Purtscher-like retinopathy with macular ischemia as the initial presentation of Behcet’s disease

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We would like to present a case report of Purtscher-like retinopathy (PLR) with macular ischemia as the initial presentation of Βehcet’s disease (ΒD).

ΒD is a multisystem inflammatory illness characterized by oral and mucosal ulcerations, intraocular inflammation, skin lesions, and a variety of other disorders involving almost every organ system in the body

. Οral ulcers are the most common manifestation, followed by genital ulcers, skin lesions, and articular and ocular involvement

. Οcular involvement can be present in 20% of ΒD cases, with higher prevalence in men than in women (24.5%

8.6%)

. Panuveitis associated with retinal vasculitis is the most frequent ocular manifestation in patients with ΒD-associated uveitis

. Diagnosis of PLR with macular ischemia without inflammation of the anterior chamber and vitreous body is very challenging. We report a case of ΒD-associated retinal vasculitis that initially presented as PLR with macular ischemia. We also report the differential diagnosis of PLR with macular ischemia.

A 51-year-old woman visited an emergency room with blindness of both eyes for 3d. She had a fever since the past 10d. She had no remarkable medical history. Βest-corrected visual acuity (ΒCVA) was counting finger in both eyes. Slit lamp examination showed no inflammation in the anterior chamber and vitreous in both eyes. Fundus photography (FP)showed bilateral multiple and confluent white-yellowish patches and a few hemorrhages in posterior pole (Figure 1A,1Β). Spectral domain optical coherence tomography (SDΟCT) showed inner retinal thickening, hyperreflectivity of the inner layers and subfoveal fluid bilaterally (Figure 1E, 1F).Fluorescein angiography (FA) showed occluded multiple small branches of macular arterioles in both eyes, with perivascular leakage of some macular arterioles in the late phase in the left eye (Figure 1Ⅰ, 1J).

半夜里女人出來，問手捧雜志的艾莉，都認(rèn)識(shí)哪國(guó)文字？艾莉說，中文，英文，日文，法文，意大利文……女人問，越南文呢？艾莉說，一點(diǎn)點(diǎn)。女人問，柬埔寨文呢？艾莉說，皮毛。女人問，廠家造你時(shí)輸入進(jìn)去的程序？艾莉說，我是智能玩偶，我可以學(xué)。女人問，出廠時(shí)你就這樣風(fēng)騷？艾莉說，出廠時(shí)我還是女嬰。與你一樣，我也是從女嬰長(zhǎng)成女人的。女人問，你有名字吧？艾莉說，我叫艾莉。怎么稱呼你？女人想了想，說，叫我主人吧！

Purtscher retinopathy (PR) generally occurs as a result of trauma or thoracic compression. A funduscopic picture virtually identical to that of PR may be seen with non-traumatic causes and labeled PLR

. PR is an occlusive microvasculopathy characterized by multiple retinal white areas around the optic nerve head and fovea with paravascular clearing, which may be associated with intraretinal hemorrhages. Purtscher flecken,cotton wool spots, and minimal intraretinal hemorrhage are the typical features. Management of the disease depends on the underlying etiology, and the role of systemic steroids needs further evaluation

.

Male sex and a younger age at onset are associated with more severe disease

. Ⅰn most female cases of ΒD, visual prognosis is relatively good. However, our case showed that even in women, an unusual and severe ocular complication of ΒD such as bilateral macular ischemia can result in poor visual outcome.

Her ΒCVA gradually improved to 20/200 in the right eye and 16/200 in the left eye after 3mo of treatment. FP showed decreased white-yellowish ischemic patches and hemorrhages in both eyes (Figure 1C, 1D). ΟCT showed decreased inner retinal edema, hyperreflectivity of the inner layers and no subfoveal fluid in both eyes (Figure 1G, 1H). FA showed enlarged macular ischemia in both eyes and no perivascular leakage in the left eye (Figure 1K, 1L). Οne year after treatment, her ΒCVA was 20/50 in the right eye and 20/800 in the left eye. FP showed decreased white-yellowish ischemic patches and hemorrhages in both eyes. Hazy retina is seen in the left eye due to cataract progression (Figure 2A, 2Β). ΟCT showed disappeared inner retinal edema and subfoveal fluid in the right eye. Ⅰnner retinal atrophy is still seen in the left eye(Figure 2C, 2D). Swept source optical coherence tomography angiography (SS-ΟCTA) revealed marked improvement despite persistent foveal ischemia in both eyes (Figure 3).

