mycophenolic-acid and Orbital-Pseudotumor

IgG4 is a newly known systemic disorder. It can present as orbital inflammation or as an orbital mass. We describe the case of a 5-year-old child with proptosis and globe dystopia who went on to have an orbital biopsy. Initially this was reported to be idiopathic orbital inflammation. After IgG4-RD was considered in the differential, the orbital biopsy was revisited, immunohistochemistry was done and the patient's serum was tested for IgG subclass levels. IgG4-RD was diagnosed and the patient was subsequently treated with prednisone and mycophenolate and made a good recovery. This is the youngest reported case of IgG4-RD in a child.

Topics: Child, Preschool; Diagnosis, Differential; Drug Therapy, Combination; Female; Humans; Hypergammaglobulinemia; Immunoglobulin G; Immunosuppressive Agents; Magnetic Resonance Imaging; Mycophenolic Acid; Orbital Neoplasms; Orbital Pseudotumor; Plasma Cells; Prednisone; Tomography, X-Ray Computed

Idiopathic orbital inflammatory syndrome (IOIS) is a nonspecific inflammation of orbital tissue. As it is a diagnosis of exclusion, systemic testing and, at times biopsy, is utilized to rule out other inflammatory etiologies. Since some inflammatory etiologies that masquerade as typical IOIS can be vision or life threatening, it is important to consider these diagnoses. Systemic lupus erythematosus (SLE) is a chronic systemic autoimmune process that can affect the eye and visual system in 20% of individuals. In this idiopathic process, the deposition of pathogenic autoantibodies and immune complexes damage tissues and cells. Some common ocular manifestations of SLE include keratoconjunctivitis sicca, periocular skin lesions, orbital inflammation, retinal hemorrhages and vasculitis, retinal vaso-occlusive disease, iritis, scleritis, optic neuritis and optic neuropathy. One rare clinical entity in the SLE spectrum is panniculitis, also known as lupus erythematosus profundus (LEP), which is a nodular inflammation of adipose tissue. Panniculitis involving orbital structures as the primary presenting symptom of SLE is quite unusual and has only rarely been previously reported in the literature and has not been reported presenting as IOIS. This uncommon presentation can make the diagnosis more difficult. We describe a patient who had presented with ptosis evolving to orbital inflammation, which was consistent with IOIS by laboratory and histologic examinations. The patient later developed extensive panniculitis and a final diagnosis of LEP was made.

Topics: Adolescent; Diagnosis, Differential; Drug Therapy, Combination; Female; Glucocorticoids; Humans; Immunosuppressive Agents; Magnetic Resonance Imaging; Mycophenolic Acid; Orbital Diseases; Orbital Pseudotumor; Panniculitis, Lupus Erythematosus

Topics: Antineoplastic Agents; B-Lymphocytes; Drug Resistance; Female; Fibrosis; Glucocorticoids; Humans; Magnetic Resonance Imaging; Middle Aged; Mycophenolic Acid; Orbit; Orbital Pseudotumor; T-Lymphocytes; Tomography, X-Ray Computed

Topics: Adult; Anti-Inflammatory Agents; Churg-Strauss Syndrome; Dacryocystitis; Diagnosis, Differential; Female; Humans; Immunosuppressive Agents; Magnetic Resonance Imaging; Methylprednisolone; Mycophenolic Acid; Orbital Pseudotumor; Prognosis

To evaluate treatment outcomes with mycophenolate mofetil in patients with inflammatory eye disease.. Retrospective case series.. Eighty-four consecutive patients with inflammatory eye disease treated with mycophenolate mofetil at an academic referral center.. Medical records were reviewed for treatment with mycophenolate mofetil. Dose of mycophenolate mofetil, response to therapy, dose of prednisone, use of other immunosuppressive drugs, and side effects associated with the use of mycophenolate mofetil were recorded.. Ability to control ocular inflammation with mycophenolate mofetil and to taper prednisone to < or =10 mg daily, and incidence of treatment-related side effects.. Of the 84 patients treated with mycophenolate mofetil, 61% had uveitis, 17% had scleritis, 11% had mucous membrane pemphigoid, and 11% had orbital or other inflammatory disease. Forty-three percent of patients treated with mycophenolate mofetil had been treated with at least one other immunosuppressive drug previously. The median dose of prednisone at the start of mycophenolate mofetil therapy was 40 mg, and 82% of the patients were considered a treatment success, as judged by the ability to control the inflammation and taper prednisone to < or =10 mg daily. Median time to treatment success was 3.5 months. Mycophenolate mofetil therapy was discontinued due to insufficient efficacy at a rate of 0.10 per person-year (PY) and due to side effects at a rate of 0.08/PY. The most frequent side effect was gastrointestinal upset, with a rate of 0.19/PY.. These data suggest that mycophenolate mofetil may be an effective corticosteroid-sparing agent in the treatment of inflammatory eye disease with a manageable side effect profile.

Topics: Adolescent; Adult; Aged; Child; Conjunctival Diseases; Drug Therapy, Combination; Female; Glucocorticoids; Humans; Immunosuppressive Agents; Male; Middle Aged; Mycophenolic Acid; Orbital Pseudotumor; Pemphigoid, Benign Mucous Membrane; Prednisone; Retrospective Studies; Scleritis; Treatment Outcome; Uveitis

To report our experience with the use of mycophenolate mofetil for treatment of idiopathic orbital inflammation (IOI).. Noncomparative interventional case series.. We reviewed the records of five patients with IOI who were treated with mycophenolate mofetil. The indications for use included recurrent inflammation after other therapies (four patients) and to avoid corticosteroid-induced blood sugar elevation in poorly controlled diabetes mellitus (one patient). Treated patients were followed for a median of 8.5 months (range, 4-30 months) after mycophenolate mofetil therapy was begun. Main outcome measures were response to treatment, treatment-related side effects, drug dosage, previous and final treatment regimens, and discontinuation of systemic corticosteroids.. Mycophenolate mofetil therapy was discontinued in one patient because of nausea. Each of the remaining four patients experienced complete resolution of orbital inflammation. Of these, only one patient remains on prednisone, but at a significantly reduced dose (1 mg/d).. Mycophenolate mofetil therapy resulted in the resolution of inflammation in patients with refractory or corticosteroid-dependent IOI and was used successfully for a first episode of IOI in a patient for whom corticosteroids were contraindicated.

Topics: Adult; Anti-Inflammatory Agents, Non-Steroidal; Female; Humans; Male; Middle Aged; Mycophenolic Acid; Orbital Pseudotumor; Prodrugs; Treatment Outcome