xRead - Nasal Obstruction (September 2024) Full Articles

20426984, 0, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/alr.23262, Wiley Online Library on [02/01/2024]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License

180

KUANetal.

TABLE XIX.B.1 (Continued)

Clinical endpoints 1. Disease

Study

Year LOE Study design Study groups

Conclusions

Gotoet al. 950

1. IgG4-ROD is characterized by multispatial and multitemporal lesion onset, relative responsiveness to steroid treatment, and recurrence following treatment tapering 2. Further studies to develop a classification system of severity specifically for ophthalmic lesions are warranted Common patterns include enlargement of V2 (most often infraorbital) with eosinophilia and multiorgan disease Common patterns include enlargement of V2 (most often infraorbital) with eosinophilia and multiorgan disease Oral steroids are first-line therapy and for those who do not respond or relapse, radiotherapy or other cytotoxic therapies may be considered

2015 5

Expert opinion N/A

characteristics

2. Diagnostic criteria

McNaband

2015 5

Expert opinion N/A

Description of presentation

McKelvie 944

and co-morbid disease

McNaband

2015 5

Expert opinion N/A

Description of presentation

McKelvie 942

and co-morbid disease

Mendenhall and Lessner 977

2010 5

Expert opinion N/A

Expert opinion on clinical progression of OPand radiotherapy Expert opinion on OP evaluation, treatment, and outcomes

Jacobs and Galetta 978 1. Recurrent or steroid-resistant case of presumed OP warrant biopsy 2. Consider radiotherapy if multiply recurrent or refractory Abbreviations: AUC, area under the curve; IgG4-ROD, IgG4-related ophthalmologic disease; NPV, negative predictive value; OP, orbital pseudotumor; PPV, positive predictive value. 2002 5 Expert opinion N/A

is typically more thoroughly defined on MRI. 952 Unfortu nately, the nonspecific nature of these imaging findings makes it difficult to distinguish IgG4-ROD from other more common orbital adnexal lymphoproliferative (OAL) disorders or in rare cases, fungal disease or vasculitis. Machine learning techniques, however, have been recently evaluated as a method to improve diagnostic accuracy. In a retrospective study of 28 OP and 28 OAL patients, Hou et al. found that machine learning reliably distinguished the two entities with a sensitivity, specificity, PPV, and NPV of 71%, 91%, 89%, and 76%, respectively. In the same study, expert radiologists reliably distinguished the two disease entities with 75%, 68%, 70%, and 73% sensitivity, specificity, PPV, and NPV, respectively. 953 Another retrospective study of 21 OP and 16 OAL patients utilized a combination of tumor blood flow and apparent diffusion coefficient (ADC) on MRI to differentiate OP from OAL with 90% sensitivity, 93% specificity, 95% PPV, 88% NPV, and 91% accuracy. 954 Radi ologic findings may have some prognostic significance in OP. A relatively large study evaluating 89 patients with OP demonstrated that the 12 patients with infraorbital nerve

has been demonstrated in up to 88% of patients in one radiographic review of 65 histologically confirmed cases. 949 Histopathologic examination of affected tissue is neces sary to establish the diagnosis, and cellular makeup reveals marked lymphocytic and plasmacytic infiltration, fibro sis (often “storiform”), a ratio of IgG4 + plasmacytes to IgG + plasmacytes of more than 40%, and more than 400 IgG4 + cells per mm 2 . “Definitive” IgG4-ROD is defined as (A) enlargement of periorbital tissue including the trigeminal nerve on imaging, (B) the previously men tioned histopathologic findings, and (C) a serum IgG4 ≥ 135 mg/dL. “Probable” disease is defined as a combina tion of points A and B, while “possible” disease is defined as having met points A and C. 950,951 Many radiographic studies have focused on OP as an entity prior to recent pathologic classification as IgG4 ROD. Contrast-enhanced MRI with fat suppression is acknowledged to be superior to CT in the initial evalua tion. Neither modality has findings specific for IgG4-ROD; however, an enhancing focal or infiltrating orbital mass