Presentation
IONE was present in 53% (8/15) of IgG4-ROD cases whereas it was never present (0/23) in cases of non-IgG4-ROD (P 0.0001). [ncbi.nlm.nih.gov]
Clinical presentation includes mass lesion or swelling of the involved structures, commonly involving lacrimal gland and duct, infraorbital and supraorbital nerves, extraocular muscles and orbital soft tissues. A systemic involvement is common. [orpha.net]
Case: An otherwise healthy 59-year-old man presented with bilateral exophthalmos and right visual disturbance. Orbital magnetic resonance imaging showed bilateral IONE and a soft tissue mass in the right orbit. [okayama.pure.elsevier.com]
Entire Body System
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Inflammation
• IONE appears when inflammation is in direct contact with the ION canal. [ncbi.nlm.nih.gov]
Conclusions In European patients suffering from orbital inflammation, the presence of IONE on an MRI is a specific sign of IgG4-ROD. [doi.org]
Other orbital masses or inflammation can result in visual disturbance (blurred vision, double vision, visual field impairment), restricted eye movements, pain or discomfort, numbness in the distribution of the supraorbital and/or infraorbital nerves, [readtiger.com]
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Soft Tissue Mass
Orbital magnetic resonance imaging showed bilateral IONE and a soft tissue mass in the right orbit. Excisional biopsy in the left infraorbital canal was performed. [ncbi.nlm.nih.gov]
tissue mass (Ormond’s disease) IgG4-related retroperitoneal fibrosis Lymphoma, sarcoma Liver Inflammatory pseudotumor IgG4-related hepatopathy Cholangiocarcinoma, hepatocellular carcinoma, metastatic cancer Aorta Periaortitis and periarteritis, inflammatory [kjpbt.org]
Skin
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Skin Lesion
The re-examined skin lesions resembled histologically the eyelid lesion. It is essential to be aware of IgG4-related disease, including in recurrent chalazia. [doi.org]
Eyes
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Lacrimation
Lacrimal gland involvement may cause swelling of the upper eyelid, or proptosis if there is severe swelling. [readtiger.com]
Clinical presentation includes mass lesion or swelling of the involved structures, commonly involving lacrimal gland and duct, infraorbital and supraorbital nerves, extraocular muscles and orbital soft tissues. A systemic involvement is common. [orpha.net]
Orbital pseudotumors are often associated with inflammation of the extraocular muscles (ORBITAL MYOSITIS) or inflammation of the lacrimal glands (DACRYOADENITIS). Orbital Myositis Inflammation of the extraocular muscle of the eye. [bioportfolio.com]
Abstract Immunoglobulin G4 (IgG4)-related disease is a novel clinical entity characterized by infiltration of IgG4-immunopositive plasmacytes and elevated serum IgG4 concentration accompanied by enlargement of and masses in various organs, including the lacrimal [ncbi.nlm.nih.gov]
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Blurred Vision
On admission, he had marked blepharoedema, conjunctival injection, exophthalmos, diplopia, and blurred vision. He also had bronchial asthma and urinary retention requiring urethral catheterization. [ncbi.nlm.nih.gov]
Other orbital masses or inflammation can result in visual disturbance (blurred vision, double vision, visual field impairment), restricted eye movements, pain or discomfort, numbness in the distribution of the supraorbital and/or infraorbital nerves, [readtiger.com]
A 62-year-old man presented with bilateral blurred vision. He had a history of sinus surgery, and a biopsy specimen showed dense infiltration of IgG4-positive plasma cells. His visual acuity was 20/25, right eye, and 20/125, left eye. [collections.lib.utah.edu]
At first visit, he complained of severe headache, fever, blurred vision and bilateral inflamed eyes. There was no previous ophthalmic history and his general health was good. [austinpublishinggroup.com]
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Eye Pain
The patient also presented with mild left-sided proptosis without red eye, pain, blurring, or diplopia, and there was no rhinorrhea, bleeding, or fever. [omicsonline.org]
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Visual Impairment
Bilateral IgG4-ROD or extra-orbital manifestations were present in only 2 of these patients and were not predictive of visual impairment. [journals.lww.com]
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Red Eye
The patient also presented with mild left-sided proptosis without red eye, pain, blurring, or diplopia, and there was no rhinorrhea, bleeding, or fever. [omicsonline.org]
Musculoskeletal
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Muscle Swelling
A case of extraocular muscle swelling due to IgG4-related sclerosing disease. Jpn J Ophthalmol. 2011;55:315–7. PubMed CrossRef Google Scholar 6. Watanabe T, Fujikawa Y, Kawakami S, Hatta T, Hamano H, Kawa S, et al. [springerlink.com]
Psychiatrical
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Withdrawn
Overall, DMARDs were poorly effective and were often withdrawn due to drug-related toxicity. In 7 cases, RTX was specifically indicated for IgG4-ROD presenting as: soft tissue (n 6), EOM (n 4), nerve (n 2), or bone (n 2) involvements. [journals.lww.com]
Urogenital
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Urinary Retention
He also had bronchial asthma and urinary retention requiring urethral catheterization. His serum immunoglobulin (Ig) G4 level was elevated to 1,830 U/mL. Fluorodeoxyglucose-positron emission tomography revealed an abnormal uptake in multiple organs. [ncbi.nlm.nih.gov]
Workup
The results of systemic workup including gallium-67 scintigraphy were normal. [karger.com]
