Idiopathic Sclerosing Inflammation Presenting as Sinusitis

Abstract

Idiopathic sclerosing orbital inflammation is a rare finding that is poorly delineated, immune mediated, and causes severe symptoms and disability. It has been described affecting the orbit in addition to other sites within the head and neck, but has rarely been described presenting as sinusitis. A case report and literature review were performed. A 14-year-old girl with right-sided face and eye pain and pressure for >1 month presented 3 days after endoscopic sinus surgery for presumed acute sinusitis. She subsequently developed ipsilateral vision loss and hypesthesia of the infraorbital nerve. MRI revealed a mildly enhancing soft tissue intensity lesion extending from the right maxillary sinus into the pterygopalatine fossa and orbital apex through the inferior orbital fissure. Biopsy specimens of the lesion were consistent with a sclerosing inflammatory lesion. High-dose steroids led to rapid improvement in vision and pain; however, the patient was unable to tolerate steroid weaning because of recurrence of eye pain and headache. Repeat imaging showed progression of the lesion. Rheumatology was consulted and the patient's steroid therapy was altered and her medications were expanded to include azathioprine. The patient's symptoms improved and subsequent imaging showed a reduction in the size and extent of the lesion. Idiopathic sclerosing inflammation is characterized by primary, chronic, and immunologically mediated fibrosis. Patients typically have a poor response to corticosteroid treatment or radiotherapy. Immunosuppressive therapy in addition to corticosteroids is the recommended treatment.

Sclerosing orbital pseudotumor

“…It showed similar condition to the inflammatory pseudotumor, but more aggressive and with worse response to steroids (8) . The most common signs and symptoms observed are:proptosis (49%), orbital pain (12.2%), palpebral edema or mass (22.4%), diplopia (6.1%), visual loss (6.1%) and ptosis (4.1%) (11) .Another study also found pain, proptosis, palpebral edema, restriction of eye movements, ptosis and visual loss as the most common clinical condition (5) .…”

“…Corticosteroids presented moderate effectiveness in sclerosing cases,and the greater success is obtained when administered early (8) . This is due to the predominance of fibrosis and collagen in histology, with little inflammatory infiltrate.Some patients respond poorly to corticotherapy and have poor visual prognosis (5) .…”

“…Others require the addition of immunosuppressants such as methotrexate, azathioprine, mycophenolate and cyclosporine (6) . Azathioprine associated to other agents can be used to control the disease and to reduce the use of corticosteroids (8) . Although presenting stroma with extensive hyalinization and fibrosis in biopsy, the patient reported had satisfactory response to the treatment.He presented remission of corticotherapy associated to azathioprine, withdrawal of corticosteroid, and he is currently in quarterly follow-up for the gradual withdrawal of azathioprine.…”

“…The sclerosing form is a rare subtype of inflammatory orbital pseudotumor, and is characterized by insidious inflammation resulting in the synthesis of fibers and collagen, with injury of orbital structures (6) . It presents a condition similar to inflammatory pseudotumor, but more aggressive and with a worse response to steroids (8) . 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) . Radiation therapy is indicated for patients with refractory disease, with inadequate response to corticosteroid, or with contraindication to its use.It is usually divided into 10 sessions for two to three weeks at a dose of 20 Gy (7) .…”