Close
New

Medscape is available in 5 Language Editions – Choose your Edition here.

 

Scleritis Workup

  • Author: Manolette R Roque, MD, MBA, FPAO; Chief Editor: John D Sheppard, Jr, MD, MMSc  more...
 
Updated: Apr 11, 2016
 

Laboratory Studies

Based on the past history, review of systems, and physical examination, select appropriate diagnostic tests to confirm or reject the following suspected associated diseases:

  • Rheumatoid factor - Rheumatoid arthritis
  • Antinuclear antibodies - Systemic lupus erythematosus, rheumatoid arthritis, polymyositis, progressive systemic sclerosis, or mixed connective tissue
  • Antineutrophil cytoplasmic antibodies (ANCA) - Granulomatosis with polyangiitis, polyarteritis nodosa, or microscopic polyangiitis
  • Human leukocyte antigen (HLA) typing - Ankylosing spondylitis, reactive arthritis, psoriatic arthritis, or arthritis associated with inflammatory bowel disease
  • Eosinophil count/immunoglobulin E (IgE) - Allergic angiitis of Churg-Strauss syndrome or atopy
  • Uric acid - Gout
  • Erythrocyte sedimentation rate (ESR) - Giant cell arteritis
  • Hepatitis B surface antigen (HBsAg) - Polyarteritis nodosa
  • Serologies - Infectious diseases, including syphilis and Lyme disease
  • Purified-protein derivative (PPD) skin test or Quantiferon gold assay - Tuberculosis
  • Anergy skin test - Sarcoidosis
  • Prick test - Atopy
Next

Imaging Studies

See the list below:

  • Chest radiography or CT scanning - Tuberculosis, Granulomatosis with polyangiitis, allergic angiitis of Churg-Strauss syndrome, sarcoidosis, or atopy
  • Sinus films - Granulomatosis with polyangiitis
  • Sacroiliac radiography - Ankylosing spondylitis, reactive arthritis, psoriatic arthritis, or arthritis associated with inflammatory bowel disease
  • Limb joint radiography - Rheumatoid arthritis, psoriatic arthritis, arthritis associated with inflammatory bowel disease, or gout
  • Ultrasonography (A-scan and B-scan) - Posterior scleritis; most helpful test to aid in diagnosing posterior scleritis, which is characterized by flattening of the posterior aspect of the globe, thickening of the posterior sclera, and retrobulbar edema[6]
  • Orbital CT scanning - Posterior scleritis; a useful diagnostic tool to aid in detecting the following, which are important to differentiate posterior scleritis from orbital inflammatory diseases and orbital neoplasm: extraocular muscle or lacrimal gland enlargement, sinus tissue involvement, and posterior scleral thickening[7]
  • Orbital MRI - Posterior scleritis
    • MRI is used to differentiate localized inflammatory pseudotumor from posterior scleritis in proptosis or choroidal tumors from posterior scleritis in subretinal mass.
    • Some orbital tumors can cause choroidal folds and retinal striae, which are also signs of posterior scleritis, and are detected reliably with MRI.
  • Related clinical guideline summary from the American College of Radiology: ACR Appropriateness Criteria: Orbits, vision, and visual loss.[8]
Previous
Next

Other Tests

See the list below:

  • Smears, cultures, and polymerase chain reaction (PCR) from conjunctival, corneal, episcleral, or scleral scraping - Infectious scleritis
    • Scleral or corneoscleral biopsy is recommended if smears and culture results are negative at 48 hours, infectious scleritis is still the primary clinical suspicion, and the patient is worsening despite targeted empirical antimicrobial therapy.
      • One third of tissue from a biopsy is sent to the microbiology department, where it is homogenized for smears, cultures, and PCR.
      • The middle third of tissue is transported to the pathology department for histopathology with special stains (eg, periodic acid-Schiff [PAS], Gomori methenamine-silver [GMS], acid-fast, calcofluor white).
      • The last third of tissue is sent to the immunology department for immunofluorescence studies with monoclonal antibodies (eg, anti–herpes simplex virus type 1, anti–varicella-zoster virus antibodies).
  • PCR of body fluids, orbital abscess drainage, aqueous humor, and vitreous - Infectious scleritis
Previous
Next

Procedures

Low-dose anterior segment fluorescein angiography (FA) combined with anterior segment indocyanine green (ICG) angiography is recommended.[9] ICG distinguishes totally occluded vessels from the temporary obstruction caused by high endothelial venules or vascular spasm seen as nonperfusion with FA. FA identifies new corneal vessels and leakage, whereas ICG does not. ICG locates the site of maximum inflammation and is more valuable in assessing the effects of treatment and when to withdraw that treatment. These anterior-segment imaging techniques require specific expertise and training and are not available in most facilities or academic departments.

