Retina Quiz
Lupus Treatment: Cause or Cure?
Mark T. Dunbar, O.D.A 30-year-old Hispanic female was referred by her general physician for a baseline evaluation so she could be started on Plaquenil (hydroxychloroquine) therapy. She had been diagnosed with systemic lupus erythematosus approximately one year ago, and was taking oral prednisone 60mg daily. Her doctor had hoped that Plaquenil would control her lupus better than the oral prednisone that she was currently on. Her only other medication was the antacid Prevacid for a stomach ulcer. She did not report any problems with her vision.
On examination, her best-corrected visual acuities were 20/20 O.D., 20/20 O.S. A 10-2 Humphrey visual field of the right eye showed a few points depressed nasally, but was otherwise normal. The pupils were equally round and reactive without an afferent pupillary defect. Amsler grid and color vision testing was normal in each eye.
The dilated fundus exam of the right eye revealed a localized area of subretinal fluid temporal to the macula, without evidence of hemorrhage. A yellow-white area of discoloration within the fluid also was apparent (figure 1). A fluorescein angiogram was performed, and an early frame is available for your review (figure 2). The remainder of the exam in the right eye was unremarkable. The fundus exam of the left eye was completely normal.
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| 1. In the right eye, a localized area of subretinal fluid can be seen temporal to the macula, with a yellow-white discoloration in the center of the localized area. 2. Fluorescein angiography shows a pinpoint spot of hyperfluorescence with staining of the fluid within the area. | |
Quiz
- How would you best describe the localized
area of subretinal fluid?
a. Pigment epithelial detachment (PED).
b. Neurosensory retinal detachment.
c. Localized area of ischemia.
d. Active retinitis. - What is the correct diagnosis?
a. Idiopathic central serous chorioretinopathy (ICSC).
b. Choroidal neovascular membrane (CNVM).
c. Lupus choroidopathy.
d. Branch retinal artery obstruction (BRAO). - How would you manage this patient?
a. Observation.
b. Laser photocoagulation.
c. Discontinue corticosteroids.
d. Increase corticosteroids.
- Which of the drugs mentioned above is
associated with exudative bullous retinal detachments when prescribed for the
treatment of this retinal condition?
a. Plaquenil.
b. Prednisone.
c. Prevacid.
d. None of the above.
Discussion
This is not a straightforward diagnosis, even though it might appear that way. More than likely, this patient has idiopathic central serous chorioretinopathy, however it is possible that this is a result her lupus manifesting in the form of lupus choroidopathy.
Systemic lupus erythematosus (SLE) is a multisystem disease of unknown origin. It’s characterized by autoantibody formation and immune complex disease. It is generally considered to be the prototypic autoimmune disease. SLE may affect almost any organ system in the body. Cutaneous disease occurs in approximately 85% of patients and often manifests in a characteristic butterfly rash across the nose and cheeks.1 Other cutaneous lesions include discoid lupus erythematosus, cutaneous ulcers or splinter hemorrhages.
Ocular manifestations of SLE include secondary Sjögren’s syndrome, neuro-ophthalmic lesions and, most commonly, retinal vascular disease. The latter manifest as cotton-wool spots with or without retinal hemorrhages. They appear in approximately 30% of patients with lupus, and are likely part of the microangiopathy that occurs with the disease.1
In more severe cases of retinal vascular disease, patients can develop artery and vein occlusions, both branch and central.
Fortunately, our patient had none of these findings. So what does all this have to do with our patient?
It is the pathologic nature of SLE to be a small vessel vaso-occlusive disease. Just as it affects the retina, so can it also affect the choroid. When this occlusive process develops in the choroid, it is usually localized. As a result, patients can develop serous elevations of the retina, most often affecting the neurosensory retina. In addition, patients can develop serous elevations of the pigment epithelium and combined elevations. When this occurs, it can appear identical to the serous elevations in ICSC.
The neurosensory detachments in ICSC have been well described. ICSC typically occurs in young and middle-aged males with type A personalities. The age range is generally 20-45 with a 10:1 incidence in males vs. females.2 Often, unusual emotional stress accompanies the onset of the visual symptoms. Patients may have a history of migraine.
A round or oval area of shallow elevation of the neurosensory retina in the macular region is the typical finding. This is often preceded by a smaller pigment epithelial detachment (PED) that may or may not be clinically visible. Sometimes, a PED will be present without a neurosensory detachment. The subretinal fluid within the neurosensory detachment is usually clear, although about 10% of patients may develop more of a fibrin response characterized as serofibrinous exudate.2 This accounts for the yellow-white discoloration in our patient.
Fluorescein angiography in patients with ICSC can show a variety of patterns. The typical pattern is a focal spot of hyperfluorescence early in the angiogram that represents the area where the RPE is detached. This is evident in this patient. We also see the fibrin within the subretinal space beginning to stain. The classic smokestack appearance of the dye diffusing out occurs in only about 10% of patients.2 Sometimes, you may see multiple areas of hyperfluorescence, especially among patients who have had recurrent bouts of ICSC.
One of the most interesting aspects of ICSC is the histopathology. Though it is not completely understood, ICSC may result from a focal increase in permeability of the choriocapillaris, causing damage to the overlying RPE. This increase in permeability of the choriocapillaris most likely results from a focal injury, though the cause of the injury is unknown. Similarly, in lupus choroidopathy there is also a focal insult to the choriocapillaris. However, this likely occurs from occlusion in the choroidal vasculature, resulting in damage to the overlying RPE. Uncontrolled systemic hypertension may also play a role in this.
So how can you determine whether the serous detachment is due to ICSC or from the lupus? Often, with lupus there are multiple leakage sites including complex combinations of RPE and neurosensory retinal detachments. By comparison, 90% of ICSC patients will have two or fewer leakage sites at the time of presentation.3
More interesting still is how to treat it. Most cases of ICSC will resolve without treatment in 6 months. In cases that don’t resolve spontaneously, laser photocoagulation at the site of the RPE detachment may be indicated. Dr. Don Gass has shown that systemic corticosteroid therapy may aggravate or even precipitate ICSC in some patients.2 Interestingly, many lupus patients take corticosteroids as a necessary means to treat the disease. Those patients who have serous detachments often improve once the their lupus is under control, and in many cases corticosteroids bring that about. Some, however, get worse. Do the steroids make it worse, or do the steroids control the disease and, once this occurs, the condition improves? The true relationship of serum cortisol levels in patients with lupus and ICSC is not completely understood.
We elected to observe our patient and made her general physician aware of the complications that systemic cortisone can have on this condition. The ultimate decision on the best systemic management of her lupus was left up to her general physician. Hopefully, Plaquenil will have a positive affect. Only time will tell the true relationship between her lupus and her neurosensory detachment. u
- Jabs DL. Rheumatic disease. In: Ryan SJ. Schachat AP, Murphy RP, ed. Retina, 2nd edition vol. II, Medical Retina. St. Louis: Mosby, 1994:1421-1429.
- Gass JDM. Stereoscopic Atlas of Macular Disease: Diagnosis and Treatment. 4th edition. St Louis: CV Mosby, 1997: 52-70.
- Jabs DL, Hanneken AM, Schachat AP, et al. Choroidopathy in systemic lupus erythematosus. Arch Ophhthalmol 1988;106:230-234.
October 15, 2000