheumatologie Apr 2024Many aspects of IgG4-related diseases were initially described during the late 19th and early 20th century. A variety of clinical presentations caused by this common...
Many aspects of IgG4-related diseases were initially described during the late 19th and early 20th century. A variety of clinical presentations caused by this common pathology have been named after the researchers who first described the disorders, such as Mikulicz, Küttner, Riedel or Ormond. However, the initial description of retroperitoneal fibrosis dates back to even 50 years earlier, when in 1846, the Prussian private practitioner Raphael Jakob Kosch described a hitherto unknown constellation of symptoms and pathological findings in a famous patient. This celebrity was the mathematician and astronomer Friedrich Wilhelm Bessel, a close friend of Alexander von Humboldt and Carl Friedrich Gauss.
PubMed: 38634904
DOI: 10.1007/s00393-024-01502-1 -
Journal of the American College of... Jun 2024Empagliflozin reduces the risk of heart failure (HF) hospitalizations but not all-cause mortality when started within 14 days of acute myocardial infarction (AMI). (Randomized Controlled Trial)
Randomized Controlled Trial
BACKGROUND
Empagliflozin reduces the risk of heart failure (HF) hospitalizations but not all-cause mortality when started within 14 days of acute myocardial infarction (AMI).
OBJECTIVES
This study sought to evaluate the association of left ventricular ejection fraction (LVEF), congestion, or both, with outcomes and the impact of empagliflozin in reducing HF risk post-AMI.
METHODS
In the EMPACT-MI (Trial to Evaluate the Effect of Empagliflozin on Hospitalization for Heart Failure and Mortality in Patients with Acute Myocardial Infarction) trial, patients were randomized within 14 days of an AMI complicated by either newly reduced LVEF<45%, congestion, or both, to empagliflozin (10 mg daily) or placebo and were followed up for a median of 17.9 months.
RESULTS
Among 6,522 patients, the mean baseline LVEF was 41 ± 9%; 2,648 patients (40.6%) presented with LVEF <45% alone, 1,483 (22.7%) presented with congestion alone, and 2,181 (33.4%) presented with both. Among patients in the placebo arm of the trial, multivariable adjusted risk for each 10-point reduction in LVEF included all-cause death or HF hospitalization (HR: 1.49; 95% CI: 1.31-1.69; P < 0.0001), first HF hospitalization (HR: 1.64; 95% CI: 1.37-1.96; P < 0.0001), and total HF hospitalizations (rate ratio [RR]: 1.89; 95% CI: 1.51-2.36; P < 0.0001). The presence of congestion was also associated with a significantly higher risk for each of these outcomes (HR: 1.52, 1.94, and RR: 2.03, respectively). Empagliflozin reduced the risk for first (HR: 0.77; 95% CI: 0.60-0.98) and total (RR: 0.67; 95% CI: 0.50-0.89) HF hospitalizations, irrespective of LVEF or congestion, or both. The safety profile of empagliflozin was consistent across baseline LVEF and irrespective of congestion status.
CONCLUSIONS
In patients with AMI, the severity of left ventricular dysfunction and the presence of congestion was associated with worse outcomes. Empagliflozin reduced first and total HF hospitalizations across the range of LVEF with and without congestion. (Trial to Evaluate the Effect of Empagliflozin on Hospitalization for Heart Failure and Mortality in Patients with Acute Myocardial Infarction [EMPACT-MI]; NCT04509674).
Topics: Humans; Benzhydryl Compounds; Glucosides; Male; Female; Myocardial Infarction; Heart Failure; Middle Aged; Aged; Sodium-Glucose Transporter 2 Inhibitors; Ventricular Function, Left; Stroke Volume; Hospitalization; Double-Blind Method; Follow-Up Studies
PubMed: 38588929
DOI: 10.1016/j.jacc.2024.03.405 -
The New England Journal of Medicine Apr 2024Empagliflozin improves cardiovascular outcomes in patients with heart failure, patients with type 2 diabetes who are at high cardiovascular risk, and patients with... (Randomized Controlled Trial)
Randomized Controlled Trial
BACKGROUND
Empagliflozin improves cardiovascular outcomes in patients with heart failure, patients with type 2 diabetes who are at high cardiovascular risk, and patients with chronic kidney disease. The safety and efficacy of empagliflozin in patients who have had acute myocardial infarction are unknown.
METHODS
In this event-driven, double-blind, randomized, placebo-controlled trial, we assigned, in a 1:1 ratio, patients who had been hospitalized for acute myocardial infarction and were at risk for heart failure to receive empagliflozin at a dose of 10 mg daily or placebo in addition to standard care within 14 days after admission. The primary end point was a composite of hospitalization for heart failure or death from any cause as assessed in a time-to-first-event analysis.
RESULTS
A total of 3260 patients were assigned to receive empagliflozin and 3262 to receive placebo. During a median follow-up of 17.9 months, a first hospitalization for heart failure or death from any cause occurred in 267 patients (8.2%) in the empagliflozin group and in 298 patients (9.1%) in the placebo group, with incidence rates of 5.9 and 6.6 events, respectively, per 100 patient-years (hazard ratio, 0.90; 95% confidence interval [CI], 0.76 to 1.06; P = 0.21). With respect to the individual components of the primary end point, a first hospitalization for heart failure occurred in 118 patients (3.6%) in the empagliflozin group and in 153 patients (4.7%) in the placebo group (hazard ratio, 0.77; 95% CI, 0.60 to 0.98), and death from any cause occurred in 169 (5.2%) and 178 (5.5%), respectively (hazard ratio, 0.96; 95% CI, 0.78 to 1.19). Adverse events were consistent with the known safety profile of empagliflozin and were similar in the two trial groups.
