Orbital Sarcoidosis

Description of Ophthalmic-Orbital Sarcoidosis

  • Sarcoidosis is a disease of unknown aetiology characterized by chronic inflammation and granuloma formation across multiple systems of {the} body. Although it predominantly affects {the} lungs and eyes, it is seen to affect {the} skin, lymph nodes, liver, spleen, heart, central and peripheral nervous systems, musculoskeletal system, and salivary glands.

Pathophysiology of Ophthalmic-Orbital Sarcoidosis

  • Histologically widespread noncaseating epithelioid granulomata in {the} presence of classic clinical and radiolologic findings aid {the} diagnosis of sarcoidosis. It commonly presents with complications such as blindness, meningitis, arthritis, kidney disease, skin disease, other systemic involvement and even death. A pulmonologist’s consultation might be {the} only way to support a diagnosis of ophthalmic or neurosarcoid using a bronchoscopic guided biopsy of concomitant chest lesions.

Symptoms and Signs of Ophthalmic-Orbital Sarcoidosis

  • Sarcoidosis might present with isolated symptoms in specific systems, but more commonly it presents with a plethora of symptoms with varying severity and affecting multiple organ systems.
  • Ophthalmologic involvement might affect {the} optic nerve pathways or various structures within {the} orbit producing {the} following symptoms and signs:

Neuro-ophthalmic involvement

Diplopia: Due to cranial nerve palsies

Reduced vision with or without scotoma: Due to oedema or infiltration of {the} optic nerve

Visual symptoms: Due to optic nerve damage caused by raised intraocular pressure

Orbital structure involvement

Uveitis results in blurring of vision, photophobia, floaters, scotomata and painful red eye.

Periocular lesions present with dry eye symptoms and disfigurement of adjacent structures

Mass lesion within {the} orbit causes bulging of {the} eyeballs and double vision

If it involves {the} orbital and adnexal structures, it can produce symptoms related to {the} lacrimal gland, lacrimal sac, eyelid and orbit.

Diagnosis of Ophthalmic-Orbital Sarcoidosis

Diagnosis of ophthalmic or orbital sarcoidosis is established through meticulous clinical examination and investigations.

Clinical examination reveals various manifestations of sarcoidosis in five different areas, namely:

  1. Neuro-ophthalmic manifestations: Rare cases of diplopia and ptosis occur due to involvement of {the} cranial nerves – oculomotor, trochlear, or abducens nerve. 5-38% of patients with neurosarcoid affect {the} optic nerve with ensuing edema, increased vascularity, nerve head elevation, haemorrhage, or optic atrophy. Retrobulbar optic nerve involvement might present as a mass lesion requiring biopsy to confirm {the} diagnosis.
  2. Ocular manifestations: 25-54% of patients with sarcoidosis present with ocular manifestations while almost all cases of ophthalmic sarcoidosis have systemic involvement.

Heerfordt Syndrome: Uveitis, parotid enlargement and occasionally papilledema.

Löfgren syndrome: Erythema nodosum, bilateral hilar adenopathy, arthralgias and uveitis.

Painless, bilateral, palpable swelling of {the} lacrimal gland is seen in some patients causing moderate to severe keratitis sicca.

  • Anterior segment disease: The following findings are seen with involvement of {the} anterior segment of {the} eye.
  • Granulomatous lesions in {the} conjunctiva
  • Keratoconjunctivitis sicca
  • Interstitial keratitis
  • Band keratopathy
  • Scleritis
  • Cataract due to uveitis or corticosteroid-induced cataract
  • Anterior uveitis is {the} most common manifestation with granulomatous or mutton-fat keratic precipitates, iris nodules, posterior synechiae and peripheral anterior synechiae
  • Complications of uveitis can also be seen.
  • Glaucoma is common. Gonioscopy should be done in all patients with raised intraocular pressure.

Posterior segment disease: Seen in 25-30% of patients with sarcoidosis. Findings include:

  • Cellular infiltrates, “String of Pearls” opacities, haze, syneresis, posterior vitreous detachment, and hemorrhages in {the} vitreous cavity.
  • Periphlebitis manifesting as perivenous exudates which look like candle-wax drippings or En taches de bougie. They resolve almost instantaneously with steroids.
  • In retinal neovascularization with vitreous haemorrhage, ischemia, and retinal detachment, panretinal photocoagulation and/or vitrectomy might be advocated.
  • Cystoid macular edema (CME) is {the} collection of extracellular fluid in {the} outer plexiform layer of {the} retina. Intravenous fluorescein angiography (IVFA) might assist in diagnosis by demonstrating leakage of dye in and around {the} fovea.
  • Focal and discrete subretinal mottling on ophthalmoscopy
  • Fibrinous exudates in {the} pars plana

Orbital disease: 20% of patients with ophthalmic manifestations of sarcoidosis involve {the} orbit or {the} lacrimal gland presenting as a mass lesion with bulging of {the} eyes, drooping of eyelids, enlarged lacrimal gland or ophthalmoplegia.

