Journal List > J Korean Ophthalmol Soc > v.50(8) > 1008337

Chang, Jung, and Hyeong: Retinopathy Associated With Systemic Lupus Erythematosus

Abstract

Purpose:

To investigate the clinical characteristics of retinopathy associated with systemic lupus erythematosus (SLE) and its risk factors.

Methods:

Medical records of patients who were diagnosed with SLE were reviewed retrospectively. The presence of retinal hemorrhage, vasculitis and a cotton wool patch were regarded as lupus retinopathy, but concomitant diabetic retinopathy and hypertensive retinopathy were excluded from the study. The correlation between the development of lupus retinopathy and the presence of positive autoantibodies was also investigated.

Results:

Ocular morbidity was found in 173 of 260 (66%) SLE patients. Retinopathy was detected in 52 eyes of 33 patients (12%), which included 36 eyes of 21 patients (63%) with classic retinopathy and 11 eyes of 10 patients (30%) with vaso-occlusive retinopathy. The presence of classic retinopathy coincided with the flare-up of lupus activity and completely resolved without visual impairment. However, vaso-occlusive retinopathy was not related with lupus activity, and resulted in significant visual impairments of 20/200 or less in six eyes of five patients. The disease activity of lupus assessed by the maximum SLE disease activity index was higher in patients with retinopathy (p<0.05), and the prevalence of antiphospholipid antibody was higher in patients with vaso-occlusive retinopathy than in patients with classic retinopathy (66.7% vs. 37.5%, p<0.05).

Conclusions:

Vaso-occlusive retinopathy in SLE can result in permanent visual impairment. Patients with high SLE activity or positive anti-phospholipid antibodies have a high possibility of developing SLE retinopathy and should be referred for ophthalmologic examination.

