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]

• Cervical Lymphadenopathy

  In the preceding 14 years, he underwent multiple biopsies for bilateral submandibular gland enlargement and right cervical lymphadenopathy, which were reported as reactive lymphoid hyperplasia with a suspicion of Hodgkin’s disease. [doi.org]

• 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]

• Hunting

  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]

  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]

  The presence of p-ANCA antibody in serum strongly supports diagnosis. [5] (V) Tolosa-Hunt syndrome This is characterized by unilateral (mostly) painful ophthalmoplegia due to granulomatous lesion at or near the orbital apex that responds dramatically [meajo.org]

• Recurrent Sinusitis

  Five patients had a history of asthma, and 3 patients had a history of recurrent sinusitis. [doi.org]

• Fever of Unknown Origin

  After the investigation for a fever of unknown origin, IgG4-related aseptic meningitis was diagnosed with a cerebrospinal fluid (CSF) examination and bone marrow biopsy. [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]

• Conjunctival Injection

  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]

• Ciliary Injection

  A slit-lamp examination revealed bilateral conjunctival/ciliary injection, scleritis, and 2+ anterior chamber cells (Figure 1a, b). [austinpublishinggroup.com]

• 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]

• 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]

• Cranial Nerve Involvement

  Sir, IgG4-related disease is a recently defined condition which may result in multi-organ involvement. 1 Cranial nerve involvement has rarely been reported. We describe a case of infraorbital nerve enlargement (IONE) due to IgG4-related disease. [doi.org]

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. [doi.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]

• 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, http://links.lww.com/MD/B594 ). 3.5 [journals.lww.com]

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]

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]

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 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]

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]

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]