Pars Planitis Epidemiology, Diagnosis, Follow-Up And Prognosis

Merve Bahar

doi:10.7197/cmj.1386749

Review

Year 2023, Volume: 45 Issue: 4, 8 - 16, 31.12.2023

Merve Bahar

https://doi.org/10.7197/cmj.1386749

Abstract

References

1. Jabs, D.A., R.B. Nussenblatt, and J.T. Rosenbaum, Standardization of uveitis nomenclature for reporting clinical data. Results of the First International Workshop. Am J Ophthalmol, 2005. 140(3): p. 509-16.
2. Smith, J.A., et al., Epidemiology and course of disease in childhood uveitis. Ophthalmology, 2009. 116(8): p. 1544-51, 1551.e1.
3. Nikkhah, H., et al., Childhood pars planitis; clinical features and outcomes. J Ophthalmic Vis Res, 2011. 6(4): p. 249-54
4. Soylu, M., G. Ozdemir, and A. Anli, Pediatric uveitis in southern Turkey. Ocul Immunol Inflamm, 1997. 5(3): p. 197-202.
5. Ozdal, P.C., et al., Patterns of childhood-onset uveitis in a referral center in Turkey. J Ophthalmic Inflamm Infect, 2012. 2(1): p. 13-9.
6. Arellanes-García, L., L. Navarro-López, and C. Recillas-Gispert, Pars planitis in the Mexican Mestizo population: ocular findings, treatment, and visual outcome. Ocul Immunol Inflamm, 2003. 11(1): p. 53-60.
7. Paroli, M.P., et al., Intermediate uveitis: comparison between childhood-onset and adult- onset disease. Eur J Ophthalmol, 2014. 24(1): p. 94-100.
8. Heinz, C., S. Schoonbrood, and A. Heiligenhaus, Intermediate uveitis in children and young adults: differences in clinical course, associations and visual outcome. Br J Ophthalmol, 2014. 98(8): p. 1107-11.
9. Paroli, M.P., et al., Intermediate uveitis in a pediatric Italian population. Ocul Immunol Inflamm, 2011. 19(5): p. 321-6.
10. Malinowski, S.M., et al., The association of HLA-B8, B51, DR2, and multiple sclerosis in pars planitis. Ophthalmology, 1993. 100(8): p. 1199-205.
11. Arellanes-García, L., et al., Idiopathic intermediate uveitis in childhood. Int Ophthalmol Clin, 2008. 48(3): p. 61-74.
12. Donaldson, M.J., et al., Pars planitis: a 20-year study of incidence, clinical features, and outcomes. Am J Ophthalmol, 2007. 144(6): p. 812-817.
13. Navarrete, A., A. Koriat, and R. Amer, Implications of pars planitis-associated cystoid macular edema on visual outcome and management in children. Graefes Arch Clin Exp Ophthalmol, 2020. 258(8): p. 1803-1811.
14. Tugal-Tutkun, I., Pediatric uveitis. J Ophthalmic Vis Res, 2011. 6(4): p. 259-69.
15. Khodadoust, A.A., et al., Pars planitis and autoimmune endotheliopathy. Am J Ophthalmol, 1986. 102(5): p. 633-9.
16. de Boer, J., et al., Long-term follow-up of intermediate uveitis in children. Am J Ophthalmol, 2006. 141(4): p. 616-21.
17. Prieto, J.F., et al., Pars planitis: epidemiology, treatment, and association with multiple sclerosis. Ocul Immunol Inflamm, 2001. 9(2): p. 93-102.
18. Yalçındağ, F.N., E. Temel, and E.G. Özgür, Spectral domain optical coherence tomography findings of patients with pars planitis and risk factors affecting visual acuity. Int Ophthalmol, 2021.
19. Finamor, L.P., C. Muccioli, and R. Belfort, Jr., Imaging techniques in the diagnosis and management of uveitis. Int Ophthalmol Clin, 2005. 45(2): p. 31-40.
20. Tran, V.T., P. LeHoang, and C.P. Herbort, Value of high-frequency ultrasound biomicroscopy in uveitis. Eye (Lond), 2001. 15(Pt 1): p. 23-30.
21. Kadayifçilar, S., B. Eldem, and B. Tumer, Uveitis in childhood. J Pediatr OphthalmolStrabismus, 2003. 40(6): p. 335-40.
22. Kalinina Ayuso, V., et al., Young age as a risk factor for complicated course and visual outcome in intermediate uveitis in children. Br J Ophthalmol, 2011. 95(5): p. 646-51.
23. Guest, S., E. Funkhouser, and S. Lightman, Pars planitis: a comparison of childhood onset and adult onset disease. Clin Exp Ophthalmol, 2001. 29(2): p. 81-4.
24. Lehpamer, B., et al., Epiretinal membranes in uveitic macular edema: effect on vision and response to therapy. Am J Ophthalmol, 2014. 157(5): p. 1048-55.
25. Albavera-Giles, T., et al., Outcomes of cataract surgery with/without vitrectomy in patients with pars planitis and immunosuppressive therapy. Graefes Arch Clin Exp Ophthalmol, 2017. 255(6): p. 1213-1219.
