Eales' disease Dr Chinmayi Vyas M.S. Dr Jyotirmay Biswas M.S., FAMS, FIC Path,FAICO Director of Uveitis and Ophthalmic pathology Sankara Nethralaya, Chennai,India

Ocular History 30 year old lady February 2012 complaints of left eye blurred vision with floaters. No h/o similar problems Systemic history : unremarkable

February 2012 : First Presentation Right eye Left eye BCVA 6/6; N6 3/60, N36 IOP 35 28 Anterior segment Normal Vitreous cells ++ Fundus findings Occlusive vasculitis with active retinitis in superiotemporal quadrant

February 2012 : First Presentation ESR:12 mm Mantoux Test : positive Serum Angiotensin converting enzyme: 8.4 U/L QuantiFeron TB gold test : positive High resolution computed tomography scan chest : non specific lesion

February 2012 Eale’s disease v/s presumed tuberculous vasculitis Started on Oral prednisolone 1 mg/kg (60 mg/day) Reviewed with chest physician: started on 2 drug anti TB Rx for 9 months. High Intra ocular pressure on first visit: steroid responder ?? Started on anti glaucoma Rx

Feb 2012 – June 2012 Improvement in vision Right eye 6/6 , N6 ; Left eye 6/9, N6 Activity reduced as compared to first visit. Oral steroid tapered, anti glaucoma treatment continued

June 2012 complains of reduced vision in Left eye Patient was on prednisolone 10 mg/day Right eye Left eye BCVA 6/6; N6 3/60, N36 IOP 10 12 Anterior segment quite Vitreous cells ++ Fundus findings Active vasculitis Occlusive vasculitis with active retinitis with macular edema

June 2012 Fundus fluorescine angiography advised Oral prednisolone dose hiked up ? ? other causes Tests done: C-ACNA: negative P- ANCA: negative HLA B51: negative

July 2012 Oral steroid dose increased Left eye sectoral panretinal photocoagulation done around area of neovascularization Anti TB treatment continued

August 2012 Sudden reduction in vision in Left eye Right eye Left eye BCVA 6/6; N6 CF 1 mt; N36 IOP 10 12 Anterior segment quite Fundus findings Resolving vasculitis Vitreous hemorrhage

August 2012

August to September 2012 August 2012 - right eye : vision maintained; disease stabilized - Left eye: non resolving vitreous haem September 2012 - Left eye: Pars plana vitrectomy with membrane peeling with Endo laser application done under steroid cover - Vitreous sample taken for Polymerase chain reaction(PCR) for Mycobacterium Tuberculosis

Diagnosis after vitrectomy PCR for M. Tuberculosis : positive Mantoux Test : positive QuantiFeron TB gold test : positive Presumed Tuberculous retinal periphlebitis

Problems Eales' disease v/s presumed tuberculous periphlebitis Negative Mantoux test does not exclude Tuberculosis QuantiFERON TB gold test : adds to the diagnosis PCR of vitreous biopsy for MPB 64 diagnostic Presumed tuberculous periphlebitis most common cause for Eales disease.

Follow-up: November 2012 continued on oral steroids tapering dose Right eye Left eye BCVA 6/6; N6 6/9, N6 IOP 10 12 Anterior segment quite Fundus findings Resolving vasculitis continued on oral steroids tapering dose

Follow-up: November 2012

Follow-up: March 2013 Right eye Left eye BCVA 6/6; N6 6/7.5, N6 IOP 8 9 Anterior segment WNL quite Fundus findings Resolving vasculitis Resolved vasculitis continued on oral prednisolone 10mg/day: stopped after 2 months

Follow-up: March 2013

FFA done: no active vasculitis Follow-up: November 2013 FFA done: no active vasculitis Off oral steroids >6 months Right eye Left eye BCVA 6/6; N6 IOP 8 Anterior segment WNL quite Fundus findings Resolved vasculitis Laser marks with resolved vasculitis

Follow-up: November 2013

Discussion Eales disease is defined as idiopathic inflammatory Retinal vasculitis with peripheral retinal revascularization primarily affecting the peripheral retina. It has high male preponderance Etiopathogenesis of Eales’ disease is still controversial and ill-understood. Tuberculosis is considered to be the most important cause for eales disease as evidenced by molecular micro biological studies.

Conclusion Treatment with anti tuberculous treatment along with oral steroids treatment is very useful especially in developing countries with high prevalence of tuberculosis Prompt retinal photocoagulation of the area of neovascularization and capillary non perfusion helps in preventing the complications

Conclusion It is in the nature of the disease to have recurrences Therefore it is prudent to have regular follow ups for early diagnosis of recurrence of vasculitis and complications like peripheral neovascularization and vitreaous hemorrhage