1096
AMERICAN JOURNAL OF OPHTHALMOLOGY
trait inherited in an autosomal dominant mode; it shows varying expressivity. The role of goniodysgenesis in open-angle glau coma is frequently underestimated; it is therefore most refreshing to find this sub ject brought up again. However, the subject is better documented than suggested by the choices of references in the article by Drs. Kimura and Levene. TORD JERNDAL,
M.D.
Goteborg, Sweden REPLY
Editor: Both our studies and those of Dr. Jerndal are similar in that they concern congenital malformation of the angle in open-angle glaucoma. They differ in that Dr. Jerndal deals with glaucoma in patients under 40 years of age (juvenile and late congenital), whereas we deal with glaucoma in patients over 40 (primary open-angle). His popula tion consists of members of one family, whereas ours consists of individuals from different families. His angle malformation (goniodysgenesis) seems to be different in many respects from that noted by us. Dr. Jerndal's excellent study should have been included in our paper and we regret this oversight. RALPH Z. LEVENE,
Birmingham,
M.D.
Alabama
JUVENILE RHEUMATOID ARTHRITIS
Editor: I read the paper "Ocular manifestations of juvenile rheumatoid arthritis," by L. T. Chylack, Jr., D. C. Bienfang, A. R. Bellows, and J. S. Stillman (Am. J. Ophthalmol. 79:1026, 1975), with both interest and dis appointment. The interest was aroused by the large series of 36 children followed-up for a mean period of 6.3 years; in this con dition too few patients are often followed-up for too short a time. The disappointment followed however on finding that the authors had failed to make
DECEMBER, 1975
the essential distinction between acute and chronic iridocyclitis.1 Since the acute inflam mation, adequately treated, had a good visual prognosis, and the chronic inflamma tion, however intensively treated, has a bad one2'3 this failure affects the value of many of their findings. Since 1968, I have been treating children much in the way described in this article, and now have a series of 61 patients with chronic anterior uveitis, followed-up for a mean period of 11 years. My results are so much worse than the claim in this paper of "no serious visual acuity loss and other complications in better than 80%" that, while congratulating the authors, I search for an explanation. Is it that a high proportion of their patients had acute iridocyclitis, or per haps the benign type of chronic inflammation which I described in 1972 ?2 Or is it possible that six years is too short a period in which to make pronouncements about prognosis? Their statement that laboratory studies are not helpful in detecting iridocyclitis is contradicted by a paper read at the Ameri can Rheumatism Association Meeting in 1972,* which established the importance of finding antinuclear antibodies in seronegative children as a guide to the identification of those likely to develop this serious eye con dition. The authors' statement that the result of surgical treatment of band keratopathy is discouraging is also incorrect. The removal of a corneal band by chelation is easy, safe, and satisfactory.6 It is discouraging that chloroquine and hydroxychloroquine can still be given to any patient for over six years continuously—with the almost predictable result of pigmentary changes in the central retina and permanent damage to sight. Iatrogenic disease of this type is readily preventable.6 W. K. SMILEY,
M.D.
Tapelow, Maidenhead, Berks, England REFERENCES
1. Smiley, W. K.: Iridocyclitis in Still's disease. Trans. Ophthalmol. Soc, U.K. 85:351, 1965.
VOL. 80, NO. 6
1097
CORRESPONDENCE
2. : Ocular involvement in juvenile rheu matoid arthritis (Still's disease). Proc. Roy Soc. Med. 66:1163, 1973. 3. :The eye in juvenile rheumatoid arthri tis. Trans. Ophthalmol. Soc. U.K. 94:817, 1974. 4. Schaller, J. G., Johnson, G. D., Holborow, E. J., Ansell, B. M., and Smiley, W. K.: The as sociation of antinuclear antibodies with the chronic iridocyclitis of juvenile rheumatoid arthritis (Still's disease). Arthr. Rheum. 17:409, 1974. 5. Grant, W. M.: New treatment for calcific corneal opacities. Arch. Ophthalmol. 48:681, 1952.
6. Percival, S. P. B., and Meanock, I.: Chloroquine: ophthalmologicai safety and clinical assess ment in rheumatoid arthritis. Br. Med. J. 3:579, 1968.
