MR Imaging of IgG4-Related Disease in the Head and Neck and Brain

Abstract

SUMMARY:IgG4-related disease is characterized by histologic fibrosis with IgG4-positive plasma cell infiltration. Our study evaluated MR imaging features of IgG4-related disease in the head and neck and brain. Images from 15 patients were retrospectively evaluated for the location, signal intensity, and enhancement patterns of lesions. Lacrimal gland enlargement was observed in 8 cases. Other lesions included orbital pseudotumor in 5, pituitary enlargement in 5, and cranial nerve enlargement in 7; the infraorbital nerve was involved in 4. All lesions were hypointense on T2-weighted images, which is typical for IgG4-related lesions. Multiple sites were involved in the head and neck and brain in 11 patients. The diagnosis of IgG4-related disease should be considered in a patient presenting with T2 hypointense lacrimal gland, pituitary, or cranial nerve enlargement, or a T2 hypointense orbital mass, especially if multiple sites in the head and neck are involved in the presence of elevated serum IgG4.ABBREVIATIONS: AIP ϭ autoimmune pancreatitis; HN ϭ head and neck; IgG4 ϭ immunoglobulin G4; IgG4-RD ϭ IgG4-related disease; IPT ϭ inflammatory pseudotumor; MD ϭ Mikulicz disease; SS ϭ Sjö gren syndrome; V1 ϭ first division of the trigeminal nerve (CN V); V2 ϭ second division of the trigeminal nerve

Immunoglobulin G4–Related Disease of the Orbit: Imaging Features in 27 Patients

“…19 Several previous publications regarding IgG4-related disease of the head and neck have focused their attention within the orbit on lacrimal gland enlargement. 4,7,17,18 Although lacrimal gland enlargement was com- mon in this case series, extraocular muscle enlargement was the most common finding. Several authors have also reported the presence of perineural spread of IgG4-related disease, with expansion of the associated neural foramina.…”

“…Several authors have also reported the presence of perineural spread of IgG4-related disease, with expansion of the associated neural foramina. 4,8,9,17,18 Limitations of our study include its retrospective nature. Additionally, the imaging-acquisition parameters were not consistent among all patients.…”

“…[1][2][3][4][5] Although initially described in association with autoimmune pancreatitis, manifestations of IgG4-related disease are now reported in nearly every organ system. [1][2][3][5][6][7][8][9][10] Multiple case reports and small case series of orbital manifestations of IgG4-related disease have noted involvement of the extraocular muscles, lacrimal glands, and infraorbital nerve, 1,2,4,[7][8][9][10][11][12][13][14][15][16][17][18][19] but these small series do not allow evaluation of the typical patterns of imaging findings in IgG4-related disease. The purpose of this study was to retrospectively identify the spectrum of imaging features seen in IgG4-related disease of the orbit.…”

IgG4-related disease: a primer on diagnosis and management

“…55 Parenchymal and structural brain lesions have been described, confirmed by biopsy examination, which showed improvement with glucocorticoids and rituximab. 56 Aside from the impingement of cranial nerves by expanding orbital tumors, the disease can cause hypertrophic pachymeningitis.…”

Immunoglobulin G4-Related Disease in the Head and Neck: Two Case Reports and Literature Review

“…Imaging findings of IgG4-related disease differ based on involved organ. Imaging of IgG4-related disease in head and neck lesions such as lacrimal gland, orbital mass and pituitary reveals hypointense signal in T 2 of MRI, 47 which was observed in MRI of our second patient. So, the diagnosis of IgG4-related disease should be suggested for T 2 -weighted hypointense enhancing lesions of the head and neck such as lacrimal glands, as in our second patient, and further investigations should be performed.…”