Sclerosing Keratitis

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Disease Entity

ICD-10 H16.33

ICD-11 9A78.A

Disease

First described by German ophthalmologist Dr. Albrecht von Gräfe, sclerosing keratitis is a type of interstitial keratitis that involves severe inflammation of the peripheral cornea and/or sclera near the cornea[1]. Sclerosing keratitis can occur alone or as a complication of scleritis, resulting in corneal scarring and potentially impacting visual acuity depending upon its progression towards the visual axis[2][3][4].

Etiology

Sclerosing keratitis has been associated with infection and autoimmunity;

Diagnosis

History

Females are affected more than males and the disease is often bilateral[12]. Thorough discussion should take place with the patient about recent travel (such as to places with high rates of leprosy, TB, onchocerciasis, or mumps) as well as family history of autoimmune conditions to determine the etiology.

Many patients with chronic scleritis develop sclerosing keratitis, while few with episcleritis develop this complication[2][13][14].

Symptoms

Similar to scleritis, sclerosing form is associated with ocular pain[3]. Vision is not affected unless the opacity crosses the visual axis, in which case vision will become significantly blurry and poor[2]. Patients will also commonly report epiphora, ocular redness, and photophobia.

Systemic signs can be associated with specific autoimmune disorders, but it is possible to have sclerosing keratitis due to autoimmunity without having systemic signs[3].

Signs

Sclerosing keratitis involves scleral inflammation invading the cornea, both by distance and depth, causing edema, opacification, as well as changes to the cornea which resemble sclera[15][16][17]. The area of sclerosing is typically next to the inflamed area of sclera but will extend regardless of active scleritis[2][14]. It must include the posterior layers of the cornea for diagnosis though it may also involve the anterior layers. Sclerosing can result in limbal guttering[2][14]. Deposits exist in the stromal opacification that have been described as “candy floss” and “crystalline” in appearance[14][16][17]. The opacification can be yellow or white/gray colored, waxy, and may develop a bordering precipitin ring[14][16][17].

Vascularization of the opacification is possible but infrequent, and this does not occur at the encroaching front of the opacity[17][18]. It may be vascularized at any layer, though usually in the posterior layer[7][16]. Rarely does vascularization reach the center of the cornea, typically only if longstanding and untreated[16][18].

Diagnostic Procedures

Sclerosing keratitis is apparent with slit lamp examination. The opacification may be better visualized with fluorescein[13][17]. Vessels will appear blue-colored[16].

Laboratory Tests

Labs should be run to differ between autoimmune and infectious etiologies, as well as to assess general function[3][4]. These should include the following;

  • Inflammatory markers: CRP, ESR
  • Autoantibody panel: ANA, ANCA, RF antibodies, anti-CCP antibodies
  • TB skin test/QuantiFERON blood test
  • Skin biopsies & cultures
  • CBC w/ diff, CMP, UA

Management

General Treatment

Treatment must address the cause of disease, either eliminating the source of infection or managing autoimmunity[3][14]. Referral to rheumatology or infectious diseases should be done once the cause is determined so that an appropriate treatment plan can be initiated.

NSAIDs, corticosteroids, and/or immunomodulatory agents may be indicated to limit ocular inflammation and scarring depending upon the severity of the disease[3][14].

Prognosis

When the cause is treated and active inflammation resolves, corneal scarring will remain[2]. This will not affect visual acuity unless it is in the visual axis. The visual axis is not commonly affected because the opacification begins on the periphery of the cornea and can be halted with early medical intervention[2]. If the visual axis is affected by scarring, keratoplasty may be indicated.

References

  1. Calhoun, FP. A Classification of Corneal Affections. Am J Ophthalmol. 1922;5(1):8–13. doi:10.1016/s0002-9394(22)91018-9
  2. 2.0 2.1 2.2 2.3 2.4 2.5 2.6 McGavin DD, Williamson J, Forrester JV, et al. Episcleritis and scleritis. A study of their clinical manifestations and association with rheumatoid arthritis. Br J Ophthalmol. 1976;60(3):192-226. doi:10.1136/bjo.60.3.192
  3. 3.0 3.1 3.2 3.3 3.4 3.5 3.6 Pereira Gomes JA, Neto AL, Scheinberg MA. ANCA associated with scleritis and sclerosing keratitis. Clin Rheumatol. 1993;12(3):429-430. doi:10.1007/bf02231597
  4. 4.0 4.1 4.2 Vignesh APP, Srinivasan R. Ocular manifestations of rheumatoid arthritis and their correlation with anti-cyclic citrullinated peptide antibodies. Clin Ophthalmol. 2015:393-397. doi:10.2147/opth.s77210
  5. Choyce DP. Ocular leprosy, with reference to certain cases shown. P Roy Soc Med. 1955;48(2):108-112. doi:10.1177/003591575504800211
  6. Choyce DP. Diagnosis and management of ocular leprosy. Br J Ophthalmol. 1969;53(4):217-223. doi:10.1136/bjo.53.4.217
  7. 7.0 7.1 Doggart JH. Vascularization of the cornea. Br J Ophthalmol. 1951;35(3):160-167. doi:10.1136/bjo.35.3.160
  8. Ellison J. Tuberculous scleritis with sclerosing keratitis. P Roy Soc Med. 1936;29(4):391-391. doi:10.1177/003591573602900445
  9. Hall LR, Pearlman E. Pathogenesis of Onchocercal keratitis (river blindness). Clin Microbiol Rev. 1999;12(3):445-453. doi:10.1128/cmr.12.3.445
  10. North DP. Ocular complications of mumps. Br J Ophthalmol. 1953;37(2):99-101. doi:10.1136/bjo.37.2.99
  11. Gheita TA, Abd El-Latif EM. Ocular presentation in granulomatosis with polyangiitis (GPA) patients: relation to autoantibodies and disease activity. Ann Rheum Dis. 2018;77:1493. doi:10.1136/annrheumdis-2018-eular.5230
  12. Duane A, Fuchs E. Diseases of the Cornea. In: Text-Book of Ophthalmology. Philadelphia, PA: J.B. Lippincott Co; 1908:211-212.
  13. 13.0 13.1 Patel SJ, Lundy DC. Ocular manifestations of autoimmune disease. Am Fam Physician. 2002 Sep 15;66(6):991-8. PMID: 12358224.
  14. 14.0 14.1 14.2 14.3 14.4 14.5 14.6 Watson PG, Hayreh SS, Awdry PN. Episcleritis and scleritis II. Br J Ophthalmol. 1968;52(4):348-349. doi:10.1136/bjo.52.4.348
  15. Viswalingam A. A case of sclerosing keratitis profunda. Br J Ophthalmol. 1936;20(8):449-455. doi:10.1136/bjo.20.8.449
  16. 16.0 16.1 16.2 16.3 16.4 16.5 Watson PG, Hayreh SS. Scleritis and episcleritis. Br J Ophthalmol. 1976;60(3):163-191. doi:10.1136/bjo.60.3.163
  17. 17.0 17.1 17.2 17.3 17.4 Watson P, Booth-Mason S. Fluorescein angiography in the differential diagnosis of sclerokeratitis. Br J Ophthalmol. 1987;71(2):145-151. doi:10.1136/bjo.71.2.145
  18. 18.0 18.1 Marshall JC. Sclerosing keratitis. P Roy Soc Med. 1931;24(8):1044-1045. doi:10.1177/003591573102400852
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