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Journal of Ophthalmic Inflammation and Infection

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Open Access 01-12-2017 | Review

IgG4-related disease presenting as panuveitis without scleral involvement

Authors: Kinda Najem, Larissa Derzko-Dzulynsky, Edward A. Margolin

Published in: Journal of Ophthalmic Inflammation and Infection | Issue 1/2017

Abstract

Background

The following case emphasizes the importance of including IgG4-related disease (RD) in the differential diagnosis of intraocular inflammation and multiple cranial nerve palsies.

Results

A 33-year-old man, with a history of idiopathic bilateral panuveitis, presented with a new right pupillary-sparing partial third nerve palsy, which spontaneously resolved in 2 weeks, but was followed 1 month later, by a right sixth nerve palsy, which also resolved within a few weeks. Motility disturbance was accompanied by a decrease in the central acuity in the right eye. Magnetic resonance imaging/angiography (MRI/MRA) demonstrated a densely enhancing osteodestructive skull base process extending through the cavernous sinus and into the right superior orbital fissure. Biopsy of the lesion was consistent with IgG4-related disease (RD).

Conclusions

This is the first reported case of IgG4-RD associated panuveitis without scleral involvement, expanding the list of clinical manifestations of the IgG4-RD.

Lapadula G, Marchesoni A, Armuzzi A, Blandizzi C, Caporali R, Chimenti S, Cimaz R, Cimino L, Gionchetti P, Girolomoni G, Lionetti P, Marcellusi A, Mennini FS, Salvarani C (2014) Adalimumab in the treatment of immune-mediated diseases. Int J Immunopathol Pharmacol 27:33–48CrossRefPubMed

Sim JK, Lee SY, Shim JJ, Kang KH (2016) Pulmonary sarcoidosis induced by adalimumab: a case report and literature review. Yonsei Med J 57:272–273CrossRefPubMed

Korta DZ, Ochieng P, Fishman D, Katz SE (2015) Pulmonary sarcoidosis and latent tuberculosis in a patient with psoriasis treated with adalimumab. Dermatol Online J. 21.

Stone JH, Zen Y, Deshpande V (2012) IgG4-related disease. N Engl J Med 366:539–551CrossRefPubMed

McNab AA, McKelvie P (2015) IgG4-related ophthalmic disease part I: background and pathology. Ophthal Plast Reconstr Surg 31:83–88CrossRefPubMed

Hamano H, Kawa S, Horiuchi A, Unno H, Furuya N, Akamatsu T, Fukushima M, Nikaido T, Nakayama K, Usuda N, Kiyosawa K (2001) High serum IgG4 concentrations in patients with sclerosing pancreatitis. N Engl J Med 344:732–738CrossRefPubMed

Ohno K, Sato Y, Ohshima K-I, Takata K, Ando M, Abd Al-Kader L, Iwaki N, Takeuchi M, Orita Y, Yoshino T (2012) IgG4-related disease involving the sclera. Mod Rheumatol. doi:10.1007/s10165-012-0758-y

Saeki T, Saitol A, Hiura T, Yamazaki H, Emura I, Ueno M, Miyamura S, Gejyo F (2006) Lymphoplasmacytic infiltration of multiple organs with immunoreactivity for IgG4: IgG4-related systemic disease. Intern Med 45:163–167CrossRefPubMed

Takahira M, Kawano M, Zen Y, Minato H, Yamada K, Sugiyama K (2007) IgG4-related chronic sclerosing dacryoadenitis. Arch Ophthalmol 125:1575–1578CrossRefPubMed

10.

Deshpande V, Zen Y, Chan JK, Yi EE, Sato Y, Yoshino T, Klöppel G, Heathcote JG, Khosroshahi A, Ferry JA, Aalberse RC, Bloch DB, Brugge WR, Bateman AC, Carruthers MN, Chari ST, Cheuk W, Cornell LD, Fernandez-Del Castillo C, Forcione DG, Hamilos DL, Kamisawa T, Kasashima S, Kawa S, Kawano M, Lauwers GY, Masaki Y, Nakanuma Y, Notohara K, Okazaki K, Ryu JK, Saeki T, Sahani DV, Smyrk TC, Stone JR, Takahira M, Webster GJ, Yamamoto M, Zamboni G, Umehara H, Stone JH (2012) Consensus statement on the pathology of IgG4-related disease. Mod Pathol 137:699–711

11.

Umehara H, Okazaki K, Masaki Y, Kawano M, Yamamoto M, Saeki T, Matsui S, Yoshino T, Nakamura S, Kawa S, Hamano H, Kamisawa T, Shimosegawa T, Shimatsu A, Nakamura S, Ito T, Notohara K, Sumida T, Tanaka Y, Mimori T, Chiba T, Mishima M, Hibi T, Tsubouchi H, Inui K, Ohara H (2012) Comprehensive diagnostic criteria for IgG4-related disease. Mod Rheumatol 22:21–30CrossRefPubMed

12.

Carruthers MN, Khosroshahi A, Augustin T, Deshpande V, Stone JH (2015) The diagnostic utility of serum IgG4 concentrations in IgG4-related disease. Ann Rheum Dis 74:14–18CrossRefPubMed

13.

