Table 3.
Disease progression in patients with retinitis pigmentosa carrying variants of the four major causative genes.
| Gene | Inheritance | No., n | Follow-up duration, years (range) | Change in BCVA | Shortening of EZ band, μm (range) | Change in EZ band | |||||||
|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
| Deterioration of BCVA, logMAR (range) | Age at exam, yearsa | Symptom duration, yearsb | Age at exam, yearsa | Symptom duration, yearsb | |||||||||
| Beta | P valuec | Beta | P valuec | Beta | P valuec | Beta | P valuec | ||||||
| EYS | AR | 15 | 2.0 (1.0–20.0) | 0.1 (−0.1−1.0) | 0.01 | 0.0003 | 0.02 | <0.0001 | −555.0 (−1615.0−158.0) | −62.28 | 0.0002 | −80.09 | <0.0001 |
| PDE6B | AR | 10 | 1.5 (1.0–4.0) | 0.0 (−0.2−0.2) | 0.02 | 0.0342 | 0.04 | <0.0001 | −247.0 (−1239.0−135.0) | −54.26 | 0.0105 | −58.60 | 0.0299 |
| RP1 | AD/AR∗ | 4/5∗ | 2.0 (2.0–8.0) | 0.1 (0.0–0.2) | 0.00 | 0.0851 | 0.01 | 0.0590 | −35.0 (−1243.0—1.0) | −140.22 | <0.0001 | −141.80 | <0.0001 |
| USH2A | AR | 12 | 2.0 (1.0–9.0) | 0.0 (−0.1 − 0.5) | 0.01 | 0.0002 | 0.02 | 0.0039 | −676.2 (−1676.0 − 0.0) | −170.37 | <0.0001 | −182.76 | <0.0001 |
| Four major causative genes | 41/42∗ | 2.0 (1.0–20.0) | 0.0 (−0.2 − 1.0) | −421.5 (−2295.0 − 158.0) | |||||||||
| Group-time interaction effectd | 0.0140 | 0.0006 | 0.0336 | 0.0050 | |||||||||
BCVA, best-corrected visual acuity; EZ, ellipsoid zone; SD, standard deviation; LogMAR, logarithm of the minimum angle of resolution; AR, autosomal recessive; AD, autosomal dominant. aLinear growth curve model: y = beta·t (model without intercept). bLinear growth curve model: y = a + beta·t. cP < 0.05 was considered significant. dP value for interaction between groups, P < 0.05 was considered significant. ∗Only patients (five out of nine) with peripheral type RP1-associated RP were analyzed.