A 29-year-old Asian Indian male was diagnosed as Eales’ disease in the left eye and was referred to the uvea clinic of our tertiary eye care center in south India for persistent inflammation in the left eye. He was treated with oral steroid and had pan-retinal laser photocoagulation of neovascularisation of retina. He had poor vision in the right eye since 10 years.
On examination, his best corrected visual acuity was no perception light in the right eye and 20/200; N36 in the left eye. Right eye was phthisical with pupillary membrane, peripheral anterior and posterior synechiae, complicated cataract and low intraocular pressure.
Anterior segment of left eye was normal. Indirect ophthlomoscopy of the fundus showed media haze at the posterior pole, a pale optic disc with attenuated and sclerosed vessels. There was few retinal hemorrhages along with collaterals temporal to the macula. Mid-peripheral retina showed laser photocoagulation scars [Fig. 1A]. Fundus fluorescein angiography in the arterio-venous phase showed disc leakage with active vasculitis temporal to fovea [Fig. 1B]. Ischemic areas were also noted temporal to macula. Fundus lesions were suggestive of active Eales’ disease in the left eye. The patient was investigated for causes of retinal vasculitis. Laboratory investigations for toxoplasma, syphilis, sarcoidosis and collagen diseases were negative. The right eye was enucleated for cosmetic reasons. The eyeball was fixed in 10% neutral buffered formalin, sectioned axially and subjected to processing for paraffin embedding. Serial sections were stained with Hematoxylin and Eosin, Masson's trichrome stain for collagen and Ziehl-Neelsen stain(ZN) for acid fast bacilli. IHCs was performed for multiple markers which included glial fibrillary acidic protein (GFAP), CD45, CD68, CD3, CD4, CD8, CD20, CD138, MPO, IgG and IgG4.
Histopathology revealed a phthisic eyeball with a thick epiretinal membrane and retinal detachment. The epiretinal membrane was densely collagenized and scarred, relatively avascular with sclerotic vessels reflecting chronicity and flanked above and below by metaplastic bone. A think band of dense fibrillary gliosis was seen in the adherent retina (Fig. 2A). Inflammation was seen forming a small aggregate beneath the ciliary body. It had an admixture of lymphocytes, histiocytes with elongated nuclei and epithelioid morphology forming loose clusters, admixed with lymphocytes and few polymorphs. The lymphoid cells were CD3 + T cells with scant to absent CD20 + B cells. The T cells were CD8 + cytotoxic cells. No CD4 + T cells or plasma cells were seen. IgG4 was negative. The inflammatory focus had elicited a fibrillary gliosis and collagenization entrapping thin vascular channels (Fig. 3A-H). ZN stain for acid fast bacilli was negative.
DNA was extracted from the paraffin section and nested PCR targeting MPB64 gene and IS6110 region of MTB genome were found to be positive [Fig. 5A]. Real time PCR showed 3,460 of copies of MTB DNA [Fig. 5B].
Patient was put on four drug antitubercular treatment with oral Prednisolone 60 mg per day which was gradually tapered over 6 weeks. On last follow up, there was complete resolution of vasculitis. However, there was no improvement of vision in OS due to macular ischaemia.