Orbital Vasculitides–Differential Diagnosis

  • Gabriela M. EspinozaEmail author
  • Jessica L. Liu
Vasculitis (L Espinoza, Section Editor)
Part of the following topical collections:
  1. Topical Collection on Vasculitis


Purpose of Review

The orbit is subject to a variety of vascular insults that manifest with both specific and nonspecific patterns of vision compromise. The aim of the following review is to highlight the ophthalmic clinical features of systemic vasculitides that most frequently involve the orbit and differentiate them from the most common non-vasculitic orbital disorders.

Recent Findings

New studies continue to explore the autoimmune nature of vasculitic disease and seek to determine optimal use of newer therapies such as biologic agents.


The pattern of ocular involvement in the context of clinical history allows the knowledgeable physician to distill a differential diagnosis into a specific or likely cause. Establishing a diagnosis in a timely fashion allows for a custom-tailored approach to therapy.


Vasculitis Behcet’s disease IgG4 disease Cogan’s disease Granulomatosis with polyangiitis Takayasu arteritis 



The authors would like to thank Dr. Gabriel Andrade from the Federal University of Sao Paulo–UNIFESP for his photographic contribution to this paper. We also thank Dr. Eduardo B. Rodrigues for his assistance.

Compliance with Ethical Standards

Conflict of Interest

The authors declare that they have no conflict of interest.

Human and Animal Rights and Informed Consent

This article does not contain any studies with human or animal subjects performed by any of the authors.


Papers of particular interest, published recently, have been highlighted as: • Of importance •• Of major importance

