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Pathology International May 2021In this review, the author shows that simultaneous multiple disorders caused by reactivation of Epstein-Barr virus can lead to salivary gland disorders as part of... (Review)
Review
In this review, the author shows that simultaneous multiple disorders caused by reactivation of Epstein-Barr virus can lead to salivary gland disorders as part of Sjogren's syndrome (SS). Therefore, clinicians must differentiate SS from other diseases when diagnosing and treating salivary gland disorders. In particular, the author explains how microbial infection in SS overcomes immunological tolerance, leading to pathological changes, and how cytokine overexpression and endocrine disrupters contribute to glandular tissue injury. Also, the author suggests that involvement of reactive oxygen species is a common pathogenesis of salivary gland disorders and SS, so regulation of oxidative stress is an effective treatment for both. The results of clinical studies on restoring salivary gland function and regenerating salivary glands with tissue stem cells may provide clues on elucidating the cause of SS.
Topics: Antioxidants; Arthritis, Rheumatoid; Autoantigens; Autoimmune Diseases; Cytokines; Diagnosis, Differential; Dioxins; Epstein-Barr Virus Infections; Estrogens; Female; Genetic Predisposition to Disease; Herpesvirus 4, Human; Humans; Interleukin-10; Lymphocytes; Male; Mikulicz' Disease; Oxidative Stress; Reactive Oxygen Species; Salivary Glands; Sjogren's Syndrome; Stem Cell Transplantation; Ubiquinone; Virus Activation; Virus Diseases
PubMed: 33751738
DOI: 10.1111/pin.13079 -
Annals of Medicine and Surgery (2012) Mar 2021and importance: this case report aimed at an unusual case of extensive rhinoscleroma with a literature review, We report this case to bring attention to the patients...
and importance: this case report aimed at an unusual case of extensive rhinoscleroma with a literature review, We report this case to bring attention to the patients with immunodeficiency problem regardless of presenting symptoms. Case presentation: a 70 -year-old patient with diabetes and hypertension, consulted for chronic median and posterior headaches have not improved with symptomatic treatment. She reported cacosmia without rhinorrhea and right otalgia. The examination of the nasal cavity showed a bleeding lesion on the lateral wall of the nasopharynx. CT-scan of the facial showed tissue lesion on the posterior and lateral walls of the nasopharynx, with infiltration of the parapharyngeal space and bone lysis right edge of the clivus. The biopsy confirmed the diagnosis of rhinoscleroma. The patient started the ciprofloxacin for 3 months, and the evolvement has noticed with the disappearance of the lesion during the first month of treatment. The patient started the ciprofloxacin for 3 months, and the evolvement was noticed with the disappearance of the lesion during the first month of treatment. Cilinical discussion: rhinoscleroma is a chronic and progressive granulomatous disease of the nasal cavities. The diagnosis is confirmed by the search for bacteria and an anatomopathological assessment of MIKULICZ cells. The medical treatment of choice has a high concentration in macrophages such as rifampicin and fluoroquinolone. Conclusion: rhinoscleroma is a granulomatous disease caused by klebsiella rhinoscleromatis, you must think about in front a nasopharyngeal lesion imitating a malignant pathology.
PubMed: 33643651
DOI: 10.1016/j.amsu.2021.02.012 -
The Pan African Medical Journal 2020Mikulicz's disease is a unique condition involving the enlargement of the lacrimal and salivary glands, similar to that observed in Sjogren's syndrome; however,...
Mikulicz's disease is a unique condition involving the enlargement of the lacrimal and salivary glands, similar to that observed in Sjogren's syndrome; however, Mikulicz's disease is clinically characterized by infrequent autoimmune reactions and responsiveness to glucocorticoid treatment. The ultrasound features of the lacrimal and salivary glands in patients with IgG4-Mikulicz's disease were characterized by multiple hypoechoic areas of varying sizes within the enlarged glands. IgG4 serum level was also elevated, in contrast to the detection of normal levels in Sjogren's syndrome. In this article, we intended to illustrate a case of Mikulicz's disease with clinical and imaging features.