References

1. Wallace DJ, Hahn BH. Dubois' Lupus Erythematosus. 7th ed.Philadelphia: Lippincott Williams & Wilkins;2007. p. 65–86.
2. Kim IT, Chang SD. Papilledema and cerebral venous thrombosis in a patient with systemic lupus erythematosus. J Korean Ophthalmol Soc. 1999; 40:2015–9.
3. Hwang HS, Kim DH. Transient myopia with severe chemosis associated with systemic lupus erythematosus. J Korean Ophthalmol Soc. 2007; 48:1445–8.
crossref
4. Im CY, Kim SS, Kim HK. Bilateral optic neuritis as first manifestation of systemic lupus erythematosus. Korean J Ophthalmol. 2002; 16:52–8.
crossref
5. Shin SY, Lee JM. A case of multiple cranial nerve palsies as the initial ophthalmic presentation of antiphospholipid syndrome. Korean J Ophthalmol. 2006; 20:76–8.
crossref
6. Oh PC, Kim GH, Jin CH, Baek HJ. A case of systemic lupus erythematous associated with neuromyelitis optica (Devic's Syndrome). J Korean Rheum Assoc. 2007; 14:263–7.
crossref
7. Yun KA, Kim JG. A case of orbital myositis secondary to systemic lupus erythematosus. J Korean Rheum Assoc. 2006; 13:171–6.
8. Kim IT, Na SC, Lee KJ. Vascular occlusion associated with antiphospholipid antibodies in systemic lupus erythematosus. J Korean Ophthalmol Soc. 2000; 41:427–32.
9. Park DH, Koo HM, Chung SK. A case of optic neuritis and central retinal vein occlusion associated with systemic lupus erythematosus. J Korean Ophthalmol Soc. 1994; 35:116–21.
10. Jung NH, Kim SY. A case of severe retinal vaso-occlusive disease in systemic lupus erythematosus. J KoreanOphthalmol Soc. 1993; 34:1287–92.
11. Yawm SC, Uhm KB, Choe JK. A case of severe vaso-occlusive disease in systemic lupus erythematosus. J Korean Ophthalmol Soc. 1986; 27:681–6.
12. Jabs DA, Fine SL, Hochberg MC, et al. Severe retinal vaso− occlusive disease in systemic lupus erythematous. Arch Ophthalmol. 1986; 104:558–63.
13. Lanham JG, Barrie T, Kohner EM, Hughes GR. SLE retinopathy: evaluation by fluorescein angiography. Ann Rheum Dis. 1982; 41:473–8.
crossref
14. Stafford-Brady FJ, Urowitz MB, Gladman DD, Easterbrook M. Lupus retinopathy. Patterns, associations, and prognosis. Arthritis Rheum. 1988; 31:1105–10.
15. Bombardier C, Gladman DD, Urowitz MB, et al. Derivation of the SLEDAI. A disease activity index for lupus patients. The Committee on Prognosis Studies in SLE. Arthritis Rheum. 1992; 35:630–40.
16. Gold DH, Morris DA, Henkind P. Ocular findings in systemic lupus erythematosus. Br J Ophthalmol. 1972; 56:800–4.
crossref
17. Au A, O'Day J. Review of severe vaso-occlusive retinopathy in systemic lupus erythematosus and the antiphospholipid syndrome: associations, visual outcomes, complications and treatment. Clin Experiment Ophthalmol. 2004; 32:87–100.
crossref
18. Giorgi D, Pace F, Giorgi A, et al. Retinopathy in systemic lupus erythematosus: pathogenesis and approach to therapy. Hum Immunol. 1999; 60:688–96.
crossref
19. Klinkhoff AV, Beattie CW, Chalmers A. Retinopathy in systemic lupus erythematosus: relationship to disease activity. Arthritis Rheum. 1986; 29:1152–6.
crossref
20. Aronson AJ, Ordonez NG, Diddie KR, Ernest JT. Immune− complex deposition in the eye in systemic lupus erythematosus. Arch Intern Med. 1979; 139:1312–3.
21. Graham EM, Spalton DJ, Barnard RO, et al. Cerebral and retinal vascular changes in systemic lupus erythematosus. Ophthalmology. 1985; 92:444–8.
crossref
22. Ushiyama O, Ushiyama K, Koarada S, et al. Retinal disease in patients with systemic lupus erythematosus. Ann Rheum Dis. 2000; 59:705–8.
crossref
23. Kleiner RC, Najarian LV, Schatten S, et al. Vaso-occlusive retinopathy associated with antiphospholipid antibodies (lupus anticoagulant retinopathy). Ophthalmology. 1989; 96:896–904.
crossref
24. Montehermoso A, Cervera R, Font J, et al. Association of anti-phospholipid antibodies with retinal vascular disease in systemic lupus erythematosus. Semin Arthritis Rheum. 1999; 28:326–32.
25. Alarcon-Segovia D, Deleze M, Oria CV, et al. Antiphospholipid antibodies and the antiphospholipid syndrome in systemic lupus erythematosus. A prospective analysis of 500 consecutive patients. Medicine (Baltimore). 1989; 68:353–65.
26. Levine JS, Branch DW, Rauch J. The antiphospholipid syndrome. N Engl J Med. 2002; 346:752–63.
crossref
27. Soo MP, Chow SK, Tan CT, et al. The spectrum of ocular involvement in patients with systemic lupus erythematosus without ocular symptoms. Lupus. 2000; 9:511–4.
28. Ho TY, Chung YM, Lee AF, Tsai CY. Severe vaso-occlusive retinopathy as the primary manifestation in a patient with systemic lupus erythematosus. J Chin Med Assoc. 2008; 71:377–80.
crossref

Figure 1.
Comparison of peak SLEDAI among patients with retinopathy, without retinopathy, and each subtype of retinopathy. ttest; †Mann Whitney-U test; ‡ Patients without retinopathy.
jkos-50-1215f1.tif
Table 1.
Ocular manifestations found in SLE
Ocular manifestations No of patients (%)
Keratoconjunctivitis sicca 80 (30.8)
Cataract 60 (23.1)
Retinopathy 33 (12.6)
Choroidopathy 8 (3.1)
Optic neuropathy 5 (1.9)
Uveitis 4 (1.5)
Others 17 (6.5)
No abnormality 87 (33.5)
Table 2.
Difference of demographics between patients with retinopathy and without retinopathy
  No retinopathy (n=227) Retinopathy (n=33) p-value
Age at ocular exam 37.2±12.5 36.2±13.7 0.657
Age at SLE diagnosis 33.6±11.9 34.3±13.3 0.749
M:F 21 (9%): 206 (91%) 5 (15%): 28 (85%) 0.346

ttest

Chi square test.

