Pseudotumor orbitário em paciente com doença de Still do adulto: associação incomum

Abstract

A doença de Still do adulto é uma desordem inflamatória rara, caracterizada por picos febris diários, rash evanescente, artrite e envolvimento de múltiplos órgãos. Manifestações oculares têm sido descritas associadas às manifestações clínicas clássicas da doença. Será descrito um caso de pseudotumor orbitário, condição clínica incomum, em uma paciente com diagnóstico de doença de Still do adulto.

Sclerosing orbital pseudotumor

“…he idiopathic orbital pseudotumor is an nonmalignant orbital inflammation without local or systemic known cause (1) . Its etiology is uncertain, with likely evidenced autoimmune process due to the response to immunosuppressive and its relation to disorders such as Crohn's disease, systemic lupus erythematosus, rheumatoid arthritis, among others (2) . It is more prevalent in adults, accounting for about 6% of all orbital lesions (3) .…”

“…It usually presents as unilateral, and bilateral is more common in children (4) . It can manifest with pain, proptosis, erythema and local edema, fibrosis, mass effect, ptosis, chemosis, motility dysfunction of the extraocular muscles, diplopia and optic neuropathy (2) .…”

“…The diagnosis is obtained by exclusion.For that, the medical history, clinical examination, laboratory tests for differential diagnosis with other diseases (T3, T4, TSH, VHS, glucose, urea, creatinine, blood cultures), CT scan, biopsy and treatment response are evaluated (9) . Systemic corticosteroids are the first line of treatment for orbital pseudotumors, usually starting with a dose of 1 to 2 mg/kg/ day of oral prednisone (2) . In sclerosing cases, efficacy is moderate, being greater when administered early (8) .Other treatment lines include immunosuppressive, cytotoxic agents, IV immune globulin, alpha TNF inhibitor, monoclonal antibody, and mycophenolate mofetil (10) .…”