Topics: Adult; Diagnosis, Differential; Female; Humans; Immunoglobulin G; Lacrimal Apparatus; Mikulicz' Disease; Salivary Glands; Sjogren's Syndrome; Ultrasonography
PubMed: 33598067
DOI: 10.11604/pamj.2020.37.252.26835 -
European Journal of Radiology Mar 2021Immunoglobulin G4-related disease (IgG4-RD) is a newly defined fibro-inflammatory multisystemic condition defined by a triad of diagnostic criteria based on clinical... (Review)
Review
PURPOSE
Immunoglobulin G4-related disease (IgG4-RD) is a newly defined fibro-inflammatory multisystemic condition defined by a triad of diagnostic criteria based on clinical presentation, biochemical and histopathological findings. Whereas some subsites of this disease have been well described in the literature so far (e.g. pancreas, kidneys, retroperitoneum, salivary glands), more recently identified anatomical sites of involvement in the head and neck are less well understood (e.g. nose, paranasal sinuses).
METHOD
This pictorial review details the imaging appearances of extracranial IgG4-RD in the Head & Neck. Multimodality imaging appearance and features are presented, with reference to the published literature to date.
RESULTS
Following a subsite-based approach, we present both the most common and the more rarely encountered imaging patterns of IgG4-RD in the extracranial head and neck, along with the relevant differential diagnoses to consider. Our institutional experience not only cements what is already known in the existing literature on this topic, but also reveals new imaging features of IgG4-RD, notably in the sinonasal tract.
CONCLUSIONS
This pictorial review of extracranial head & neck IgG4-RD will enable radiologists to recognise the features of this condition and propose it as a differential diagnosis to include alongside other probable entities. It establishes the place of the radiologist in the diagnosis and management of IgG4-RD.
Topics: Autoimmune Diseases; Head; Humans; Immunoglobulin G; Immunoglobulin G4-Related Disease; Neck
PubMed: 33524920
DOI: 10.1016/j.ejrad.2021.109560 -
International Journal of Surgery Case... Feb 2021Segmental aganglionosis (the absence of ganglions) is a rare presentation of Hirschsprung's disease, whereby only limited segment/segments of aganglionic bowel is...
INTRODUCTION
Segmental aganglionosis (the absence of ganglions) is a rare presentation of Hirschsprung's disease, whereby only limited segment/segments of aganglionic bowel is interposed between segments of innervated bowel, or "skip'' area of normal innervations is present within an area of aganglionosis.
PRESENTATION OF CASE
We reported a case of a 3 day old male newborn who presented with failure to pass meconium along with progressive abdominal distension. There were skip lesions present in between. Mikulicz double barrel enterostomy was carried out, which was followed by an uneventful postoperative period. Four months later, the patient was admitted for levelling biopsies which revealed the absence of ganglions in the terminal ileum as well as in the rectosigmoid junction. But the ganglions were present in between and proximal to the terminal ileum where the previously dilated small bowel segment was resected. This presentation was contradicted the most accepted migration theory of Hirschsprung's disease.
DISCUSSION
As seen in our case, and in21 other cases published between 1954-2016, we highly recommend that leveling/mapping biopsies should definitely include the cecal pole and the small bowel segments proximal to the ileocecal valve as well as the multilevel colonic biopsies down till the rectum.
CONCLUSION
Reporting of these cases brings out interesting questions with respect to the pathogenesis and serves to highlight the existence of several variants within the spectrum of Hirschsprung's disease.
PubMed: 33485175
DOI: 10.1016/j.ijscr.2021.01.049 -
Scientific Reports Jan 2021The clinical utility of serum immunoglobulin free light chains (sFLC) in IgG4-related disease (IgG4-RD) is unknown. Herein we evaluated their association with clinical...