26. Jalil, A., F.E. Dhawahir-Scala, and N.P. Jones, Nonprogressive tractional inferior retinal elevation in intermediate uveitis. Ocul Immunol Inflamm, 2010. 18(1): p. 60-3.
27. Pollack, A.L., et al., Peripheral retinoschisis and exudative retinal detachment in pars planitis. Retina, 2002. 22(6): p. 719-24.
28. Zierhut, M., et al., Therapy of uveitis in children. Int Ophthalmol Clin, 2008. 48(3): p. 131-52.
29. Quinones, K., et al., Pars plana vitrectomy versus immunomodulatory therapy for intermediate uveitis: a prospective, randomized pilot study. Ocul Immunol Inflamm, 2010. 18(5): p. 411-7.
30. Maccora, I., E.S. Sen, and A.V. Ramanan, Update on noninfectious uveitis in children and its treatment. Curr Opin Rheumatol, 2020. 32(5): p. 395-402.
31. Ozdal, P.C., N. Berker, and I. Tugal-Tutkun, Pars Planitis: Epidemiology, Clinical Characteristics, Management and Visual Prognosis. J Ophthalmic Vis Res, 2015. 10(4): p. 469 80.
32. Silman, A.J., et al., Lymphoproliferative cancer and other malignancy in patients with rheumatoid arthritis treated with azathioprine: a 20 year follow up study. Ann Rheum Dis, 1988. 47(12): p. 988-92.
33. Budde, K., et al., Pharmacodynamic monitoring of mycophenolate mofetil. Clin Chem Lab Med, 2000. 38(11): p. 1213-6.
34. Vitale, A.T., A. Rodriguez, and C.S. Foster, Low-dose cyclosporin A therapy in treating chronic, noninfectious uveitis. Ophthalmology, 1996. 103(3): p. 365-73; discussion 373-4.
35. Pasadhika, S. and J.T. Rosenbaum, Update on the use of systemic biologic agents in the treatment of noninfectious uveitis. Biologics, 2014. 8: p. 67-81.
36. Rifkin, L.M., A.D. Birnbaum, and D.A. Goldstein, TNF inhibition for ophthalmic indications: current status and outlook. BioDrugs, 2013. 27(4): p. 347-57.
37. Cordero-Coma, M. and L. Sobrin, Anti-tumor necrosis factor-α therapy in uveitis. Surv Ophthalmol, 2015. 60(6): p. 575-89.
38. Hu, S., et al., Comparison of the inhibition mechanisms of adalimumab and infliximab in treating tumor necrosis factor α-associated diseases from a molecular view. J Biol Chem,2013. 288(38): p. 27059-27067.
39. Nguyen, Q.D., 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. 388(10050): p. 1183-92.
40. Díaz-Llopis, M., et al., Treatment of refractory uveitis with adalimumab: a prospective multicenter study of 131 patients. Ophthalmology, 2012. 119(8): p. 1575-81.
41. Fabiani, C., et al., Comparative efficacy between adalimumab and infliximab in the treatment of non-infectious intermediate uveitis, posterior uveitis, and panuveitis: a retrospective observational study of 107 patients. Clin Rheumatol, 2019. 38(2): p. 407-415.
42. Vazquez-Cobian, L.B., T. Flynn, and T.J. Lehman, Adalimumab therapy for childhood uveitis. J Pediatr, 2006. 149(4): p. 572-5.
43. Simonini, G., et al., Superior efficacy of Adalimumab in treating childhood refractory chronic uveitis when used as first biologic modifier drug: Adalimumab as starting anti-TNF-α therapy in childhood chronic uveitis. Pediatr Rheumatol Online J, 2013. 11: p. 16.
44. Simonini, G., et al., Prevention of flare recurrences in childhood-refractory chronic uveitis: an open-label comparative study of adalimumab versus infliximab. Arthritis Care Res (Hoboken),2011. 63(4): p. 612-8.
45. Oray, M. and İ. Tuğal-Tutkun, Treatment of Juvenile Idiopathic Arthritis-Associated Uveitis. Turk J Ophthalmol, 2016. 46(2): p. 77-82.
46. Najjar, D.M., et al., EDTA chelation for calcific band keratopathy: results and long-term follow-up. Am J Ophthalmol, 2004. 137(6): p. 1056-64.
47. Holland, G.N., Intraocular lens implantation in patients with juvenile rheumatoid arthritis- associated uveitis: an unresolved management issue. Am J Ophthalmol, 1996. 122(2): p. 255-7.
48. Sijssens, K.M., et al., Long-term ocular complications in aphakic versus pseudophakic eyes of children with juvenile idiopathic arthritis-associated uveitis. Br J Ophthalmol, 2010. 94(9): p. 1145-9.
49. Ladas, J.G., et al., Relationship between aqueous humor protein level and outflow facility in patients with uveitis. Invest Ophthalmol Vis Sci, 2001. 42(11): p. 2584-8.