Editor: In their article, "Ocular manifestations of juvenile rheumatoid arthritis," Chylack and associates noted that 4 1 % of children with uveitis related to juvenile rheumatoid ar thritis, "did not respond to more than six months of intensive topical treatment with corticosteroid drops." That is an extremely important observation and admission. Many ophthalmologists are determined to use oph thalmic topical corticosteroid preparations for all cases of uveitis regardless of whether or not they improve the inflammation. The prognosis of cataract surgery in chil dren with juvenile rheumatoid arthritis is poor. Any medication that promotes the formation of lens opacification and does not materially improve an intraocular inflamma tion should not be used. Many children with juvenile rheumatoid arthritis and smoldering chronic iridocylitis require only prolonged use of cycloplegics. There are three forms of juvenile rheuma toid arthritis: 1. The acute systemic type is character ized by an acute onset of rash, fever, leukocytosis, and hepatosplenomegaly. It occurs especially between ages 1 and 3 years and predominantly affects boys. Initially there may be no arthritis. Iridocyclitis is uncom mon. 2. The poly-arthritis type is usually acute in onset and is seen more frequently in girls. Classically, over five joints are inflammed. Uveitis may occur. 3. The pauciarticular or monoarticular
type is insidious in nature. From one to four joints may be involved. Characteristically, the arthritis is minor. Uveitis occurs in as high as 30% of children in this group. Schaller and associates2 noted antinuclear antibodies in 52 of 69 children with rheuma toid disease and iridocyclitis. Certainly all children with juvenile rheumatoid arthritis should have periodic eye examinations by an ophthalomologist; however, being aware of the presence or absence of antinuclear antibodies and also of the type of juvenile rheumatoid arthritis (monoarticular), will alert the physician to those cases that require especially careful observation. J. T. COYLE,
Bellevue,
M.D.
Washington
REFERENCES
1. Komreich, H., and Hanson, V.: Rheumatic disease. Current Problems in Pediatrics. 4:6, 1974.
2. Schaller, J., Johnson, G., Ansell, B., and Holborrow, E.: Anti-nuclear antibodies in patients with iridocyclitis and juvenile rheumatoid arthritis. Arthr. Rheum. 16:130, 1973. REPLY
Editor: While Mr. Smiley's experience in this field is well recognized, we feel that our series of patients offers some new and sig nificant perspectives on the problems of iridocyclitis in juvenile rheumatoid arthritis. Mr. Smiley has emphasized the significance of differentiating between the acute and chronic forms of iridocyclitis.1"3 As he states in his letter, the acute form "adequately treated, has a good visual prognosis, and the chronic inflammation, however intensively treated, has a bad one." While we grant that this classification may be an interesting retro spective maneuver when reviewing a large series of cases, we have not found it par ticularly useful in dealing with new patients and their parents. Some of the reasons for this are mentioned by Mr. Smiley himself: 9/15 of his cases of acute iridocyclitis had recurrences 1 and there were exacerbations in his series of chronic cases "so severe as to produce hypopon." Therefore, in dealing
AMERICAN JOURNAL OF OPHTHALMOLOGY
trait inherited in an autosomal dominant mode; it shows varying expressivity. The role of goniodysgenesis in open-angle glau coma is frequently underestimated; it is therefore most refreshing to find this sub ject brought up again. However, the subject is better documented than suggested by the choices of references in the article by Drs. Kimura and Levene. TORD JERNDAL,
M.D.
Goteborg, Sweden REPLY
Editor: Both our studies and those of Dr. Jerndal are similar in that they concern congenital malformation of the angle in open-angle glaucoma. They differ in that Dr. Jerndal deals with glaucoma in patients under 40 years of age (juvenile and late congenital), whereas we deal with glaucoma in patients over 40 (primary open-angle). His popula tion consists of members of one family, whereas ours consists of individuals from different families. His angle malformation (goniodysgenesis) seems to be different in many respects from that noted by us. Dr. Jerndal's excellent study should have been included in our paper and we regret this oversight. RALPH Z. LEVENE,
Birmingham,
M.D.
Alabama
JUVENILE RHEUMATOID ARTHRITIS
Editor: I read the paper "Ocular manifestations of juvenile rheumatoid arthritis," by L. T. Chylack, Jr., D. C. Bienfang, A. R. Bellows, and J. S. Stillman (Am. J. Ophthalmol. 79:1026, 1975), with both interest and dis appointment. The interest was aroused by the large series of 36 children followed-up for a mean period of 6.3 years; in this con dition too few patients are often followed-up for too short a time. The disappointment followed however on finding that the authors had failed to make
DECEMBER, 1975
the essential distinction between acute and chronic iridocyclitis.1 Since the acute inflam mation, adequately treated, had a good visual prognosis, and the chronic inflamma tion, however intensively treated, has a bad one2'3 this failure affects the value of many of their findings. Since 1968, I have been treating children much in the way described in this article, and now have a series of 61 patients with chronic anterior uveitis, followed-up for a mean period of 11 years. My results are so much worse than the claim in this paper of "no serious visual acuity loss and other complications in better than 80%" that, while congratulating the authors, I search for an explanation. Is it that a high proportion of their patients had acute iridocyclitis, or per haps the benign type of chronic inflammation which I described in 1972 ?2 Or is it possible that six years is too short a period in which to make pronouncements about prognosis? Their statement that laboratory studies are not helpful in detecting iridocyclitis is contradicted by a paper read at the Ameri can Rheumatism Association Meeting in 1972,* which established the importance of finding antinuclear antibodies in seronegative children as a guide to the identification of those likely to develop this serious eye con dition. The authors' statement that the result of surgical treatment of band keratopathy is discouraging is also incorrect. The removal of a corneal band by chelation is easy, safe, and satisfactory.6 It is discouraging that chloroquine and hydroxychloroquine can still be given to any patient for over six years continuously—with the almost predictable result of pigmentary changes in the central retina and permanent damage to sight. Iatrogenic disease of this type is readily preventable.6 W. K. SMILEY,
M.D.