  • Diffuse scleritis
    • FA - Rapid filling, short transit time, extensive leakage, normal vascular pattern, and deep sclera leakage in early disease
    • ICG - Rapid filling, short transit time, no leakage except for local vascular damage, and occasionally late deep leakage
  • Nodular scleritis
    • FA - Rapid transit time, abnormal leakage pattern, and staining nodules
    • ICG - Rapid filling, short transit time, and stained nodules
  • Necrotizing scleritis
    • FA - Hypoperfusion and venular occlusion, increased transit time, new vessel formation, and deep staining
    • ICG - Hypoperfusion and venular occlusion, increased transit time, and late leakage from new or damaged vessels
  • Scleromalacia perforans
    • FA - Virtually no perfusion
    • ICG - Leakage in area of necrotic tissue
  • Posterior scleritis
    • FA - Retinal pigment epithelial detachments, serous retinal detachment, cystoid edema, choroidal folds, and hyperfluorescent and hypofluorescent areas
    • ICG - Diffuse zonal choroidal hyperfluorescence intermediate or late phase, pinpoint leakage, delayed choroidal perfusion, and hyperfluorescence in areas of maximal activity
Previous
Next

Histologic Findings

Diffuse scleritis or nodular scleritis

A nongranulomatous inflammatory reaction occurs that is characterized by infiltration of mononuclear cells, such as macrophages, lymphocytes, and plasma cells. In the most severe cases, mononuclear cells may organize into granulomatous lesions. Mast cells, neutrophils, and eosinophils may also be present.

Necrotizing scleritis

A granulomatous inflammatory reaction occurs that is characterized by a central area of fibrinoid necrosis, surrounded by epithelioid cells, multinucleated giant cells, lymphocytes, and plasma cells. Neutrophils, mast cells, and eosinophils are dispersed throughout the inflamed tissue and around vessels. Inflammatory microangiopathy, which is characterized by neutrophilic infiltration in and around the episclera and sclera that perforates the vessel walls with or without fibrinoid necrosis, is frequently seen.

Previous
 
 
Contributor Information and Disclosures
Author

Manolette R Roque, MD, MBA, FPAO Section Chief, Ocular Immunology and Uveitis, Department of Ophthalmology, Asian Hospital and Medical Center; Section Chief, Ocular Immunology and Uveitis, International Eye Institute, St Luke's Medical Center Global City; Senior Eye Surgeon, The LASIK Surgery Clinic; Director, AMC Eye Center, Alabang Medical Center

Manolette R Roque, MD, MBA, FPAO is a member of the following medical societies: American Academy of Ophthalmology, American Society of Cataract and Refractive Surgery, Philippine Medical Association, American Uveitis Society, International Ocular Inflammation Society, Philippine Ocular Inflammation Society, American Society of Ophthalmic Administrators, American Academy of Ophthalmic Executives, Philippine Society of Cataract and Refractive Surgery

Disclosure: Nothing to disclose.

Specialty Editor Board

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Received salary from Medscape for employment. for: Medscape.

R Christopher Walton, MD Professor, Director of Uveitis and Ocular Inflammatory Disease Service, Department of Ophthalmology, University of Tennessee College of Medicine

R Christopher Walton, MD is a member of the following medical societies: American Academy of Ophthalmology, Association for Research in Vision and Ophthalmology, Retina Society, American College of Healthcare Executives, American Uveitis Society

Disclosure: Nothing to disclose.

Chief Editor

John D Sheppard, Jr, MD, MMSc Professor of Ophthalmology, Microbiology and Molecular Biology, Clinical Director, Thomas R Lee Center for Ocular Pharmacology, Ophthalmology Residency Research Program Director, Eastern Virginia Medical School; President, Virginia Eye Consultants

John D Sheppard, Jr, MD, MMSc is a member of the following medical societies: American Academy of Ophthalmology, American Society for Microbiology, American Society of Cataract and Refractive Surgery, Association for Research in Vision and Ophthalmology, American Uveitis Society

Disclosure: Nothing to disclose.