  1. 1.
    Azad SV, Takkar B, Venkatesh P. Eye and Vasculitis. J Vasc. 2016;2:108. Scholar
  2. 2.
    Zlatanović G, Veselinović D, Cekić S, Zivković M, Dorđević-Jocić J, Zlatanović M. Ocular manifestation of rheumatoid arthritis-different forms and frequency. Bosn J Basic Med Sci. 2010;10:323–7. Scholar
  3. 3.
    Shoughy SS, Tabbara KF. Ocular findings in systemic lupus erythematosus. Saudi J Ophthalmol. 2016;30:117–21. Scholar
  4. 4.
    Groen F, Rothova A. Ocular involvement in sarcoidosis. Semin Respir Crit Care Med. 2017;38:514–22. Scholar
  5. 5.
    Gauthier AS, Noureddine S, Delbosc B. Interstitial keratitis diagnosis and treatment. J Fr Ophthalmol. 2019;42:e229–37. Scholar
  6. 6.
    Jennette JC, Falk RJ, Bacon PA, Basu N, Cid MC, Ferrario L, et al. 2012 revised international Chapel Hill consensus conference nomenclature of Vasculitides. Arthritis Rheum. 2013;65:1–11. Scholar
  7. 7.
    Rothschild PR, Pagnoux C, Seror R, Brézin AP, Delair E, Guillevin L. Ophthalmologic Manifestations of Systemic Necrotizing Vasculitides at Diagnosis: A Retrospective Study of 1286 Patients and Review of the Literature. Semin Arthritis Rheum. 2013;42:507–14. Scholar
  8. 8.
    Grayson PC, Cuthbertson D, Carette S, Hoffman GS, Khalidi NA, Koening CL, et al. New Features of Disease After Diagnosis in 6 Forms of Systemic Vasculitis. J Rheumatol. 2013;40:1905–12. Scholar
  9. 9.
    • Mombaerts I, Bilyk JR, Rose GE, McNab AA, Fay A, Dolman PJ, et al. Consensus on diagnostic criteria of idiopathic orbital inflammation using a modified Delphi approach. JAMA Ophthalmol. 2017;135:769–76. This study provides a structure to evaluate idiopathic orbital inflammation and help limit the differential diagnosis in this diagnosis of exclusion. CrossRefPubMedPubMedCentralGoogle Scholar
  10. 10.
    •• Watts RA, Robson J. Introduction, epidemiology and classification of vasculitis. Best Prct Res Clin Rheumatol. 2018;32:3–20. This is a good review of currently accepted definitions of vasculitis and epidemiology. CrossRefGoogle Scholar
  11. 11.
    Cotch MF, Hoffman GS, Yerg DE, Kaufman GI, Targonski P, Kaslow RA. The epidemiology of Wegener's granulomatosis: estimates of the five-year period prevalence, annual mortality, and geographic disease distribution from population-based data sources. Arthritis Rheum. 1996;39:87–92.CrossRefPubMedGoogle Scholar
  12. 12.
    Panupattanapong S, Stwalley DL, White AJ, Olsen MA, French AR, Hartman ME. Epidemiology and Outcomes of Granulomatosis With Polyangiitis in Pediatric and Working-Age Adult Populations In the United States: Analysis of a Large National Claims Database. Arthritis Rheum. 2018;70:2067–76. Scholar
  13. 13.
    Sfiniadaki E, Tsiara I, Theodossiadis P, Chatziralli I. Ocular manifestations of granulomatosis with Polyangiitis: a review of the literature. Ophthalmol Therapy. 2019;8:227–34. Scholar
  14. 14.
    Csernok E, Gross WL. Current understanding of the pathogenesis of granulomatosis with polyangiitis (Wegener’s). Expert Rev Clin Immunol. 2013;9:641–8. Scholar
  15. 15.
    Tarabishy AB, Schulte M, Papaliodis GN, Hoffman GS. Wegener’s granulomatosis: clinical manifestations, differential diagnosis, and management of ocular and systemic disease. Surv Ophthalmol. 2010;55:429–44. Scholar
  16. 16.
    • Pagnoux C. Updates in ANCA-associated vasculitis. Eur J Rheumatol. 2016;3:122–33. A thorough review with excellent figures to characterize findings. CrossRefPubMedPubMedCentralGoogle Scholar
  17. 17.
    Tan LT, Davagnanam I, Isa H, Taylor SR, Rose GE, Verity DH, et al. Clinical and imaging features predictive of orbital granulomatosis with polyangiitis and the risk of systemic involvement. Ophthalmology. 2014;121:1304–9. Scholar
  18. 18.
