Idiopathic orbital inflammation with sclerosing mesenteritis: a new association?

Abstract

A 70-year-old man, who was recently diagnosed with sclerosing mesenteritis following an abdominal biopsy, presented with an acute onset of left upper eyelid swelling, moderate ptosis, mild chemosis and restriction of movements. A computed tomography scan showed an enlarged lateral rectus muscle with surrounding soft tissue changes. A diagnosis of orbital inflammation manifesting as myositis was made and the patient was commenced on high-dose prednisolone, which showed a rapid response. It is believed that this may be a new association similar to that previously reported with retroperitoneal fibrosis and orbital inflammation.

Sclerosing Orbital Inflammation and Systemic Disease

“…Of the non-MFF cases, orbital location was reported in 30 cases and was quite varied. The most common locations were diffuse/apical (11) and the inferior/temporal quadrants (8). Only 6 of the 30 non-MFF cases had lacrimal gland involvement and, of those, 3 (50%) were associated with systemic inflammation other than MFF (Table 3).…”

“…Since then, this entity has been described in several case reports, and its name was been shortened to "multifocal fibrosclerosis." [7][8][9][10][11][12][13][14] Systemic involvement, particularly in the retroperitoneum and mediastinum, is not altogether uncommon in the setting of a range of fibrotic inflammatory, histiocytic, and xanthogranulomatous orbital diseases, including Erdheim-Chester disease, Rosai-Dorfman disease, hyper-IgG-4 disease, and multifocal fibrosclerosis. In systemic xanthogranulomatous, histiocytic, and IgG4-related disease, orbital involvement is frequently bilateral.…”

Orbital pseudotumor: Distinct diagnostic features and management

“…The etiology of orbital pseudotumor is unknown, but infection, autoimmune disorder, and aberrant wound healings have been put forward as possibilities 2. The disorder has also been associated with infectious diseases such as Streptococcal pharyngitis, viral upper respiratory infection and Borrelia burgdorferi infection 9102223. Pathological findings may be non-specific and may only reveal benign lymphoid hyperplasia and inflammatory cell infiltration with necrotizing vasculitis 24.…”

Idiopathic retroperitoneal fibrosis presenting with recurrent bilateral uveitis

“…RPF has been associated with other ocular disorders including episcleritis, orbital pseudotumour and xanthomatosis 13–15. To our knowledge, this constitutes the second report in the English literature of RPF associated with isolated uveitis, in the absence of an underlying systemic disorder 7.…”