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ERROR: type should be string, got "https://profreg.medscape.com/px/getpracticeprofile.do?method=getProfessionalProfile&urlCache=aHR0cHM6Ly9lbWVkaWNpbmUubWVkc2NhcGUuY29tL2FydGljbGUvMTIwODc5NC1jbGluaWNhbA==\nIntermediate Uveitis Clinical Presentation\nAuthor: Robert H Janigian, Jr, MD; Chief Editor: Hampton Roy, Sr, MD more...\nIntermediate Uveitis\nSections Intermediate Uveitis\nImmunosuppressives\nHistamine H2-receptor antagonist\nGastric pump inhibitors\nDMARDs, TNF Inhibitors\nCorticosteroid Ophthalmic Implants\nThe most common symptoms of intermediate uveitis are blurry vision and floaters.\nPain and photophobia are the exception.\nBilateral involvement at initial presentation approaches 80%, although it is frequently asymmetric. Eventually, approximately one third of unilateral cases will become bilateral.\nLater in the disease course, more severe visual loss may occur secondary to chronic CME (28-50%), uveitic glaucoma (15%), rhegmatogenous retinal detachment (3-22%), vitreous hemorrhage (6-28%), cataracts (15-20%), or cyclitic membrane development.\nSee the list below:\nOn ocular examination, the ophthalmologist encounters vitritis that ranges in severity. The absence of cellular activity in the vitreous precludes the diagnosis of active intermediate uveitis.\nThe presenting visual acuity is often reduced to 20/40 (mild visual loss) due to mild vitritis and CME.\nAnterior segment inflammation is infrequent and more commonly associated with pediatric intermediate uveitis. On occasion, patients with MS develop granulomatous anterior uveitis with characteristic mutton keratic precipitates.\nAggregates of inflammatory cells may appear in the inferior vitreous as white or yellow tufts termed vitreous snowballs. A snowbank, the requisite finding in pars planitis, may be seen as a grayish yellow exudate along the inferior ora serrata, frequently extending over the pars plana. Not all patients with intermediate uveitis manifest snowbanks.\nIn severe cases, the exudates may coalesce across the entire periphery for 360°, albeit rarely.\nScleral depression is usually required to appreciate snowbanks, but, sometimes, they can be seen with the eye infraducted using an indirect ophthalmoscope without the 20 D-lens.\nIn fact, snowbanks may be fibroglial masses and not a true protein exudate (see Histologic Findings).\nPeripheral retinal vascular abnormalities are not uncommon but may become obscured by the dense vitritis.\nSheathing or obliteration of small venules may be noted. This finding may appear months or years after initial presentation.\nLess often, a periarteritis or a combined perivasculitis is present with exudates.\nPeripheral retinal neovascularization can occur as a result of ischemia, causing vitreous hemorrhages; this occurs more commonly in children.\nThe neovascularization can evolve into a vascular cyclitic membrane in the rare patient, exercising traction on the ciliary body and leading to hypotony and phthisis bulbi.\nCME may be seen. Severe macular edema can be appreciated clinically. Angiographic study or optical coherence tomography is often necessary for a definitive diagnosis, especially if the edema is subtle or if the media are hazy. Some patients with angiographic CME may present with 20/20 acuity.\nEstimates of the incidence of macular edema vary.\nMost early reports have noted this complication in 28-50% of cases.\nOptic nerve edema is not uncommon, especially in pediatric cases where the disk is edematous at least half of the time. In a retrospective study, optic disk edema was found in 71% of patients with onset of the disease before age 16 years.\nIn the anterior segment, late findings include anterior and posterior synechiae, band keratopathy, cataracts, and glaucoma.\nThe glaucoma may be related to both the uveitis and/or corticosteroid use.\nThe incidence of cataract formation, most often a posterior subcapsular opacity, has been reported in approximately 15-20% of cases and may not be independent to the use of steroids for treatment.\nThe late complications of intermediate uveitis are important to recognize early.\nA combination of vitreous hemorrhage and vitreous fibrosis can cause traction on the peripheral retina and lead to retinal detachment. Studies vary widely in the frequency of this late complication, ranging from 3-22%. Some detachments may become complete, leading to a phthisical eye.\nChronic CME may cause moderate-to-severe vision loss. Treating CME, regardless of how good the vision may be, is therefore imperative.\nPeripheral retinal neovascularization can occur as a result of ischemia, causing vitreous hemorrhages, as discussed above.\nA vascular cyclitic membrane can exercise traction on the ciliary body and lead to hypotony and phthisis bulbi.