Two ultraviolet radiation-induced episodes of optic neuritis in a patient with antinuclear antibody

G. Neumann-Andersen

Department of Rheumatology, University Hospital of Umeå, 901 85 Umeå, Sweden

SIR, Exposure to the ultraviolet (UV) radiation of sunlight may induce disease flares with extracutaneous manifestations in systemic lupus erythematosus (SLE) [1]. There is only one previous report on UV-induced neurological manifestations in SLE [2]. Here the case is presented of a 49-yr-old Caucasian female who suffered two episodes of UV-induced optic neuritis.

In April 1997, the patient presented with deteriorating vision in her right eye, proceeding to blindness within 2 weeks (Fig. 1Go). The ophthalmologist found lack of vision in the upper half of the vision field, ability to count fingers only at 20 cm distance in the lower half and lack of colour vision. The papill was swollen with blurred margins. After 1 week on 60 mg prednisolone, the patient regained almost normal vision. For half a year, however, the papill remained slightly swollen and the field of vision slightly concentrically restricted in the upper part. In May 1995, the patient had experienced decreased vision and lightning phenomenon in her left eye. Vision and fundoscopy were normal; perimetry was not performed. Only after half a year, the patient recovered spontaneously. Both incidents occurred 1 week after returning from a 2-week-long ski-ing holiday to Arjeplog, northern Sweden (66°N, 18°E, 7–800 m above sea level). Sunshine, which always made the patient feel dizzy with nausea and fatigue, was difficult to avoid, since she enjoyed taking holidays with family members. The patient's mother had suffered from juvenile diabetes mellitus since the age of 23 yr. The patient herself had been treated with levothyroxine for 2 yr because of a slight hypothyreosis.



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FIG. 1.  Serial data on two flares of optic neuritis in a 49-yr-old ANA-positive Caucasian female.

 
Antinuclear antibody (ANA) on HEp 2-cells was positive at 1:800 (<1:100), mixed homogeneous and speckled pattern; anti-SS-A/Ro, anti-SS-B/La, anti-Sm, anti-RNP, anti-native DNA and IgM RF (Waaler Rose) were all negative at the time of presentation with right optic neuritis. Magnetic resonance imaging of the brain and orbita after 5 days of treatment was normal. Haemoglobin was 135 g/l (119–153), white blood cell count 9.3x109 /l (3.5–9.0) and platelets 257x109 /l (150–350) before treatment. On examination at the University Hospital of Umeå in July 1997, there was no history or signs of SLE, Sjögren's syndrome or Raynaud's phenomenon. Cerebrospinal fluid (CSF) analysis showed no cells, slightly elevated protein 614 mg/l (150–450 mg/l), CSF albumin 419 mg/l (75–400 mg/l) and CSF IgG 69 mg/l (13–68 mg/l). The CSF IgG index (CSF-IgG/P-IgG/CSF-albumin/P-albumin) was normal, i.e. 0.58 (0.70). There was no oligoclonal banding on liquor-elphoresis. Plasma (P)-IgG was 10.2 g/l (6.9–15.7), with no monoclonal banding, P-albumin 37.5 g/l (39.6–51.1), C3 0.84 g/l (0.53–1.20), C4 0.25 g/l (0.21–0.49), C1q 139% (76–136) and C3d 5% (<6%). Antibody to cardiolipin was negative, APTT normal, and VDRL, lupus anticoagulans and Borrelia serology all negative. The patient had the HLA type A24(9), 26(10); B27,45 with DRB1*08; DQB1*04 homozygosity. A thorough neurological examination was normal. A psychological test showed slightly decreased visual attention and ability to concentrate. Because she developed nausea on chloroquine, the patient was started on azathioprine. On this regimen, avoiding sun exposure, the patient has experienced no further episodes of optic neuritis.

The highest ozone depletion rate of the hemisphere, accentuated by frequent sudden ozone losses in springtime, intensifies the UV-B radiation in the mountains of Northern Sweden. UV-B is further enhanced by height over sea level and reflection from snow. All gases in the atmosphere attenuate UV-B by scattering, corresponding to an 8% increase per kilometre above sea level. Dependent on quality, snow reflects UV-B, which may increase by 20–35% [3].

The UV radiation of sunlight is generally divided into UV-C (200–290 nm: far UV, germicidal UV), UV-B (290–320 nm: midrange UV, sunburn radiation) and UV-A (320–400 nm: near UV, black light). UV-C radiation is totally, and UV-B partially, absorbed by the stratospheric ozone layer. While UV-B does not penetrate window glass and only reaches the epidermis, UV-A penetrates average, but not tinted glass, and the epidermis [4, 5]. UV-B in small doses causes DNA damage, lymphocyte apoptosis [6], and induction of pro-inflammatory cytokines, adhesion molecules [4] and nitric oxide synthase [7] in keratinocytes. This may even have systemic effects. The UV-B-induced change of trans- to cis-urocanoic acid suppresses cell-mediated immunity. Moreover, UV-B decreases the ability of Langerhans cells to stimulate CD4+ Th1 cells and activates CD4+CD45RA+ suppressor-inducer T cells, probably changing the immunological scene in favour of B-cell activation [4, 8]. UV-A may provoke systemic flares directly by penetrating into the subcutis vasculature. On the other hand, longer wavelength UV light, i.e. UV-A1 (340–400 nm), may have flare-preventing effects in SLE [9].

The only characteristic of SLE in the present patient was a high-titre ANA. However, optic neuritis might be the debuting symptom in SLE [10]. The only report on UV-induced CNS lupus is that of a Swedish female SLE patient, suffering two attacks of myelitis after a vacation to the Balearics [2]. Optic neuritis is often combined with myelitis in SLE, a combination named Devic's opticomyelitis. The manifestations constituting Devic's syndrome might thus be more easily inducible by UV light than other CNS manifestations in SLE.

Generally, there are several ways to avoid UV radiation: use of sunscreens, tinted car windows [5], avoidance of cool, white, fluorescent light. Moreover, thanks to the Montreal protocol, it is hoped that the ozone depletion rate will decrease [3].

References

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  3.  Björn LO, Callaghan TV, Gehrke C, Johanson U, Sonesson M, Gwynn-Jones D. The problem of ozone depletion in Northern Europe. Ambio 1998;27:275–9.[ISI]
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  6.  Abe M, Ishikawa O, Miyachi Y, Kanai Y. In vitro spontaneous and UVB-induced lymphocyte apoptosis are not specific to SLE. Photodermatol Photoimmunol Photomed 1997;13:204–7.[ISI][Medline]
  7.  Kuhn A, Fehsel K, Krutman J, Lehmann P, Ruzicka T, Kolb-Bachofen V. Aberrant timing in epidermal expression of inducible nitric oxide synthase after UV irradiation in cutaneous lupus erythematosus. J Invest Dermatol 1998;111:149–53.[Abstract]
  8.  Norris DA. Pathomechanisms of photosensitive lupus erythematosus. J Invest Dermatol 1993;100:58S–68S.[Abstract]
  9.  Molina JF, McGrath H Jr. Longterm ultraviolet-A1 irradiation therapy in systemic lupus erythematosus. J Rheumatol 1997; 24:1072–4.[ISI][Medline]
  10.  Tola MR, Granieri E, Caniatti L, Paolino E, Monetti C, Dovigo L et al. Systemic lupus erythematosus presenting with neurological disorders. J Neurol 1992;239:61–4.[ISI][Medline]
Accepted 21 June 1999





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