Pars Planitis

Pars Planitis

National Organization for Rare Disorders, Inc.

Important

It is possible that the main title of the report Pars Planitis is not the name you expected. Please check the synonyms listing to find the alternate name(s) and disorder subdivision(s) covered by this report.

Synonyms

  • Peripheral Retinal Inflammation
  • PP
  • Vitritis
  • Intermediate Uveitis (UI)

Disorder Subdivisions

  • None

General Discussion

Although pars planitis is generally benign, there can be significant vision loss in extreme cases. It is an immunological disorder of the eye characterized by inflammation of a part of the uvea, the layer of tissue between the sclera and the retina, the membranes protecting the eyeball. The uvea, in turn, is made up of three portions: the iris, the ciliary body, and the choroid. In addition, the uvea contains many of the blood vessels that supply the eye.



The pars plana is a narrow section of the ciliary body, inflammation of which is known as pars planitis. In association with the inflammation or immunological response, fluid and cells infiltrate the clear gelatin-like substance (vitreous humor) of the eyeball, near the retina and/or pars plana. As a result, swelling of the eye or eyes can also occur, but more importantly blurred vision and progressive increase in the vision of floaters is reported as main symptoms by patients suffering this condition as a result of the infiltration of the vitreous humor.



The inflammation occurs in the intermediate zone of the eye; that is, between the anterior part(s) of the eye (iris) and the posterior part(s), the retina and/or choroid. It has therefore been designated as one of the diseases of a family of intermediate uveitis.



In some cases, the disturbance of vision may be slightly progressive.

Symptoms

The symptoms of pars planitis include blurred vision and dark floating spots that intrude upon clarity of eyesight. Swelling can occur inside the eye, particularly on the peripheral retina or macula that may lead to decreased vision. Glaucoma may occur as well.



The clinical picture may be complicated by cataracts, retinal detachment, or fluid within the retina (macular edema)



More often the examining ophthalmologist (specialist in diseases of the eye) will see clusters of white blood cells trapped within the eyeball that are called snowballs. The term physicians may use for these is inflammatory exudate. These are clusters of white blood cells trapped within the eyeball. If these clusters are located on the pars plana, they are known as snowbanks.

Causes

Pars planitis is thought to be an autoimmune disorder. An autoimmune reaction causes the inflammation that is thought to cause symptoms of this disorder.



Autoimmune disorders are caused when the body's natural defenses against foreign or invading organisms (e.g., antibodies) begin to attack healthy tissue for unknown reasons. In rare cases, pars planitis has occurred within families; however, as yet, no genetic inheritance pattern has been identified.



Some clinicians have put forward the idea that the immunological response may be the result of one of two possible causes: (1) an isolated set of events internal to the patient (endogenous), or (2) an association with some other disorder (exogenous). Among the disorders with which pars planitis has been associated are: multiple sclerosis, Lyme disease, Behcet disease, sarcoidosis, and tuberculosis, among others.

Affected Populations

Pars planitis is an uncommon ocular disorder of children and young adults that affects males slightly more frequently than females.

Standard Therapies

Diagnosis

Pars planitis is usually diagnosed by means of a thorough physical evaluation, detailed patient history, and specialized eye examination.



Treatment

If no external causes of Pars planitis are found, treatment typically consists of corticosteroid drugs to control inflammation. If steroids are used, the patient must be carefully monitored.



If an external association of pars planitis is identified, then treatment of the associated disorder may take care of the inflammation.



Pars planitis may be accompanied by blood leaking from ruptured small blood vessels in the eye. These are generally not serious and, if necessary, can be treated by laser or cryotherapy (freezing of tissue) to seal blood vessels and arrest leakage.



Other treatments are symptomatic and supportive, as well as treatment of the complications (cataracts, glaucoma, cystoid macular edema, etc).

Investigational Therapies

Information on current clinical trials is posted on the Internet at www.clinicaltrials.gov. All studies receiving U.S. government funding, and some supported by private industry, are posted on this government web site.



Also website: www.uveitis.org has information regarding this condition and current trials available.



For information about clinical trials being conducted at the NIH Clinical Center in Bethesda, MD, contact the NIH Patient Recruitment Office:



Tollfree: (800) 411-1222

TTY: (866) 411-1010

Email: prpl@cc.nih.gov



For information about clinical trials sponsored by private sources, contact:

www.centerwatch.com

References

TEXTBOOKS

Guest S. Pars Planitis. In: NORD Guide to Rare Disorders. Lippincott Williams & Wilkins. Philadelphia, PA. 2003:656-57.