Lindfield D, Attfield K, McElvanney A (2012) Sytemic immunoglobulin G4 (IgG4) disease and idiopathic orbital inflammation; removing “idiopathic” from nomenclature. Eye 26:623–629CrossRefPubMedPubMedCentral

14.

Sogabe Y, Ohshima K, Azumi A, Takahira M, Kase S, Tsuji H, Yoshikawa H, Nakamura T (2014) Location and frequency of lesions in patients with IgG4-related ophthalmic diseases. Graefes Arch Clin Exp Ophthalmol 252:531–538CrossRefPubMed

15.

Paulus YM, Cocklerham KP, Cockerham GC, Gratzinger D (2012) IgG4-positive sclerosing orbital inflammation involving the conjunctiva: a case report. Ocul Immunol Inflamm 20:375–377CrossRefPubMed

16.

Lee CS, Harocopos GJ, Kraus CL, Lee AY, Van Stavern GP, Couch SM, Rao PK (2015) IgG-associated orbital and ocular inflammation. J Ophthalmic Inflamm Infect 5:1–8CrossRef

17.

McNab AA, McKelvie P (2015) IgG4-related ophthalmic disease part II: clinical aspects. Ophthal Plast Reconstr Surg 31:1–12CrossRef

18.

Michel L, Clairand R, Néel A, Masseau A, Frampas E, Hamidou M (2011) Association of IgG4-related disease and sarcoidosis. Thorax 66:920–921CrossRefPubMed

19.

Mudhar HS, Bhatt R, Sandramouli S (2011) Xanthogranulomatous variant of immunoglobulin G4 sclerosing disease presenting as ptosis, proptosis and eyelid skin plaques. Int Ophthalmol 31:245–248CrossRefPubMed

20.

Singh K, Rajan KDA, Eberhart C (2010) Orbital necrobiotic xanthogranuloma associated with systemic IgG4 disease. Ocul Immunol Inflamm 18:373–378CrossRefPubMed

21.

Chang Y, Keogh KA, Lewis JE, Ryu JH, Cornell LD, Garrity JA, Yi ES (2013) IgG4-positive plasma cells in granulomatosis with polyangiitis (Wegener’s): a clinicopathologic and immunohistochemical study on 43 granulomatosis with polyangiitis and 20 control cases. Hum Pathol 44:2432–2437CrossRefPubMed

22.

Cheuk W, Yuen HK, Chan AC, Shih LY, Kuo TT, Ma MW, Lo YF, Chan WK, Chan JK (2008) Ocular adnexal lymphoma associated with IgG4+ chronic sclerosing dacryoadenitis: a previously undescribed complication of IgG4-related sclerosing disease. Am J Surg Pathol 32:1159–1167CrossRefPubMed

23.

Oyama T, Takizawa J, Nakamura N, Aoki S, Aizawa Y, Abe H (2011) Multifocal mucosa-associated lymphoid tissue lymphoma associated with IgG4-related disease; a case report. Jpn J Ophthalmol 55:304–306CrossRefPubMed

24.

Sato Y, Ohshima K, Takata K, Huang X, Cui W, Ohno K, Yoshino T (2012) Ocular adnexal IgG4-producing mucosa-associated lymphoid tissue lymphoma mimicking IgG4-related disease. J Clin Exp Hematop 52:51–55CrossRefPubMed

25.

Kase S, Yamamoto T, Ishijima K, Noda M, Ishida S (2013) Spontaneous regression of IgG4-related dacryoadenitis. Mod Rheumato 23:1018–1021CrossRef

26.

Kohsroshahi A, Stone JH (2011) Treatment approaches to IgG4-related systemic disease. Curr Opin Rheumatol 23:67–71CrossRef

27.

Kohsroshahi A, Bloch DB, Deshpande V, Stone JH (2010) Rituximab therapy leads to rapid decline of serum IgG4 levels and prompts clinical improvement in IgG4-related systemic disease. Arthritis Rheum 62:1755–1762CrossRef

28.

Kohsroshahi A, Carruthers MN, Deshpande V, Unizony S, Bloch DB, Stone JH (2012) Rituximab for the treatment of IgG4-related systemic disease: lessons from 10 consecutive patients. Medicine (Baltimore) 91:57–66CrossRef

29.

Kubota T, Moritani S, Katayama M, Terasaki H (2010) Ocular adnexal IgG4-related lymphoplasmacytic infiltrative disorder. Arch Ophthalmol 128:577–584CrossRefPubMed

Title: IgG4-related disease presenting as panuveitis without scleral involvement
Authors: Kinda Najem
Larissa Derzko-Dzulynsky
Edward A. Margolin
Publication date: 01-12-2017
Publisher: Springer Berlin Heidelberg
Published in: Journal of Ophthalmic Inflammation and Infection / Issue 1/2017
Electronic ISSN: 1869-5760
DOI: https://doi.org/10.1186/s12348-017-0125-4

Keynote webinar | Spotlight on medication adherence

Springer Medicine

IgG4-related disease presenting as panuveitis without scleral involvement

Abstract

Background

Results

Conclusions

Keynote webinar | Spotlight on medication adherence

Springer Medicine

Abstract

Background

Results

Conclusions

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