The clinical utility of serum immunoglobulin free light chains (sFLC) in IgG4-related disease (IgG4-RD) is unknown. Herein we evaluated their association with clinical phenotypes, serology and activity in patients with IgG4-RD. Cross-sectional study that included 45 patients with IgG4-RD, and as controls 25 with Sjögren's syndrome (SS) and 15 with sarcoidosis. IgG4-RD patients were classified in clinical phenotypes: pancreato-hepato-biliary, retroperitoneum/aorta, head/neck-limited and Mikulicz/systemic; as well as proliferative vs. fibrotic phenotypes. We assessed the IgG4-RD Responder Index (IgG4-RD RI) at recruitment and measured IgG1, IgG4, κ and λ sFLC serum levels by turbidometry. sFLC levels were similar among IgG4-RD, SS and sarcoidosis groups. Regarding the IgG4-RD patients, the mean age was 49 years, 24 (53.3%) were men and 55.5% had activity. Eight (17.7%) belonged to pancreato-hepato-biliary, 6 (13.3%) to retroperitoneum/aorta, 14 (31.1%) to head/neck-limited, 16 (35.5%) to Mikulicz/systemic phenotypes, whereas 36 (80%) to proliferative and 9 (20%) to fibrotic phenotypes. High κ sFLC, λ sFLC and κ/λ ratio were present in 29 (64.4%), 13 (28.9%) and 13 (28.9%) of IgG4-RD patients, respectively. There were no differences in sFLC among IgG4-RD phenotypes. κ sFLC and κ/λ ratio correlated positively with the number of involved organs and IgG4-RD RI. Patients with renal involvement had higher κ sFLC and λ sFLC. The AUC for κ sFLC and λ sFLC, for renal involvement was 0.78 and 0.72, respectively. Active IgG4-RD had higher levels of κ sFLC and more frequently a high κ/λ ratio. The AUC for κ sFLC and κ/λ ratio for predicting active IgG4-RD was 0.67 and 0.70, respectively. sFLC correlated positively with IgG1 and IgG4 levels. sFLC may be useful as a biomarker of disease activity as well as multiorgan and renal involvement. In particular, a high κ/λ ratio may identify patients with active disease.
Topics: Adult; Biomarkers; Female; Humans; Immunoglobulin G; Immunoglobulin G4-Related Disease; Immunoglobulin Light Chains; Immunosuppressive Agents; Male; Middle Aged; Phenotype
PubMed: 33469111
DOI: 10.1038/s41598-021-81321-5 -
Modern Rheumatology May 2021IgG4-related disease (IgG4-RD) is a fascinating clinical entity first reported in this century in Japan, and includes a wide variety of diseases, such as formerly named...
IgG4-related disease (IgG4-RD) is a fascinating clinical entity first reported in this century in Japan, and includes a wide variety of diseases, such as formerly named Mikulicz's disease (MD), autoimmune pancreatitis (AIP), interstitial nephritis, prostatitis and retroperitoneal fibrosis. The Japanese IgG4 team organized by the Ministry of Health, Labor and Welfare (MHLW) of Japan has published the first criteria, comprehensive diagnostic (CD) criteria for IgG-RD 2011. Thereafter, IgG4-RD has been accepted widely and many cases have been reported from all over the world. Several problems have arisen in clinical practice, however, including the difficulty obtaining biopsy samples, and the sensitivity and specificity in cut off level of serum IgG4 and impaired immunostaining of IgG4. Given these situations, the Japanese IgG4 team has updated the 2011 comprehensive diagnostic criteria for IgG4-RD and propose the 2020 revised comprehensive diagnostic (RCD) criteria for IgG4-RD, which consists of 3 domains; 1) Clinical and radiological features, 2) Serological diagnosis and 3) Pathological diagnosis. In addition, the new pathological diagnosis is composed by three sub-items including storiform fibrosis and obliterative phlebitis.