Additional Contributors

Maite Sainz de La Maza, MD, PhD Associate Professor, Department of Ophthalmology, Division of Ocular Immunology and Uveitis, Hospital Clinico of Provincial, Barcelona, Spain

Maite Sainz de La Maza, MD, PhD is a member of the following medical societies: American Academy of Ophthalmology, American Uveitis Society

Disclosure: Nothing to disclose.

John D Sheppard, Jr, MD, MMSc Professor of Ophthalmology, Microbiology and Molecular Biology, Clinical Director, Thomas R Lee Center for Ocular Pharmacology, Ophthalmology Residency Research Program Director, Eastern Virginia Medical School; President, Virginia Eye Consultants

John D Sheppard, Jr, MD, MMSc is a member of the following medical societies: American Academy of Ophthalmology, American Society for Microbiology, American Society of Cataract and Refractive Surgery, Association for Research in Vision and Ophthalmology, American Uveitis Society

Disclosure: Nothing to disclose.

References
  1. González-López JJ, Lavric A, Dutta Majumder P, Bansal N, Biswas J, Pavesio C, et al. Bilateral Posterior Scleritis: Analysis of 18 Cases from a Large Cohort of Posterior Scleritis. Ocul Immunol Inflamm. 2015 Oct 16. 1-8. [Medline].

  2. Sims J. Scleritis: presentations, disease associations and management. Postgrad Med J. 2012 Sep 12. [Medline].

  3. Sainz de la Maza M, Foster CS, Jabbur NS. Scleritis associated with rheumatoid arthritis and with other systemic immune-mediated diseases. Ophthalmology. 1994 Jul. 101(7):1281-6; discussion 1287-8. [Medline].

  4. Sainz de la Maza M, Foster CS, Jabbur NS. Scleritis associated with systemic vasculitic diseases. Ophthalmology. 1995 Apr. 102(4):687-92. [Medline].

  5. French DD, Margo CE. Postmarketing surveillance rates of uveitis and scleritis with bisphosphonates among a national veteran cohort. Retina. 2008 Jun. 28(6):889-93. [Medline].

  6. Agrawal R, Lavric A, Restori M, Pavesio C, Sagoo MS. NODULAR POSTERIOR SCLERITIS: Clinico-Sonographic Characteristics and Proposed Diagnostic Criteria. Retina. 2015 Aug 19. [Medline].

  7. Axmann S, Ebneter A, Zinkernagel MS. Imaging of the Sclera in Patients with Scleritis and Episcleritis using Anterior Segment Optical Coherence Tomography. Ocul Immunol Inflamm. 2015 Aug 10. 1-6. [Medline].

  8. [Guideline] Zimmerman RD, Seidenwurm DJ, Davis PC, et al. ACR Appropriateness Criteria. Orbits, vision, and visual loss. National Guideline Clearinghouse. 2006.

  9. Nieuwenhuizen J, Watson PG, Emmanouilidis-van der Spek K, Keunen JE, Jager MJ. The value of combining anterior segment fluorescein angiography with indocyanine green angiography in scleral inflammation. Ophthalmology. 2003 Aug. 110(8):1653-66. [Medline].

  10. Sainz de la Maza M, Molina N, Gonzalez-Gonzalez LA, Doctor PP, Tauber J, Foster CS. Clinical characteristics of a large cohort of patients with scleritis and episcleritis. Ophthalmology. 2012 Jan. 119(1):43-50. [Medline].

  11. Hakin KN, Ham J, Lightman SL. Use of orbital floor steroids in the management of patients with uniocular non-necrotising scleritis. Br J Ophthalmol. 1991 Jun. 75(6):337-9. [Medline].

  12. Galor A, Jabs DA, Leder HA, Kedhar SR, Dunn JP, Peters GB 3rd. Comparison of antimetabolite drugs as corticosteroid-sparing therapy for noninfectious ocular inflammation. Ophthalmology. 2008 Oct. 115(10):1826-32. [Medline].

  13. Sobrin L, Christen W, Foster CS. Mycophenolate mofetil after methotrexate failure or intolerance in the treatment of scleritis and uveitis. Ophthalmology. 2008 Aug. 115(8):1416-21, 1421.e1. [Medline].

