Match!

IgG4-related disease causing facial nerve and optic nerve palsies: Case report and literature review

Published on Nov 1, 2016in American Journal of Otolaryngology0.93
· DOI :10.1016/j.amjoto.2016.08.005
Cameron C. Wick5
Estimated H-index: 5
(UTSW: University of Texas Southwestern Medical Center),
Joseph Zachariah1
Estimated H-index: 1
(St Mary's Hospital)
+ 5 AuthorsCliff A. Megerian28
Estimated H-index: 28
Cite
Abstract
Abstract IgG4-related disease (IgG4-RD) is increasingly being recognized as an entity effecting the head and neck region. Although most commonly seen with salivary gland or paranasal sinus involvement, IgG4-RD may also involve the temporal bone and skull base. We report a rare care of a 61-year-old female with IgG4-RD presenting as synchronous lesions of the middle ear and middle cranial fossa with polyneuropathy of cranial nerves II, VI, and VII. Initial histopathological evaluation of her resected ear mass suggested a benign inflammatory process but no specific diagnosis. Her symptoms progressed over 10 months prompting re-evaluation of the specimen and consideration of the IgG4-RD diagnosis. Key pathologic features included prominent lymphoplasmacytic population, storiform fibrosis, obliterative phlebitis, and IgG4 specific staining. The patient was treated with high-dose intravenous and oral steroids but was transitioned to azathioprine secondary to steroid-induced myopathy. Radiographic studies before and after treatment reveal marked improvement of the intracranial and extracranial disease. Correspondingly, her cranial neuropathies resolved. A high degree of clinical suspicion is necessary to diagnosis IgG4-RD. The diagnosis can be supported by elevated serum IgG, elevated IgG index, and pathognomonic histopathological findings. Primary treatment is with corticosteroids. However, immunotherapy using azathioprine or rituximab can be utilized in recurrent disease or patients with steroid intolerance.
  • References (44)
  • Citations (4)
Cite
References44
Newest
Published on Oct 1, 2016in Joint Bone Spine3.28
Gyuhwan Hwang1
Estimated H-index: 1
(SCH: Soonchunhyang University),
Sy Jin12
Estimated H-index: 12
(SCH: Soonchunhyang University),
Hyun-Sook Kim4
Estimated H-index: 4
(SCH: Soonchunhyang University)
Joint Bone Spine - In Press.Proof corrected by the author Available online since vendredi 8 avril 2016
Published on May 1, 2016in American Journal of Otolaryngology0.93
Wai Keat Wong1
Estimated H-index: 1
,
Randall P. Morton12
Estimated H-index: 12
(University of Auckland)
Abstract IgG4-related disease (IgG4-RD) is a novel clinicopathological entity characterised by elevated tissue levels of IgG4-positive plasma cells. It can present in almost every organ systems. We present a case of a 48 year-old man with recurrent intra-orbital and cervical lymph node swelling and found to have greater auricular nerve involvement intraoperatively during open surgical biopsy. Histopathological evaluation of biopsied specimens from these lesions yielded IgG4-positive plasma cell ...
Published on Nov 1, 2015in Laryngoscope2.34
Eleonora F. Spinazzi3
Estimated H-index: 3
,
Stuti V. Desai3
Estimated H-index: 3
+ 4 AuthorsJean Anderson Eloy37
Estimated H-index: 37
(RU: Rutgers University)
Objective Inflammatory pseudotumor (IPT) of the lateral skull base is a rare nonneoplastic inflammatory process of unknown cause often mistaken for malignancy or infection. This systematic review aims to analyze all reported cases of lateral skull base IPT to date in order to provide insight into the management of this uncommon lesion. Data Sources MEDLINE/PubMed database. Review Methods The MEDLINE/PubMed databases were searched for articles related to lateral skull base IPT. A bibliography rev...
Published on Sep 1, 2015in Laryngoscope2.34
Xia Hong4
Estimated H-index: 4
,
Zhi-peng Sun4
Estimated H-index: 4
+ 12 AuthorsL. Zhang8
Estimated H-index: 8
Objectives/Hypothesis To further recognize the comorbid diseases of immunoglobulin G4-related sialadenitis (IgG4-RS) in the head and neck region and to observe the response of these conditions to immunomodulatory therapy. Study Design Retrospective review. Methods The symptoms of comorbid diseases and medical histories in 51 patients (24 men, 27 women; median age, 55 years) diagnosed with IgG4-RS were analyzed. Thirty-six patients received immunomodulatory therapy and were followed up for 10.4 ±...
