Intermediate Uveitis: Background, Pathophysiology, Epidemiology

Sections

Sections Intermediate Uveitis
Overview
Presentation
- History
- Physical
- Causes
- Show All
DDx
Workup
Treatment
Medication
Follow-up
References

Overview

Background

Intermediate uveitis refers to inflammation localized to the vitreous and peripheral retina. Intermediate uveitis was first described in the literature as chronic cyclitis by Fuchs in 1908. According to the Standardization of Uveitis Nomenclature Working Group, the primary site of inflammation is the vitreous and such entities as pars planitis, posterior cyclitis, and hyalitis are encompassed. Intermediate uveitis may be initially associated with the development of a systemic disease, such as multiple sclerosis (MS) or sarcoidosis. As such, intermediate uveitis may be the first expression of autoimmunity in these patients.

Pars planitis is considered a subset of intermediate uveitis and is characterized by the presence of white exudates (snowbanks) over the pars plana and ora serrata or by aggregates of inflammatory cells in the vitreous (snowballs) in the absence of an infectious etiology (eg, Lyme disease) or a systemic disease (eg, sarcoidosis). Some authorities believe that patients with pars planitis have worse vitritis, more severe macular edema, and a guarded prognosis compared to other patients with intermediate uveitis. This primary form accounts for over 50% of patients with intermediate uveitis.

Pathophysiology

There may be an immunogenetic predisposition for the disorder in some cases (see Causes), accounting for family clustering that is seen on occasion, indicating environmental or hereditary associations. The available evidence would suggest an autoimmune mechanism; however, the antigenic stimulus remains elusive.

Frequency

United States

Traditionally, the proportion of patients with intermediate uveitis is estimated to be 4-8% of uveitis cases in referral centers. The National Institutes of Health (NIH) reports a higher percentage (15%), which may indicate improved awareness or the nature of the uveitis referral clinic. In the pediatric population, intermediate uveitis can account for up to 25% of uveitis cases.

Mortality/Morbidity

Permanent loss of vision is most commonly seen in patients with chronic cystoid macular edema (CME). Every effort must be made to eradicate CME when present. Other less common causes of visual loss include rhegmatogenous retinal detachment, glaucoma, band keratopathy, cataracts, vitreous hemorrhage, epiretinal membrane, and choroidal neovascularization.

Race

No racial predilection exists for this disease.

Sex

The incidence of intermediate uveitis is equal in men and women.

Age

Although intermediate uveitis can develop at any age, it primarily afflicts children and young adults. There is a bimodal distribution with one peak in the second decade and another peak in the third or fourth decade.

In the pediatric age group, intermediate uveitis is associated with a worse presenting visual acuity. Poorer outcomes may be related to delayed presentation/diagnosis, the inherent difficulties of immunosuppression in children, or a more aggressive disease.

