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Reumatologia 2020According to a new concept for the classification and division of autoimmune diseases, Mikulicz's disease and Küttner's tumor belong to immunoglobulin G4-related... (Review)
Review
According to a new concept for the classification and division of autoimmune diseases, Mikulicz's disease and Küttner's tumor belong to immunoglobulin G4-related diseases (IgG4-RD) and fulfil their diagnostic criteria. The aim of this study was to summarize the new classification concepts of IgG4-RD in the head and neck area and to review their clinical, histopathological and serologic criteria and the methods used in the diagnostic workup with respect to their advantages, limitations and differentiative value. The PubMed, Web of Science, Google Scholar, and Scopus databases were searched for articles published between 2009 and 2019 using the following key words: IgG4-related diseases, Mikulicz's disease, Küttner's tumor, salivary glands, xerostomia. Results of the review of the literature revealed that Mikulicz's disease and Küttner's tumor fulfil the same diagnostic criteria but may manifest different clinical symptoms which determine the choice of the different diagnostic tools.
PubMed: 32921832
DOI: 10.5114/reum.2020.98437 -
Journal of Cardiovascular Translational... Jun 2021Critical leg ischemia (CLI) complicated by diabetes mellitus (DM), which is a very common and dangerous disease, represents the ultimate stage of peripheral arterial... (Randomized Controlled Trial)
Randomized Controlled Trial
Critical leg ischemia (CLI) complicated by diabetes mellitus (DM), which is a very common and dangerous disease, represents the ultimate stage of peripheral arterial disease. Patients are treated with antiplatelet drugs, statins and limb revascularization, but a significant number of patients are not candidate for revascularization. Literature shows that in such cases, gene therapy could be a perfect therapeutic option. The aim of our study was to evaluate efficacy of double vascular endothelial growth factor/hepatocyte growth factor (VEGF/HGF) gene therapy in patients with CLI complicated by DM. We observed that 90 days after administration, serum level of VEGF and ankle-brachial index increased significantly (p < 0.001) and rest pain decreased significantly compared with the control group (p < 0.002). Moreover considerable improvement in vascularization was observed in computed tomography angiography (P = 0.04). Based on the results of this study, we suggest that the therapy with pIRES/VEGF165/HGF bicistronic plasmid administration is a safe and effective method of treatment of patients with both CLI and DM. Graphical abstract.
Topics: Aged; Aged, 80 and over; Biomarkers; Blood Glucose; Critical Illness; Diabetes Mellitus, Type 2; Female; Functional Status; Genetic Therapy; Hepatocyte Growth Factor; Humans; Internal Ribosome Entry Sites; Ischemia; Male; Middle Aged; Neovascularization, Physiologic; Peripheral Arterial Disease; Plasmids; Poland; Recovery of Function; Time Factors; Treatment Outcome; Vascular Endothelial Growth Factor A
PubMed: 32875492
DOI: 10.1007/s12265-020-10066-9 -
The Indian Journal of Surgery Jun 2020The earliest available evidence attributes the discovery of droplets as a mode of transmission of disease to Carl Flügge, a German bacteriologist, a contemporary of...
The earliest available evidence attributes the discovery of droplets as a mode of transmission of disease to Carl Flügge, a German bacteriologist, a contemporary of Emil Kocher, in 1897. This finding was instrumental in the development of the gauze mask introduced by Johann von Mikulicz Radecki in the same year. A surgical mask has become an indispensable tool in the armamentarium to fight the COVID 19 pandemic. Surgical masks which were once limited to the confines of healthcare setups are now donned by the members of the general public. It has become imperative that a healthcare worker selects the right kind of respiratory protective equipment to protect himself and his patients. The surgical mask has become essential, in a way, for survival.
PubMed: 32837074
DOI: 10.1007/s12262-020-02438-x -
Cureus Aug 2020Immunoglobulin G4-related disease (IgG4-RD) is a new disease entity of rare and complex immune-mediated fibroinflammatory conditions that can affect any organ. The...
Immunoglobulin G4-related disease (IgG4-RD) is a new disease entity of rare and complex immune-mediated fibroinflammatory conditions that can affect any organ. The concomitance of IgG4 sclerosing sialadenitis and dacryoadenitis with rhinosinusitis is extremely rare. We report a case of IgG4 sclerosing sialadenitis and dacryoadenitis (Mikulicz's disease) diagnosed in a middle-aged African American man with a long-standing history of chronic rhinosinusitis who presented with progressively worsening bilateral salivary and lacrimal glands swelling. Imaging revealed pansinusitis, symmetric enlargement of the lacrimal glands, parotid glands, and submandibular glands. Serological IgG4 level was significantly elevated and the diagnosis of IgG4 sclerosing sialadenitis was confirmed by histopathology. A robust clinical response in the facial swelling and nasal manifestations was noted after the initiation of immunotherapy with corticosteroids.