Tapelow, Maidenhead, Berks, England REFERENCES
1. Smiley, W. K.: Iridocyclitis in Still's disease. Trans. Ophthalmol. Soc, U.K. 85:351, 1965.
VOL. 80, NO. 6
1097
CORRESPONDENCE
2. : Ocular involvement in juvenile rheu matoid arthritis (Still's disease). Proc. Roy Soc. Med. 66:1163, 1973. 3. :The eye in juvenile rheumatoid arthri tis. Trans. Ophthalmol. Soc. U.K. 94:817, 1974. 4. Schaller, J. G., Johnson, G. D., Holborow, E. J., Ansell, B. M., and Smiley, W. K.: The as sociation of antinuclear antibodies with the chronic iridocyclitis of juvenile rheumatoid arthritis (Still's disease). Arthr. Rheum. 17:409, 1974. 5. Grant, W. M.: New treatment for calcific corneal opacities. Arch. Ophthalmol. 48:681, 1952.
6. Percival, S. P. B., and Meanock, I.: Chloroquine: ophthalmologicai safety and clinical assess ment in rheumatoid arthritis. Br. Med. J. 3:579, 1968.
Editor: In their article, "Ocular manifestations of juvenile rheumatoid arthritis," Chylack and associates noted that 4 1 % of children with uveitis related to juvenile rheumatoid ar thritis, "did not respond to more than six months of intensive topical treatment with corticosteroid drops." That is an extremely important observation and admission. Many ophthalmologists are determined to use oph thalmic topical corticosteroid preparations for all cases of uveitis regardless of whether or not they improve the inflammation. The prognosis of cataract surgery in chil dren with juvenile rheumatoid arthritis is poor. Any medication that promotes the formation of lens opacification and does not materially improve an intraocular inflamma tion should not be used. Many children with juvenile rheumatoid arthritis and smoldering chronic iridocylitis require only prolonged use of cycloplegics. There are three forms of juvenile rheuma toid arthritis: 1. The acute systemic type is character ized by an acute onset of rash, fever, leukocytosis, and hepatosplenomegaly. It occurs especially between ages 1 and 3 years and predominantly affects boys. Initially there may be no arthritis. Iridocyclitis is uncom mon. 2. The poly-arthritis type is usually acute in onset and is seen more frequently in girls. Classically, over five joints are inflammed. Uveitis may occur. 3. The pauciarticular or monoarticular
type is insidious in nature. From one to four joints may be involved. Characteristically, the arthritis is minor. Uveitis occurs in as high as 30% of children in this group. Schaller and associates2 noted antinuclear antibodies in 52 of 69 children with rheuma toid disease and iridocyclitis. Certainly all children with juvenile rheumatoid arthritis should have periodic eye examinations by an ophthalomologist; however, being aware of the presence or absence of antinuclear antibodies and also of the type of juvenile rheumatoid arthritis (monoarticular), will alert the physician to those cases that require especially careful observation. J. T. COYLE,
Bellevue,
M.D.
Washington
REFERENCES
1. Komreich, H., and Hanson, V.: Rheumatic disease. Current Problems in Pediatrics. 4:6, 1974.
2. Schaller, J., Johnson, G., Ansell, B., and Holborrow, E.: Anti-nuclear antibodies in patients with iridocyclitis and juvenile rheumatoid arthritis. Arthr. Rheum. 16:130, 1973. REPLY
Editor: While Mr. Smiley's experience in this field is well recognized, we feel that our series of patients offers some new and sig nificant perspectives on the problems of iridocyclitis in juvenile rheumatoid arthritis. Mr. Smiley has emphasized the significance of differentiating between the acute and chronic forms of iridocyclitis.1"3 As he states in his letter, the acute form "adequately treated, has a good visual prognosis, and the chronic inflammation, however intensively treated, has a bad one." While we grant that this classification may be an interesting retro spective maneuver when reviewing a large series of cases, we have not found it par ticularly useful in dealing with new patients and their parents. Some of the reasons for this are mentioned by Mr. Smiley himself: 9/15 of his cases of acute iridocyclitis had recurrences 1 and there were exacerbations in his series of chronic cases "so severe as to produce hypopon." Therefore, in dealing
