Article Text

Retinal pigment epithelial detachment: an unusual presentation in ocular sarcoidosis
  1. T BOURCIER,
  2. L LUMBROSO,
  3. N CASSOUX,
  4. C FARDEAU,
  5. P LEHOANG
  1. Ophthalmology Department, Pitié-Salpêtrière Hospital, Paris, France, 47–83, boulevard de l’Hôpital, Paris Cedex 13, France
  1. Professor P Lehoang.

Statistics from Altmetric.com

Editor,—Sarcoidosis is a systemic granulomatous disease of unknown aetiology that most often affects lungs, lymph nodes, liver, and the skin. Ocular involvement occurs in about a quarter of patients.1 2 More often it affects the anterior segment and the adnexal tissues. However, posterior segment involvement in sarcoidosis is not uncommon3 4 and various manifestations include vitreous inflammation, retinal periphlebitis, candlewax drippings, optic nerve, choroidal and chorioretinal granulomas. Optic disc swelling and macular oedema can also be seen. Choroidal and optic disc neovascularisation are a rare occurrence as well as optic nerve atrophy. Exudative detachments of neurosensory retina have been described in a few patients1 5 but to the best of our knowledge retinal pigment epithelial (RPE) detachments have never been described. We report the clinical features of a case of RPE detachment in sarcoidosis.

CASE REPORT

A 47 year old black woman presented with sudden bilateral vision decrease. Her medical history was remarkable for histologically proved sarcoidosis that had been diagnosed 6 years previously during an episode of lupus pernio associated with pulmonary disease. This patient was treated for 3 years with hydroxychloroquine sulphate (200 mg a day) for the dermatological manifestations of the disease. The pulmonary disease was thought quiet until the occurrence of the ocular symptoms.

On examination, her best corrected visual acuity was 20/70 in the right eye and 20/30 in left eye. Slit lamp examination showed mutton fat keratic precipitates with 2+ cells and flare in both eyes. Intraocular pressure was normal in both eyes. On fundus examination, mild vitreous haze, yellowish small choroidal infiltrates, as well as RPE detachments could be seen scattered throughout the posterior pole in both eyes (Fig1). There was no overlying sensory retinal detachment. The retinal vessels seemed to be normal. Fluorescein (Fig 2) and indocyanine green angiography showed early hypofluorescence and late hyperfluorescence corresponding to RPE detachments. There was no prior history of ocular trauma or surgery and no extraocular signs (no neurological, skin, or hair manifestations). The systemic sarcoidosis was not active at the time of ocular manifestations.

Figure 1

Fundus photograph showing small choroidal infiltrates as well as RPE detachments scattered throughout the posterior pole of the left eye.

Figure 2

Late venous phase (10 minutes) of fluorescein angiogram of the same eye showing multiple hyperfluorescence areas of different sizes in the posterior pole.

Systemic (1 g intravenous pulses of methylprednisone daily for 3 days followed by oral prednisone 1 mg/kg a day), periocular, and topical corticosteroids were then administered. On follow up, 1 month later, visual acuity improved to 20/25 in both eyes and fluorescein angiograms showed complete disappearance of the abnormal leakage with only transmission defects due to pigment epithelial atrophy. Systemic steroids were then tapered. Two months later a recurrence of inflammation and the same fundus presentation occurred.

In spite of an initial sensitivity to systemic steroid therapy, four new relapses occurred and corticosteroid dependence at around 40 mg a day was observed. Azathioprine therapy was necessary to obtain a complete remission.

COMMENT

The aetiology of most RPE detachments occurring in young patients (under 50 years old) is unknown. These detachments are usually solitary and unilateral.6 RPE detachments are also commonly seen in association with inflammatory diseases such as sympathetic ophthalmia and Harada’s disease. In this last disease, multiple bilateral RPE detachments associated with secondary sensory retinal detachments may occur, and extraocular signs including alopecia, vitiligo, hearing loss, and encephalitis are often noted. RPE detachments may be associated with systemic corticotherapy.6

To the best of our knowledge, RPE detachments have never been associated with sarcoidosis.

In this case, there was no concurrent diagnosis other than sarcoidosis that could account for RPE detachments. Indeed, this patient had no other sign for Vogt–Koyanagi–Harada syndrome or sympathetic ophthalmia and no prior steroid therapy. The appearance of RPE detachments was concomitant with the onset of a typical anterior granulomatous uveitis and the lesions were dramatically improved by steroid therapy. These facts as well as the dependence on steroid therapy strongly suggest that RPE detachments and the choroidal infiltrates are inflammatory in nature. We can hypothesise that choriocapillary inflammation is a possible patho-aetiology of these manifestations as it can be seen in other inflammatory diseases.

In conclusion, RPE detachments were identified in one patient with biopsy proved sarcoidosis and could be considered as a rare manifestation of ocular sarcoidosis.

References

View Abstract

Request permissions

If you wish to reuse any or all of this article please use the link below which will take you to the Copyright Clearance Center’s RightsLink service. You will be able to get a quick price and instant permission to reuse the content in many different ways.