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

Published on Nov 1, 2016in American Journal of Otolaryngology0.932
· DOI :10.1016/j.amjoto.2016.08.005
Cameron C. Wick6
Estimated H-index: 6
(UTSW: University of Texas Southwestern Medical Center),
Joseph Zachariah1
Estimated H-index: 1
(St Mary's Hospital)
+ 5 AuthorsCliff A. Megerian29
Estimated H-index: 29
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 (5)
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References44
Newest
#1Gyuhwan Hwang (SCH: Soonchunhyang University)H-Index: 1
#2Sy Jin (SCH: Soonchunhyang University)H-Index: 12
Last. Hyun-Sook Kim (SCH: Soonchunhyang University)H-Index: 5
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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 ...
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#2Stuti V. DesaiH-Index: 3
Last. Jean Anderson Eloy (RU: Rutgers University)H-Index: 39
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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...
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Last. Arias MH-Index: 1
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Introduccion. La paquimeningitis hipertrofica es un trastorno infrecuente que produce un engrosamiento focal o difuso de la duramadre. Puede ser idiopatica o secundaria a procesos infecciosos, autoinmunes o neoplasicos. La recientemente descrita ‘enfermedad relacionada con IgG4’ podria ser la causa de bastantes cuadros considerados criptogenicos. Caso clinico. Mujer de 54 anos, con historia de asma bronquial, que consulto por cefalea, vertigo y perdida de audicion por su oido izquierdo. En la re...
1 CitationsSource
#1Masaru KojimaH-Index: 35
#2Yoshimasa NakazatoH-Index: 9
Last. Naoya NakamuraH-Index: 28
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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...
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#1Jennifer C. AlyonoH-Index: 4
#2Yangyang ShiH-Index: 2
Last. C. Eduardo CorralesH-Index: 12
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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
6 CitationsSource
#1Zachary S. Wallace (Harvard University)H-Index: 18
#2Vikram Deshpande (Harvard University)H-Index: 90
Last. John H. Stone (Harvard University)H-Index: 70
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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...
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#1Xia HongH-Index: 4
#2Zhipeng SunH-Index: 7
Last. Guang-Yan YuH-Index: 8
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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 ±...
7 CitationsSource
#1Allyson L. Hughes (UK: University of Kentucky)H-Index: 1
#2Brett T. Comer (UK: University of Kentucky)H-Index: 5
1 CitationsSource
#1Arezou Khosroshahi (Emory University)H-Index: 26
#2Zachary S. Wallace (Harvard University)H-Index: 18
Last. John H. Stone (Harvard University)H-Index: 70
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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....
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#1John P. Marinelli (Mayo Clinic)H-Index: 3
#2Chiara Marvisi (University of Modena and Reggio Emilia)H-Index: 2
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OBJECTIVE: IgG4-related disease (IgG4-RD) is a recently recognized disease characterized by fibroinflammatory infiltrates rich in IgG4+ plasma cells that can present as isolated tumor-like lesions of the head and neck. The objective of the current study was to describe the cranial base manifestations of IgG4-RD. METHODS: Review of all cases at three tertiary-referral centers since disease description in 2003. RESULTS: Eleven patients were identified at a median age at presentation of 58 years (I...
Source
#1Cléa Melenotte (AMU: Aix-Marseille University)H-Index: 8
#2Julie Seguier (AMU: Aix-Marseille University)H-Index: 2
Last. Nicolas Schleinitz (AMU: Aix-Marseille University)H-Index: 28
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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...
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#1Carolina Wuesthoff (Kolling Institute of Medical Research)H-Index: 1
#2Alexandra Allende (Macquarie University)H-Index: 1
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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 or...
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#1Mahmoud A. AbdelRazek (Harvard University)H-Index: 1
#2Nagagopal Venna (Harvard University)H-Index: 16
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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...
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#1A. Thompson (RPH: Royal Perth Hospital)H-Index: 1
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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 ...
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#1Bernardo BaptistaH-Index: 1
#2Alina Casian (Guy's and St Thomas' NHS Foundation Trust)H-Index: 7
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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...
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