Orbital Inflammatory Complications of Crohn’s Disease: A Rare Case Series

Abstract

Orbital inflammatory disease is a rare ophthalmic manifestation of Crohn’s disease. Inflammation is characteristically nonspecific, involving one or multiple structures of the orbit. Mechanisms of disease and optimal methods of treatment are poorly understood. The aim of this report is to present 3 cases of orbital involvement in Crohn’s disease. A retrospective case note review of patients with orbital inflammatory disease and Crohn’s disease was performed at our academic center to determine the clinical, imaging, and histopathologic features of this condition and its relationship to intestinal Crohn’s disease. Three patients were identified with orbital inflammatory manifestations complicating Crohn’s disease. All patients described were women with active intestinal disease and had a history of treatment with immunosuppressive therapies. Similarities were observed in clinical presentations with variance noted in radiologic and histopathologic findings. In all cases, symptoms improved with oral corticosteroids or nonsteroidal drugs in combination with anti-tumor necrosis factor agents. Inflammatory bowel disease–related orbital complications are rare but potentially vision-threatening. It is important to consider mimics of orbital inflammatory disease such as systemic inflammatory disease, malignancy, congenital malformations, infection, and trauma when formulating a comprehensive differential diagnosis. Therapeutic intervention is directed toward preservation of vision and orbital function and reducing the acute inflammatory process. Corticosteroids are typically the initial treatment of choice for moderate-to-severe disease, although several classes of immunomodulatory agents have been variably useful in treating this condition. Heightened awareness and close cooperation between gastroenterologists and ophthalmologists are mandatory.

Nonspecific Orbital Inflammation (NSOI): Unraveling the Molecular Pathogenesis, Diagnostic Modalities, and Therapeutic Interventions

“…In fact, TED is the most commonly associated disease with NSOI. Other conditions linked to NSOI are Crohn's disease [10][11][12], psoriasis [13], discoid lupus [14][15][16], Behçet disease [17,18], sarcoidosis [19], Wegener's granulomatosis [20], Churg-Strauss syndrome [21,22], and giant cell arthritis [23][24][25]. It is important to remember that NSOI may present with other connective tissue diseases, and it should always remain on the list of differentials until another cause can be identified.…”

Identification of rare HIV-1–infected patients with extreme CD4+ T cell decline despite ART-mediated viral suppression

“…EXID4 maintained stable CD4 + T cells below the pre-ART level, 68 cells/μl (11%) with 4.3% naive (Supplemental Material Case Reports), despite 3 years of consistent suppression of HIV-1 pVL, but eventually developed right eye pain due to an infiltrating, hypermetabolic right orbital mass causing displacement of the right superior extraocular muscle and modeling of the superior right orbital bone as seen on MRI and PET-CT. A biopsy of the mass documented dense fibrosis and granulomas of epithelioid myeloid cells consistent with idiopathic orbital inflammation (IOI) (Supplemental Figure 8). This rare clinical entity has unknown etiology, but is associated with other inflammatory diseases and can cause a local infiltrating, destructive sclerosing process (14,15). His inguinal lymph node histology and quantitative imaging documented a prominent IFN-α signature, increased collagen-1 deposition with accumulation of myeloid cells, and overall preserved CD4 + T cell density in the follicular area and T cell zone compared with HC and paired HIV/AIDS untreated and ART-treated IR subjects (Supplemental Figures 8 and 9).…”