Author: Sergio Matas Granados
Co-authors: WILFREDO MENJiVAR AGUILAR, ANDReS ROMERO MARTiNEZ
Abstract
PURPOSE: 100 WORDS MAX.To report a case of a Purtscher-like retinopathy that was diagnosed in a 52-year-old woman with visual acuity loss in the course of idiopathic pneumonia, who was later diagnosed with adult-onset Still’s disease (AOSD). The objective is to show this rare association between both diseases.
Initially described secondary to massive head trauma and other types of traumas, Purtscher-like retinopathy has been reported in diverse conditions, including acute pancreatitis, thrombotic microangiopathies, hypercoagulable states, chronic renal failure, and autoimmune rheumatic disorders such as systemic lupus erythematosus, juvenile dermatomyositis, and scleroderma.
SETTING/VENUE 50 WORDS MAX.
Purtscher retinopathy is characterized by the appearance of cottony exudates and localized hemorrhages around the optic disc. Its appearance responds to ischemic phenomena in the posterior retinal pole.
AOSD is a rare multisystemic autoinflammatory disease with quotidian spiking fever, evanescent rash, arthralgia, and multiple organ involvement, including the eye.
METHODS 200 WORDS MAX.
We present the case of a 52-year-old woman admitted to our hospital for atypical community-acquired pneumonia treated with systemic broad-spectrum antibiotics with poor outcome. She had several feverish peaks since admission despite having made several changes in systemic antibiotic treatment, in addition to polyserositis and congestive heart failure. The patient began treatment with intravenous corticosteroids from day 7 after admission.
After 12 days from admission, the patient complained of blurred vision in both eyes. Her visual acuity was 0.1 in the right eye and 0.3 in the left eye. Her anterior pole was normal in both eyes. In the fundus, cottony exudates and hemorrhages were observed in the peripapillary region of both eyes.
In the macular optical coherence tomography (OCT) we observed a serous detachment of the subfoveal neuroepithelium together with intraretinal fluid and edema of the nerve fiber layer in the papillomacular bundle. Papillary OCT showed peripapillary nerve fiber layer edema in all quadrants of both eyes.
RESULTS 200 WORDS MAX
The patient presented clinical improvement and was discharged 5 weeks after admission. She reported a generalized skin rash prior to the onset of respiratory symptoms; therefore, she was diagnosed as adult Still's disease.
3 months later, she was assessed again in our consultation. Visual acuity in her right eye was 0.4 and in her left eye 0.6. Peripapillary lesions in the fundus decreased in size and number. There was an arteriolar narrowing in both eyes.
The macular OCT showed a macular retinal thinning without intra or subretinal edema. The OCT of the papillae also showed thinning in the peripapillary nerve fiber layer of both eyes.
CONCLUSIONS 200 WORDS MAX
AOSD, which is characterized by nonspecific clinical findings such as high fever, arthritis, and rash, should be considered in the differential diagnosis of cases that present with fever of unknown origin and blurred vision. Purtscher-like retinopathy can be seen in all stages of AOSD. Multidisciplinary approaches could prevent severe complications and allow early diagnosis to help initiate treatment in a timely manner.
Regarding treatment, several systematic reviews did not find statistically significant difference for patients treated with corticosteroids compared with observation. However, some case reports showed that corticosteroids use did improve visual outcome.
