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Rinsho Byori. the Japanese Journal of... Feb 2012IgG4-related disease is a recently recognized systemic syndrome characterized by mass-forming lesions, mainly in exocrine tissue, that consist of lymphoplasmacytic... (Review)
Review
IgG4-related disease is a recently recognized systemic syndrome characterized by mass-forming lesions, mainly in exocrine tissue, that consist of lymphoplasmacytic infiltrates and sclerosis. There are numerous IgG4+ plasma cells in the affected tissues, and the serum IgG4 level is elevated in these patients. Ocular adnexal IgG4-related disease frequently involves bilateral lacrimal glands, and obliterative phlebitis is rare. Moreover, some malignant lymphomas, especially mucosa-associated lymphoid tissue lymphoma, arise from ocular adnexal IgG4-related disease. It is known that hyper IL-6 syndromes, such as multicentric Castleman's disease, rheumatoid arthritis, and other autoimmune diseases, fulfill the histological diagnostic criteria for IgG4-related disease; therefore, hyper IL-6 syndromes and IgG4-related disease cannot be differentially diagnosed by immunohistochemical staining alone. However, upon laboratory examination, hype IL-6 syndromes show elevation of the CRP level, polyclonal hyper gamma-globulinemia, anemia, and hypoalbuminemia. These findings are quite different from IgG4-related disease, which is not characterized by elevated serum IgA, IgM, and CRP levels. Therefore, laboratory findings are crucial for the differential diagnosis.
Topics: Arthritis, Rheumatoid; Biomarkers; C-Reactive Protein; Castleman Disease; Diagnosis, Differential; Humans; Immunoglobulin G; Interleukin-6; Lymphoma; Syndrome
PubMed: 22568099
DOI: No ID Found -
Pathology International Apr 2010IgG4-related diseases comprise a recently recognized systemic syndrome characterized by mass-forming lesions in mainly exocrine tissue that consist of lymphoplasmacytic... (Review)
Review
IgG4-related diseases comprise a recently recognized systemic syndrome characterized by mass-forming lesions in mainly exocrine tissue that consist of lymphoplasmacytic infiltrates and sclerosis. There are numerous IgG4-positive plasma cells in the affected tissues, and the serum IgG4 level is increased in these patients. The present study describes the history, autoimmune pancreatitis (AIP), IgG4-related lymphadenopathy and lymphomagenesis based upon ocular adnexal IgG4-related disease. Lymphoplasmacytic sclerosing pancreatitis, a prototypal histological type of AIP, is now recognized as a systemic IgG4-related disease. Lymph node lesions can be subdivided into at least five histological subtypes, and systemic IgG4-related lymphadenopathy should be distinguished from multicentric Castleman's disease. Interleukin-6 and CRP levels are abnormally high in multicentric Castleman's disease, but are normal in the majority of systemic IgG4-related lymphadenopathy. Ocular adnexal IgG4-related disease frequently involves bilateral lacrimal glands swelling, and obliterative phlebitis is rare. Moreover, some malignant lymphomas, especially mucosa-associated lymphoid tissue lymphoma, arise from ocular adnexal IgG4-related disease. In addition, IgG4-producing lymphoma also exists.
Topics: Autoantibodies; Autoimmune Diseases; Hematologic Diseases; Humans; Immunoglobulin G; Lymphatic Diseases; Pancreatitis; Plasma Cells; Sclerosis; Syndrome
PubMed: 20403026
DOI: 10.1111/j.1440-1827.2010.02524.x -
BMJ Case Reports Apr 2022Mucus fishing syndrome is a chronic inflammatory ocular surface condition characterised by repetitive self-extraction of mucous strands from the eye.A man in his 30s...
Mucus fishing syndrome is a chronic inflammatory ocular surface condition characterised by repetitive self-extraction of mucous strands from the eye.A man in his 30s presented with bilateral ocular redness, itch, irritation, tearing and sticky mucoid discharge for 3 months. Examination disclosed bilateral bulbar and tarsal conjunctival injection. Fluorescein staining disclosed a well-circumscribed area of tarsal conjunctival epithelial defect near the inferior lacrimal punctum in both eyes. The patient admitted to a habit of mechanically removing mucus from his eyes several times a day. Demonstration of the mucus extraction process disclosed direct contact of his fingers with the excoriated tarsal conjunctiva in each eye. He was diagnosed with mucus fishing syndrome and his condition resolved within a month after he stopped fishing mucus from his eyes and had a course of topical antibiotics and steroids.Mucus fishing syndrome is an important diagnostic consideration in patients with chronic conjunctivitis.