\nThe cause of intermediate uveitis or pars planitis has not been elucidated. Intermediate uveitis is a category of uveitis based on an anatomical classification system that can include diseases of various etiology and clinical manifestations. Associations of the disease with such entities as MS, sarcoidosis, or inflammatory bowel disease suggest an autoimmune component in at least a subset of patients. The clustering of familial cases has led to the investigation of human leukocyte antigen (HLA) associations. The inciting event appears to be peripheral retinal perivasculitis and vascular occlusion, leading to ocular inflammation, vitritis, and snowbank formation. The etiology of the antigenic stimulus is not clear and may be either vitreal or perivascular in nature.\nIn 1963, Kimura and Hogan first noted several members of one family to be afflicted with chronic cyclitis. [1] Since then, there have been multiple case reports of intermediate uveitis in families, including a case report in identical twins.\nSeveral studies show that the HLA-DR2 histocompatibility complex gene is associated with intermediate uveitis, suggesting an immunogenetic predisposition for the disorder in some cases.\nIn a prospective study of 53 patients with pars planitis by Raja et al, an association was found with the HLA-DR15, a subtype of HLA-DR2. [2] In addition, there was a suggestion that the association was stronger for patients with both pars planitis and MS. This supports previous studies showing a similar relationship.\nOther associations include HLA-A28, HLA-B8, and HLA-B51.\nIt is evident that genetics plays some role in the pathophysiology of intermediate uveitis, but the importance remains unclear.\nIn addition, cytokine gene polymorphism may be associated with disease development and visual prognosis in patients with intermediate uveitis. In particular, TT homozygotes for the interferon-gamma (INF-gamma) gene may be at a higher risk of disease development and may also run a more severe course.\nDespite a high prevalence of intermediate uveitis and pars planitis in uveitis clinics, the causative factors are still unknown. Apart from idiopathic forms of the disease, there are known associations with such entities as MS, sarcoidosis, and inflammatory bowel disease. Evidence of a systemic disorder can be found in up to one third of patients with intermediate uveitis. Infectious etiologies include Epstein-Barr virus (EBV) infection, Lyme disease, human T-cell lymphotrophic virus type1 (HTLV-1) infection, cat scratch disease, and hepatitis C.\nThe association between MS and intermediate uveitis is well documented. Raja et al reported a 16.2% prevalence rate of MS in a small population with pars planitis, which agrees with the findings of Malinowski and his colleagues. [2, 3]\nRetinal phlebitis, vitreous cells or snowball opacities, posterior synechiae, iritis, iridocyclitis, and retinal neovascularization are common manifestations of ocular sarcoidosis, which emphasizes the overlap of ophthalmologic signs between idiopathic intermediate uveitis and ocular sarcoidosis.\nKimura SJ, Hogan MJ. Chronic Cyclitis. Arch of Ophthalmol. 1964. 71:193-201.\nRaja SC, Jabs DA, Dunn JP, et al. Pars Planitis: Clinical Features and Class II HLA Associations. Ophthalmology. 1999. 106 (3):594-599. [Medline].\nMalinowski 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].\nHenderly 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].\nRodriguez 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].\nKaplan HJ. Intermediate Uveitis (Pars Planitis, Chronic Cyclitis)- A Four Step Approach to Treatment. Saari KM, ed. Uveitis Update. Amsterdam: Exerpta Medica; 1984. 169-172.\nHogewind 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].\nMurphy 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].\nJaffe 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].\nNguyen 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].\nMarkomichelakis 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].\nRajaraman 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].\nDevenyi 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].\nPark SE, Mieler WF, Pulido JS. 2 peripheral scatter photocoagulation for neovascularization associated with pars planitis. Arch Ophthalmol. 1995 Oct. 113(10):1277-80. [Medline].\nBecker M, Davis J. Vitrectomy in the treatment of uveitis. Am J Ophthalmol. 2005 Dec. 140(6):1096-105. [Medline].\nQuinones 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].\nJaffe 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].\nLowder 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].\nThorne 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].\nKalinina 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].\nAbu 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].\nAndroudi 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].\nBecker 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].\nBiswas 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].\nBloch-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].