IgG4-related disease is a newly described systemic autoimmune and allergic disease, characterized histologically by a fibroinflammatory response with IgG4 plasma cells. It was initially described as affecting the pancreas, but commonly involves the head and neck region as well. While a biopsy is essential for definitive diagnosis, cross sectional imaging may be the initial modality which may suggest this entity. We describe a case of pathologically proven IgG4 related disease which highlights some key radiologic features seen in this entity. Our case highlights some key radiological features of IgG4- related disease in the head and neck, with involvement of the lacrimal glands, pituitary gland and cranial nerves on CT.
Lacrimal gland involvement in granulomatosis with polyangiitis (GPA) commonly accompanies orbital disease, but occasionally may be the sole presentation preceding any other organ manifestation or systemic disease. Diagnosis of orbital GPA, especially in patients with lacrimal involvement as the initial presentation, can be difficult because of nonspecific clinical features and lack of diagnostic specificity on histologic and antineutrophilic cytoplasmic antibody (ANCA) testing. Orbital GPA can be associated with a high morbidity from potential visual loss or rapid progression of latent systemic disease, making early diagnosis important. The purpose of this study was to describe the clinical and imaging features of patients with lacrimal gland involvement secondary to GPA and to compare them with those of other orbital inflammatory conditions in the lacrimal gland fossa.