    Tan LT, Davagnanam I, Isa H, Rose GE, Verity DH, Pusey CD, et al. Clinical and imaging features of lacrimal gland involvement in granulomatosis with polyangiitis. Ophthalmology. 2015;122:2125–9. Scholar
  19. 19.
    Isse N, Nagamatsu Y, Yoshimatsu N, Obata T, Takahara N. Granulomatosis with polyangiitis presenting as an orbital inflammatory pseudotumor: a case report. J Med Case Rep. 2013;7:110. Scholar
  20. 20.
    Hibino M, Kondo T. Dacryoadenitis with ptosis and diplopia as the initial presentation of granulomatosis with polyangiitis. Intern Med. 2017;56:2649–53. Scholar
  21. 21.
    Muller K, Lin JH. Orbital granulomatosis with polyangiitis (Wegener granulomatosis): clinical and pathologic findings. Arch Pathol Lab Med. 2014;138:1110–4. Scholar
  22. 22.
    • Tugal-Tutkun I. Systemic vasculitis and the eye. Curr Opin Rheumatol. 2017;29:24–32. This review is a good overview of current studies into gene expression profiling of orbital tissues in vasculitis. CrossRefPubMedGoogle Scholar
  23. 23.
    Isa H, Luthert P, Rose G, Verity D, Pusey C, Tomkins-Netzer O, et al. Tissue interleukin-17 and interleukin-23 as biomarkers for orbital granulomatosis with polyangiitis. Ophthalmology. 2015;122:2140–2. Scholar
  24. 24.
    Rosenbaum JT, Choi D, Wilson DJ, Grossniklaus HE, Sibley CH, Harrington CA, et al. Orbital Disease Consortium Molecular diagnosis of orbital inflammatory disease. Exp Mol Pathol. 2015;98:225–9. Scholar
  25. 25.
    Rosenbaum JT, Choi D, Wilson DJ, Grossniklaus HE, Harrington CA, Sibley CH, et al. Orbital pseudotumor can be a localized form of granulomatosis with polyangiitis as revealed by gene expression profiling. Exp Mol Pathol. 2015;99:271–8. Scholar
  26. 26.
    Pakrou N, Selva D, Leibovitch I. Wegener’s granulomatosis: ophthalmic manifestations and management. Semin Arthritis Rheum. 2006;35:284–92. Scholar
  27. 27.
    Perumal B, Black EH, Levin F, Servat JJ. Non-infectious orbital vasculitides. Eye (Lond). 2012;26:630–9. Scholar
  28. 28.
    Stone JH, Merkel PA, Spiera R, Seo P, Langford CA, Hoffman GS, et al. Rituximab versus cyclophosphamide for ANCA-associated vasculitis. N Engl J Med. 2010;363:221–32. Scholar
  29. 29.
    Gopaluni S, Smith RM, Lewin M, McAlear CA, Mynard K, et al. Rituximab versus azathioprine as therapy for maintenance of remission for anti-neutrophil cytoplasm antibody-associated vasculitis (RITAZAREM): study protocol for a randomized controlled trial. Trials. 2017;18:112. Scholar
  30. 30.
    Wiwatwongwana D, Esdaile JM, White VA, Dolman PJ. Intravenous immunoglobulin (IVIG) for orbital Wegener’s granulomatosis. Can J Ophthalmol. 2012;47:82–3. Scholar
  31. 31.
    Blum M, Andrassy K, Adler D, Hartmann M, Volcker HE. Early experience with intravenous immunoglobulin treatment in Wegener’s granulomatosis with ocular involvement. Graefes Arch Clin Exp Ophthalmol. 1997;235:599–602.CrossRefPubMedGoogle Scholar
  32. 32.
    Carmona FD, Mackie SL, Martin JE, Taylor JC, Vaglio A, Eyre S, et al. A large-scale genetic analysis reveals a strong contribution of the HLA class II region to giant cell arteritis susceptibility. Am J Hum Genet. 2015;96(4):565–80. Scholar
  33. 33.
    Carmona FD, Gonzalez-Gay MA, Martin J. Genetic component of giant cell arteritis. Rheumatology (Oxford). 2014;53:6–18. Scholar
  34. 34.
    Duhaut P, Bosshard S, Ducroix JP. Is giant cell arteritis an infectious disease? Biological and epidemiological evidence. Presse Med. 2004;33:1403–8.CrossRefPubMedGoogle Scholar
  35. 35.
    Nagel MA, White T, Khmeleva N, Rempel A, Boyer PJ, Bennett JL, et al. Analysis of varicella-zoster virus in temporal arteries biopsy positive and negative for giant cell arteritis. JAMA Neurol. 2015;72:1281–7. Scholar
  36. 36.