\nBonfioli AA, Damico FM, Curi AL, et al. Intermediate uveitis. Semin Ophthalmol. 2005 Jul-Sep. 20(3):147-54. [Medline].\nBora 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].\nBoyd SR, Young S, Lightman S. Immunopathology of the noninfectious posterior and intermediate uveitides. Surv Ophthalmol. 2001 Nov-Dec. 46(3):209-33. [Medline].\nBrockhurst RJ, Schepens CL, Okamura ID. Uveitis II. Peripheral Uveitis: Clinical Descriptions, Complications and Differential Diagnosis. Am J Ophthalmol. 1960. 49:1257-1266.\nCapone A Jr, Aaberg TM. Intermediate Uveitis. In: Albert, Jacobiec FA, eds. Principles and Practice of Ophthalmology. 1994. Philadelphia: WB Saunders:423-442.\nDoro 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].\nDuguid 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].\nGanesh 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].\nGuest 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].\nHolland GN. The enigma of pars planitis, revisited. Am J Ophthalmol. 2006 Apr. 141(4):729-30. [Medline].\nJabs 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].\nJain 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].\nKaplan HJ. Surgical Treatment of Intermediate Uveitis. Developments in Ophthalmology. 1992. 23:185-189. [Medline].\nMalik 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].\nMiserocchi 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].\nMurphy 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].\nNussenblatt RB, Whitcup SM, Palestine AG. Uveitis: Fundamentals and Clinical Practice. 3rd ed. St. Louis: CV Mosby; 2004. 291-300.\nOkada AA. Noninfectious uveitis: a scarcity of randomized clinical trials. Arch Ophthalmol. 2005 May. 123(5):682-3. [Medline].\nPotter 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].\nReinthal 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].\nSmith RE. Pars Planitis. Ryan SJ, ed. Retina. St. Louis: CV Mosby; 1973. Vol. 2: 637-646.\nSmith 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].\nSmith RE, Nozik RA. Uveitis: A Clinical Approach to Diagnosis and Management. 2nd ed. Baltimore: Williams and Wilkins; 1989. 166-170.\nStanford 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].\nStavrou 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].\nTabbara 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].\nTessler 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].\nTrittibach 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].\nTugal-Tutkun I, Havrlikova K, Power WJ, et al. Changing patterns in uveitis of childhood. Ophthalmology. 1996 Mar. 103(3):375-83. [Medline].\nOzzello DJ, Palestine AG. Factors affecting therapeutic decisions in intermediate and posterior uveitis. Am J Ophthalmol. 2015 Feb. 159 (2):213-20.e3. [Medline].\nRobert H Janigian, Jr, MD Clinical Associate Professor, Department of Surgery (Ophthalmology), The Warren Alpert Medical School of Brown University\nRobert 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\nTheodoros Filippopoulos, MD Head of Glaucoma Clinic, Athens Vision Eye Institute; Clinical Lecturer, Department of Ophthalmology, Second Ophthalmology Clinic, University of Athens Medical School, Greece\nTheodoros 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\nBrian A Welcome, MD Staff Physician, Department of Ophthalmology, Rhode Island Hospital\nBrian 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\nR Christopher Walton, MD Adjunct Professor, Department of Ophthalmology, University of Texas Health Science Center at San Antonio\nR Christopher Walton, MD is a member of the following medical societies: American Academy of Ophthalmology, American Uveitis Society\nHampton Roy, Sr, MD Associate Clinical Professor, Department of Ophthalmology, University of Arkansas for Medical Sciences\nHampton Roy, Sr, MD is a member of the following medical societies: American Academy of Ophthalmology, American College of Surgeons, Pan-American Association of Ophthalmology\nJohn 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\nJohn 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\nDisclosure: 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.\nencoded search term (Intermediate Uveitis) and Intermediate Uveitis\nUveitis Evaluation and Treatment\nUveitis Classification\nIritis and Uveitis\nGranulomatous Iritis (Anterior Uveitis)\nJuvenile Idiopathic Arthritis Uveitis\nUveitis, Anterior, Childhood\nOn Pembrolizumab Therapy for Stage III Melanoma, Side Effects Linked to Longer Recurrence-Free Survival\nEfficacy and Safety of Apremilast for Behçet's Syndrome\nM. Pneumoniae May Trigger Inflammatory Skin and Mucous Membrane Lesions in Children\nHidden Heart Disease: 19 Dermatologic Clues You Should Know\nDiseases & Conditions Uveitis Evaluation and Treatment\nDiseases & Conditions Uveitis Classification\nDiseases & Conditions Intermediate Uveitis\nDiseases & Conditions Juvenile Idiopathic Arthritis Uveitis"