Pars Planitis Epidemiology, Diagnosis, Follow-Up And Prognosis

Year 2023, Volume: 45 Issue: 4, 8 - 16, 31.12.2023

Merve Bahar

https://doi.org/10.7197/cmj.1386749

Abstract

Pars planitis (PP) is an idiopathic intermediate uveitis accompanied by snowbanks and snowballs that often affects the pediatric and adolescent age groups. PP accounts for 5-26.7% of pediatric uveitis in different series. Histopathological and clinical findings indicate autoimmune etiology. It shows bilateral and asymmetrical involvement. While patients often complain of blurred vision and floaters, sometimes PP can be asymptomatic. Complications develop as a result of chronic involvement. Diagnosis is made by clinical examination and imaging methods. Treatment aims to suppress inflammation in the acute period and to reduce the frequency, severity and complications of exacerbations in the long term. The ultimate goal is to prevent ocular morbidity by providing complete remission. Conventional treatments include corticosteroids and immunomodulatory (IMT) agents such as methotrexate (MTX), azathioprine (AZA), cyclosporine A (CSA), mycophenolate mofetil (MFM). In recent years, new treatment options including biological agents such as anti-TNF-α therapy have become widespread and are used effectively in treatment. The most important point regarding the necessity of surgical treatment is that surgical success depends on the complete suppression of ocular inflammation with medical treatment. Therefore, it must be ensured that full inflammation control is achieved before surgery.

