Unusual presentation of CREST retinal vasculitis



To report a case of CREST (calcinosis, Raynaud’s phenomenon, esophageal dysmotility, sclerodactyly, telangiectasias) syndrome-induced retinal vasculitis in the setting of ocular inflammation soon after recent micropulse cyclophotocoagulation (mTS-CPC).


Our patient developed CREST associated retinal vasculitis in both eyes (right > left) eight days after receiving mTS-CPC in her left eye. There was initial concern for sympathetic ophthalmia due to the resulting bilateral inflammation. The patient was treated with prednisone with resolution of her symptoms.


This is the first case of CREST retinal vasculitis that appears to be directly triggered by inflammation caused by mTS-CPC.


CREST syndrome (calcinosis, Raynaud’s phenomenon, esophageal dysmotility, sclerodactyly, telangiectasias) is a limited form of scleroderma, primarily affecting specific organ systems rather than the entire body. Most ocular symptoms of CREST are limited to the anterior segment and include keratoconjunctivitis sicca and eyelid telangectasias. Posterior pole findings typically mimic those of hypertensive retinopathy and include vascular tortuosity, cotton wool spots (CWS), and mild retinal microvascular abnormalities. Additional posterior pole findings are quite rare – to date only two cases of CREST vasculitis have ever been published. Other rare ocular CREST presentations include two cases of juxtafoveal telangectasias, one central retinal artery occlusion (CRAO), two branch retinal artery occlusions (BRAO), , and three cases of granulomatous uveitis. ,

We describe a novel case of CREST vasculitis in which our patient presented with concern for sympathetic ophthalmia due to the resulting bilateral inflammation that ensued following a transscleral micropulse cyclophotocoagulation (mTS-CPC) treatment for end-stage glaucoma in her left eye eight days prior. To date, this is the first reported case of CREST vasculitis that may have been triggered by intraocular inflammation following mTS-CPC. This is also the first case report in which sympathetic ophthalmia was a differential diagnosis for CREST vasculitis.

Case report

A 65-year-old female presented with sudden onset of painless, decreased vision in both eyes. The patient had been feeling weak at the time of presentation. She had a history of difficulty with swallowing which had flared over the past few days. The patient also reported chest tightness and pain radiating down her left arm with exertion. Eight days prior to the above presentation, the patient had received mTS-CPC for refractory glaucoma in her left eye. Her past medical history included CREST syndrome with known Raynaud’s phenomenon, diabetes mellitus type 2, asthma, chronic kidney disease stage 3, osteoporosis, anemia, hearing loss, pancreatitis and pulmonary hypertension. Surgical history included hemorrhoidectomy, adenoidectomy and hysterectomy. Her ocular history was significant for cataract surgery, chronic angle closure glaucoma, laser peripheral iridotomy (LPI) and trabeculectomy OU. She was currently taking timolol OU, latanoprost OD, prednisolone acetate OS, moxifloxacin OS, and oral ranitidine, nystatin, pantoprazole, KCl, atorvastatin, amlodipine, prednisone, cetirizine, metformin, aspirin and sertraline. She had drug allergies to clindamycin and sulfa drugs. She denied alcohol, tobacco or drug use. Her family history was significant for primary open angle glaucoma, Parkinson’s disease and coronary artery disease (CAD) in her father and CAD in her mother. Review of systems was positive for dyspnea on exertion and difficulty swallowing. On examination best corrected Snellen visual acuity (BCVA) was 20/30 OD and 20/100 OS. Her visual field was constricted in all four quadrants OS. Pupils measured 4 mm in dark and 3 mm in light, and no relative afferent pupillary defect (RAPD) was noted. Intraocular pressure measured 9 mm Hg and 15 mm Hg respectively. On anterior segment exam a cystic bleb was noted superiorly in both eyes and endopigment was noted across her posterior lens capsule OS. There was 1+ anterior chamber cell in the right eye and no inflammation noted in her left eye. The patient had patient LPIs in both eyes and scatter iris-corneal touch and lens-iris touch in the left eye. A posterior chamber intraocular lens was present OD and sulcus lens was present OS. On dilated exam, a mild vitritis with a mix of white blood cells and pigment was noted in the right eye. She had a 0.8 and 0.9 cup to disk ratio respectively with inferior thinning OU. Retinal pigment epithelial (RPE) mottling was noted in both eyes. In the right eye, CWS were visible and white membranes were noted to emanate from the disc. Vessels were sclerotic with mild perivascular sheathing OU. In her left eye, CWS were noted along the nasal arcade.

Our patient was started on prednisone 40 mg by mouth and prednisolone acetate 1 % ophthalmic suspension was started six times a day OD. She returned for follow-up a day later and was found to have CWS and vasculitis in all four quadrants OU. At this time optos photos ( Fig. 1 : A-B) were taken. White membranes extended from the disc nasally, and vasculitis was noted superiorly, nasally and temporally OD. Vasculitis was observed nasally and temporally OS. Fluorescein angiogram (FA) ( Fig. 2 : A-B) was taken and demonstrated significant hypofluorescence from nonperfusion during the arteriovenous phase and perivascular hyperfluorescence with leakage during the late recirculation phase OU. Optical coherence tomography (OCT) macula demonstrated retinal thickening OD > OS. Uveitis and infectious work-up was negative, including antineutrophil cytoplasmic antibodies (ANCA), rheumatoid factor (RF), human leukocyte antigen-B27 (HLA-B27), angiotensin converting enzyme (ACE), lysozyme, interferon gamma releasing assay, bilirubin, Bartonella antibodies, rapid plasma reagin (RPR), reticulocyte count and pancreatic elastase-1. However, antinuclear antibody (ANA) with reflex titer was positive > 1:640.

Jan 3, 2022 | Posted by in OPHTHALMOLOGY | Comments Off on Unusual presentation of CREST retinal vasculitis

Full access? Get Clinical Tree

Get Clinical Tree app for offline access