    • De Smit E, O’Sullivan E, Mackey DA, Hewitt AW. Giant cell arteritis: ophthalmic manifestations of a systemic disease. Graefes Arch Clin Exp Ophthalmol. 2016; 254: 2291-2306. Thorough review of ophthalmic findings in giant cell arteritis.
  37. 37.
    Borchers AT, Gershwin ME. Giant cell arteritis: a review of classification, pathophysiology, geoepidemiology and treatment. Autoimmun Rev. 2012;11:A544–54. Scholar
  38. 38.
    Salvarani C, Cimino L, Macchioni P, Consonni D, Cantini F, Bajocchi G, et al. Risk factors for visual loss in an Italian population-based cohort of patients with giant cell arteritis. Arthritis Rheum. 2005;53:293–7. Scholar
  39. 39.
    Salvarani C, Pipitone N, Versari A, Hunder GG. Clinical features of polymyalgia rheumatica and giant cell arteritis. Nat Rev Rheumatol. 2012;8:509–21. Scholar
  40. 40.
    Carroll SC, Gaskin BJ, Danesh-Meyer HV. Giant cell arteritis. Clin Exp Ophthalmol. 2006;34:159–73. Scholar
  41. 41.
    Ninan JV, Lester S, Hill CL. Giant cell arteritis: beyond temporal artery biopsy and steroids. Intern Med J. 2017;47:1228–40. Scholar
  42. 42.
    Hayreh SS, Podhajsky PA, Zimmerman B. Occult giant cell arteritis: ocular manifestations. Am J Ophthalmol. 1998;125:521–6. Scholar
  43. 43.
    Mehler MF, Rabinowich L. The clinical neuro-ophthalmologic spectrum of temporal arteritis. Am J Med. 1988;85:839–44. Scholar
  44. 44.
    Hayreh SS. Posterior ischaemic optic neuropathy: clinical features, pathogenesis, and management. Eye (Lond). 2004;18:1188–206. Scholar
  45. 45.
    Hayreh SS, Podhajsky PA, Zimmerman B. Ocular manifestations of giant cell arteritis. Am J Ophthalmol. 1998;125:509–20. Scholar
  46. 46.
    Bayar SA, Gokmen O, Pinarci EY, Altinors DD, Gedik S. Corneal endothelial decompensation and ocular hypotony in a case with temporal arteritis. J Neuroophthalmol. 2012;32:385. Scholar
  47. 47.
    Pellegrini F, Prosdocimo G, Bonsanto D, Machin P, Foroozan R. Thick corneas, large pupils, and a giant problem. Surv Ophthalmol. 2018;63:595–9. Scholar
  48. 48.
    Radda TM, Bardach H, Riss B. Acute ocular hypotony. A rare complication of temporal arteritis. Ophthalmologica. 1981;182:148–15. Scholar
  49. 49.
    Seror R, Baron G, Hachulla E, Debandt M, Larroche C, Puechal X, et al. Adalimumab for steroid sparing in patients with giant-cell arteritis: results of a multicentre randomised controlled trial. Ann Rheum Dis. 2014;73:732074–81. Scholar
  50. 50.
    Hoffman GS, Cid MC, Hellmann DB, Guillevin L, Stone JH, Schousboe J, et al. A multicenter, randomized, double-blind, placebo-controlled trial of adjuvant methotrexate treatment for giant cell arteritis. Arthritis Rheum. 2002;46:1309–18. Scholar
  51. 51.
    • Villiger PM, Adler S, Kuchen S, Wemelinger F, Dan D, Fiege V, et al. Tocilizumab for induction and maintenance of remission in giant cell arteritis: a phase 2, randomised, double-blind, placebo-controlled trial. Lancet. 2016;387:1921–7. First trial showing tocilizumab inducing and maintaining remission in patients with giant cell arteritis. CrossRefPubMedGoogle Scholar
  52. 52.
    Stone JH, Tuckwell K, Dimonaco S, Klearman M, Aringer M, Blockmans D, et al. Trial of tocilizumab in giant-cell arteritis. N Engl J Med. 2017;377:317–28. Scholar
  53. 53.
    Ly KH, Stirnemann J, Liozon E, Michel M, Fain O, Fauchais AL. Interleukin-1 blockade in refractory giant cell arteritis. Joint Bone Spine. 2014;81:76–8. Scholar
  54. 54.
    Zhang H, Watanabe R, Berry GJ, Tian L, Goronzy JJ, Weyand CM. Inhibition of JAK-STAT signaling suppresses pathogenic immune responses in medium and large vessel vasculitis. Circulation. 2018;137:1934–48. Scholar
  55. 55.
    • Seyahi E. Takayasu arteritis: an update. Curr Opin Rheumatol. 2017;29:51–6. Concise review of Takayasu arteritis. CrossRefPubMedGoogle Scholar
  56. 56.
