|Year : 2022 | Volume
| Issue : 3 | Page : 131-134
Ocular Disorders Complicating Systemic Lupus Erythematosus in a Nigerian
Chizoba U Uba-Obiano1, Sebastian N.N Nwosu1, Emeka A Chianakwalam2, Henry Mmadu Nwankwo3
1 Department of Ophthalmology, Nnamdi Azikiwe University, Awka; Guinness Eye Centre, Onitsha, Nigeria
2 Guinness Eye Centre, Onitsha, Nigeria
3 Department of Medicine, Nnamdi Azikiwe University, Awka, Nigeria
|Date of Submission||21-Apr-2021|
|Date of Decision||23-Feb-2022|
|Date of Acceptance||08-Mar-2022|
|Date of Web Publication||26-Dec-2022|
Dr. Chizoba U Uba-Obiano
Department of Ophthalmology, Nnamdi Azikiwe University, Awka, 420007
Source of Support: None, Conflict of Interest: None
Systemic lupus erythematosus (SLE) is a connective tissue disorder involving a myriad of organs and structures in the body. We present a 45-year-old woman diagnosed with SLE at the rheumatology clinic and complained of gradual decrease in vision in the course of treatment. Analysis of case history, clinical features, and results of investigation was carried out. Ocular features reported were keratoconjunctivitis, mild visual impairment, nystagmus, and optic neuropathy. This is to report a case of ocular involvement in a Nigerian patient with SLE. SLE may be complicated by ocular disorders leading to visual impairment. Early ophthalmic assessment is therefore recommended for patients with SLE.
Keywords: keratoconjunctivitis sicca, nigeria, ocular features, optic neuropathy, systemic lupus erythematosus
|How to cite this article:|
Uba-Obiano CU, Nwosu SN, Chianakwalam EA, Nwankwo HM. Ocular Disorders Complicating Systemic Lupus Erythematosus in a Nigerian. Niger J Ophthalmol 2022;30:131-4
| Introduction|| |
Systemic lupus erythematosus (SLE) is a prototype connective tissue disorder characterized by recurrent episodes of inflammation and progressive damage to the joints, tendons, and vital organs, such as the heart, lungs, brain, kidneys, eyes, and skin.,
The revised 1997 American College of Rheumatology criterion is intended to assist physicians in making a diagnosis of SLE with a high sensitivity and specificity., The criteria range from highly pathognomic clinical signs and symptoms to derangement in the function of multiple organ systems., However, the 2012 Systemic Lupus International Collaborating Clinics (SLICC) diagnostic criterion has gained wide acceptance for being able to identify certain subsets of patient especially when some of the cardinal antibodies are negative, in children and lupus nephritis with limited systemic manifestations.
The etiology of SLE is multifactorial with significant contribution from genetic, hormonal, and environmental factors.,, Epidemiologic studies have shown marked gender, age, racial, temporal, and regional variations.,
There is worldwide variation in the reported incidence and prevalence of SLE in all nationalities. The lowest incidences of SLE were reported in Africa and Ukraine (0.3/100,000), whereas the highest incidence was reported in North America (23.2/100,000 persons)., Studies of racial tendencies showed that SLE more frequently affected non-Caucasian individuals. SLE accounted for 5.28% of all the 1250 cases reported over the study period of 6 years in a rheumatology clinic in Nigeria, whereas 33 cases were diagnosed in 14 years in Cotonou.
There is higher disease prevalence in women compared to men for every age and ethnic group with a female to male ratio that ranges from 2:1 to 15:1.,,, Although not conclusive, these observations suggest that hormonal factors may play important role in SLE pathogenesis. The age distribution of SLE cases is usually broad, ranging from children as young as 2 years old to adults 80 years of age, with women of child bearing age being at most risk.,,
It is estimated that up to 30% of patients with SLE may have ocular complications. SLE affects most ocular structures such as the periorbita, ocular adnexea, cornea, uvea, sclera, optic nerve, and the retina.,, The most common ophthalmic manifestation of lupus is retinal vascular lesions manifesting as inflammation or thrombosis especially in SLE with antiphospholipid syndrome, followed by keratoconjunctivitis sicca. However, the most vision-threatening sequelae are due to optic nerve involvement and retinal vaso-occlusion.