PubMed: 32821631
DOI: 10.7759/cureus.9756 -
Cureus Jun 2020Chronic sclerosing sialadenitis (CSS) or Küttner tumor is an under-recognized, benign fibroinflammatory disease most commonly seen in the submandibular gland of older...
Chronic sclerosing sialadenitis (CSS) or Küttner tumor is an under-recognized, benign fibroinflammatory disease most commonly seen in the submandibular gland of older adult males. Sialolithiasis or bacterial infection was first suspected as an etiology, but CSS is now considered an immunoglobulin G4-related disease (IgG4-RD). IgG4-RD can affect almost every organ in the body, characterized by organ fibrosis with IgG4-positive plasmacytes. Numerous autoimmune-related diseases have been unified under IgG4-RD, including Mikulicz disease (MD), autoimmune pancreatitis, Reidel's thyroiditis, and others. In any organ, IgG4-RD can present similar to malignancy. Due to the ability to mimic malignancy, it is crucial to be aware of this under-recognized clinical entity. CSS is currently of broad and high clinical interest due to increased understanding, multiorgan involvement, and more clearly defined criteria. To increase awareness of this disease, we describe a rare presentation of CSS with a literature review.
PubMed: 32754389
DOI: 10.7759/cureus.8846 -
BMC Immunology Jul 2020Primary immunodeficiences (PIDs) are a group of chronic, serious disorders in which the immune response is insufficient. In consequence, it leads to an increased...
BACKGROUND
Primary immunodeficiences (PIDs) are a group of chronic, serious disorders in which the immune response is insufficient. In consequence, it leads to an increased susceptibility to infections. Up to date, there are about 350 different disorders classified in that group. There are also patients suffering from recurrent respiratory tract infections (RRTI), however that group doesn't present any abnormalities in terms of conducted immunological tests. Many factors, including medical, can have an impact on physical development of a child. Data such as birth weight and length, also weight, height, BMI during admission to the hospital were collected from 195 patients' medical histories from their hospitalization at Clinical Immunology and Paediatrics Ward of J. Gromkowski Hospital in Wrocław. Investigated groups included patients with PIDs, RRTI and a control group of healthy children. Our purpose was to evaluate the physical growth of children with PID and children with RRTI by assessment of their height and weight. All of parameters were evaluated using centile charts, suitable best for the Polish population.
RESULTS
The lowest mean birth weight and height was found among the PIDs patients group. Children with PIDs during hospitalization had statistically relevant lower mean weight than the control group and almost 18% of them had their height situated below 3rd percentile. The statistically relevant differences have been found between them and RRTI group in terms of weight, height and nutritional status. The statistically significant difference was detected between the nutritional status of PID and control group.
CONCLUSIONS
There is a higher percentage of PID patients with physical growth abnormalities in comparison to healthy children. Our findings indicate a need for further investigation of immune system irregularities and their influence on physical growth of children.
Topics: Adolescent; Body Height; Body Mass Index; Body Weight; Child; Child, Preschool; Chronic Disease; Female; Growth Disorders; Humans; Male; Primary Immunodeficiency Diseases; Recurrence; Respiratory Tract Infections
PubMed: 32677887
DOI: 10.1186/s12865-020-00372-x -
Rheumatology (Oxford, England) Jan 2021Several IgG4-related disease (IgG4-RD) phenotypes have been proposed and the first set of classification criteria have been recently created. Our objectives were to...
OBJECTIVES
Several IgG4-related disease (IgG4-RD) phenotypes have been proposed and the first set of classification criteria have been recently created. Our objectives were to assess the phenotype distribution and the performance of the classification criteria in Spanish patients as genetic and geographical differences may exist.
METHODS
We performed a cross-sectional multicentre study (Registro Español de Enfermedad Relacionada con la IgG4, REERIGG4) with nine participating centres from Spain. Patients were recruited from November 2013 to December 2018. The 2019 American College of Rheumatology/European League Against Rheumatism classification criteria (AECC) were used.
RESULTS
We included 105 patients; 88% had Caucasian ethnicity. On diagnosis, 86% met the international pathology consensus while 92% met the Japanese comprehensive criteria. The phenotype distribution was head and neck 25%, Mikulicz and systemic (MS) 20%, pancreato-hepato-biliary (PHB) 13%, retroperitoneal and aorta (RA) 26%. Sixteen per cent had an undefined phenotype. Seventy-seven per cent of the cases met the AECC. From the 24 patients not meeting the AECC, 33% met exclusion criteria, and 67% did not get a score ≥20 points. Incomplete pathology reports were associated to failure to meet the AECC.
CONCLUSIONS
The PHB phenotype was rare among Spanish IgG4-RD patients. The MS phenotype was less frequent and the RA phenotype was more prevalent than in other, Asian patient series. An undefined phenotype should be considered as some patients do not fall into any of the categories. Three quarters of the cases met the 2019 AECC. Incomplete pathology reports were the leading causes of failure to meet the criteria.
