Idiopathic retinal vasculitis, aneurysms, and neuro-retinitis

T. S. Chang, G. W. Aylward, Janet L Davis, W. F. Mieler, G. L. Oliver, A. L. Maberley, J. D M Gass, D. Callanan, J. S. Duker, J. H. Drouilhet, D. E. Eifrig, R. B. Feldman, R. E. Kalina, J. H. Killian, R. B. Nussenbatt, C. A. Puliafito, T. A. Rice, H. Schatz, S. M. Whitcup

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Abstract

Purpose: The authors describe the clinical feature of ten patients with a new syndrome characterized by the presence of retinal vasculitis, multiple macroaneurysms, neuro-retinitis, and peripheral capillary nonperfusion. Methods: The authors evaluated ten patients identified to have clinical features compatible with the syndrome of idiopathic retinal vasculitis, aneurysms and neuroretinitis (IRVAN). Clinical examination findings, sequential fundus photographs (when available), fluorescein angiograms, systemic investigations, response to therapy, and visual outcomes were reviewed. Results: Seven eyes of four patients sustained a marked decrease in visual acuity of 20/200 or worse. Visual loss was due to a combination of an exudative maculopathy and sequelae of retinal ischemia. Capillary nonperfusion was seen in all ten patients and was severe enough to warrant panretinal laser photocoagulation in six patients. Systemic investigations were uniformly noncontributory. Oral prednisone appears to have little beneficial effects on patients with this disorder. Conclusions: Patients with IRVAN have characteristic retinal features that readily identify this syndrome. An increased awareness of this rare syndrome may help to identify sight-threatening complications at an earlier stage. The authors caution against extensive medical investigations.

Original languageEnglish
Pages (from-to)1089-1097
Number of pages9
JournalOphthalmology
Volume102
Issue number7
StatePublished - Jan 1 1995

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Retinal Vasculitis
Retinitis
Aneurysm
Light Coagulation
Prednisone
Fluorescein
Visual Acuity
Angiography
Lasers
Ischemia

ASJC Scopus subject areas

  • Ophthalmology

Cite this

Chang, T. S., Aylward, G. W., Davis, J. L., Mieler, W. F., Oliver, G. L., Maberley, A. L., ... Whitcup, S. M. (1995). Idiopathic retinal vasculitis, aneurysms, and neuro-retinitis. Ophthalmology, 102(7), 1089-1097.

Idiopathic retinal vasculitis, aneurysms, and neuro-retinitis. / Chang, T. S.; Aylward, G. W.; Davis, Janet L; Mieler, W. F.; Oliver, G. L.; Maberley, A. L.; Gass, J. D M; Callanan, D.; Duker, J. S.; Drouilhet, J. H.; Eifrig, D. E.; Feldman, R. B.; Kalina, R. E.; Killian, J. H.; Nussenbatt, R. B.; Puliafito, C. A.; Rice, T. A.; Schatz, H.; Whitcup, S. M.

In: Ophthalmology, Vol. 102, No. 7, 01.01.1995, p. 1089-1097.

Research output: Contribution to journalArticle

Chang, TS, Aylward, GW, Davis, JL, Mieler, WF, Oliver, GL, Maberley, AL, Gass, JDM, Callanan, D, Duker, JS, Drouilhet, JH, Eifrig, DE, Feldman, RB, Kalina, RE, Killian, JH, Nussenbatt, RB, Puliafito, CA, Rice, TA, Schatz, H & Whitcup, SM 1995, 'Idiopathic retinal vasculitis, aneurysms, and neuro-retinitis', Ophthalmology, vol. 102, no. 7, pp. 1089-1097.
Chang TS, Aylward GW, Davis JL, Mieler WF, Oliver GL, Maberley AL et al. Idiopathic retinal vasculitis, aneurysms, and neuro-retinitis. Ophthalmology. 1995 Jan 1;102(7):1089-1097.
Chang, T. S. ; Aylward, G. W. ; Davis, Janet L ; Mieler, W. F. ; Oliver, G. L. ; Maberley, A. L. ; Gass, J. D M ; Callanan, D. ; Duker, J. S. ; Drouilhet, J. H. ; Eifrig, D. E. ; Feldman, R. B. ; Kalina, R. E. ; Killian, J. H. ; Nussenbatt, R. B. ; Puliafito, C. A. ; Rice, T. A. ; Schatz, H. ; Whitcup, S. M. / Idiopathic retinal vasculitis, aneurysms, and neuro-retinitis. In: Ophthalmology. 1995 ; Vol. 102, No. 7. pp. 1089-1097.
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AU - Aylward, G. W.

AU - Davis, Janet L

AU - Mieler, W. F.

AU - Oliver, G. L.

AU - Maberley, A. L.

AU - Gass, J. D M

AU - Callanan, D.

AU - Duker, J. S.

AU - Drouilhet, J. H.

AU - Eifrig, D. E.

AU - Feldman, R. B.

AU - Kalina, R. E.

AU - Killian, J. H.

AU - Nussenbatt, R. B.

AU - Puliafito, C. A.

AU - Rice, T. A.

AU - Schatz, H.

AU - Whitcup, S. M.

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N2 - Purpose: The authors describe the clinical feature of ten patients with a new syndrome characterized by the presence of retinal vasculitis, multiple macroaneurysms, neuro-retinitis, and peripheral capillary nonperfusion. Methods: The authors evaluated ten patients identified to have clinical features compatible with the syndrome of idiopathic retinal vasculitis, aneurysms and neuroretinitis (IRVAN). Clinical examination findings, sequential fundus photographs (when available), fluorescein angiograms, systemic investigations, response to therapy, and visual outcomes were reviewed. Results: Seven eyes of four patients sustained a marked decrease in visual acuity of 20/200 or worse. Visual loss was due to a combination of an exudative maculopathy and sequelae of retinal ischemia. Capillary nonperfusion was seen in all ten patients and was severe enough to warrant panretinal laser photocoagulation in six patients. Systemic investigations were uniformly noncontributory. Oral prednisone appears to have little beneficial effects on patients with this disorder. Conclusions: Patients with IRVAN have characteristic retinal features that readily identify this syndrome. An increased awareness of this rare syndrome may help to identify sight-threatening complications at an earlier stage. The authors caution against extensive medical investigations.

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