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]
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]
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]
Conclusions : The findings of the present study suggest that IgG4-ROD occurs in various regions of the ocular adnexa. Keywords: 744 tumors • 631 orbit © 2014, The Association for Research in Vision and Ophthalmology, Inc., all rights reserved. [iovs.arvojournals.org]
Cardiovascular
- Thrombosis
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]
Courtesy of James Chelnis MD Figure 2: B, Orbital MRI following thrombosis of left cavernous, sigmoid, and transverse sinuses. Courtesy of James Chelnis MD Imaging Figure 2: C, Orbital MRI showing resolution of intracavernous thrombosis. [eyewiki.aao.org]
Cubbon Animal Models of Ischemic Heart Disease: From Atherosclerosis and Thrombosis to Myocardial Infarction, Pages 97-110, R. Covarrubias, A.S. Major, R.J. [ebookshop.ro]
Musculoskeletal
- Osteoporosis
(o A) 2001 NIH Consensus Development Panel on Osteoporosis Prevention, Diagnosis, and Therapy, March 7–29, 2000: highlights of the conference. South Med J 94: 569–573 Google Scholar 37. Reinshagen M (2008) Osteoporosis in inflammatory bowel disease. [link.springer.com]
Skin
- Blister
However, IgG4 is known to play a role in protection against type 1 hypersensitivity reactions, for example to bee venom, and in the pathogenesis of autoimmune blistering diseases such as pemphigus vulgaris, helminth infections, and malignancy including [dermnetnz.org]
Neurologic
- Headache
Case report: A 63-year-old man, who was referred to our clinic with refractory uveitis, presented initially with severe headache, fever and ocular inflammations. [austinpublishinggroup.com]
Workup
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]
Diagnostic and treatment workup for IgG4-related disease. Expert Rev Clin Immunol. 2017;13(9):867-875. ↑ Woo YJ, Kim JW, Yoon JK. Clinical Implications of serum IgG4 levels in patients with IgG4-related ophthalmic disease. [eyewiki.aao.org]
[…] 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]
Serum
- Hypercholesterolemia
Kilic Familial Hypercholesterolemia, Pages 285-297, A. Pirillo, A.L. Catapano Frailty in Heart Failure Patients, Pages 298-302, S. Pedraza, T.A. Barrett, C.M. Hritz Genetic Atrial Fibrillation, Pages 303-312, R. Chia, A. Mehta, H. Huang, D. [ebookshop.ro]
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]
[…] 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]
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]
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 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]
Epidemiology
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]
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]
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]
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]
This report highlights that neurologists and ophthalmologists should be aware that patients presenting with topiramate-associated maculopathy should be advised to discontinue topiramate promptly to prevent irreversible loss of vision. [dovepress.com]