History of Present Illness
A 36-year-old female presented with complaints of blurred vision in both eyes (OU). In 1990, at the age of 15, she had developed butterfly-shaped skin lesions on the face; treatment was unspecific. In 1993, swelling and pain of the knee and small joints of both hands occurred. She was now found positive for antinuclear antibodies (ANA) leading to the diagnosis of systemic lupus erythematosus (SLE). She was treated with prednisone 10 to 20 mg per day for years.
In 1998 she had developed proteinuria leading to the diagnosis of lupus nephritis. She had developed arterial hypertension and moderate anemia (both were controlled with medications). Additional immunosuppressive treatment was initiated with Resochin, azathioprine, and pulsed cyclophosphamide, but the patient either did not tolerate the medication or it was inefficient. Finally, mycophenolate mofetil was introduced and continued for the next 4 years. This improved the patient’s condition.
In 2005, 1 year after she stopped mycophenolate in remission, she was found to have large areas of retinal ischemia, occlusive retinal vasculitis, and retinal neovascularization elsewhere (NVE) in the temporal retina ( Figs. 52.1 and 52.2 ). Laser photocoagulation of the ischemic areas was performed. In 2011 she needed peritoneal dialysis due to terminal renal insufficiency. Systemic treatment included prednisone 20 mg daily, which caused moderate osteoporosis and cataracts in both eyes. She had one episode of peritonitis in 2012 (due to Klebsiella ).
At the first examination in our clinic, she complained about deterioration of vision. Optical coherence tomography (OCT) revealed macular edema OU and a chorioretinal scar in the left macula. The patient received sub-Tenon triamcinolone injections bilaterally, and macular edema resolved. Due to retinal ischemia, laser photocoagulation was performed.
| OD | OS | |
|---|---|---|
| Visual acuity | 20/25 | 20/250 |
| Intraocular pressure (IOP) | 14 | 14 |
| Sclera/conjunctiva | Clear, no hyperemia | Clear, no hyperemia |
| Cornea | Clear | Clear |
| Anterior chamber (AC) | Deep, no cells, no flare | Deep, no cells, no flare |
| Iris | Unremarkable | Unremarkable |
| Lens | Posterior subcapsular cataract | Posterior subcapsular cataract |
| Anterior vitreous | Clear | Clear |
| Fundus | Occlusive retinal vasculitis in superotemporal retina with old inactive NVE and chorioretinal scars after laser photocoagulation. Macular edema and large pigmented chorioretinal scar below the macula | Occlusive retinal vasculitis in superotemporal retina and chorioretinal scars after laser photocoagulation. Macular edema |
Questions to Ask
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Have you ever had any problems with sugar levels, excessive thirst, urination, night sweats, or loss of body mass?
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Have you ever had any numbness, tingling, weakness on one side of the body, or bowel or bladder problems? Is there any history of neurologic disorders in the family?
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Have you had any cough, lung problems, or episodes of fever?
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Have you noticed any recent ticks or tick bites on your body?
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Have you ever had a cat scratch?
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Do you have a travel history?
She answers no to all of the questions.
Assessment
Occlusive retinal vasculitis (arteritis) OU, macular edema
Differential Diagnosis
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SLE vasculitis
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Sarcoid vasculitis
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Multiple sclerosis (MS)–associated retinal vasculitis
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Less likely: Lyme-associated, cat scratch disease–associated, or tuberculous retinal vasculitis
Working Diagnosis
Occlusive retinal vasculitis (arteritis), OU
Testing
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In patients with a medical history of lupus-related renal insufficiency, the workup includes urinalysis with microscopy (proteinuria, hematuria), basic biochemistry with ESR, C-reactive protein (CRP), serum creatinine, ANA, anti–double-stranded deoxyribonucleic acid (DNA) antibodies, anti-Ro/SSA antibodies, anti-SSB (La) antibodies, anticardiolipin antibodies, anti-B2 glycoprotein antibodies, lupus anticoagulant, complement levels, circulating immune complexes, neurologic investigation for neurolupus (nuclear magnetic resonance [NMR] of the brain, if neurologic review of systems is positive).
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For atypical cases, check:
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Angiotensin-converting enzyme (ACE), lysozyme, soluble IL-2 receptors
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QuantiFERON gold test or purified protein derivative (PPD), antineutrophil cytoplasmic antibodies (c-ANCA)
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Fluorescent treponemal antibody absorption (FTA-ABS), rapid plasma reagin (RPR)
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Lyme antibodies with Western blot
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Bartonella antibodies
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Neurologic investigation for MS or vasculitis of the brain
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Management
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Systemic prednisone 1 mg/kg for 1 week then slowly taper by 10 mg every week to 20 mg, then further reduction by 2.5 mg every week to 10 mg. Then very slow taper depending on the activity of uveitis.
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In severe cases, immediate addition of an immunosuppressive drug is necessary.
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Local treatment: topical nonsteroidal antiinflammatory drug (NSAID) three to four times per day and sub-Tenon or intravitreal steroid injections for macular edema.
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Intravitreal anti–vascular endothelial growth factor (VEGF) for choroidal neovascularization (CNV). Anti-VEGF may be used as additional therapy for severe cases of retinal neovascularization (NVE or NVD—neovascularization of the optic disc) in occlusive retinal vasculitis.
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Follow up in 2 weeks.
Follow-up
Since 2013, the patient was negative for ANA, anti–double-stranded DNA (anti-dsDNA) antibodies, anti-Ro/SSA antibodies, anti-myeloperoxidase (anti-MPO) antibodies, anticardiolipin antibodies, and anti-B2 glycoprotein antibodies and had a normal range of C3 and C4 complement components. Circulating immune complexes were detected occasionally (maximum level in 2014 was 0.600). Since 2016, the patient was planned for renal transplantation but could not perform it due to physical distress and frequent systemic infections (including skin abscesses). Arterial hypertension and anemia responded well to medical treatment. In 2018, the patient had developed congestive cardiomyopathy with pleural effusion, which has been punctured (punctate was sterile and QuantiFERON Gold Test was negative). She also developed an abscess of the skin in the gluteal region and was treated with antibiotics. Under this treatment, the patient was stable.
Regarding the ophthalmologic findings, in 2013 the patient had exudative retinal detachment ( Fig. 52.3 ) in the right eye (OD), which resolved after an increased dose of steroids (prednisone 1 mg/kg). In 2016, a decrease in visual acuity (VA) occurred (OD 20/30 and left eye [OS] 20/250) and active CNV with cystoid macular edema (CME) was documented in both eyes ( Fig. 52.4 ). The patient received sub-Tenon triamcinolone injections OU, after which reduction of intraretinal fluid occurred but was not complete in the OS. Therefore an intravitreal anti-VEGF agent was given in the OS and fluid resorbed. Also, mycophenolate mofetil was reintroduced along with prednisone 10 mg. After 3 months, VA in the OD was 20/25 and 20/200 in the OS. In 2019, progression of cataract in both eyes was detectable (OD 20/30, OS 20/250). The final fundus photographs ( Fig. 52.5 ), fluorescein angiography (FA) ( Fig. 52.6 ), OCT, and OCT angiography (OCTA) ( Fig. 52.7 ) are shown.