Published on Sep 1, 2015in Ear, nose, & throat journal
Masaru Kojima35
Estimated H-index: 35
,
Yoshimasa Nakazato9
Estimated H-index: 9
+ 2 AuthorsNaoya Nakamura28
Estimated H-index: 28
We report 2 rare cases of IgG4-related plasma cell granuloma of the maxillary sinus. Histologically, both lesions were characterized by severe lymphoplasmacytic infiltration. In 1 case, fibrous sclerosis and obliterative arteritis were observed. Immunohistochemical study demonstrated that IgG4-positive cells comprised more than 40% of the IgG-positive plasma cells. Moreover, the serum IgG4 level was elevated in both cases. A good responsiveness to steroid therapy has been seen in IgG4-related di...
Published on Sep 1, 2015in Otolaryngology-Head and Neck Surgery2.31
Allyson L. Hughes1
Estimated H-index: 1
(UK: University of Kentucky),
Brett T. Comer5
Estimated H-index: 5
(UK: University of Kentucky)
Published on Sep 1, 2015in Otology & Neurotology2.06
Jennifer C. Alyono3
Estimated H-index: 3
,
Yangyang Shi1
Estimated H-index: 1
+ 4 AuthorsCarleton Eduardo Corrales3
Estimated H-index: 3
ObjectiveTo describe the presentation, treatment, and outcome of inflammatory pseudotumors (IPs) of the skull base.Data SourcesEnglish-language articles in PubMed, Web of Science, and EMBASE from earliest available through April 2014.Study SelectionArticles were identified using a keyword search for
Published on Sep 1, 2015in Arthritis & Rheumatism9.00
Zachary S. Wallace17
Estimated H-index: 17
(Harvard University),
Vikram V. Deshpande85
Estimated H-index: 85
(Harvard University)
+ 4 AuthorsJohn H. Stone67
Estimated H-index: 67
(Harvard University)
Objective IgG4-related disease (IgG4-RD) is an immune-mediated fibroinflammatory condition that can affect nearly any organ. Prior studies have focused on individual cases of IgG4-RD or small case series. This study was undertaken to report detailed clinical and laboratory findings in a larger group of patients with IgG4-RD whose diagnosis was established by strict clinicopathologic correlation. Methods The baseline features of 125 patients with biopsy-proven IgG4-RD were reviewed. The diagnosis...
Published on Jul 1, 2015in Arthritis & Rheumatism9.00
Arezou Khosroshahi25
Estimated H-index: 25
(Emory University),
Zachary S. Wallace17
Estimated H-index: 17
(Harvard University)
+ 39 AuthorsHiroshi Goto24
Estimated H-index: 24
(TMU: Tokyo Medical University)
A. Khosroshahi, Z. S. Wallace, J. L. Crowe, T. Akamizu, A. Azumi, M. N. Carruthers, S. T. Chari, E. Della-Torre, L. Frulloni, H. Goto, P. A. Hart, T. Kamisawa, S. Kawa, M. Kawano, M. H. Kim, Y. Kodama, K. Kubota, M. M. Lerch, M. L€ ohr, Y. Masaki, S. Matsui, T. Mimori, S. Nakamura, T. Nakazawa, H. Ohara, K. Okazaki, J. H. Ryu, T. Saeki, N. Schleinitz, A. Shimatsu, T. Shimosegawa, H. Takahashi, M. Takahira, A. Tanaka, M. Topazian, H. Umehara, G. J. Webster, T. E. Witzig, M. Yamamoto, W. Zhang, T....