CONCLUSIONS
Among patients at increased risk for heart failure after acute myocardial infarction, treatment with empagliflozin did not lead to a significantly lower risk of a first hospitalization for heart failure or death from any cause than placebo. (Funded by Boehringer Ingelheim and Eli Lilly; EMPACT-MI ClinicalTrials.gov number, NCT04509674.).
Topics: Aged; Female; Humans; Male; Middle Aged; Benzhydryl Compounds; Double-Blind Method; Follow-Up Studies; Glucosides; Heart Failure; Hospitalization; Kaplan-Meier Estimate; Myocardial Infarction; Sodium-Glucose Transporter 2 Inhibitors; Treatment Outcome; Heart Disease Risk Factors
PubMed: 38587237
DOI: 10.1056/NEJMoa2314051 -
Circulation May 2024Empagliflozin reduces the risk of heart failure (HF) events in patients with type 2 diabetes at high cardiovascular risk, chronic kidney disease, or prevalent HF... (Randomized Controlled Trial)
Randomized Controlled Trial
BACKGROUND
Empagliflozin reduces the risk of heart failure (HF) events in patients with type 2 diabetes at high cardiovascular risk, chronic kidney disease, or prevalent HF irrespective of ejection fraction. Whereas the EMPACT-MI trial (Effect of Empagliflozin on Hospitalization for Heart Failure and Mortality in Patients With Acute Myocardial Infarction) showed that empagliflozin does not reduce the risk of the composite of hospitalization for HF and all-cause death, the effect of empagliflozin on first and recurrent HF events after myocardial infarction is unknown.
METHODS
EMPACT-MI was a double-blind, randomized, placebo-controlled, event-driven trial that randomized 6522 patients hospitalized for acute myocardial infarction at risk for HF on the basis of newly developed left ventricular ejection fraction of <45% or signs or symptoms of congestion to receive empagliflozin 10 mg daily or placebo within 14 days of admission. In prespecified secondary analyses, treatment groups were analyzed for HF outcomes.
RESULTS
Over a median follow-up of 17.9 months, the risk for first HF hospitalization and total HF hospitalizations was significantly lower in the empagliflozin compared with the placebo group (118 [3.6%] versus 153 [4.7%] patients with events; hazard ratio, 0.77 [95% CI, 0.60, 0.98]; =0.031, for first HF hospitalization; 148 versus 207 events; rate ratio, 0.67 [95% CI, 0.51, 0.89]; =0.006, for total HF hospitalizations). Subgroup analysis showed consistency of empagliflozin benefit across clinically relevant patient subgroups for first and total HF hospitalizations. The need for new use of diuretics, renin-angiotensin modulators, or mineralocorticoid receptor antagonists after discharge was less in patients randomized to empagliflozin versus placebo (all <0.05).
CONCLUSIONS
Empagliflozin reduced the risk of HF in patients with left ventricular dysfunction or congestion after acute myocardial infarction.
REGISTRATION
URL: https://www.clinicaltrials.gov; Unique identifier: NCT04509674.
Topics: Humans; Glucosides; Benzhydryl Compounds; Heart Failure; Male; Female; Myocardial Infarction; Aged; Middle Aged; Double-Blind Method; Sodium-Glucose Transporter 2 Inhibitors; Hospitalization; Treatment Outcome; Stroke Volume
PubMed: 38581389
DOI: 10.1161/CIRCULATIONAHA.124.069217 -
Cancers Feb 2024The role of immune checkpoint inhibitors (ICIs; anti-PD1) in the treatment of childhood cancers is still evolving. The aim of this nationwide retrospective study was to...
BACKGROUND/AIM
The role of immune checkpoint inhibitors (ICIs; anti-PD1) in the treatment of childhood cancers is still evolving. The aim of this nationwide retrospective study was to assess the safety and effectiveness of ICIs used in a group of 42 patients, with a median age of 13.6 years, with various types of advanced malignancies treated in pediatric oncology centers in Poland between 2015 and 2023.
RESULTS
The indications for treatment with anti-PD1 were as follows: Hodgkin lymphoma (11); malignant skin melanoma (9); neuroblastoma (8); and other malignancies (14). At the end of follow-up, complete remission (CR) was observed in 37.7% (15/42) of children and disease stabilization in 9.5% (4/42), with a mean survival 3.6 (95% CI = 2.6-4.6) years. The best survival (OS = 1.0) was observed in the group of patients with Hodgkin lymphoma. For malignant melanoma of the skin, neuroblastoma, and other rare malignancies, the estimated 3-year OS values were, respectively, 0.78, 0.33, and 0.25 ( = 0.002). The best progression-free survival value (0.78) was observed in the group with malignant melanoma. Significantly better effects of immunotherapy were confirmed in patients ≥ 14 years of age and good overall performance ECOG status. Severe adverse events were observed in 30.9% (13/42) patients.
PubMed: 38473329
DOI: 10.3390/cancers16050968