Pars planit (PP) sıklıkla pediatrik ve adölesan yaş grubunu etkileyen snowbank ve snowball’ların eşlik ettiği idiyopatik intermediate üveittir. PP farklı serilerde pediatrik üveitlerin %5-26,7'sini oluşturmaktadır. Histopatolojik ve klinik bulgular otoimmün etiyolojiye işaret eder. Bilateral, asimetrik tutulum gösterir. Hastalar sıklıkla bulanık görme ve uçuşma şikayetiyle başvurur. Bazen de asemptomatik seyreder. Kronik tutulum sonucu komplikasyon gelişimine rastlanabilir. Tanı klinik muayene ve görüntüleme yöntemleriyle konur. Tedavinin amacı akut dönemde enflamasyonu baskılamak, uzun dönemde ise atakların sıklığını, şiddetini ve komplikasyonları azaltmaktır. Nihai amaç, tam bir remisyon sağlanarak oküler morbiditenin önlenmesidir. Geleneksel tedaviler arasında steroitler ve metotreksat (MTX), azatioprin (AZA), siklosporin A (CSA), mikofenolat mofetil (MFM) gibi immunomodülatuar (İMT) ajanlar bulunmaktadır. Son yıllarda; anti-TNF-α tedavisi gibi biyolojik ajanları kapsayan yeni tedavi seçenekleri yaygınlaşmış olup tedavide etkin şekilde kullanılmaktadır. Cerrahi tedavi gerektiğinde ise dikkat edilmesi gereken en önemli nokta; cerrahi başarının oküler enflamasyonun medikal tedavi ile tamamen baskılanmasına bağlı olduğudur. Bu nedenle cerrahi öncesi tam enflamasyon kontrolü sağlandığından emin olunmalıdır.

