Evaluate the Differences in CT Features and Serum IgG4 Levels between Lymphoma and Immunoglobulin G4-Related Disease of the Orbit

Abstract

Background: Benign immunoglobulin G4 (IgG4)-related orbital disease (IgG4-ROD)—characterized as tumors mimicking malignant orbital lymphoma (OL)—responds well to steroids, instead of chemotherapy, radiotherapy and/or surgery of OL. The objective of this study was to report the differences in computed tomography (CT) features and- serum IgG4 levels of IgG4-ROD and OL. Methods: This study retrieved records for patients with OL and IgG4-ROD from a pathology database during an eight-year-and-five-month period. We assessed the differences between 16 OL patients with 27 lesions and nine IgG4-ROD patients with 20 lesions according to prebiopsy CT features of lesions and prebiopsy serum IgG4 levels and immunoglobulin G (IgG) levels This study also established the receiver-operating curves (ROC) of precontrast and postcontrast CT Hounsfield unit scales (CTHU), serum IgG4 levels, serum IgG levels and their ratios. Results: Significantly related to IgG4-ROD (all p < 0.05) were the presence of lesions with regular borders, presence of multiple lesions—involving both lacrimal glands on CT scans—higher median values of postcontrast CTHU, postcontrast CTHU/precontrast CTHU ratios, serum IgG4 levels and serum IgG4/IgG level ratios. Compared to postcontrast CTHU, serum IgG4 levels had a larger area under the ROC curve (0.847 [95% confidence interval (CI): 0.674–1.000, p = 0.005] vs. 0.766 [95% CI: 0.615–0.917, p = 0.002]), higher sensitivity (0.889 [95% CI: 0.518–0.997] vs. 0.75 [95% CI: 0.509–0.913]), higher specificity (0.813 [95% CI: 0.544–0.960] vs. 0.778 [95% CI: 0.578–0.914]) and a higher cutoff value (≥132.5 mg/dL [milligrams per deciliter] vs. ≥89.5). Conclusions: IgG4-ROD showed distinct CT features and elevated serum IgG4 (≥132.5 mg/dL), which could help distinguish IgG4-ROD from OL.

Radiological Determinants of Complicated Immunoglobulin G4–Related Ophthalmic Disease: A Territory-Wide Cohort Study

“…Klingenstein et al 22 reported that there is no radiological difference between these 2 conditions, and it is impossible to differentiate the 2 entities clinically. Yuan et al 38 reported that orbital lymphoma was more commonly unilateral and showed molding features radiologically when compared with Ig-G4-ROD. Yoo et al 39 reported that T2 isointensity on MRI and hyperattenuation on precontrast CT images were observed in some IgG4-mucosa-associated lymphoid tissue lymphoma cases.…”

IgG4-Related Disease

“…Compared with orbital lymphoma, IgG4-ROD presents with regular rather than irregular lesion borders, presents more frequently with lacrimal gland involvement, and with bilateral and multiple lesions according to a CT study from Taiwan 41 . However, another study in German patients did not find any CT imaging differences between both diseases 42 …”

Autoinflammation It Is!

“…This Special Issue on auto-inflammation also contains relevant contribution that have been rarely addressed in such detail elsewhere. To highlight just a few points, one can read about the current options of imaging in auto-inflammation [ 5 ] with special attention to IgG4 related diseases [ 6 ] or about the issue of dysphagia in myositis [ 7 ]. Furthermore, this Special Issue also contains valuable original work, such as studies providing basic data on a novel variant of TNF receptor-associated periodic syndrome [ 8 ], drug hepatotoxicity in the treatment of gouty arthritis [ 9 ], the impact of certain anti-rheumatic drugs on DNA repair [ 10 ], genetic background in association with response to anti-rheumatic drugs [ 11 ], and the impact of IL1-inhibition on the cytokine milieu in adult-onset Still’s disease [ 12 ].…”