Topics: Humans; Male; Conjunctiva; Conjunctivitis; Eye Diseases; Mucus; Syndrome
PubMed: 35396241
DOI: 10.1136/bcr-2022-249188 -
Ophthalmic Plastic and Reconstructive... 2017To review and summarize the syndromic, nonsyndromic, and systemic associations of congenital lacrimal drainage anomalies. (Review)
Review
PURPOSE
To review and summarize the syndromic, nonsyndromic, and systemic associations of congenital lacrimal drainage anomalies.
METHODS
The authors performed a PubMed search of all articles published in English on congenital lacrimal anomalies (1933-2016). Patients of these articles were reviewed along with the literature of direct references to syndromes and other systemic associations. Data reviewed included syndromic descriptions, systemic details, demographics, lacrimal presentations, management, and outcomes.
RESULTS
Syndromic and systemic associations with congenital lacrimal drainage disorders are not known to be common. Although familial presentations have been reported, the inheritance patterns are unclear for most anomalies. There is an increasingly growing evidence of a genetic basis to many lacrimal anomalies. However, few syndromes have either widespread or are frequently associated with lacrimal anomalies. Few sequences of distinct lacrimal presentations and intraoperative findings are seen. Surgical challenges in these patients are distinct and a thorough pre and intraoperative anatomical assessment, detailed imaging when indicated, and assessment and correction of associated periocular and facial abnormalities may facilitate good outcomes.
CONCLUSIONS
Lacrimal drainage anomalies associated with syndromic and nonsyndromic systemic conditions have certain unique features of their own and their surgical and anesthesia challenges are distinct. Diagnosis of congenital lacrimal drainage anomalies should prompt consideration of the possible presence of associated systemic abnormalities.
Topics: Humans; Lacrimal Apparatus; Lacrimal Apparatus Diseases; Syndrome
PubMed: 28472008
DOI: 10.1097/IOP.0000000000000923 -
Current Eye Research Sep 2023The current study seeks to investigate the association between lacrimal disorders and the use of docetaxel and paclitaxel.
PURPOSE
The current study seeks to investigate the association between lacrimal disorders and the use of docetaxel and paclitaxel.
METHODS
A disproportionality analysis was conducted using the United States FDA Adverse Event Reporting System (FAERS). All adverse event reports containing the term docetaxel or paclitaxel were selected. Lacrimal adverse events were identified using the lacrimal disorders Standardized MedDRA Query (SMQ), which includes disorders that affect lacrimal gland and drainage system including blockage of nasolacrimal duct, occlusion/stenosis of punctum, lacrimal gland neoplasms, and inflammations and infections.
RESULTS
The proportionate reporting ratio (PRR) comparing lacrimal events among docetaxel to paclitaxel users was 2.47 (95% CI, 2.03-3.02). With respect to specific lacrimal events, dacryostenosis (PRR 19.54 [95% CI, 7.19-53.13]), increased lacrimation (PRR 3.2 [95% CI, 2.42-4.23]), lacrimation disorder ( = 0.02), and xeropthalmia reports ( > 0.001) were significantly more common.
CONCLUSIONS
The growing body of epidemiologic, clinical, and pathophysiologic research supports the case that docetaxel leads to adverse lacrimal events in certain patients and should be taken into consideration when oncologists consider docetaxel vs. paclitaxel.
Topics: Humans; United States; Docetaxel; Taxoids; Paclitaxel; Lacrimal Apparatus Diseases
PubMed: 37232564
DOI: 10.1080/02713683.2023.2219041 -
Nihon Rinsho Men'eki Gakkai Kaishi =... Feb 2008Mikulicz's disease represents persistent enlargement of the lacrimal and salivary glands, and autoimmune pancreatitis is shown with diffuse pancreatic swelling. Both... (Review)
Review
Mikulicz's disease represents persistent enlargement of the lacrimal and salivary glands, and autoimmune pancreatitis is shown with diffuse pancreatic swelling. Both diseases are characterized with elevated IgG4 concentrations in the serum and prominent infiltration by plasmacytes expressing IgG4 in the glands. Clinical analyses were performed in 40 patients with systemic IgG4-related plasmacytic syndrome (SIPS) who consulted the doctors in Sapporo Medical University Hospital. Our patients were mainly middle-aged or elderly females. The average age was 58.9 years. The diagnosis was following ; 33 cases with Mikulicz's disease, 3 cases with Küttner's tumor, and 4 cases with IgG4-related dacryoadenitis. Slight dysfunction of lacrimal and salivary gland was observed in about 60% of them. Antinuclear antibodies were detected in only 15% of the cases with SIPS. Almost all, except one case, did not have anti-SS-A or anti-SS-B antibodies. Interestingly, hypocomplementemia was revealed in 30% of them. The complications of SIPS include autoimmune pancreatitis, tubulointerstitial nephritis, retroperitoneal fibrosis, prostatitis, and so on. SIPS is mainly treated by the administration of steroids. We started to prescribe much quantity of prednisolone to the patients with organ failure. The recurrence was admitted in the 3 patients for the followed 16 years. We present here the problems and prospects in SIPS.