Keywords

pars planitis, pediatric uveitis, biological agent

References

1. Jabs, D.A., R.B. Nussenblatt, and J.T. Rosenbaum, Standardization of uveitis nomenclature for reporting clinical data. Results of the First International Workshop. Am J Ophthalmol, 2005. 140(3): p. 509-16.
2. Smith, J.A., et al., Epidemiology and course of disease in childhood uveitis. Ophthalmology, 2009. 116(8): p. 1544-51, 1551.e1.
3. Nikkhah, H., et al., Childhood pars planitis; clinical features and outcomes. J Ophthalmic Vis Res, 2011. 6(4): p. 249-54
4. Soylu, M., G. Ozdemir, and A. Anli, Pediatric uveitis in southern Turkey. Ocul Immunol Inflamm, 1997. 5(3): p. 197-202.
5. Ozdal, P.C., et al., Patterns of childhood-onset uveitis in a referral center in Turkey. J Ophthalmic Inflamm Infect, 2012. 2(1): p. 13-9.
6. Arellanes-García, L., L. Navarro-López, and C. Recillas-Gispert, Pars planitis in the Mexican Mestizo population: ocular findings, treatment, and visual outcome. Ocul Immunol Inflamm, 2003. 11(1): p. 53-60.
7. Paroli, M.P., et al., Intermediate uveitis: comparison between childhood-onset and adult- onset disease. Eur J Ophthalmol, 2014. 24(1): p. 94-100.
8. Heinz, C., S. Schoonbrood, and A. Heiligenhaus, Intermediate uveitis in children and young adults: differences in clinical course, associations and visual outcome. Br J Ophthalmol, 2014. 98(8): p. 1107-11.
9. Paroli, M.P., et al., Intermediate uveitis in a pediatric Italian population. Ocul Immunol Inflamm, 2011. 19(5): p. 321-6.
10. Malinowski, S.M., et al., The association of HLA-B8, B51, DR2, and multiple sclerosis in pars planitis. Ophthalmology, 1993. 100(8): p. 1199-205.
11. Arellanes-García, L., et al., Idiopathic intermediate uveitis in childhood. Int Ophthalmol Clin, 2008. 48(3): p. 61-74.
12. Donaldson, M.J., et al., Pars planitis: a 20-year study of incidence, clinical features, and outcomes. Am J Ophthalmol, 2007. 144(6): p. 812-817.
13. Navarrete, A., A. Koriat, and R. Amer, Implications of pars planitis-associated cystoid macular edema on visual outcome and management in children. Graefes Arch Clin Exp Ophthalmol, 2020. 258(8): p. 1803-1811.
14. Tugal-Tutkun, I., Pediatric uveitis. J Ophthalmic Vis Res, 2011. 6(4): p. 259-69.
15. Khodadoust, A.A., et al., Pars planitis and autoimmune endotheliopathy. Am J Ophthalmol, 1986. 102(5): p. 633-9.
16. de Boer, J., et al., Long-term follow-up of intermediate uveitis in children. Am J Ophthalmol, 2006. 141(4): p. 616-21.
17. Prieto, J.F., et al., Pars planitis: epidemiology, treatment, and association with multiple sclerosis. Ocul Immunol Inflamm, 2001. 9(2): p. 93-102.
18. Yalçındağ, F.N., E. Temel, and E.G. Özgür, Spectral domain optical coherence tomography findings of patients with pars planitis and risk factors affecting visual acuity. Int Ophthalmol, 2021.
19. Finamor, L.P., C. Muccioli, and R. Belfort, Jr., Imaging techniques in the diagnosis and management of uveitis. Int Ophthalmol Clin, 2005. 45(2): p. 31-40.
20. Tran, V.T., P. LeHoang, and C.P. Herbort, Value of high-frequency ultrasound biomicroscopy in uveitis. Eye (Lond), 2001. 15(Pt 1): p. 23-30.
21. Kadayifçilar, S., B. Eldem, and B. Tumer, Uveitis in childhood. J Pediatr OphthalmolStrabismus, 2003. 40(6): p. 335-40.
22. Kalinina Ayuso, V., et al., Young age as a risk factor for complicated course and visual outcome in intermediate uveitis in children. Br J Ophthalmol, 2011. 95(5): p. 646-51.
23. Guest, S., E. Funkhouser, and S. Lightman, Pars planitis: a comparison of childhood onset and adult onset disease. Clin Exp Ophthalmol, 2001. 29(2): p. 81-4.
24. Lehpamer, B., et al., Epiretinal membranes in uveitic macular edema: effect on vision and response to therapy. Am J Ophthalmol, 2014. 157(5): p. 1048-55.
25. Albavera-Giles, T., et al., Outcomes of cataract surgery with/without vitrectomy in patients with pars planitis and immunosuppressive therapy. Graefes Arch Clin Exp Ophthalmol, 2017. 255(6): p. 1213-1219.
26. Jalil, A., F.E. Dhawahir-Scala, and N.P. Jones, Nonprogressive tractional inferior retinal elevation in intermediate uveitis. Ocul Immunol Inflamm, 2010. 18(1): p. 60-3.
27. Pollack, A.L., et al., Peripheral retinoschisis and exudative retinal detachment in pars planitis. Retina, 2002. 22(6): p. 719-24.
