Management of orbital IgG4-related disease

Glass, Lora R. Dagi; Freitag, Suzanne K.

doi:10.1097/icu.0000000000000204

Cited by 14 publications

(6 citation statements)

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“…The standard treatment for both IgG4-RD and IgG4-ROD is oral steroids,7 14 15 but the disease has a high recurrence rate. Ebbo et al reported that 13 of 19 cases (68.4%) of IgG4-ROD relapsed after the first course of oral steroids 16.…”

Section: Discussionmentioning

confidence: 99%

“…Oral steroid administration is the first-line therapy for IgG4-ROD and patients generally exhibit a good treatment response. However, recurrence occurs in more than half of the patients during oral steroid dose tapering, prolonging treatment and subsequently increasing the risk of steroid-related complications 7–9. Therefore, alternative therapies, including immunosuppressive drugs and rituximab, have been examined and validated 10 11.…”

Section: Introductionmentioning

confidence: 99%

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The natural course of IgG4-related ophthalmic disease after debulking surgery: a single-centre retrospective study

et al. 2019

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ObjectiveThis study aimed to examine the natural course and relapse rate of IgG4-related ophthalmic disease (IgG4-ROD) after debulking surgery in Japanese patients.Methods and analysisThis retrospective review included patients with IgG4-ROD who did not undergo further treatment following debulking surgery. The patients were diagnosed between January 2009 and December 2018 at the Department of Ophthalmology and Pathology, Niigata University Medical and Dental Hospital. The main outcome measures included postoperative IgG4-ROD recurrence rate and differences between patients with and without recurrent disease.ResultsFifteen patients (six male, 9 female; 61.8±16.2 years) were included. Twelve patients (80.0%) had dacryoadenitis disease and three patients (20.0%) had orbital fat tissue disease. About 70%–100% of the lesion was resected in the debulking surgery and the pathological diagnosis was rendered. A definitive diagnosis was made in 13 cases (86.7%) and a probable diagnosis in 2 cases (13.3%). Patients were followed up for 39.0±25.5 months following operation. All patients had lesion volume reduction and patients with dacryoadenitis had eyelid swelling improvement after surgery. Two patients (13.3%) had disease recurrence and six patients (40.0%) had extraophthalmic lesions. There was no statistically significant difference in clinical features between relapsed and non-recurring cases.ConclusionWe observed a 13.3% relapse rate following debulking surgery in patients with IgG4-ROD who did not undergo further treatment. This rate is lower than the documented relapse rate of 30%–70% following oral prednisolone therapy. Therefore, debulking surgery may be a treatment option for IgG4-ROD.

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Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

The natural course of IgG4-related ophthalmic disease after debulking surgery: a single-centre retrospective study

et al. 2019

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“…Unfortunately, the patient's ocular symptoms recurred under oral immunosuppressants including azathioprine, hydroxychloroquine, and prednisolone. Elevated IgG4 level was also detected, which suggested a more complicated clinical situation that might be responsive to higher dose of steroids [13]. The repeated use of IV pulse steroid treatments did not lead to any recurrence of generalized MG, but resulted in full recovery of the ocular symptoms.…”

Section: Discussionmentioning

confidence: 91%

“…Meanwhile, steroid-induced symptom exacerbation should be taken into consideration while initially treating the relatively elderly patients with severe MG [7]. However, the phenomenon of persistent ocular symptoms disappeared after repeated doses of IV pulse steroid, indicating that early deterioration should not preclude this beneficial treatment option during later followups of these patients [13]. We propose that the IV pulse steroid treatments were even more indicated if an IgG4 disease co-exists with MG and thyroid-associated orbitopathy.…”

Section: Resultsmentioning

confidence: 99%

A 49-Year-Old Man with Combined Myasthenia Gravis, Autoimmune Thyroiditis and IgG4 Orbitopathy

Chang¹,

Tsai²,

Chen³

et al. 2017

Transl Biomed

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“…A diagnózis felál-lításában, a jellegzetes klinikai tünetek mellett, az emelkedett IgG 4 -szint segít, de a szövettani vizsgálat minden esetben elengedhetetlen, amely az érintett szervben IgG 4 -pozitív plazmasejt-infiltrációt mutat. Az első vá-lasztandó terápia az orális kortikoszteroid, amelyre álta-lában gyors és látványos javulás észlelhető, míg súlyo-sabb esetekben biológiai kezelésre (rituximab) lehet szükség [11][12][13][14].…”

