Nonspecific Orbital Inflammation (NSOI) Howard R. Krauss MD - PowerPoint PPT Presentation

Nonspecific Orbital Inflammation (NSOI) Howard R. Krauss MD Professor of Ophthalmology and Neurosurgery Pacific Neuroscience Institute, Santa Monica, CA P A C I F I C N E U R O . O R G

… … nothing to disclothes … … P A C I F I C N E U R O . O R G

NSOI • Is: Is not: • Orbital pseudotumor Infection (viral, bacterial, parasitic) • Idiopathic orbital inflammatory Thyroid Eye Disease (IOI) disease Metastatic disease • Nonspecific orbital inflammation Lymphoproliferative disease (NSOI) Sarcoid • (Tolosa-Hunt syndrome is dx’d Wegener’s granulomatosis when NSOI occurs at the orbital C-C fistula apex) P A C I F I C N E U R O . O R G

NSOI is not: P A C I F I C N E U R O . O R G

NSOI Pseudotumor is a pseudodiagnosis. P A C I F I C N E U R O . O R G

NSOI Pseudotumor is a pseudodiagnosis. P A C I F I C N E U R O . O R G

This young woman has had several days of progressively worsening pain and swelling

T1, fat-sat, with gado

CT findings, in similar case

Orbital echography, in similar case

Normal echography

64-year-old man with several weeks of progressive swelling and redness OS

CT

Utility of adjunctive echography

NSOI Rao Chundury, MD MBA, Anupama Chundury, MS, Gabriella Original article contributed by: Espinoza, MD Andrew Go Lee, MD, Cat Nguyen Burkat, MD FACS, Guy V. All contributors: Jirawuthiworavong, M.D., M.A. and Rao V. Chundury, MD Assigned editor: Guy V. Jirawuthiworavong, M.D., M.A. Assigned status Up to Date by Cat Nguyen Burkat, MD FACS on Review: October 7, 2017. P A C I F I C N E U R O . O R G

NSOI • May present with, or be accompanied by: • Pain and/or tenderness (may be worse on eye movement) • Eyelid edema and/or erythema • Injection and/or chemosis • Proptosis • Diplopia • Uveitis • Serous retinal detachment P A C I F I C N E U R O . O R G

NSOI • What is the antigen? • Episcleritis • Scleritis • Trochleitis • Dacryoadenitis • Myositis • Perioptic neuritis • Diffuse orbital inflammatory disease P A C I F I C N E U R O . O R G

NSOI • Myositis & Dacryoadenitis – CT courtesy of Courtesy of Z.X. Ding P A C I F I C N E U R O . O R G

Dacryoadenitis (courtesy of Chua) P A C I F I C N E U R O . O R G

Diffuse orbital inflammatory disease CT courtesy of Courtesy of Z.X. Ding P A C I F I C N E U R O . O R G

NSOI • What are associated? • Viral prodrome • Autoimmune disease: – Crohn’s – systemic lupus erythematosus – rheumatoid arthritis – ankylosing spondylitis P A C I F I C N E U R O . O R G

NSOI • What is the inflammatory process? • nondiagnostic and diverse: – diverse polymorphous infiltrate – atypical granulomatous inflammation – tissue eosinophilia – infiltrative sclerosis – IgG4-related disease P A C I F I C N E U R O . O R G

NSOI • Work-up: • complete blood count • basic metabolic panel • thyroid function studies • erythrocyte sedimentation rate • antinuclear antibodies, antineutrophil cytoplasmic antibodies, angiotensin- converting enzyme level, rapid plasma reagin test, and rheumatoid factor. P A C I F I C N E U R O . O R G

NSOI • Treatment: • Corticosteroids • Surgery • Radiation therapy • Non-steroidal anti-inflammatory drugs • Cytotoxic agents (chlorambucil, cyclophosphamide) • Corticosteroid sparing immunosuppressants (methotrexate, cyclosporine, azathioprine) • IV immune-globin • Plasmapheresis • Biologic treatments (such as TNF- α inhibitors ) P A C I F I C N E U R O . O R G

Calcineurin inhibitors Cyclosporine-A inhibits synthesis of T-cell growth cytokines, IL-2 and IFN- γ Tacrolimus P A C I F I C N E U R O . O R G

Antiproliferative drugs (Cytotoxic) Azathioprine mercaptopurine analog which inhibits purine metabolism enzymes Cyclophosphamide B-cell cytotoxic alkylating agent Methotrexate inhibitor of dihydrofolate reductase, an enzyme needed in folic acid synthesis. This results in suppression of both T-cell and B- cell functions. Methotrexate is also known to enhance the release of adenosine, which has potent anti-inflammatory effects P A C I F I C N E U R O . O R G

Cytokine/protein specific biologic agents “It’s a mab mab mab mab world.” Adalimumab (IgG1 monoclonal antibody targeting TNF- α) (IgG1 monoclonal antibody targeting TNF- α ) Etanercept Infliximab (inhibits binding of TNF- α with its receptors) Rituximab (induces apoptosis of CD20+ B cells) Tocilizumab (anti-interleukin-6 receptor antibody) P A C I F I C N E U R O . O R G

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NSOI P A C I F I C N E U R O . O R G

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NSOI “NSOI is diagnosis of exclusion … Many therapeutic regimens exist … Steroids are the initial treatment of choice for moderate to severe NSOI .” P A C I F I C N E U R O . O R G

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