Topics: Consensus Development Conferences as Topic; Humans; Immunoglobulin G4-Related Disease; Japan; Male; Middle Aged; Practice Guidelines as Topic
PubMed: 33274670
DOI: 10.1080/14397595.2020.1859710 -
Rheumatology (Oxford, England) Jun 2021The aim of this study was to compare the clinical characteristics of IgG4-related disease (IgG4-RD) among different age groups.
OBJECTIVE
The aim of this study was to compare the clinical characteristics of IgG4-related disease (IgG4-RD) among different age groups.
METHODS
We conducted a prospective study of 737 patients who were newly diagnosed with IgG4-RD and compared detailed demographic features, organ involvements, laboratory tests, treatments and outcomes across age groups. The patients were divided into five groups according to their age at diagnosis: ≤39, 40-49, 50-59, 60-69 and ≥70 years. The clinical characteristics of paediatric patients were also described.
RESULTS
Sex ratio, disease duration, allergy history and clinical symptoms were significantly different across age groups. Besides, the proportions of superficial organ involvement (lacrimal gland and sinus) decreased with age, while the proportions of internal organ involvement (pancreas, biliary tract, retroperitoneal tissue, lung and prostate) increased with age, which was more prominent in male patients. Mikulicz's disease was the most common manifestation (70%) in paediatric IgG4-RD patients. Multiple Cox analysis identified that age ≤56 years at diagnosis was an independent risk factor of relapse.
CONCLUSION
We revealed the impact of age on clinical characteristics of IgG4-RD, which indicated that different management might be required among different age groups.
Topics: Adolescent; Adult; Age Factors; Aged; B-Lymphocyte Subsets; Child; Female; Humans; Immunoglobulin G4-Related Disease; Male; Middle Aged; Mikulicz' Disease; Proportional Hazards Models; Prospective Studies; Recurrence; Sex Factors; Young Adult
PubMed: 33211878
DOI: 10.1093/rheumatology/keaa651 -
AME Case Reports 2020Immunoglobulin G4 (IgG4) related disease is a rare autoimmune disease involving multiple organs and tissues. A diagnosis of IgG4-related disease (IgG4-RD) is mainly...
Immunoglobulin G4 (IgG4) related disease is a rare autoimmune disease involving multiple organs and tissues. A diagnosis of IgG4-related disease (IgG4-RD) is mainly based on serum IgG4 concentration, imaging, pathology, and effective glucocorticoids therapy. In this paper, we report a 53-year-old male with typical signs and symptoms of IgG4-RD successfully treated with glucocorticoids. This patient had experienced bilateral mumps for more than 8 months and intermittent abdominal pain spreading to his lower back for 2 months before his admission. During his hospitalization, based on the characteristic appearance of magnetic resonance imaging (MRI), magnetic resonance cholangiopancreatography (MRCP), and computed tomography with positron emission tomography, a diagnosis of autoimmune pancreatitis (AIP), cholangitis with biliary obstruction, lachrymal adenitis, and submandibular adenitis was made. A high serum IgG4 concentration further supported a diagnosis of IgG4-RD. Then, the treatment was promptly initiated with corticosteroids. MRI, MRCP, and IgG4 concentration were re-examined during his follow up, suggesting that glucocorticoid treatment resulted in a resolution of his disease. The dosage of glucocorticoid had been gradually decreased. Now, he is stable with oral low-dose glucocorticoids. Certainly, long-term follow up of this patient with such a rare disease is very essential to observe the possibility of disease recurrence and glucocorticoids related complications.
PubMed: 33178994
DOI: 10.21037/acr-20-43 -
Annals of the Rheumatic Diseases Jan 2021
Topics: Eponyms; History, 19th Century; History, 20th Century; History, 21st Century; Humans; Immunoglobulin G4-Related Disease; Mikulicz' Disease; Sjogren's Syndrome; Terminology as Topic
PubMed: 32988841
DOI: 10.1136/annrheumdis-2020-218843