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

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

Aug 10, 2023 8 likes •329 views

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

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Nonspecific Orbital Inflammation (NSOI)

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Howard R. Krauss MD

Professor of Ophthalmology and Neurosurgery Pacific Neuroscience Institute, Santa Monica, CA

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… … nothing to disclothes … …

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NSOI

  • Is:
  • Orbital pseudotumor
  • Idiopathic orbital inflammatory

(IOI) disease

  • Nonspecific orbital inflammation

(NSOI)

  • (Tolosa-Hunt syndrome is dx’d

when NSOI occurs at the orbital apex) Is not: Infection (viral, bacterial, parasitic) Thyroid Eye Disease Metastatic disease Lymphoproliferative disease Sarcoid Wegener’s granulomatosis C-C fistula

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NSOI is not:

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NSOI

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Pseudotumor is a pseudodiagnosis.

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NSOI

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Pseudotumor is a pseudodiagnosis.

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

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T1, fat-sat, with gado

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CT findings, in similar case

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Orbital echography, in similar case

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Normal echography

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

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CT

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Utility of adjunctive echography

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NSOI

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Original article contributed by: Rao Chundury, MD MBA, Anupama Chundury, MS, Gabriella Espinoza, MD All contributors: Andrew Go Lee, MD, Cat Nguyen Burkat, MD FACS, Guy V. Jirawuthiworavong, M.D., M.A. and Rao V. Chundury, MD Assigned editor: Guy V. Jirawuthiworavong, M.D., M.A. Review: Assigned status Up to Date by Cat Nguyen Burkat, MD FACS on October 7, 2017.

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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

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NSOI

  • What is the antigen?
  • Episcleritis
  • Scleritis
  • Trochleitis
  • Dacryoadenitis
  • Myositis
  • Perioptic neuritis
  • Diffuse orbital inflammatory disease

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NSOI

  • Myositis & Dacryoadenitis

– CT courtesy of Courtesy of Z.X. Ding

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Dacryoadenitis (courtesy of Chua)

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Diffuse orbital inflammatory disease

CT courtesy of Courtesy of Z.X. Ding

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NSOI

  • What are associated?
  • Viral prodrome
  • Autoimmune disease:

– Crohn’s – systemic lupus erythematosus – rheumatoid arthritis – ankylosing spondylitis

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NSOI

  • What is the inflammatory process?
  • nondiagnostic and diverse:

– diverse polymorphous infiltrate – atypical granulomatous inflammation – tissue eosinophilia – infiltrative sclerosis – IgG4-related disease

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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.

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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)

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Calcineurin inhibitors

Cyclosporine-A inhibits synthesis of T-cell growth cytokines, IL-2 and IFN-γ Tacrolimus

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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

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Cytokine/protein specific biologic agents

“It’s a mab mab mab mab world.”

Adalimumab (IgG1 monoclonal antibody targeting TNF-α) Etanercept (IgG1 monoclonal antibody targeting TNF-α) Infliximab (inhibits binding of TNF-α with its receptors) Rituximab (induces apoptosis of CD20+ B cells) Tocilizumab (anti-interleukin-6 receptor antibody)

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NSOI

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NSOI

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NSOI

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NSOI

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“NSOI is diagnosis of exclusion … Many therapeutic regimens exist … Steroids are the initial treatment of choice for moderate to severe NSOI.”

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