Topics: Adult; Aged; Aged, 80 and over; Female; Humans; Immunoglobulin G; Male; Middle Aged; Mikulicz' Disease; Plasma Cells; Sjogren's Syndrome; Syndrome
PubMed: 18311037
DOI: 10.2177/jsci.31.1 -
Annals of Internal Medicine May 2012
Topics: Adult; Autoimmune Diseases; Granuloma, Plasma Cell; Humans; Immunoglobulin G; Kidney Diseases; Lacrimal Apparatus; Male; Pancreatitis; Syndrome; Young Adult
PubMed: 22547483
DOI: 10.7326/0003-4819-156-9-201205010-00020 -
Annals of Anatomy = Anatomischer... Jan 2021To review and update the syndromic and non-syndromic systemic associations of congenital lacrimal drainage anomalies. (Review)
Review
BACKGROUND
To review and update the syndromic and non-syndromic systemic associations of congenital lacrimal drainage anomalies.
METHODS
The authors performed a PubMed search of all articles published in English on congenital lacrimal drainage anomalies (CLDA). The current review provides an update from January 2017 to August 2020 on all CLDA associated with clinical syndromes or non-syndromic systemic disorders. The update intends to appraise the readers on all papers that were published in the interim 3-year period since the publication of the previous major review by authors (1933-2016). Patients with specific syndromes or systemic disorders were then reviewed. Pertinent cross-references from each of the short-listed articles were also included. Data reviewed include syndromic descriptions, systemic details, demographics, lacrimal presentations, management, and outcomes.
RESULTS
There have been significant new updates. Eleven new syndromes have been added to the list of syndromic associations, of which three were suspects. Among the new syndromic associations, three (PHACE, Williams-Beuren, and Peter's plus syndromes) described CLDA details in depth. Several new non-syndromic systemic conditions with associated CLDA also came to light, the foremost amongst them being proboscis lateralis, diprosopus dirrhinus, cerebral palsy, and NGLY-1 related disorders. Although familial presentations have been reported, the inheritance patterns are unclear for most anomalies. Surgical challenges in these patients are distinct, and a thorough pre-operative assessment, including detailed imaging when indicated, may facilitate good outcomes. Two updated tables reflecting the summary of syndromic and non-syndromic systemic associations are provided to capture the details at a glance.
CONCLUSION
It is not very uncommon to find CLDA in syndromic or non-syndromic systemic disorders. Diagnosis of a craniofacial syndrome should prompt the physician to look out for CLDA. Similarly, a diagnosis of multiple CLDA should alert the examiner for the possible presence of associated systemic anomalies.
Topics: Abnormalities, Multiple; Humans; Lacrimal Apparatus; Lacrimal Apparatus Diseases; Syndrome
PubMed: 33098980
DOI: 10.1016/j.aanat.2020.151613 -
Clinical and Experimental Rheumatology 2013IgG4-related Mikulicz's disease (IgG4-MD) has been recently established as a subtype of IgG4-related diseases involving the salivary and lacrimal glands, and the... (Comparative Study)
Comparative Study
OBJECTIVES
IgG4-related Mikulicz's disease (IgG4-MD) has been recently established as a subtype of IgG4-related diseases involving the salivary and lacrimal glands, and the diseased glands are characteristically and highly responsive to corticosteroid therapy. We retrospectively evaluated ultrasonography (US) of the salivary and lacrimal glands for monitoring the efficacy of corticosteroid treatment in patients with IgG4-MD.
METHODS
US features of the salivary and lacrimal glands were assessed and compared with the serum IgG4 levels in 8 patients with IgG4-MD before and at various stages after initiation of oral corticosteroids.
RESULTS
US features of the lacrimal and salivary glands of patients with IgG4-MD were characterised by multiple hypoechoic areas in enlarged glands. The submandibular glands were most frequently involved by the disease, and bilateral glands of the same type were similarly affected exhibiting the same hypoechoic pattern. Alleviations of abnormal gland architecture and size in response to corticosteroid therapy were effectively detected with US. The US findings of the involved glands were proportional to the serum IgG4 level before and during the corticosteroid therapy.
CONCLUSIONS
US helps monitor the efficacy of corticosteroid treatment in patients with IgG4-MD.
Topics: Administration, Oral; Adrenal Cortex Hormones; Aged; Drug Monitoring; Female; Humans; Immunoglobulin G; Lacrimal Apparatus; Magnetic Resonance Imaging; Male; Middle Aged; Mikulicz' Disease; Predictive Value of Tests; Retrospective Studies; Salivary Glands; Time Factors; Treatment Outcome; Ultrasonography
PubMed: 23806290
DOI: No ID Found