Table 3.
Funduscopic findings in each subtype of lupus etinopathy
Retinopathy type Funduscopic finding No (%)
Classic retinopathy Hemorrhage 13 (61.9)
(N=21) Cotton wool spot 13 (61.9)
  Focal vasculitis 2 (9.5)
  Hard exudate 1 (4.7)
Vaso occlusive CRVO 2 (20)
retinopathy BRVO 5 (50)
(N=10) CRAO 1 (10)
  BRAO 1 (10)
  Diffuse obstruction 1 (10)
Others (N=2)   2  
Table 4.
Difference of systemic manifestations between patients with retinopathy and without retinopathy
Systemic manifestations No retinopathy N=148 (%) Retinopathy N=30 p-value
Rash 64 (43.2) 19 (63.3) 0.048#
Photosensitivity 26 (17.6) 4 (13.3) 0.790
Mucosal ulcer 35 (23.6) 6 (20.0) 0.814
Arthritis 62 (41.9) 13 (43.3) 1.000
Serositis 26 (17.6) 8 (26.7) 0.307
Renal involvement 63 (42.6) 20 (66.7) 0.026
Neurological involvement 7 (4.7) 2 (6.7) 0.649
Hematologic abnormality 103 (69.6) 17 (56.7) 0.201
Immunologic abnormality§ 120 (81.1) 26 (86.7) 0.606

Proteinuria > 500 mg/day, hematuria (RBC > 5/HPF), pyuria (WBC > 5/HPF) or cellular or granular casts

Seizure, organic brai syndrome, cranial nerve disorder, cerebral vascular accident, or psychosis

Hemolytic anemia, leukopenia (<4000/mm3) or lymphopeni (<1500/mm3)

§ LE cell(+), Sm antibody (+), decreased C3/C4 level or increased dsDNA

Fisher's exact test.

# p<0.05

Table 5.
Relationship between common autoantibodies found in SLE and lupus retinopathy
Autoantibodies No retinopathy N(%) All retinopathies N (%) Classic retinopathy N (%) occlusive retinopathy N (%) p p p
APL§ 53 (30.1) 13 (48.1) 6 (37.5) 6 (66.7) 0.078 0.576 0.031‡‡
LE cell 27 (44.3) 4 (40.0) 3 (42.9) 1 (50.0) 1.000 1.000 1.000
Ro / La 67 (63.2) 10 (58.8) 7 (63.6) 3 (60.0) 0.790 1.000 1.000
Sm 32 (27.8) 6 (33.3) 5 (45.5) 1 (20.0) 0.779 0.298 1.000
RNP∗∗ 30 (52.6) 5 (50.0) 5 (62.5) 0 (0) 1.000 0.716 0.237
RF†† 27 (19.3) 4 (25.0) 3 (21.4) 1 (100.0) 0.526 0.737 0.199

Fisher's exact test between “no retinopathy” and “all retinopathies”

Fisher's exact test between “no retinopathy” and “classic retinopathy”

Fisher's exact test between “no retinopathy” and “occlusive retinopathy”

§ Antiphospholipid antibody: Lupus anticoagulant or anticardiolipine antibody

Anti-Ro (SS-A) antibodies/anti-La (SS-B) antibodies

Anti-Sm antibodies

∗∗ Anti-snRNP antibodies

†† Rheumatoid factor

‡‡ p<0.05.

TOOLS
Similar articles