Systemic diseases with panuveitis

Systemic diseases with panuveitis (Orphanet code 280933) is a clinical grouping of rare systemic conditions in which panuveitis — inflammation affecting all layers of the uveal tract (iris, ciliary body, and choroid) — occurs as a prominent ocular manifestation of an underlying systemic disease. This category encompasses a heterogeneous set of disorders where widespread inflammatory or autoimmune processes affect multiple organ systems, including the eyes, joints, skin, lungs, nervous system, and vascular system. Panuveitis in these contexts is not an isolated ocular finding but rather a sign of systemic immune dysregulation or inflammatory disease. Conditions that may present with systemic disease and panuveitis include Behçet disease, sarcoidosis, Vogt-Koyanagi-Harada disease, and certain systemic vasculitides, among others. Key symptoms depend on the specific underlying systemic condition but commonly include blurred vision, eye pain, photophobia, floaters, and redness of the eyes due to the panuveitis itself. Systemic features may include fever, skin lesions, oral or genital ulcers, joint pain, lymphadenopathy, neurological symptoms, or pulmonary involvement. If left untreated, panuveitis can lead to serious ocular complications such as cataracts, glaucoma, macular edema, retinal detachment, and permanent vision loss. Treatment typically involves addressing both the ocular inflammation and the underlying systemic disease. Corticosteroids (topical, periocular, intravitreal, or systemic) are often used as first-line therapy for acute inflammation. Steroid-sparing immunosuppressive agents such as methotrexate, azathioprine, mycophenolate mofetil, cyclosporine, or biologic therapies (e.g., anti-TNF agents like adalimumab or infliximab) may be required for chronic or refractory cases. Management is typically multidisciplinary, involving ophthalmologists, rheumatologists, and other specialists depending on the organs affected.

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