Topics: Age Factors; Cross-Sectional Studies; Databases, Factual; Female; Humans; Immunoglobulin G; Immunoglobulin G4-Related Disease; Male; Middle Aged; Phenotype; Prohibitins; Registries; Sex Factors; Spain; White People
PubMed: 32658963
DOI: 10.1093/rheumatology/keaa247 -
In Vivo (Athens, Greece) 2020In the variety of congenital abdominal cystic lesions (CACL) of different origin, ovarian cyst is the most common intra-abdominal pathology in female neonates. The...
BACKGROUND/AIM
In the variety of congenital abdominal cystic lesions (CACL) of different origin, ovarian cyst is the most common intra-abdominal pathology in female neonates. The prognosis and timing of treatment varies depending on the nature of CACL. This study aimed to assess the results of diagnostics and treatment of CACL.
PATIENTS AND METHODS
A retrospective analysis was performed of 39 cases of CACL, with the spectrum including ovarian, enteric, mesenteric and pancreatic origin. Outcome of minimally invasive surgery, open surgery or conservative approach was analyzed.
RESULTS
Twenty-eight neonates underwent surgery, while 11 were treated conservatively. Twenty patients were treated with a laparoscopic technique and eight with laparotomy combined with laparoscopy. Final diagnosis included: Fifteen cases of ovarian pathology (ovarian torsion in 11 cases), 12 treated laparoscopically and three with laparotomy, six enteric duplications (four laparoscopic and two laparotomic), three mesenteric cysts (one laparoscopic and two laparotomic), two pancreatic cysts (both laparoscopic only), two duodenal stenoses, including duodenal septum (both laparotomies with Heineke-Mikulicz plasty). No blood transfusion apart from two cases requiring re-laparotomy and no early complications were observed in any case; no death occurred.
CONCLUSION
With the strategy of management based on ultrasound and laboratory data, a laparoscopically assisted minimal access approach resulted in minimal risk of complications and complete recovery in all patients, leading to exclusion of oncological risk.
Topics: Abdominal Cavity; Adult; Clinical Decision-Making; Comorbidity; Cysts; Disease Management; Disease Susceptibility; Female; Humans; Infant; Infant, Newborn; Laparoscopy; Minimally Invasive Surgical Procedures; Treatment Outcome
PubMed: 32354912
DOI: 10.21873/invivo.11895 -
Journal of Oral and Maxillofacial... Feb 2020Chronic sclerosing sialadenitis is associated with the immunoglobulin G4 (IgG4)-related disease (RD) spectrum. IgG4-RD is a newly recognized immunomediated...
Chronic sclerosing sialadenitis is associated with the immunoglobulin G4 (IgG4)-related disease (RD) spectrum. IgG4-RD is a newly recognized immunomediated fibroinflammatory condition characterized by several features: a tendency to form tumefactive lesions at multiple sites, lymphoplasmacytic infiltrate, fibrosis and obliterative phlebitis. Often but not always, the serum IgG4 concentrations are also elevated. Immunohistochemistry for IgG4 is helpful to clinch the diagnosis. Here, we describe a case of 65-year-old male with IgG4-related chronic sclerosing sialadenitis of the s ubmandibular gland. We have discussed the histopathological criteria to diagnose this entity.
PubMed: 32189922
DOI: 10.4103/jomfp.JOMFP_83_17 -
Journal of the Korean Association of... Feb 2020Immunoglobulin G4 (IgG4)-related dacryoadenitis and sialoadenitis (IgG4-DS) are part of a multiorgan fibroinflammatory condition of unknown etiology termed IgG4-related... (Review)
Review
Immunoglobulin G4 (IgG4)-related dacryoadenitis and sialoadenitis (IgG4-DS) are part of a multiorgan fibroinflammatory condition of unknown etiology termed IgG4-related disease (IgG4-RD), which has been recognized as a single diagnostic entity for less than 15 years. Histopathologic examination is critical for diagnosis of IgG4-RD. CD4+ T and B cells, including IgG4-expressing plasma cells, constitute the major inflammatory cell populations in IgG4-RD and are thought to cause organ damage and tissue fibrosis. Patients with IgG4-RD who have active, untreated disease exhibit significant increase of IgG4-secreting plasmablasts in the blood. Considerable insight into the immunologic mechanisms of IgG4-RD has been achieved in the last decade using novel molecular biology approaches, including next-generation and single-cell RNA sequencing. Exploring the interactions between CD4+ T cells and B lineage cells is critical for understanding the pathophysiology of IgG4-RD. Establishment of pathogenic T cell clones and identification of antigens specific to these clones constitutes the first steps in determining the pathogenesis of the disease. Herein, the clinical features and mechanistic insights regarding pathogenesis of IgG4-RD were reviewed.
PubMed: 32158675
DOI: 10.5125/jkaoms.2020.46.1.3