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  • Severe eye pain, often at night (sometimes radiating to the forehead, brow, jaw), exacerbated by eye movements and touch
  • Acute or gradual onset of redness in the eyes
  • Sometimes tearing, photophobia, and visual impairment


  • Often (up to 50%) associated with systemic diseases, including:
    • Rheumatoid arthritis
    • Granulomatosis with polyangiitis
    • Recurrent polychondritis
    • Polyarteritis nodosa
    • Chronic inflammatory bowel diseases
  • Less common causes:
    • Infectious: Herpes Zoster, Tuberculosis, Leprosy, Syphilis, Lyme disease, Fungi
    • Surgically induced: Typically 3 weeks after surgery (strabismus, trabeculectomy, implant, pterygium surgery, etc.)

Forms of immune-mediated scleritis

  • Anterior Scleritis
    • Non-necrotizing anterior scleritis
      • Diffuse: Generalised or restricted to one quadrant, with extended scleral, episcleral, and conjunctival vessels
      • Nodular: Single or multiple scleral nodules, often in the palpebral fissure, non-movable, intensely dark blue compared to episcleral nodules
      • Anterior Segment OCT
    • Necrotizing anterior scleritis with inflammation
      • Extreme pain
      • Forms:
        • Vasoocclusive
        • Granulomatous
      • Anterior segment angiography, ultrasound biomicroscopy (UBM): Early detection of necrotic areas
    • Scleromalacia perforans/necrotizing anterior scleritis without inflammation
      • Often asymptomatic/mild symptoms
      • Necrotic sclera plaques near the limbus without vascular congestion. Connection and extension of necrotic areas. Rarely perforation.
      • Typically in women with rheumatoid arthritis
  • Posterior Scleritis
    • Scleral inflammation posterior to the ora serrata; only 2% of all scleritis cases
    • Often associated with anterior scleritis
    • Presentation: Choroidal folds, papilledema, macular edema, exudative retinal detachment, uveal effusion, yellow-brown subretinal mass, exophthalmos
    • Diagnosis:
      • Ultrasound: “T-sign” (retrobulbar edema in the optic sheath and Tenon’s space), thickened sclera-choroid in comparison
      • OCT: choroidal folds, choroidal thickening
      • MRI and CT: Thickening of the sclera

Differential Diagnosis


  • Phenylephrine test: Administer 1 drop of Phenylephrine 2.5%, evaluate after 10-15min
    • if redness persists: Scleritis
    • if redness disappears: Episcleritis
  • Anterior segment OCT: Helpful for distinguishing between episcleritis and scleritis
  • Work-up for systemic diseases recommended
  • Differential blood count, ESR, CRP, ANA, ANCA, RF, anti-CCP, ACE, syphilis, , Quantiferon test, chest X-ray
  • In case of suspected posterior scleritis: Ultrasound with “T-sign”? MRI/CT: Scleral thickening?

Therapy for immune-mediated anterior and posterior scleritis

  • First choice: Systemic NSAIDs for non-necrotizing disease.
    • E.g., Froben (flurbiprofen) 50mg 3x daily or Diclofenac 2×50-75mg for 2 weeks, then 1×50-75mg per day
    • Often requires several months of administration
  • In case of pronounced findings with severe pain, poor response to NSAIDs, or necrotizing form:
    • Systemic steroids: Prednisolone initially 1-1.5mg/kg body weight/day
    • CAUTION: Periocular steroid injection is contraindicated in the necrotizing form due to the risk of necrosis!
    • CAUTION: In the necrotizing form, in addition to steroids, immediate initiation of immunomodulatory therapy is needed!
  • Possible Medications:
    • Cytostatics: Cyclophosphamide, Azathioprine, Mycophenolate mofetil, Methotrexate
    • Immunomodulators: Ciclosporin, Tacrolimus (mainly used for long-term therapy)
    • Specific antibodies: Infliximab, Rituximab, Adalimumab (CAUTION: Rule out tuberculosis!)
  • Possibly local steroids for symptom relief (response rate 0-50%), no effect on the course
  • Last resort in (threatening) perforation is surgery (then also diagnostic for pathogens)


  • EyeWiki Scleritis
  • Tappeiner C, Walscheid K, Heiligenhaus A. Diagnose und Therapie der Episkleritis und Skleritis [Diagnosis and treatment of episcleritis and scleritis]. Ophthalmologe. 2016;113(9):797-810. doi:10.1007/s00347-016-0344-3
  • The Wills Eye Manual: Office and Emergency Room Diagnosis and Treatment of Eye Disease; Nika Bagheri MD, Brynn Wajda MD, et al; Lippincott Williams&Wilkins; 7th Edition (2016)
  • Kanski’s Clinical Ophthalmology: A Systematic Approach; Jack J. Kanski MD, Brad Bowling MD; Saunders Ltd.; 8th Edition (2015)