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ICAR SINONASAL TUMORS

TABLE XIX.B.1 Evidence surrounding IgG4-related ophthalmic disease (formerly idiopathic orbital inflammation/orbital pseudotumor).

Clinical endpoints 1. Clinical features 2.

Study

Year LOE Study design Study groups

Conclusions

Wuet al. 947

No gender predilection, bilateral in > 50% of cases, steroid-responsive but recurrent

2015 2

Systematic review

Biopsy-confirmed

cases of IgG4-ROD with available histopathologic features ( n = 172)

Histopathologic features

Suhlr et al. 967

2014 2

Double

Orbital inflammation patients refractory to systemic corticosteroids and at least one other immunosuppressant ( n = 10) Cases of “definite” or “probable” IgG4-ROD identified in the literature ( n = 88)

1. Validated

1. Rituximab was safe and effective in seven out of 10 refractory OP patients, without toxicity 2. Rituximab should be considered in steroid-refractory orbital inflammation Compared to pancreatic IgG4-RD, IgG4-ROD affects younger patients and is associated with salivary gland lesions Periocular tissue other than the lacrimal gland may be affected in IgG4-ROD 1. IgG4-ROD had fewer symptoms and longer duration at presentation than IgG4-negative orbital inflammatory pseudotumor 2. IgG4 positivity portended a greater rate of recurrence in the subgroup of patients treated with surgery plus oral glucocorticoids IgG4-ROD more likely to have storiform fibrosis, plasmacytic infiltrate, and periphlebitis Histopathologic findings of collagenous fibrosis, count and ratio of IgG4-positive plasma cells, and anatomic location of lesion (lacrimal gland) all point to IgG4-ROD; in their absence, OP is likely IgG4-ROD more frequent in patients with infraorbital nerve involvement and bilateral disease

blinded RCT

orbital disease grading scale 2. Corticosteroid dose reduction

Andrew et al. 943 2013 2

Clinical features

Systematic

reviewand meta analysis

Gotoet al. 946

2021 3

Retrospective cohort

Patients diagnosed with IgG4-ROD ( n = 378) Idiopathic orbital inflammatory

Clinical features

Chenet al. 948

1. Clinical features 2. Recurrence

2021 3

Retrospective cohort

disease patients with and without IgG4-positive histopathology ( n = 165)

Abadet al. 951

2019 3

Prospective cohort

Patients with orbital tumors and histologically

1. IgG4 positivity onbiopsy 2. Other histopatho logic features

documented ( n = 35)

Minet al. 956

Histopathologic features

2019 3

Retrospective cohort

Orbital inflammatory pseudotumor patients diagnosed via surgical pathology ( n = 16)

Aryasit et al. 957

2021 4

Retrospective case series

Patients with a

1. Clinical features 2.

histopathologic diagnosis of idiopathic orbital inflammation ( n = 45)

Histopathologic features

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