RSS-Feed abonnieren
PDF herunterladen
DOI: 10.1055/a-0825-6550
Orbitale Manifestation der IgG4-assoziierten Erkrankung: eine Fallserie
Orbital Manifestation of IgG4-Related Disease: Three Case ReportsPublikationsverlauf
eingereicht 13. September 2018
akzeptiert 04. Dezember 2018
Publikationsdatum:
28. Januar 2019 (online)
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.
-
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
- 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
- 3 Takahira M, Kawano M, Zen Y. et al. IgG4-Related Chronic Sclerosing Dacryoadenitis. Arch Ophthalmol 2007; 125: 1575-1578
- 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;
- 5 Ebbo M, Patient M, Grados A. et al. Ophthalmic manifestations in IgG4-related disease. Medicine (Baltimore) 2017; 96: e605
- 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
- 7 Deshpande V, Zen Y, Chan JK. et al. Consensus statement on the pathology of IgG4-related disease. Mod Pathol 2012; 25: 1181-1192
- 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;
- 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
- 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
- 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
- 12 Andrew N, Kearney D, Selva D. IgG4-related orbital disease: a meta-analysis and review. Acta Ophthalmol 2013; 91: 694-700
- 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