[…] xanthogranuloma, Erdheim-Chester and Rosai-Dorfman diseases. [7,8] Because serum IgG4 (sIgG4) elevation and IgG4 plasma cells tissue infiltration are not specific of IgG4-RD, such diagnosis should only be retained after that an extensive diagnostic workup [journals.lww.com]
As part of their workup before the biopsy, the patients underwent imaging studies of the orbit. Orbital computed tomography was performed in 6 patients and orbital magnetic resonance imaging in 5 patients; 1 patient underwent both tests. [doi.org]
Serum
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Antinuclear Autoantibodies
Antinuclear autoantibodies were found in 7 of 17 patients, between 1/80 and 1/320 but no patient had either dsDNA or extractable nuclear antigen (especially SSA or SSB) positive autoantibodies (Supplemental Table 2, ). 3.5 Response to treatment All patients [journals.lww.com]
Treatment
All patients improved clinically after corticosteroid treatment. Serum IgG 4 levels decreased after steroid treatment (p 0.005) and normalised in nine patients. [ncbi.nlm.nih.gov]
Prognosis
Consideration of IgG4-RD and accurate diagnosis by biopsy have important implications for prognosis and treatment following the distinction of this condition from Sjögren syndrome (SjS), granulomatosis with polyangiitis (GPA, formerly Wegener’s), sarcoidosis [acrabstracts.org]
The prognosis is good, because systemic rituximab and steroid have been reported to well control ocular inflammation [13,14], however; its etiology still remained to be elucidated. There are few cases which IgG4-RD caused scleritis [13,15]. [austinpublishinggroup.com]
[…] region typically hypointense due to fibrosis and with more progression of fibrosis it becomes more hypointense, but the signal can also be iso- to hyperintense to extra-ocular muscles 2 T1 C (Gd): moderate to a marked diffuse enhancement Treatment and prognosis [radiopaedia.org]
What is the prognosis of IgG4-related disease? Prognosis of IgG4-related disease is variable. It may spontaneously resolve or persist, with remitting and relapsing symptoms. [dermnetnz.org]
Bugiardini STEMI: Prognosis, Pages 489-498, E. Cenko, B. Ricci, R. Bugiardini Structure and Function of the Adult Vertebrate Cardiovascular System, Pages 499-510, J.D. Schultz, D.M. Bader Sudden Cardiac Death, Pages 511-520, G.L. Sumner, V.P. [ebookshop.ro]
Etiology
For each disorder, the etiology, clinical manifestations, imaging characteristics, differential diagnoses, and management options are described and discussed. [books.google.de]
Ultrasound Exam With Doppler for the Etiological Diagnosis of Orbital Masses (PUCE) Orbital masses develop at the expense of the orbital structures lacrimal glands, oculomotor muscles, optic nerve, meningeal spaces, peripheral nerves, bone wall, orbital [bioportfolio.com]
They are organised into groups, and further divided into clinical, etiological or histopathological sub-types. [orpha.net]
The prognosis is good, because systemic rituximab and steroid have been reported to well control ocular inflammation [13,14], however; its etiology still remained to be elucidated. There are few cases which IgG4-RD caused scleritis [13,15]. [austinpublishinggroup.com]
The exact etiology is not known but an association with many inflammatory/autoimmune diseases is reported. [radiopaedia.org]
Epidemiology
Epidemiology There is a little publication on epidemiology of IgG4-ROD mainly because of lacking awareness of IgG4-ROD before 2007. [11] By pooling 95 cases from seven studies of IgG4-ROD from Japan, [9], [12], [13], [14] Korea, [15] America, [16] and [e-tjo.org]
His epidemiological research has focused on 4 inter-related areas: a. the epidemiology of congestive heart failure, including identifying risk factors for the disease, characterizing the subgroups with diastolic heart failure, asymptomatic LV systolic [ebookshop.ro]
As recognition of IgG4-RD is relatively recent, there are limited studies on its epidemiology. It is therefore difficult to make an accurate estimation of prevalence. [en.wikipedia.org]
Discussion The etiology and epidemiology of IgG4-RD are unknown. IgG4-RD often occurs in elderly males. It has been estimated that the incidence of this disorder in Japan is 0.28–1.08/100,000 [ 4, 5 ]. [omicsonline.org]
Epidemiology in the pediatric population is also unknown, as there are few reported cases among children. [eyewiki.aao.org]
Pathophysiology
Offers an in-depth and thorough approach to the pathophysiology of oculoplastics and orbital disease, incorporating the perspectives of numerous specialties – all in one convenient volume. [books.google.de]
Although it responds well to steroid therapy, recurrence and relapse occur following the early reduction or withdrawal of medication. [2] As for the pathophysiologic correlation between LC and IgG4-RD, there is no literature available to illustrate the [ijo.in]
Antzelevitch Cardiac Anatomy, Physiology, and Pathophysiology, Pages 373-383, K.N. Hor, A.J. Trask Cardiac and Induced Pluripotent Stem Cells, Pages 384-393, A. Salerno, W. Balkan, K. Hatzistergos, J.M. [ebookshop.ro]
This review summarizes current knowledge on clinical manifestations, pathophysiology and treatment of IgG4-RD. [ojrd.biomedcentral.com]
Prevention
Early treatment may prevent destructive changes in affected tissues. [ncbi.nlm.nih.gov]
In patients with an extraophthalmic involvement, prednisolone combined with an immunosuppressive agent was more effective for preventing recurrence than prednisolone alone. orbit inflammation lacrimal gland pathology Statistics from Altmetric.com Request [bjo.bmj.com]
Kahn SR et al (2012) Prevention of VTE in nonsurgical patients: antithrombotic therapy and prevention of thrombosis, 9th ed: American College of Chest Physicians Evidence-Based Clinical Practice Guidelines. [link.springer.com]