    Matos KT, Arantes T, Souza AW, Ramos MH, Allemann N, Muccioli C. Retinal angiography and colour Doppler of retrobulbar vessels in Takayasu arteritis. Can J Ophthalmol. 2014;49:80–6. Scholar
  57. 57.
    Peter J, David S, Danda D, Peter JV, Horo S, Joseph G. Ocular manifestations of Takayasu arteritis: a cross-sectional study. Retina. 2011;31:1170–8. Scholar
  58. 58.
    Shukla E, Desai A, Malkan N, Gokhale Y. Nodular scleritis as a presenting feature of Takayasu’s arteritis. Indian J Ophthalmol. 2016;64:312–4. Scholar
  59. 59.
    Young SM, Chan AS, Jajeh IA, Shen S, Seah LL, Choo CT, et al. Clinical features and treatment outcomes of orbital inflammatory disease in Singapore: a 10-year clinicopathologic review. Ophthal Plast Reconstr Surg. 2017;33:182–8. Scholar
  60. 60.
    • Misra DP, Wakhlu A, Agarwal V, Danda D. Recent advances in the management of Takayasu arteritis. Int J Rheum Dis. 2019;22:60–8. Good review with an excellent table summarizing current evidence based therapies for Takayasu arteritis. CrossRefPubMedGoogle Scholar
  61. 61.
    Tayer-Shifman OE, Ilan O, Tovi H, Tal Y. Cogan’s syndrome—clinical guidelines and novel therapeutic approaches. Clin Rev Allergy Immunol. 2014;47:65–72. Scholar
  62. 62.
    Vollertsen RS, McDonald RJ, Younge BR, Banks PM, Stanson AW, Ilstrup DM. Cogan's syndrome: 18 cases and a review of the literature. Mayo Clin Proc. 1986;61:344–61. Scholar
  63. 63.
    Cogan DG. Syndrome of non-syphilitic interstitial keratitis and vestibuloauditory symptoms. Arch Ophthalmol. 1945;33:144–9.CrossRefGoogle Scholar
  64. 64.
    Espinoza GM, Prost A. Cogan’s syndrome and other ocular vasculitides. Curr Rheumatol Rep. 2015;17:24. Scholar
  65. 65.
    Pollard ZF, Greenberg M, Bashinsky A, Bordenca M. Uveitis associated with atypical Cogan syndrome in children. Arch Ophthalmol. 2007;125:1574–5. Scholar
  66. 66.
    Grasland A, Pouchot J, Hachulla E, Blétry O, Papo T, Vinceneux P, et al. Typical and atypical Cogan's syndrome: 32 cases and review of the literature. Rheumatology (Oxford). 2004;43:1007–15. Scholar
  67. 67.
    Colodetti R, Spina G, Leal T, Oliveira M Jr, Soeiro A. Cogan’s syndrome – a rare aortitis, difficult to diagnose but with therapeutic potential. Rev Assoc Med Bras. 2017;63:1028–31. Scholar
  68. 68.
    Stone JR, Bruneval P, Angelini A, Bartoloni G, Basso C, Batoroeva L, et al. Consensus statement on surgical pathology of the aorta from the Society for Cardiovascular Pathology and the Association for European Cardiovascular Pathology: I. Inflammatory diseases Cardiovasc Pathol. 2015;24:267–78. Scholar
  69. 69.
    Ferrari E, Taillan B, Garnier G, Dor V, Morand P, Dujardin P. Manifestations cardiovasculaires du syndrome de Cogan. A propos d'un cas. Arch Mal Coeur Vaiss. 1992;85:913–6.PubMedGoogle Scholar
  70. 70.
    Antonios N, Silliman S. Cogan syndrome: an analysis of reported neurological manifestations. Neurologist. 2012;18:55–63. Scholar
  71. 71.
    Ho AC, Roat MI, Venbrux A, Hellmann DB. Cogan's syndrome with refractory aortitis and mesenteric vasculitis. J Rheumatol. 1999;26:1404–7.PubMedGoogle Scholar
  72. 72.
    Bonaguri C, Orsoni J, Russo A, Rubino P, Bacciu S, Lippi G, et al. Cogan’s syndrome: anti-Hsp70 antibodies are a serological marker in the typical form. Isr Med Assoc J. 2014;16:285–8.PubMedGoogle Scholar
  73. 73.
    Lydon EJ, Barisoni L, Belmont HM. Cogan’s syndrome and development of ANCA-associated renal vasculitis after lengthy disease remission. Clin Exp Rheumatol. 2009;27:S144.PubMedGoogle Scholar
  74. 74.
    Suzuki M, Arimura Y, Minoshima S, Fukuoka K, Miyake N, Ishizuka S, et al. A case of myeloperoxidase-specific antineutrophil cytoplasmic antibody (MPO-ANCA)-related glomerulonephritis associated with Cogan’s syndrome. Nihon Jinzo Gakkai Shi. 1996;38:423–7.PubMedGoogle Scholar
  75. 75.