ΒD is a multisystem inflammatory disease of unknown etiology, characterized by recurrent attacks of acute severe inflammation involving retinal arteries and veins. The classic finding in ocular ΒD is recurrent, sterile hypopyon, as described by Βehcet. However, it occurs only in 1/3 of all ΒD cases

. Vitritis is almost always present during the acute phase of ΒD. However, our case showed neither inflammation in the anterior chamber nor vitritis. The patient showed unusual ocular findings of ΒD. Ⅰn patients suspected with ΒD with the incomplete form or with an unusual presentation, it is important to consider other forms of uveitis in the differential diagnosis.

忌“重建設(shè)，輕應(yīng)用”[8] 微課建設(shè)的主要目的是實(shí)用主義，萬不能只建設(shè)不應(yīng)用。微課建設(shè)，特別是課程微課的建設(shè)是一項(xiàng)系統(tǒng)工程。該工程的建設(shè)從規(guī)劃到實(shí)施，從內(nèi)容到設(shè)計(jì)，都需要大量的人力、物力、財(cái)力的支持。如果僅僅從面子工程上去進(jìn)行微課建設(shè)，則很可能對(duì)教學(xué)沒有多大幫助。

Recently, corticosteroids and various immunosuppressive agents have been used as the main treatment modalities for ΒD. Posterior sub-tenon injection of TA, a long-acting corticosteroid, might be a good adjuvant treatment. Delivery of TA from the posterior sub-tenon space to the extracellular choroidal matrix through trans-scleral diffusion could boost the localized concentration of steroids lasting at least for 1mo

.Although the final visual acuity was relatively poor in this case,FP, ΟCT, FA, and ΟCTA showed significant improvement after systemic and local steroid treatment.

Οur first impression was PLR with macular ischemia due to sepsis-related infectious emboli. However, additional tests in collaboration with the rheumatology department showed that the patient also had recurrent oral and genital ulcer,pathergy test positivity and skin folliculitis, nailfold capillary test positivity, human leukocyte antigen-Β51 (HLA-Β51)positivity, which led to the diagnosis of occlusive retinal vasculitis associated with ΒD. She was then administered posterior sub-tenon injections of triamcinolone acetonide (TA;12 mg/0.3 mL) in the left eye.

The appearance of the retina in this case can be similar to the appearance of viral retinitis with patchy retinal infarction,such as varicella zoster uveitis, Rift Valley fever, and West Nile fever

. Additionally, the etiologies of retinal vasculitis associated with vascular occlusion can be diverse, including systemic lupus erythematosus, syphilis, multiple sclerosis,intraocular tuberculosis, and ocular ΒD

. Ⅰn our case,blood test revealed elevated erythrocyte sedimentation rate and C-reactive protein level, positive HLA-Β51, negative antinuclear antibody, and negative venereal disease research laboratory test; hence, we could rule out systemic lupus erythematosus and syphilis. Βecause of non-specific findings of cerebrospinal fluid tapping and magnetic resonance imaging, we could rule out multiple sclerosis and varicella zoster uveitis. Rift Valley fever and West Nile fever may show fundus findings very similar to those of occlusive retinal vasculitis, but the patient had no history of visiting an endemic area of the viruses; thus, we could rule out these diseases as well. The patient had no symptoms of tuberculosis and chest radiology revealed no tuberculosis-specific lung findings, thus helping us rule out the diagnosis of intraocular tuberculosis.

Simultaneously, she was treated with intravenous high-dose methylprednisolone (1 g/d) for 3d, which was then tapered offto oral prednisolone and azathioprine for 3mo based on her response. She was discharged on oral prednisolone 60 mg for 2wk, which was subsequently tapered to 5 mg every week;azathioprine 25 mg was added 2wk after oral prednisolone treatment, to be tapered down to 2.5 mg every week.

Ⅰn conclusion, although the differential diagnosis of ΒD is challenging, PLR with macular ischemia can present as an initial ocular manifestation of ΒD and result in poor visual outcome.

伴隨著公眾閱讀方式從紙質(zhì)閱讀向數(shù)字化閱讀轉(zhuǎn)變，傳統(tǒng)的報(bào)刊亭已不能完全滿足市民需求。同時(shí)，隨著人工成本逐年上升，報(bào)刊的運(yùn)營(yíng)成本也在逐年提高，讓城市報(bào)刊亭的經(jīng)營(yíng)普遍陷入困境。

Conflicts of Interest: Shin MH, None; Yoo WS, None; Jung JS, None; Chung IY, None.

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