Table 1. Patient demographics and characteristics.
The Early-Onset Disease (EOD) group consisted of a younger cohort of patients with increased frequency of oral MMP. Direct immunofluorescence (DIF) and Indirect immunofluorescence (IIF) refer to the proportion of patients who demonstrated the linear deposition of immunoglobulin G, A or complement (C3) along the basement membrane zone (BMZ) or had measurable titres of immunoglobulin in the serum respectively. Comparisons were undertaken with Fishers-exact test, Kendall’s Tau b for rank correlations and continuous variables were analysed by nonparametric tests (Mann-Whitney U test).
| All patients | Early-onset disease |
Established disease |
p value |
|
|---|---|---|---|---|
| Total no. of patients | 50 | 26 | 24 | - |
| Total no. of eyes | 99 | 51 | 48 | - |
| Male: Female (% Female) | 23:27 (54) | 11:15 (58) | 12:12 (50) | - |
| Median age (yrs) [Range] | 67 (32-91) | 62 (32-82) | 69 (39-91) | P=0.02 |
|
Median duration of
symptoms (yrs) [Range] |
3 (0-41) | 1.5 (0-3) | 14 (5-41) | P<0.001 |
|
Patient follow-up (eyes)
12 months 24 months |
43 (85) 35 (69) |
23 (45) 19 (37) |
20 (40) 16 (32) |
- - |
|
No of patients discharged back to referring hospital ¶ |
||||
| Total number discharged | 14 | 7 | 7 | - |
| 12 months follow-up | 4 | 1 | 3 | - |
| 24 months follow-up | 10 | 6 | 4 | - |
|
Biopsy
* DIF +ve IIF +ve † |
87.2% (34/39) 34.8% (8/23) |
92% (23/25) 42.9 % (6/14) |
78.6% (11/14) 2.2% (2/9) |
P=0.33 P=0.4 |
|
Extraocular mucocutaneous
involvement All mucocutaneous tissues Skin Oral |
52% (26/50) 18% (9/50) 40% (20/50) |
62% (16/26) 7.7% (2/26) 57.7% (15/26) |
42% (10/24) 29.2% (7/24) 20.8% (5/24) |
P=0.26 - P=0.01 |
| Visual Acuity ‡ | ||||
| Normal: 6/6 −>6/18 | 80.6% (29/36) | 95.2% (20/21) | 60% (9/15) | P<0.01 |
| Visual impairment: <6/18- 6/60 | 8.3% (3/36) | 4.8% (1/21) | 13.3% (2/15) | |
| Severe visual impairment: 6/60-3/60 | 0% (0/36) | 0% (0/21) | 0% (0/15) | |
| Blind: <3/60 | 11.1% (4/36) | 0% (0/21) | 26.7% (4/15) | |
| ‡ Excluded due to other causes | 28% (14/50) | 19% (5/26) | 38% (9/24) |
Follow-up: one patient from the Established Disease (EstD) group died before 12 months FU, and one from the EstD group failed to attend between 12 and 24 months and was referred back to their local hospital for continuing care.
Ten patients in total did not undergo a conjunctival biopsy (7 patients with advanced age (> 80 years) and immunosuppression was systemically contraindicated and the remaining 3 patients had end stage disease (defined as Mondino/Foster stage 4)). Data were missing for one individual and this patient was excluded from analysis.
All patients who were IIF were also DIF positive. There were no patients who were IIF positive in the absence of positive DIF studies.
VA represents a comparison of visual acuity in the better-seeing eye, after exclusion of other causes of reduced vision such as cataract, glaucoma, age-related macular degeneration and diabetic retinopathy (n=14, EOD 5; EstD 9).