  14. Wakefield D, McCluskey P. Cyclosporin therapy for severe scleritis. Br J Ophthalmol. 1989 Sep. 73(9):743-6. [Medline].

  15. Doctor P, Sultan A, Syed S, Christen W, Bhat P, Foster CS. Infliximab for the treatment of refractory scleritis. Br J Ophthalmol. 2010 May. 94(5):579-83. [Medline]. [Full Text].

  16. Huynh N, Cervantes-Castaneda RA, Bhat P, Gallagher MJ, Foster CS. Biologic response modifier therapy for psoriatic ocular inflammatory disease. Ocul Immunol Inflamm. 2008 May-Jun. 16(3):89-93. [Medline].

  17. Murphy CC, Ayliffe WH, Booth A, Makanjuola D, Andrews PA, Jayne D. Tumor necrosis factor alpha blockade with infliximab for refractory uveitis and scleritis. Ophthalmology. 2004 Feb. 111(2):352-6. [Medline].

  18. Ahmadi-Simab K, Lamprecht P, Nolle B, Ai M, Gross WL. Successful treatment of refractory anterior scleritis in primary Sjogren's syndrome with rituximab. Ann Rheum Dis. 2005 Jul. 64(7):1087-8. [Medline].

  19. Cheung CM, Murray PI, Savage CO. Successful treatment of Wegener's granulomatosis associated scleritis with rituximab. Br J Ophthalmol. 2005 Nov. 89(11):1542. [Medline].

  20. Taylor SR, Salama AD, Joshi L, Pusey CD, Lightman SL. Rituximab is effective in the treatment of refractory ophthalmic Wegener's granulomatosis. Arthritis Rheum. 2009 May. 60(5):1540-7. [Medline].

  21. Recillas-Gispert C, Serna-Ojeda JC, Flores-Suárez LF. Rituximab in the treatment of refractory scleritis in patients with granulomatosis with polyangiitis (Wegener's). Graefes Arch Clin Exp Ophthalmol. 2015 Oct 27. [Medline].

  22. Tuft SJ, Watson PG. Progression of scleral disease. Ophthalmology. 1991 Apr. 98(4):467-71. [Medline].

  23. Sainz de la Maza M, Jabbur NS, Foster CS. Severity of scleritis and episcleritis. Ophthalmology. 1994 Feb. 101(2):389-96. [Medline].

  24. Sobrin L, Kim EC, Christen W, Papadaki T, Letko E, Foster CS. Infliximab therapy for the treatment of refractory ocular inflammatory disease. Arch Ophthalmol. 2007 Jul. 125(7):895-900. [Medline].

  25. Fong LP, Sainz de la Maza M, Rice BA, Kupferman AE, Foster CS. Immunopathology of scleritis. Ophthalmology. 1991 Apr. 98(4):472-9. [Medline].

  26. Heinz C, Bograd N, Koch J, Heiligenhaus A. Ocular hypertension and glaucoma incidence in patients with scleritis. Graefes Arch Clin Exp Ophthalmol. 2012 Jul 22. [Medline].

  27. Onal S, Kazokoglu H, Koc A, Yavuz S. Rituximab for remission induction in a patient with relapsing necrotizing scleritis associated with limited Wegener´s granulomatosis. Ocul Immunol Inflamm. Sep-Oct 2008. 16(5):230-232.

  28. Sainz de la Maza M, Jabbur NS, Foster CS. An analysis of therapeutic decision for scleritis. Ophthalmology. 1993 Sep. 100(9):1372-6. [Medline].

  29. Sainz de la Maza M, Tauber J, Foster CS. Scleral grafting for necrotizing scleritis. Ophthalmology. 1989 Mar. 96(3):306-10. [Medline].

  30. Watson PG, Hayreh SS. Scleritis and episcleritis. Br J Ophthalmol. 1976 Mar. 60(3):163-91. [Medline].

  31. de Fidelix TS, Vieira LA, de Freitas D, Trevisani VF. Biologic therapy for refractory scleritis: a new treatment perspective. Int Ophthalmol. 2015 Aug 29. [Medline].

Previous
Next
 
 
 
 
All material on this website is protected by copyright, Copyright © 1994-2016 by WebMD LLC. This website also contains material copyrighted by 3rd parties.