Download PDF
Klin Monbl Augenheilkd 2019; 236(01): 25-30
DOI: 10.1055/a-0825-6550
Kasuistik
Georg Thieme Verlag KG Stuttgart · New York

Orbitale Manifestation der IgG4-assoziierten Erkrankung: eine Fallserie

Orbital Manifestation of IgG4-Related Disease: Three Case Reports
Judith Kroll
1   Augenklinik, Augenklinik Bürgerhospital, Frankfurt am Main
,
Susanne Pitz
2   Orbitazentrum, Augenklinik Bürgerhospital, Frankfurt am Main
,
Marc A. Brockmann
3   Klinik und Poliklinik für Neuroradiologie, Johannes Gutenberg-Universität Universitätsmedizin, Mainz
› Author Affiliations
Further Information

Publication History

eingereicht 13 September 2018

akzeptiert 04 December 2018

Publication Date:
28 January 2019 (online)

Also available at
    Permissions and Reprints

Zusammenfassung

Die IgG4-assoziierte Orbitopathie (IgG4-related orbital disease/IgG4 ROD) ist selten und gehört zu der erst in den letzten Jahren beschriebenen Multisystemerkrankung, der IgG4-assoziierten Erkrankung. Differenzialdiagnostisch ist sie von der idiopathischen orbitalen Entzündung, der endokrinen Orbitopathie und von einer orbitalen Neoplasie zu unterscheiden. Die Bildgebung mit MRT und eine Probenentnahme sind entscheidend für die Diagnose. Patienten mit dieser Erkrankung sprechen gut auf Steroide an. Im Folgenden werden 3 klinisch sehr unterschiedliche Manifestationen der orbitalen IgG4-assoziierten Erkrankung vorgestellt.

Abstract

IgG4-related orbital disease is rare. It belongs to an entity of a multisystemic disorder – IgG4-related disease – which has emerged recently. Differential diagnosis comprises idiopathic orbital inflammation, endocrine orbitopathy and orbital neoplasms. MRI imaging and orbital biopsy are essential in making the diagnosis. Patients respond well to systemic steroids. We now describe three clinically markedly different manifestations of IgG4-related orbital disease.

Schlüsselwörter

Orbita - IgG4-assoziierte Erkrankung - orbitale Entzündung - orbitales Lymphom - endokrine Orbitopathie

Key words

orbit - IgG4-related disease - orbital inflammation - orbital lymphoma - endocrine orbitopathy
 

  • Literatur

  • 1 Hamano H, Kawa S, Horiuchi A. et al. High serum IgG4 concentrations in patients with sclerosing pancreatitis. N Engl J Med 2001; 344: 732-738
    CrossrefPubMedGoogle Scholar
  • 2 Yamarnoto M, Ohara M, Suzuki C. et al. Elevated IgG4 concentrations in serum of patients with Mukuliczʼs disease. Scand J Rheumatol 2004; 33: 432-433
    CrossrefPubMedGoogle Scholar
  • 3 Takahira M, Kawano M, Zen Y. et al. IgG4-Related Chronic Sclerosing Dacryoadenitis. Arch Ophthalmol 2007; 125: 1575-1578
    CrossrefPubMedGoogle Scholar
  • 4 Uchida K, Masamune A, Shimosegawa T. et al. Prevalence of IgG4-related disease in Japan based on nationwide survey in 2009. Int J Rheumatol 2012; DOI: 10.1155/2012/358371.
    CrossrefPubMedGoogle Scholar
  • 5 Ebbo M, Patient M, Grados A. et al. Ophthalmic manifestations in IgG4-related disease. Medicine (Baltimore) 2017; 96: e605
    PubMedGoogle Scholar
  • 6 Sogabe Y, Oshima K, Azumi A. et al. Location and frequency of lesions in patients with IgG4-related ophthalmic diseases. Grafes Arch Clin Exp Ophthalmol 2014; 252: 531-538
    CrossrefPubMedGoogle Scholar
  • 7 Deshpande V, Zen Y, Chan JK. et al. Consensus statement on the pathology of IgG4-related disease. Mod Pathol 2012; 25: 1181-1192
    CrossrefPubMedGoogle Scholar
  • 8 Okazaki K, Umehara H. Are classification criteria for IgG4-RD now possible? The concept of IgG4-related disease and proposal of comprehensive diagnostic criteria in Japan. Int J Rheumatol 2012; DOI: 10.1155/2012/357071.
    CrossrefPubMedGoogle Scholar
  • 9 Nishida K, Sogabe Y, Makihara A. et al. Ocular adnexal marginal zone lymphoma arising in a patient with IgG4-related ophthalmic disease. Mod Rheumatol 2016; 11: 1-5
    PubMedGoogle Scholar
  • 10 Cheuk W, Yuen HK, Chan AC. et al. Ocular adnexal lymphoma associated with IgG4+ chronic sclerosing dacryoadenitis: a previously undescribed complication of IgG4-related sclerosing disease. Am J Surg Pathol 2008; 32: 1159-1167
    CrossrefPubMedGoogle Scholar
  • 11 Sato Y, Notohara K, Kojima M. et al. IgG4-related disease: historical overview and pathology of hematological disorders. Pathol Int 2010; 60: 247-258
    CrossrefPubMedGoogle Scholar
  • 12 Andrew N, Kearney D, Selva D. IgG4-related orbital disease: a meta-analysis and review. Acta Ophthalmol 2013; 91: 694-700
    CrossrefPubMedGoogle Scholar
  • 13 Soussan JB, Deschamps R, Sadik JC. et al. Infraobital nerve involment on magnetic resonance imaging in European patients with IgG4- related ophthalmic disease: a specific sign. Eur Radiol 2017; 27: 1335-1343
    CrossrefPubMedGoogle Scholar