This is a case report of a patient who presented at the rheumatology clinic of a tertiary institution with ocular features in the course of management of the disease. This presentation highlights the need for closer collaboration between rheumatologists and ophthalmologists in the management of patients with SLE to obviate visual loss in these patients.
| Case Report|| |
A 45-year-old nulliparous woman presented on April 2019 at the rheumatology clinic of Nnamdi Azikiwe University Teaching Hospital, Nnewi, with fatigue, malaise, recurrent fever, alternating pain, and numbness of the fingers and toes of 8 months duration. Examination findings revealed polyarthritis, alopecia, oral ulcers, subacute cutaneous lupus rash, and photosensitivity. Laboratory investigations showed hematologic abnormalities (Coomb negative anemia, lymphopenia, and neutropenia), as well as lupus nephritis (proteinuria, red blood cell, and granular cast). Inflammatory markers were found to be elevated; erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) were 120 mm/hour and 28 mg/L, respectively. Serologies revealed antinuclear antibodies (ANA) positivity with titer of 1:640 and a dense fine speckled pattern. Serum urea and creatinine were normal at 2.4 and 87.6 mmol/L, respectively, whereas complements C3/C4 were low. The double-stranded deoxyribonucleic acid antibody was negative, as were extractable nuclear antigens: anti-Smith, anti-Ro, anti–La, and ribonucleoprotein. The results of antiphospholipid antibody screening with lupus anticoagulant, anticardiolipin antibody, and B2 glycoprotein 1 were all negative. Pericardial effusion was revealed on 2D echocardiography. A diagnosis of SLE was made based on the 2012 SLICC diagnostic criteria (cutaneous lupus/alopecia, oral ulcers, inflammatory polyarthritis, serositis, abnormal urine sediment, and ANA positivity). She was commenced on oral mycofenolate mofetil (MMF), hydroxychloroquine, and prednisolone. Pericardial effusion was managed medically with oral colchicine 1 mg daily and frusemide 40 mg daily by the cardiologist. Fundoscopy was performed with ophthalmoscope before commencing hydroxychloroquine. Her clinical and laboratory parameters improved after commencement of treatment. Oral ulcers, skin rashes, and arthritis regressed. Repeat echocardiography revealed no pericardial effusion. ESR was 30 mm/hour, CRP was 7 mg/L, and complements C3/C4 levels were normal after treatment.
She was regularly followed up at the rheumatology clinic. A year later, she complained of blurring of vision and seeing haloes of 1-week duration; decrease in vision was sudden in onset, painless, and nonprogressive. There were no other associated ocular symptoms. She had no history of headaches, pain on ocular movement, nor double vision. The best corrected visual acuity of the right eye was 6/18 and that of the left eye was 6/12. Ocular examination revealed periorbital edema, with darkening of the upper lids, and jerk nystagmus with the null point at the left lateral position on both eyes. Extraocular muscle movements were full and normal. The anterior chamber did not reveal any abnormalities, and pupils were round and reacted sluggishly to light. There was no obvious relative afferent pupillary defect.
Binocular indirect ophthalmoscopy showed hyperemic disks with blurred disk margins in each eye. There was absence of physiologic cup in the right eye, whereas the cup–disk ratio of the left eye was 0.1 [Figure 1] and [Figure 2], the fundi photographs].
|Figure 1 Binocular indirect ophthalmoscopy showed hyperemic disks with blurred disk margins in right eye.|
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|Figure 2 Binocular indirect ophthalmoscopy showed hyperemic disks with blurred disk margins in left eye.|
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The intraocular pressure carried out with Goldman applanation tononometer was 11 and 12 mmHg in the right and left eyes, respectively. Investigations carried out include Schirmer test which was 5 mm/5 minutes on the right eye and 3 mm/5 minutes on the left, whereas tear break-up time (TBUT) was 8 and 7 seconds on the right and left eyes, respectively. Visual field test showed mild generalized depression in each eye. Ishihara color vision test was normal.