Published on Jun 1, 2015in Annals of the Rheumatic Diseases14.30
Mollie N. Carruthers15
Estimated H-index: 15
(Harvard University),
Topazian1
Estimated H-index: 1
(Mayo Clinic)
+ 7 AuthorsJohn H. Stone67
Estimated H-index: 67
(Harvard University)
Objectives To evaluate the efficacy of rituximab (RTX) in IgG4-related disease (IgG4-RD) in an open-label pilot trial. Methods We treated 30 IgG4-RD patients with two doses of RTX (1000 mg each). The participants were either treated with RTX alone (n = 26; 87%) or required to discontinue baseline glucocorticoids (GC) within 2 months (n = 4; 13%). Disease activity was measured by the IgG4-RD Responder Index (IgG4-RD RI) and physician9s global assessment (PGA). Disease response was defined as the ...
Cited By4
Newest
Published on May 1, 2019in Seminars in Arthritis and Rheumatism5.07
Cléa Melenotte5
Estimated H-index: 5
,
Julie Seguier1
Estimated H-index: 1
+ 20 AuthorsLaurent Daniel30
Estimated H-index: 30
Abstract Pachymeningitis is rare, either idiopathic or secondary to inflammatory disorders, after tumoral, surgical or infectious causes have been excluded. The fibroinflammatory IgG4-related disease is one of the etiologies of pachymeningitis with only few cases reported yet. From a single referral regional center, we evaluated the frequency of IgG4-related disease as the cause of inflammatory pachymeningitis in 10% of cases. From a National case registry of IgG4-related disease the pachymening...
Published on Feb 1, 2018in Lancet Neurology28.75
Mahmoud A. AbdelRazek1
Estimated H-index: 1
(Harvard University),
Nagagopal Venna16
Estimated H-index: 16
(Harvard University),
John H. Stone67
Estimated H-index: 67
(Harvard University)
Summary IgG4-related disease can involve nearly any organ system, including the central and peripheral nervous systems. The pathology findings are consistent from organ to organ, but careful clinicopathological correlation is necessary to establish the diagnosis. Many non-neurological and neurological inflammatory conditions, previously regarded as idiopathic in nature, are now recognised to fall within the spectrum of IgG4-related disease. The condition is highly treatable, but probably remains...
Published on Jan 1, 2018in Clinical Radiology2.08
A. Thompson1
Estimated H-index: 1
(RPH: Royal Perth Hospital),
A. Whyte1
Estimated H-index: 1
(University of Melbourne)
IgG4-related disease is a systemic, inflammatory disorder typically involving multiple organ systems. Several eponymous conditions described previously in the clinical and radiology literature are now recognised to be part of the IgG4-related disease spectrum. This includes multiple manifestations in the head and neck region, which are the subject of this review. Imaging can occasionally suggest the specific diagnosis of IgG4 disease. More commonly, it will be included in a limited differential ...
Published on Jan 1, 2018in SAGE open medical case reports
Carolina Wuesthoff (Kolling Institute of Medical Research), Alexandra Allende (Macquarie University), Nirmal Patel3
Estimated H-index: 3
(Kolling Institute of Medical Research)
In recent years, an immune-mediated disorder involving IgG4 has been described, which targets multiple organs and explains a number of disorders previously regarded as “idiopathic” or of unknown origin. Furthermore, the discovery of IgG4-related disease (IgG4-RD) has placed a number of pathologies within its spectrum, linking symptoms and conditions formerly considered isolated. Reports of the manifestations of IgG4-RD in the head and neck are scarce. Otological manifestations have been reported...
Published on Apr 1, 2017in Current Treatment Options in Neurology3.57
Bernardo Baptista1
Estimated H-index: 1
,
Alina Casian6
Estimated H-index: 6
(Guy's and St Thomas' NHS Foundation Trust)
+ 2 AuthorsClaire M Rice2
Estimated H-index: 2
(Southmead Hospital)
IgG4-related disease (IgG4-RD) is a multisystem inflammatory disorder. Early recognition of IgG4-RD is important to avoid permanent organ dysfunction and disability. Neurological involvement by IgG4-RD is relatively uncommon, but well recognised—hypertrophic pachymeningitis and hypophysitis are the most frequent manifestations. Although the nervous system may be involved in isolation, this more frequently occurs in conjunction with involvement of other systems. Elevated circulating levels of IgG...
View next paperIgG4-Related Disease Manifesting as Middle Cranial Fossa Mass: A Case Report (P5.165)