28. Zierhut, M., et al., Therapy of uveitis in children. Int Ophthalmol Clin, 2008. 48(3): p. 131-52.
29. Quinones, K., et al., Pars plana vitrectomy versus immunomodulatory therapy for intermediate uveitis: a prospective, randomized pilot study. Ocul Immunol Inflamm, 2010. 18(5): p. 411-7.
30. Maccora, I., E.S. Sen, and A.V. Ramanan, Update on noninfectious uveitis in children and its treatment. Curr Opin Rheumatol, 2020. 32(5): p. 395-402.
31. Ozdal, P.C., N. Berker, and I. Tugal-Tutkun, Pars Planitis: Epidemiology, Clinical Characteristics, Management and Visual Prognosis. J Ophthalmic Vis Res, 2015. 10(4): p. 469 80.
32. Silman, A.J., et al., Lymphoproliferative cancer and other malignancy in patients with rheumatoid arthritis treated with azathioprine: a 20 year follow up study. Ann Rheum Dis, 1988. 47(12): p. 988-92.
33. Budde, K., et al., Pharmacodynamic monitoring of mycophenolate mofetil. Clin Chem Lab Med, 2000. 38(11): p. 1213-6.
34. Vitale, A.T., A. Rodriguez, and C.S. Foster, Low-dose cyclosporin A therapy in treating chronic, noninfectious uveitis. Ophthalmology, 1996. 103(3): p. 365-73; discussion 373-4.
35. Pasadhika, S. and J.T. Rosenbaum, Update on the use of systemic biologic agents in the treatment of noninfectious uveitis. Biologics, 2014. 8: p. 67-81.
36. Rifkin, L.M., A.D. Birnbaum, and D.A. Goldstein, TNF inhibition for ophthalmic indications: current status and outlook. BioDrugs, 2013. 27(4): p. 347-57.
37. Cordero-Coma, M. and L. Sobrin, Anti-tumor necrosis factor-α therapy in uveitis. Surv Ophthalmol, 2015. 60(6): p. 575-89.
38. Hu, S., et al., Comparison of the inhibition mechanisms of adalimumab and infliximab in treating tumor necrosis factor α-associated diseases from a molecular view. J Biol Chem,2013. 288(38): p. 27059-27067.
39. Nguyen, Q.D., 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. 388(10050): p. 1183-92.
40. Díaz-Llopis, M., et al., Treatment of refractory uveitis with adalimumab: a prospective multicenter study of 131 patients. Ophthalmology, 2012. 119(8): p. 1575-81.
41. Fabiani, C., et al., Comparative efficacy between adalimumab and infliximab in the treatment of non-infectious intermediate uveitis, posterior uveitis, and panuveitis: a retrospective observational study of 107 patients. Clin Rheumatol, 2019. 38(2): p. 407-415.
42. Vazquez-Cobian, L.B., T. Flynn, and T.J. Lehman, Adalimumab therapy for childhood uveitis. J Pediatr, 2006. 149(4): p. 572-5.
43. Simonini, G., et al., Superior efficacy of Adalimumab in treating childhood refractory chronic uveitis when used as first biologic modifier drug: Adalimumab as starting anti-TNF-α therapy in childhood chronic uveitis. Pediatr Rheumatol Online J, 2013. 11: p. 16.
44. Simonini, G., et al., Prevention of flare recurrences in childhood-refractory chronic uveitis: an open-label comparative study of adalimumab versus infliximab. Arthritis Care Res (Hoboken),2011. 63(4): p. 612-8.
45. Oray, M. and İ. Tuğal-Tutkun, Treatment of Juvenile Idiopathic Arthritis-Associated Uveitis. Turk J Ophthalmol, 2016. 46(2): p. 77-82.
46. Najjar, D.M., et al., EDTA chelation for calcific band keratopathy: results and long-term follow-up. Am J Ophthalmol, 2004. 137(6): p. 1056-64.
47. Holland, G.N., Intraocular lens implantation in patients with juvenile rheumatoid arthritis- associated uveitis: an unresolved management issue. Am J Ophthalmol, 1996. 122(2): p. 255-7.
48. Sijssens, K.M., et al., Long-term ocular complications in aphakic versus pseudophakic eyes of children with juvenile idiopathic arthritis-associated uveitis. Br J Ophthalmol, 2010. 94(9): p. 1145-9.
49. Ladas, J.G., et al., Relationship between aqueous humor protein level and outflow facility in patients with uveitis. Invest Ophthalmol Vis Sci, 2001. 42(11): p. 2584-8.

There are 49 citations in total.

Details

Primary Language	English
Subjects	Health Services and Systems (Other)
Journal Section	Reviews
Authors	Merve Bahar 0000-0002-3800-7617
Publication Date	December 31, 2023
Submission Date	November 6, 2023
Acceptance Date	December 26, 2023
Published in Issue	Year 2023Volume: 45 Issue: 4

Cite

AMA	Bahar M. Pars Planitis Epidemiology, Diagnosis, Follow-Up And Prognosis. CMJ. December 2023;45(4):8-16. doi:10.7197/cmj.1386749

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