A diagnosis of bilateral optic neuropathy, dry eye, and neurologic involvement was made in a patient with SLE. Intravenous methylprednisolone pulse therapy (1 g/day) was administered for 3 days and afterwards continued with oral prednisolone (1 mg/kg/day) and MMF she was previously on. Ocular lubricant (carboxymethylcellulose) was commenced. There was marked visual improvement (best corrected visual acuity of 6/9 in both the eyes) on subsequent ophthalmic evaluation which the patient was happy about.
| Discussion|| |
Our patient met the diagnostic criteria for SLE according to the 2012 SLICC diagnostic criteria following extensive search for implicated autoantibodies.
Ocular involvement may correlate with systemic disease activity, precede other systemic symptoms, or be a complication of treatment.,,
The patient presented with ocular disturbances after a year of commencement of treatment and routine follow-up by the rheumatologist. She was promptly referred to the eye clinic for ophthalmic review, and a diagnosis of optic neuropathy, keratoconjunctivitis sicca, and neurologic involvement was made.
Keratoconjuctivitis sicca is a common ocular manifestation with a prevalence of 25% in patients with SLE; the majority of these patients usually present with at least one dry-eye symptom., The hallmark of this ocular manifestation of the disease is decreased production of the aqueous layer of the tear film. Our patient had asymptomatic dry-eye disease which was confirmed by the reduced TBUT and Schirmer test.
Periorbital edema is an uncommon manifestation of SLE with an overall incidence of 4.8%. It is mostly reported in patients of African descent and may mimic chronic blepharitis., However, periorbital edema could also be due to renal involvement in SLE or from local immune-mediated inflammatory response. Our patient’s serum electrolyte, urea, and creatinine levels were normal, indicating that the periorbital edema was not of renal origin but rather due to local inflammatory response.
Neuro-ophthalmic features include optic neuritis, optic neuropathy, cranial nerve abnormalities, nystagmus, and visual field defects., Optic nerve disease could be a manifestation of SLE and consists of optic neuritis and ischemic optic neuropathy. However, it is the most common neurologic involvement (with bilateral involvement being commoner than unilateral) and occurs in about 1% of all patients with SLE.,, Another neurologic feature observed in our patient was nystagmus and this could be due to vasculitic lesions in the brainstem. Magnetic resonance imaging which would have defined and localized the neurologic features was not carried out due to financial constraint on the part of the patient and this posed as a limitation.
Visual acuity is usually poor with most patients seeing worse than 6/60,, but our patient still had relatively good vision in both the eyes at the time of presentation and treatment further improved vision to 6/9 on both eyes and other ocular parameters as noted by reduction of oscillatory movement (nystagmus). Thus, early presentation and treatment are keys for optimal visual outcome.
There was marked visual recovery with intravenous methyl prednisolone which is in keeping with documented evidence that optic nerve disease of vasculitic origin associated with SLE generally responds well to corticosteroids if therapy is instituted early in the course of the disease. This may suggest the etiology of optic neuropathy in our patient may be of vasculitic origin.,,,
In conclusion, we presented a patient with SLE who developed ophthalmic symptoms and signs in the course of treatment. As it was not clear whether the ocular ailments were complications of the treatment or a late manifestation of disease, it is gratifying that they resolved and vision improved following prompt treatment. Therefore, it is recommended that patients diagnosed with SLE should be sent to the ophthalmologist for a detailed ophthalmic evaluation by the managing physician before commencement of therapy and during the course of treatment. Prompt referral to the ophthalmologist is also important once these patients have any ocular complaints.
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Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2]