    Gluth MB, Baratz KH, Matteson EL, Driscoll CL. Cogan syndrome: a retrospective review of 60 patients throughout a half century. Mayo Clin Proc. 2006;81(4):483–8. Scholar
  76. 76.
    Durtette C, Hachulla E, Resche-Rigon M, Papo T, Zénone T, Lioger B, et al. Cogan syndrome: characteristics, outcome and treatment in a French nationwide retrospective study and literature review. Autoimmun Rev. 2017;16:1219–23. Scholar
  77. 77.
    Padoan R, Cazzador D, Pendolino AL, Felicetti M, De Pascalis S, Zanoletti E, et al. Cogan’s syndrome: new therapeutic approaches in the biologic era. Expert Opin Biol Ther. 2019;6:1–8. Scholar
  78. 78.
    Kokturk A. Clinical and pathological manifestations with differential diagnosis in Behçet’s disease. Pathol Res Int. 2012;2012:1–9. Scholar
  79. 79.
    Alami A, Kriet M, Reda K, Laktaui A, Qubaaz A. Ocular Behçet. Pan Afr Med J. 2017;26:237. Scholar
  80. 80.
    Tanaka T, Yamakawa N, Koike N, Suzuki J, Mizuno F, Usui M. Behçet's disease and antibody titers to various heat-shock protein 60s. Ocul Immunol Inflamm. 1999;7:69–74.CrossRefPubMedGoogle Scholar
  81. 81.
    Chambers JC, Haskard DO, Kooner JS. Vascular endothelial function and oxidative stress mechanisms in patients with Behçet's syndrome. J Am Coll Cardiol. 2001;37:517–20. Scholar
  82. 82.
    Horie Y, Meguro A, Ohta T, Lee EB, Namba K, Mizuuchi K, et al. HLA-B51 carriers are susceptible to ocular symptoms of Behçet disease and the association between two becomes stronger towards the east along the silk road: a literature survey. Ocul Immunol Inflamm. 2017;25:37–40. Scholar
  83. 83.
    Davatchi F, Chams-Davatchi C, Shams H, Nadjii A, Faezi T, Akhlaghi M, et al. Adult Behcet’s disease in Iran: analysis of 6075 patients. Int J Rheum Dis. 2016;19:95–103. Scholar
  84. 84.
    Arevalo JF, Lasave AF, Al Jindan MY, Al Sabaani NA, Al-Mahmood AM, Al-Zahrani YA, et al. Uveitis in Behcet disease in a tertiary center over 25 years: the KKESH Uveitis Survey Study Group. Am J Ophthalmol. 2015;159:177–84.e1–2. Scholar
  85. 85.
    • Tanaka R, Murata H, Takamoto M, Ohtomo K, Okinaga K, Yoshida A, et al. Behcet’s disease ocular attack score 24 and visual outcome in patients with Behcet’s disease. Br J Ophthalmol. 2016;100:990–4. Provides data for a prognostic scoring system to predict deterioration of vision in patients with ocular Behcet’s disease. CrossRefPubMedGoogle Scholar
  86. 86.
    Garcher C, Bielefeld P, Desvaux C, Besancenot JF, Bron A. Bilateral loss of vision and macular ischemia related to Behçet disease. Am J Ophthalmol. 1997;124:116–7. Scholar
  87. 87.
    Nussenblatt RB. Macular alterations secondary to intraocular inflammatory disease. Ophthalmology. 1986;93:984–8.CrossRefPubMedGoogle Scholar
  88. 88.
    Alpsoy E. New evidence-based treatment approach in Behçet's disease. Pathol Res Int. 2012;2012:1–11. Scholar
  89. 89.
    Tugal-Tutkun I, Cingü K, Kir N, Yeniad B, Urgancioglu M, Gül A. Use of laser flare-cell photometry to quantify intraocular inflammation in patients with Behcet uveitis. Graefes Arch Clin Exp Ophthalmol. 2008;246:1169–77. Scholar
  90. 90.
    Alpsoy E. Behçet’s disease: A comprehensive review with a focus on epidemiology, etiology and clinical features, and management of mucocutaneous lesions. J Dermatol. 2016;43:620–32. Scholar
  91. 91.
    Yazici H, Pazarli H, Barnes CG, Tüzün Y, Ozyazgan Y, Silman A, et al. A controlled trial of azathioprine in Behçet's syndrome. N Engl J Med. 322:281–5. Scholar
  92. 92.
    Masuda K, Nakajima A, Urayama A, Nakae K, Kogure M, Inaba G. Double-masked trial of cyclosporin versus colchicine and long-term open study of cyclosporin in Behçet's disease. Lancet. 1989;1:1093–6. Scholar
  93. 93.
    BenEzra D, Cohen E, Chajek T, Friedman G, Pizanti S, de Courten C, et al. Evaluation of conventional therapy versus cyclosporine a in Behçet's syndrome. Transplant Proc. 1988;20:136–43.Google Scholar