Beers MH, Berkow R, eds. The Merck Manual, 17th ed. Whitehouse Station, NJ: Merck Research Laboratories; 1999:726.



Kanski JJ, ed. Clinical Ophthalmology. 4th ed. Butterworth-Heinemann. Oxford, UK; 1999:309-11.



Newell FW, ed. Ophthalmology: Principles and Concepts. 7th ed. Mosby Year Book, St. Louis, MO; 1991:333-34.



JOURNAL ARTICLES

Abu El-Asrar A, Geboes K. An immunohistochemical study of the 'snowbank' in a case of pars planitis. Ocul Immunol Inflamm. 2002;10:117-23.



Greiner KH, Kilmartin DJ, Forrester JV, et al. Grading of pars planitis by ultrasound biomicroscopy/echographic and clinical study. Eur J Ultrasound.2002;15:139-44.



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;109:137-42.



Lauer AK, Smith JR, Robertson JE, et al. Vitreous hemorrhage is a common complication of pediatric pars planitis. Ophthalmology. 2002;109:95-98.



Prieto JF, Dios E, Gutierrez JM, et al. Pars planitis: epidemiology, treatment, and association with multiple sclerosis. Ocul Immunol Inflamm. 2001;9:93-102.



Guest S, Funkhouser E, Lightman S. Pars planitis: a comparison of childhood onset and adult onset disease. Clin Experiment Ophthalmol. 2001;29:81-84.



Biousse V, Trichet C, Bloch-Michel E, et al. Multiple sclerosis associated with uveitis in two large clinic-based series. Neurology. 1999;52:179-81.



FROM THE INTERNET

McKusick VA, ed. Online Mendelian Inheritance in Man (OMIM). The Johns Hopkins University. Pars Planitis. Entry Number; 606177: Last Edit Date; 8/8/2001.



Douglas RS. Uveitis. Medical Encylopedia. MEDLINEplus. Update: 12/22/2002. 3pp.

www.nlm.nih.gov/medlineplus/print/ency/article/001005.htm



Pars Planitis: A Syndrome of Unknown Etiology or a Clinical Picture of Multiple Etiologies.

Massachusetts Eye and Ear Infirmary, Immunology Service, 1999. 1p.

www.uveitis.org/enhanced/Backup/webeyemd_htmbank/pars.htm



Ocular Immunology. MEEI. Last Update: 04/08/2003. 3pp.

www.uveitis.org/enhanced/index.htm



pars planitis. General Practice Notebook. © 2003. 1p.

www.gpnotebook.co.uk/simplepage.cfm?ID=772145184



Pars Planitis. Handbook of Ocular Disease management. nd. 2pp.

www.revoptom.com/handbook/SECT35b.HTM

Resources

American Autoimmune Related Diseases Association, Inc.

22100 Gratiot Ave.

Eastpointe, MI 48021

Tel: (586)776-3900

Fax: (586)776-3903

Tel: (800)598-4668

Email: aarda@aarda.org

Internet: http://www.aarda.org/



Association for Macular Diseases, Inc.

210 E. 64th St.

8th Floor

New York, NY 10065

Tel: (212)605-3719

Fax: (212)605-3795

Email: association@retinal-research.org

Internet: http://www.macula.org/



NIH/National Eye Institute

31 Center Dr

MSC 2510

Bethesda, MD 20892-2510

United States

Tel: (301)496-5248

Fax: (301)402-1065

Email: 2020@nei.nih.gov

Internet: http://www.nei.nih.gov/



Ocular Immunology and Uveitis Foundation

5 Cambridge Center

8th Floor

Cambridge, MA 02142

Tel: (617)621-6377

Fax: (781)431-2042

Tel: (866)353-6377

Email: fosters@comcast.net

Internet: http://www.uveitis.org



Genetic and Rare Diseases (GARD) Information Center

PO Box 8126

Gaithersburg, MD 20898-8126

Tel: (301)251-4925

Fax: (301)251-4911

Tel: (888)205-2311

TDD: (888)205-3223

Internet: http://rarediseases.info.nih.gov/GARD/



AutoImmunity Community

Email: moderator@autoimmunitycommunity.org

Internet: http://www.autoimmunitycommunity.org



For a Complete Report

This is an abstract of a report from the National Organization for Rare Disorders, Inc.® (NORD). Cigna members can access the complete report by logging into myCigna.com. For non-Cigna members, a copy of the complete report can be obtained for a small fee by visiting the NORD website. The complete report contains additional information including symptoms, causes, affected population, related disorders, standard and investigational treatments (if available), and references from medical literature. For a full-text version of this topic, see http://www.rarediseases.org/search/rdblist.html.

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