Clinical Presentation

Kimura SJ, Hogan MJ. Chronic Cyclitis. Arch of Ophthalmol. 1964. 71:193-201.
Raja SC, Jabs DA, Dunn JP, et al. Pars Planitis: Clinical Features and Class II HLA Associations. Ophthalmology. 1999. 106 (3):594-599. [Medline].
Malinowski SM, Pulido JS, Folk JC. Long-term visual outcome and complications associated with pars planitis. Ophthalmology. 1993 Jun. 100(6):818-24; discussion 825. [Medline].
Henderly DE, Haymond RS, Rao NA, et al. The significance of the pars plana exudate in pars planitis. Am J Ophthalmol. 1987 May 15. 103(5):669-71. [Medline].
Rodriguez A, Calonge M, Pedroza-Seres M, et al. Referral Patterns of Uveitis in a Tertiary Care Center. Archives of Ophthalmology. 1996. 114 (5):593-599. [Medline].
Kaplan HJ. Intermediate Uveitis (Pars Planitis, Chronic Cyclitis)- A Four Step Approach to Treatment. Saari KM, ed. Uveitis Update. Amsterdam: Exerpta Medica; 1984. 169-172.
Hogewind BF, Zijlstra C, Klevering BJ, et al. Intravitreal triamcinolone for the treatment of refractory macular edema in idiopathic intermediate or posterior uveitis. Eur J Ophthalmol. 2008 May-Jun. 18(3):429-34. [Medline].
Murphy CC, Greiner K, Plskova J, et al. Cyclosporine vs tacrolimus therapy for posterior and intermediate uveitis. Arch Ophthalmol. 2005 May. 123(5):634-41. [Medline].
Jaffe GJ, Dick AD, Brézin AP, Nguyen QD, Thorne JE, Kestelyn P, et al. Adalimumab in Patients with Active Noninfectious Uveitis. N Engl J Med. 2016 Sep 8. 375 (10):932-43. [Medline].
Nguyen QD, Merrill PT, Jaffe GJ, Dick AD, Kurup SK, Sheppard J, et al. Adalimumab for prevention of uveitic flare in patients with inactive non-infectious uveitis controlled by corticosteroids (VISUAL II): a multicentre, double-masked, randomised, placebo-controlled phase 3 trial. Lancet. 2016 Sep 17. 388 (10050):1183-92. [Medline].
Markomichelakis NN, Theodossiadis PG, Pantelia E, et al. Infliximab for chronic cystoid macular edema associated with uveitis. Am J Ophthalmol. 2004 Oct. 138(4):648-50. [Medline].
Rajaraman RT, Kimura Y, Li S, et al. Retrospective case review of pediatric patients with uveitis treated with infliximab. Ophthalmology. 2006 Feb. 113(2):308-14. [Medline].
Devenyi RG, Mieler WF, Lambrou FH, et al. Cryopexy of the vitreous base in the management of peripheral uveitis. Am J Ophthalmol. 1988 Aug 15. 106(2):135-8. [Medline].
Park SE, Mieler WF, Pulido JS. 2 peripheral scatter photocoagulation for neovascularization associated with pars planitis. Arch Ophthalmol. 1995 Oct. 113(10):1277-80. [Medline].
Becker M, Davis J. Vitrectomy in the treatment of uveitis. Am J Ophthalmol. 2005 Dec. 140(6):1096-105. [Medline].
Quinones K, Choi JY, Yilmaz T, Kafkala C, Letko E, Foster CS. Pars plana vitrectomy versus immunomodulatory therapy for intermediate uveitis: a prospective, randomized pilot study. Ocul Immunol Inflamm. 2010 Oct. 18(5):411-7. [Medline].
Jaffe GJ, Martin D, Callanan D, et al. Fluocinolone acetonide implant (Retisert) for noninfectious posterior uveitis: thirty-four-week results of a multicenter randomized clinical study. Ophthalmology. 2006 Jun. 113(6):1020-7. [Medline].
Lowder C, Belfort R Jr, Lightman S, Foster CS, Robinson MR, Schiffman RM, et al. Dexamethasone intravitreal implant for noninfectious intermediate or posterior uveitis. Arch Ophthalmol. 2011 May. 129(5):545-53. [Medline].
Thorne JE, Daniel E, Jabs DA, et al. Smoking as a risk factor for cystoid macular edema complicating intermediate uveitis. Am J Ophthalmol. 2008 May. 145(5):841-6. [Medline].
Kalinina Ayuso V, Ten Cate HA, van den Does P, Rothova A, de Boer JH. Young age as a risk factor for complicated course and visual outcome in intermediate uveitis in children. Br J Ophthalmol. 2011 May. 95(5):646-51. [Medline].
Abu El-Asrar AM, Geboes K. An immunohistochemical study of the 'snowbank' in a case of pars planiti. Ocul Immunol Inflamm. 2002 Jun. 10(2):117-23. [Medline].
Androudi S, Ahmed M, Fiore T, et al. Combined pars plana vitrectomy and phacoemulsification to restore visual acuity in patients with chronic uveitis. J Cataract Refract Surg. 2005 Mar. 31(3):472-8. [Medline].
Becker MD, Heiligenhaus A, Hudde T, et al. Interferon as a treatment for uveitis associated with multiple sclerosis. Br J Ophthalmol. 2005 Oct. 89(10):1254-7. [Medline].
Biswas J, Raghavendran SR, Vijaya R. Intermediate uveitis of pars planitis type in identical twins. Report of a case. Int Ophthalmol. 1998. 22(5):275-7. [Medline].
Bloch-Michel E, Nussenblatt RB. International Uveitis Study Group recommendations for the evaluation of intraocular inflammatory disease. Am J Ophthalmol. 1987 Feb 15. 103(2):234-5. [Medline].
Bonfioli AA, Damico FM, Curi AL, et al. Intermediate uveitis. Semin Ophthalmol. 2005 Jul-Sep. 20(3):147-54. [Medline].