Investigations form {the} basis of confirming a diagnosis of sarcoidosis due to {the} wide variety of eye disorders that mimic its manifestations. The various investigations that could lead to a diagnosis of sarcoidosis include:

  1. Neuroimaging: Magnetic Resonance Imaging (MRI) is more useful than Computed Tomography (CT). Lesions detected on MRI are multiple white matter lesions, meningeal enhancement, optic nerve enhancement and mass lesions in {the} brain parenchyma.
  2. Nuclear Studies: Gallium-67 citrate scanning helps in identifying potential biopsy sites to aid tissue diagnosis.
  3. Vitreous Fluid Analysis: Tapping of vitreous for immunological analysis provides supportive evidence for a diagnosis of sarcoidosis.
  4. Biopsy: Most cases of sarcoidosis require a biopsy from {the} most accessible tissues to confirm {the} diagnosis.
  • Biopsy of {the} conjunctiva should be done as it is readily accessible and associated with low morbidity.
  • A transconjunctival lacrimal gland biopsy might confirm a diagnosis suspected during a gallium scan.
  • Salivary gland biopsy might be considered in cases where sarcoid uveitis is suspected

Treatment of Ophthalmic-Orbital Sarcoidosis

Oophthalmicor orbital sarcoidosis is treated using both medications and surgery.

Pharmacologic Therapy might include topical or systemic steroids, cycloplegics, topical nonsteroidal anti-inflammatory agents and corticosteroid-sparing agents.

  • Corticosteroids: They are {the} mainstay of treatment of ophthalmic and orbital sarcoidosis. Routes of administration include topical application, depot periocular injections, oral corticosteroids, and injectable corticosteroids. Immunosuppression and other adverse effects due to systemic corticosteroids should be discussed with {the} patient.
    • The treatment of choice for anterior uveitis is topical corticosteroids and might be used with cycloplegics.
    • Predinisolone acetate 1% is {the} standard topical steroid. Prednisolone phosphate is of lesser potency.
    • Newer corticosteroids such as loteprednol etabonate are effective maintenance drugs having lesser incidence of steroid-induced glaucoma.
    • It is important to monitor improvement or progress of {the} disease with readjustments of steroid dosages to meet {the} requirements.
  • Retrobulbar Corticosteroid Injections: Depot or sub-Tenon corticosteroid injections subvert {the} inaccessibility to topical steroids in cases of intermediate and posterior uveitis. They are given weekly, bi-weekly, or monthly, up to 3 or 4 times.
  • Oral Corticosteroids: They are preferred in uveitis with optic nerve involvement; intermediate uveitis, posterior uveitis, and panuveitis; bilateral disease; and coexisting threshold systemic disease.
  • Cycloplegics: They are indicated to relieve ciliary spasm due to {the} severe inflammatory process and to prevent {the} formation of posterior synechiae.
  • Topical Non-Steroidal Anti-inflammatory Agents: These might be used when {the} risk for steroid-induced cataract formation or steroid-induced glaucoma is high.
  • Corticosteroid-Sparing Agents: These agents help when there is intolerance to steroids or treatment failure.
    • Cyclosporine A
    • Methotrexate in recalcitrant uveitis
    • Oral monoclonal antibody therapy and interferon is being tried for nonresponsive uveitis
    • Anti-tumor necrosis factor-alpha (TNF-alpha) therapy (ie, infliximab, etanercept, adalimumab) are used in recalcitrant sarcoid uveitis
    • Intravitreal vascular endothelial growth factor (VEGF) agents (bevacizumab) is used in cases of choroidal or retinal neovascularization

Surgical treatment includes cataract surgery, vitrectomy or trabeculectomy.

  • Cataract surgery is done only with complete control or resolution of ocular inflammation
  • vitreous opacification requires vitrectomy
  • Trabeculectomy or glaucoma drainage device implant procedures are needed when medical therapy fails to treat glaucoma.
Procedures
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