Title: Retinal Vasculitis Case Report
Author: Sherief Raouf, Visiting Medical Student from the Stony Brook School of Medicine
Photographer: Glen Jenkins
Date: Friday, August 19, 2016
Diagnosis: Idiopathic mixed arterial/venous occlusive vasculitis
Secondary CORE Category: Retina and Vitreous / Focal and Diffuse Choroidal and Retinal Inflammation
Description of Image:
|Negative Laboratory Workup|
|ANCA||Systemic necrotizing vasculitis|
|Serine Proteinase Ab||Systemic vasculitis|
|HbSAg, HbSAb||Hep B|
|ANA||Sensitive for SLE|
|Anti-Smith Ab||Specific for SLE|
|dsDNA Ab||Lupus Nephritis & SLE|
|Rh Factor||Rheumatoid Arthritis|
|Cardiolipin Ab and B2GP Ab||Anti-phospholipid syndrome|
|SSA Ab and SSB Ab||Sjogren’s & SLE|
|Ribonucleic Protein Ab||SLE & Mixed connective tissue disease|
|PT, PTT, dRVVT||Hypercoagulability|
Background: The patient is a 52 year old woman with a past medical history of diabetes mellitus and hypothyroidism who presented complaining of reduced vision OD, described as a “large grey spot in the center.” Visual acuity was 20/30 OD, 20/20 OS, pupils equal and reactive to light with no afferent pupillary defect, and the anterior chamber deep and quiet OU. The vitreous OD demonstrated +1 cells and the macula OD exhibited pigmentary atrophy and a small superior branch retinal artery occlusion (BRAO), while both eyes showed signs of periphlebitis.
There were no obvious signs of systemic inflammatory disease including Lupus, Sjogren’s, Giant-cell Arteritis, Granulomatosis with polyangiitis, or other systemic vasculitis. The review of systems was negative for cough, fever, orogenital ulcerations (Behçet disease), hearing loss and encephalopathy (Susac syndrome), or skin rash. Initial laboratory workup is shown in Table 1.
A laboratory investigation of infectious causes was performed and the following tests were negative: quantiferon, FTA-ABS, RPR, B. Burgdorferi Ab, toxoplasmosis and HIV ELISA. A laboratory workup for hypercoagulability was also negative (including PT, PTT, dRVVT). Brain MRI was negative for any acute intracranial process or findings of vascular inflammation or stenosis. Chest X-ray showed normal vascular markings, and a lack of any nodules, consolidations or hilar adenopathy.
The diagnosis of an arterial occlusive vasculitis was made, and treatment with oral Prednisolone and Mycophenolate mofetil was begun. One month later, the patient returned complaining of worsening blurriness OD. Exam revealed decreased vitreous cells with a persistent superior BRAO, periphlebitis and retinal neovascularization OD. The decision to treat the right eye with peripheral panretinal photocoagulation (PRP) was made. Over the ensuing months, the retinal vasculitis was medically managed with courses of prednisolone, mycophenolate and methotrexate and regular follow-up maintained. The photos above are taken from a visit 12 months after the initial visit, upon which a new BRAO was discovered.
The fundus photo demonstrates the gray-white sheathing of a retinal branch artery that is characteristic of a retinal vasculitis.1 The perivascular sheathing in occlusive vasculitis is thought to be an exudate of inflammatory cells around the vessel that leads to occlusion (Figure A, yellow arrow). Occlusion of the retinal vessel can result in ischemia of the retina and areas of capillary non-perfusion. This fundus photo demonstrates such an area of ischemic retina that is seen downstream and superior to the occluded vessel. This retina also exhibits neovascularization coincident with the ischemic areas of retina.
The late frame fluorescein angiography shows evidence of vascular obstruction in the inferior arcade, distal to the sheathed retinal branch artery. There is evidence of diffuse retinal vasculitis and vessel leakage. In addition, we can see the area of prior PRP of the areas of retinal capillary non-perfusion (Figure B, blue arrow). The angiography is able to additionally define the zones where the loss of retinal perfusion (Figure A, white arrow) has led to new areas of leaking neovascularization. Notably, there is a pronounced macular hyperfluorescence that corresponds to neovascularization (Figure B, green arrow). Given that neovascularization has continued, and that the vasculitis is active, the decision to restart corticosteroid treatment was undertaken with a plan for another round of PRP. Typically it would be preferable to ensure that the vasculitis is inactive when PRP is implemented, as its use can result in the release of more angiogenic factors, aggravating neovascularization.2
A key distinction to be made in making this diagnosis is between that of primary branch retinal vein occlusions, which closely mimics idiopathic retinal vasculitis. In BRVO, the occlusions typically occur at arteriovenous crossings, and are not multiple nor as peripheral as they tend to be in vasculitic obstructions.3 Finally, it should be said that there is some consensus to label idiopathic retinal vasculitis as Eales disease when vascular occlusions and neovascularization lead to recurrent vitreous hemorrhage in the presence of serological evidence of tuberculosis.3 Admittedly, this distinction may be a semantic one, as the yet unsolved pathophysiology may one day reveal these two entities to lie on one spectrum.
The differential for retinal vasculature occlusions is very broad and can be usefully divided into non-inflammatory (Diabetes, BRVO) and inflammatory causes. Inflammatory etiologies were examined with a careful history and an extensive laboratory and radiologic workup, yielding no clear cause. Indeed, many inflammatory retinal vascular obstructions are secondary to a systemic inflammatory process (infectious and non-infectious). However, primary idiopathic retinal vasculitis often is isolated to the eye and absent of any systemic involvement, frequently making this a diagnosis of exclusion.4
Format: Fundus photography and fluorescein angiography
- Gass, J. Donald M. Stereoscopic Atlas of Macular Diseases: Diagnosis and Treatment. St. Louis: Mosby, 1997. Print.
- Biswas, J. et al. Eales disease–an update. Surv Ophthalmol 47, 197–214 (2002).
- Namperumalsamy, P., and Dhananjay S. “Eales ” Retina. Stephen Ryan MD et al. 5th ed. Oxford: Saunders, 2013. 1479-1485. Print.
- Saurabh, K., Das, R., Biswas, J. & Kumar, A. Profile of retinal vasculitis in a tertiary eye care center in Eastern India. Indian Journal of Ophthalmology 59, 297 (2011).
Faculty Approval By: Dr. Akbar Shakoor, Dr. Griffin Jardine
Disclosure: No financial disclosures to share