Bora NS, Bora PS, Tandhasetti MT, et al. Molecular cloning, sequencing, and expression of the 36 kDa protein present in pars planitis. Sequence homology with yeast nucleopore complex protein. Invest Ophthalmol Vis Sci. 1996 Aug. 37(9):1877-83. [Medline].
Boyd SR, Young S, Lightman S. Immunopathology of the noninfectious posterior and intermediate uveitides. Surv Ophthalmol. 2001 Nov-Dec. 46(3):209-33. [Medline].
Brockhurst RJ, Schepens CL, Okamura ID. Uveitis II. Peripheral Uveitis: Clinical Descriptions, Complications and Differential Diagnosis. Am J Ophthalmol. 1960. 49:1257-1266.
Capone A Jr, Aaberg TM. Intermediate Uveitis. In: Albert, Jacobiec FA, eds. Principles and Practice of Ophthalmology. 1994. Philadelphia: WB Saunders:423-442.
Doro D, Manfre A, Deligianni V, et al. Combined 50- and 20-MHz frequency ultrasound imaging in intermediate uveitis. Am J Ophthalmol. 2006 May. 141(5):953-5. [Medline].
Duguid IG, Ford RL, Horgan SE, et al. Combined orbital floor betamethasone and depot methylprednisolone in uveitis. Ocul Immunol Inflamm. 2005 Feb. 13(1):19-24. [Medline].
Ganesh SK, Babu K, Biswas J. Phacoemulsification with intraocular lens implantation in cases of pars planitis. J Cataract Refract Surg. 2004 Oct. 30(10):2072-6. [Medline].
Guest S, Funkhouser E, Lightman S. Pars planitis: a comparison of childhood onset and adult onset disease. Clin Experiment Ophthalmol. 2001 Apr. 29(2):81-4. [Medline].
Holland GN. The enigma of pars planitis, revisited. Am J Ophthalmol. 2006 Apr. 141(4):729-30. [Medline].
Jabs DA, Nussenblatt RB, Rosenbaum JT, et al. Standardization of uveitis nomenclature for reporting clinical data. Results of the First International Workshop. Am J Ophthalmol. 2005 Sep. 140(3):509-16. [Medline].
Jain R, Ferrante P, Reddy GT, et al. Clinical features and visual outcome of intermediate uveitis in children. Clin Experiment Ophthalmol. 2005 Feb. 33(1):22-5. [Medline].
Kaplan HJ. Surgical Treatment of Intermediate Uveitis. Developments in Ophthalmology. 1992. 23:185-189. [Medline].
Malik AR, Pavesio C. The use of low dose methotrexate in children with chronic anterior and intermediate uveitis. Br J Ophthalmol. 2005 Jul. 89(7):806-8. [Medline].
Miserocchi E, Baltatzis S, Ekong A, et al. Efficacy and safety of chlorambucil in intractable noninfectious uveitis: the Massachusetts Eye and Ear Infirmary experience. Ophthalmology. 2002 Jan. 109(1):137-42. [Medline].
Murphy CC, Hughes EH, Frost NA, et al. Quality of life and visual function in patients with intermediate uveitis. Br J Ophthalmol. 2005 Sep. 89(9):1161-5. [Medline].
Nussenblatt RB, Whitcup SM, Palestine AG. Uveitis: Fundamentals and Clinical Practice. 3rd ed. St. Louis: CV Mosby; 2004. 291-300.
Okada AA. Noninfectious uveitis: a scarcity of randomized clinical trials. Arch Ophthalmol. 2005 May. 123(5):682-3. [Medline].
Potter MJ, Myckatyn SO, Maberley AL, et al. Vitrectomy for pars planitis complicated by vitreous hemorrhage: visual outcome and long-term follow-up. Am J Ophthalmol. 2001 Apr. 131(4):514-5. [Medline].
Reinthal EK, Volker M, Freudenthaler N, et al. [Optical coherence tomography in the diagnosis and follow-up of patients with uveitic macular edema]. Ophthalmologe. 2004 Dec. 101(12):1181-8. [Medline].
Smith RE. Pars Planitis. Ryan SJ, ed. Retina. St. Louis: CV Mosby; 1973. Vol. 2: 637-646.
Smith RE, Godfrey WA, Kimura SJ. Chronic cyclitis. I. Course and visual prognosis. Trans Am Acad Ophthalmol Otolaryngol. 1973 Nov-Dec. 77(6):OP760-8. [Medline].
Smith RE, Nozik RA. Uveitis: A Clinical Approach to Diagnosis and Management. 2nd ed. Baltimore: Williams and Wilkins; 1989. 166-170.
Stanford MR, Vaughan RW, Kondeatis E, et al. Are cytokine gene polymorphisms associated with outcome in patients with idiopathic intermediate uveitis in the United Kingdom?. Br J Ophthalmol. 2005 Aug. 89(8):1013-6. [Medline].
Stavrou P, Baltatzis S, Letko E, et al. Pars plana vitrectomy in patients with intermediate uveitis. Ocul Immunol Inflamm. 2001 Sep. 9(3):141-51. [Medline].
Tabbara KF, Al-Kaff AS, Al-Rajhi AA, et al. Heparin Surface-Modified Intraocular Lenses in Patients with Inactive Uveitis or Diabetes. Ophthalmology. 1998. 105(5):843-845. [Medline].
Tessler HH, Fraber MD. Intraocular Lens Implantation Versus no Intraocular Lens Implantation in Patients with Chronic Iridocyclitis and Pars Planitis: A Randomized Prospective Study. Ophthalmology. 1993. 100 (8):1206-1209. [Medline].
Trittibach P, Koerner F, Sarra GM, et al. Vitrectomy for juvenile uveitis: prognostic factors for the long-term functional outcome. Eye. 2006 Feb. 20(2):184-90. [Medline].
Tugal-Tutkun I, Havrlikova K, Power WJ, et al. Changing patterns in uveitis of childhood. Ophthalmology. 1996 Mar. 103(3):375-83. [Medline].
Ozzello DJ, Palestine AG. Factors affecting therapeutic decisions in intermediate and posterior uveitis. Am J Ophthalmol. 2015 Feb. 159 (2):213-20.e3. [Medline].

