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IgG4-Related Ophthalmic Disease


  • 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]
  • 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.[ncbi.nlm.nih.gov]
  • In this review, we present the demographic, clinical, histological, and treatment data for 172 published cases of biopsy-confirmed IgG4-ROD. Results are accompanied by a review of the relevant literature.[ncbi.nlm.nih.gov]
  • CONCLUSIONS: Patients presenting with orbital inflammatory lesions should have biopsies obtained whenever possible.[ncbi.nlm.nih.gov]
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]
  • Tiegs-Heiden CA, Eckel LJ, Hunt CH et al (2014) Immunoglobulin G4-related disease of the orbit: imaging features in 27 patients. AJNR Am J Neuroradiol 35:1393–1397 CrossRef PubMed Google Scholar 22.[doi.org]
  • Classification Division into a number of subgroups according to location has been proposed: lacrimal pseudotumour anterior pseudotumour: immediately behind the globe posterior pseudotumour: distinguished from Tolosa-Hunt syndrome in that the cavernous[radiopaedia.org]
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]
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]
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]


  • The results of systemic workup including gallium-67 scintigraphy were normal.[karger.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.[archopht.jamanetwork.com]
Lymphocytic Infiltrate
  • Moderate to marked lymphocytic infiltrate was present in 11 biopsy specimens, accompanied by marked (3 ) lymphoid follicular hyperplasia in 8 patients ( Figure 2 ).[doi.org]
  • infiltration [ 1 - 3 ].[hoajonline.com]


  • Treatment outcome was evaluated by clinical symptoms, radiologic examination, and change of serum IgG4 level in these patients. All patients had elevated serum IgG4 before treatment (462, 338, and 780 mg/dL respectively.)[ncbi.nlm.nih.gov]
  • 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]
  • Active flare-up with increased serum IgG4 levels may produce a large angle of eye deviation, but mostly respond well to steroid treatment.[ncbi.nlm.nih.gov]
  • CONCLUSIONS: Intraorbital injection of corticosteroid may be a useful treatment option for IgG4-ROD. It may have a role as a first-line therapy in selected cases of IgG4-ROD centred in the anterior orbit.[ncbi.nlm.nih.gov]
  • The material is in no way intended to replace professional medical care by a qualified specialist and should not be used as a basis for diagnosis or treatment.[orpha.net]


  • 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]
  • […] 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]
  • 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]
  • 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]


  • 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 exact etiology is not known but an association with many inflammatory/autoimmune diseases is reported.[radiopaedia.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]


  • 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]
  • 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][e-tjo.org]
Sex distribution
Age distribution


  • 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]
  • He added, “The precise role of IgG4 in disease pathophysiology, if any, remains unclear.[healio.com]


  • Early treatment may prevent destructive changes in affected tissues.[ncbi.nlm.nih.gov]
  • A continuing maintenance dose of oral steroid is recommended to prevent relapse, even when clinical remission is achieved. Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to .[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]

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