  94. 94.
    Urruticoechea-Arana A, Cobo-Ibáñez T, Villaverde-García V, Santos Gomez M, Loza E, Vargas-Osorio K, et al. Efficacy and safety of biological therapy compared to synthetic immunomodulatory drugs or placebo in the treatment of Behçet’s disease associated uveitis: a systematic review. Rheumatol Int. 2019;39:47–58. Scholar
  95. 95.
    Davatchi F, Shams H, Rezaipoor M, et al. Rituximab in intractable ocular lesions of Behçet's disease; randomized single-blind control study (pilot study). Int J Rheum Dis. 2010;13:246–52. Scholar
  96. 96.
    Davatchi F, Shahram F, Chams H, Jamshidi AR, Nadji A, Chams C, et al. High dose methotrexate for ocular lesions of Behçet's disease. Preliminary short-term results. Adv Exp Med Biol. 2003;528:579–84.CrossRefPubMedGoogle Scholar
  97. 97.
    Neri P, Mariotti C, Cimino L, Mercanti L, Giovannini A. Long-term control of cystoid macular oedema in noninfectious uveitis with mycophenolate Mofetil. Int Ophthalmol. 2009;29:127–33. Scholar
  98. 98.
    Bettiol A, Silvestri E, Di Scala G, Amedei A, Becatti M, Fiorillo C, et al. The right place of interleukin-1 inhibitors in the treatment of Behçet’s syndrome: a systematic review. Rheumatol Int. 2019;39:971–90. Scholar
  99. 99.
    Carruthers MN, Stone JH, Khosroshahi A. The latest on IgG4-RD: a rapidly emerging disease. Curr Opin Rheumatol. 2012;24:60–9. Scholar
  100. 100.
    Stone JH, Khosroshahi A, Deshpande V, Chan JK, Heathcote JG, Aalberse R, et al. IgG4-related disease: recommendations for the nomenclature of this condition and its individual organ system manifestations. Arthritis Rheum. 2012;64:3061–7. Scholar
  101. 101.
    Ebbo M, Patient M, Grados A, Groh M, Desblaches J, Hachulla E, et al. Ophthalmic manifestations in IgG4-related disease Clinical presentation and response to treatment in a French case-series. Medicine (Baltimore). 2017;96:e6205. Scholar
  102. 102.
    Perez Alamino R, Martinez C, Espinoza LR. IgG4-associated Vasculitis. Curr Rheumatol Rep. 2013;15:348. Scholar
  103. 103.
    Wallave ZS, Deshpande V, Stone JH. Ophthalmic manifestations of IgG4-related disease: single-center experience and literature review. Semin Arthritis Rheum. 2014;43:806–17. Scholar
  104. 104.
    Johnston J, Allen JE. IgG4-related disease in the head and neck. Curr Opin Otoloaryngol Head Neck Surg. 2018;26:403–8. Scholar
  105. 105.
    Andrew N, Kearney D, Selva D. IgG4-related orbital disease: a meta-analysis and review. Acta Ophthalmol. 2013;91:694–700. Scholar
  106. 106.
    Kubota T, Moritani S. Orbital IgG4-related disease: clinical features and diagnosis. ISRN Rheumatol. 2012;2012:412896–5. Scholar
  107. 107.
    Wu A, Andrew NH, McNab AA, Selva D. IgG4-related ophthalmic disease: pooling of published cases and literature review. Curr Allergy Asthma Rep. 2015;15:27. Scholar
  108. 108.
    Andrew NH, Sladden N, Kearney DJ, Selva D. An analysis of IgG4-related disease (IgG4-RD) among idiopathic orbital inflammations and benign lymphoid hyperplasias using two consensus-based diagnostic criteria for IgG4-RD. Br J Ophthalmol. 2015;99:376–81. Scholar
  109. 109.
    •• Detiger SE, Karim AF, Verdijk RM, Van Hagen PM, Van Laar JAM, Paridaens D. The treatment outcomes in IgG4-related orbital diseaese: a systematic review of the literature. Acta Ophthalmol. 2019;97:451–9. A good review of the literature and recommendation to initiate rituximab early in the case of refractory and organ- or life-threatening disease. CrossRefPubMedGoogle Scholar

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Authors and Affiliations

  1. 1.Department of OphthalmologySaint Louis University School of MedicineSaint LouisUSA

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