Media Gallery

of 0

Author

Robert H Janigian, Jr, MD Clinical Assistant Professor, Department of Surgery (Ophthalmology), The Warren Alpert Medical School of Brown University

Robert H Janigian, Jr, MD is a member of the following medical societies: American Academy of Ophthalmology, American Society of Retina Specialists, Rhode Island Medical Society

Disclosure: Shareholder for: Abbvie.

Coauthor(s)

Theodoros Filippopoulos, MD Head of Glaucoma Clinic, Athens Vision Eye Institute; Clinical Lecturer, Department of Ophthalmology, Second Ophthalmology Clinic, University of Athens Medical School, Greece

Theodoros Filippopoulos, MD is a member of the following medical societies: American Academy of Ophthalmology, American Medical Association, American Society of Cataract and Refractive Surgery, Association for Research in Vision and Ophthalmology, American Glaucoma Society

Disclosure: Nothing to disclose.

Brian A Welcome, MD Staff Physician, Department of Ophthalmology, Rhode Island Hospital

Brian A Welcome, MD is a member of the following medical societies: American Academy of Ophthalmology, American Medical Association, American Glaucoma Society, American 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

Hampton Roy, Sr, MD Associate Clinical Professor, Department of Ophthalmology, University of Arkansas for Medical Sciences

Hampton Roy, Sr, MD is a member of the following medical societies: American Academy of Ophthalmology, American College of Surgeons, Pan-American Association of Ophthalmology

Disclosure: Nothing to disclose.

Additional Contributors

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, American Uveitis Society, Association for Research in Vision and Ophthalmology

Disclosure: Serve(d) as a director, officer, partner, employee, advisor, consultant or trustee for: 1-800-DOCTORS; AbbVie; Alcon; Aldeyra; Allergan; Alphaeon; ArcScan; Baush+Lomb; Bio-Tissue; Clearside; EyeGate; Hovione; Mededicus; NovaBay; Omeros; Pentavision; Portage; Santen; Science Based Health; Senju; Shire; Sun Pharma; TearLab;TearScience;Topivert<br/>Serve(d) as a speaker or a member of a speakers bureau for: AbbVie; Alcon; Allergan; Bausch+Lomb; Bio-tissue; EyeGate;Hovione;LayerBio; NovaBay;Omeros;Portage; Santen; Shire; Stemnion; Sun Pharma;TearLab;TearScience; Topivert <br/>Received research grant from: Alcon; Aldeyra; allergan; Baush+ Lomb; EyeGate; Hovione; Kala; Ocular Therapeutix;Pfizer; RPS; Santen;Senju;Shire;Topcon; Xoma.

Sections Intermediate Uveitis
Overview
Presentation
- History
- Physical
- Causes
- Show All
DDx
Workup
Treatment
Medication
Follow-up
References

Intermediate Uveitis

Background

Pathophysiology

Epidemiology

Frequency

Mortality/Morbidity

Race

Sex

Age

About

Membership

Apps

WebMD Network

Editions

What to Read Next on Medscape

Intermediate Uveitis

Background

Pathophysiology

Epidemiology

Frequency

Mortality/Morbidity

Race

Sex

Age

References

Tables

Contributor Information and Disclosures

What would you like to print?

Find Us On

About

Membership

Apps

WebMD Network

Editions

What to Read Next on Medscape

Medscape Consult

Tools

Most Popular Articles