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Az endokrin orbitopathia differenciáldiagnosztikája

et al. 2016

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Az endokrin orbitopathia a leggyakoribb exophthalmust okozó betegség, a Graves-kór extrathyreoidalis manifesztá-ciója. Mivel a kórkép az esetek legnagyobb részében a hyperthyreosis típusos tüneteivel együtt jelentkezik, általában nem jelent gondot a betegség felismerése. A szerzők az endokrin orbitopathia differenciáldiagnosztikájában szereplő kórképeket foglalják össze a rendelkezésre álló irodalmi adatok áttekintése mellett saját 4 esetük bemutatásával. Kiemelik, hogy endokrin orbitopathiához hasonló tünetek -bár általában könnyen felismerhető a betegség -más kór-képekben is jelentkezhetnek. Az endokrin orbitopathiával foglalkozó szakembereknek ismerni kell a hasonló tünetek-kel járó kórállapotokat, hogy a beteg a megfelelő kezelést időben megkapja. Orv. Hetil., 2016, 157(8), 310-315.Kulcsszavak: endokrin orbitopathia, idiopathiás inflammatorikus orbitopathia, occularis lymphoma, IgG 4 -asszociált betegség, myasthenia gravis Differential diagnosis of Graves' orbitopathy Case reportGraves' orbitopathy is the extrathyroidal manifestation of Graves' disease, which is the most common cause of exophthalmos. As eye symptoms usually coincide with the development of thyrotoxicosis, the diagnosis of the disease is rarely difficult. The aim of the authors was to summarize the differential diagnosis of Graves' orbitopathy based on literature review and presentation of their own four problematic cases on this topic. They conclude that symptoms similar to endocrine orbitopathy are present in other disorders. Endocrinologists need to be aware of these other conditions to avoid treatment failures.Keywords: Graves' orbitopathy, idiopathic inflammatory orbitopathy, ocular lymphoma, IgG4-related disease, myasthenia gravis Erdei, A., Steiber, Z., Gazdag, A., Bodor, M., Berta, E., Szász, R., Szántó, A., Ujhelyi, B., Barna, S., Berényi, E., Nagy, V. E. [Differential diagnosis of Graves' orbitopathy. Case report]. Orv. Hetil., 2016, 157(8), 310-315. (Beérkezett: 2015 elfogadva: 2016. január 7.) Rövidítések CLL = krónikus lymphoid leukaemia; CT = komputertomográfia; EOP = endokrin orbitopathia; IgG 4 = immunglobulin G 4 ; ISZB = ischaemiás szívbetegség; MALT = mucosa associated lymphoid tissue; MRI = magnetic resonance imaging; SPECT = single photon emission computer tomograph; TRAb = TSH-receptor-ellenes antitest; TSH = thyreoideastimuláló hormon Az endokrin orbitopathia (EOP) a Graves-kór leggyakoribb extrathyreoidalis szövődménye és a leggyakoribb exophthalmust okozó kórkép. A szemtünetek az esetek döntő részében (körülbelül 80%-ában) a pajzsmirigy-túl-működés típusos tüneteivel együtt jelentkeznek, így a betegség felismerése általában nem jelent gondot. Az endokrin orbitopathia esetén az autoimmun gyulladás az ESETISMERTETÉS

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Management of orbital IgG4-related disease

Abstract: The management of orbital IgG4-RD will gain from more targeted therapy in the future as the underlying cause is better understood. In the meantime, randomized, controlled trials of varying treatment regimens would be of benefit.

Cited by 14 publications

References 36 publications

The natural course of IgG4-related ophthalmic disease after debulking surgery: a single-centre retrospective study

The natural course of IgG4-related ophthalmic disease after debulking surgery: a single-centre retrospective study

A 49-Year-Old Man with Combined Myasthenia Gravis, Autoimmune Thyroiditis and IgG4 Orbitopathy

Az endokrin orbitopathia differenciáldiagnosztikája

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