Glaucoma in Thomas S.
Episcleritis
Harbin, Jr., MD, Irvin P. Pollack,
\s=b\ Two patients had episcleritis and a secondary open angle glaucoma. Ante-
rior uveitis was absent in three of the four eyes. The low facility of outflow suggests that increased episcleral venous pressure was not the mechanism of the glaucoma. The response of the intraocular pressure and, in one case, the outflow facility to steroid therapy suggests that inflammation of angle structures was the pathogenesis of the raised intraocular pressure.
(Arch Ophthalmol 93:948-950, 1975)
An association between episcleritis and open angle glaucoma is known to many clinical investigators; however, a search of the literature failed to disclose documentation of this association or description of the
MD
The collagen-vascular diseases are the most common systemic illnesses that are associated with scleritis and, occasionally, episcleritis. Of these dis¬ eases, rheumatoid arthritis is the
frequent. complications rarely described in
most
Ocular
of episcleritis are the literature and in none of these was there an as¬ sociated glaucoma. The current inves¬ tigation describes two patients, each of whom had rheumatoid arthritis, episcleritis, and open angle glaucoma. REPORT OF CASES
Jr\.
glaucoma. Episcleritis typically ap¬ pears by causing inflammation of the episclera without affecting the vision, and it produces discomfort rather than frank pain. It is distinguished from the more severe scleritis by the absence of scierai thinning, edema, and nodules in episcleritis. Further¬ more, scleritis may be extremely painful and is frequently accom¬ panied by photophobia and tearing.1·2
Submitted for publication July 11, 1974. From the Wilmer Institute, Johns Hopkins University School of Medicine, Baltimore. Read in part before the Wilmer Residents Association meetings, Baltimore, April 18-20,1974. Reprint requests to the Wilmer Institute, Johns Hopkins University School of Medicine, 601 N Broadway, Baltimore, MD 21205 (Dr.
Pollack).
Case L—A 58-year-old woman was first by us in June 1972 because of epi¬ scleritis and glaucoma. She had had excel¬ lent ocular health until six weeks prior to referral here. She had noted mild painless redness of her eyes. When the vascular in¬ jection increased, she consulted her oph¬ thalmologist, who found her intraocular pressure to be 50 mm Hg in the right eye and 35 mm Hg in the left eye. The in¬ traocular pressure did not respond to a therapeutic regimen of pilocarpine or 0.125% echothiophate iodide, and the pa¬ tient was referred to the Wilmer Institute. She had had rheumatoid arthritis for 16 years, and she had been hospitalized two years previously for anemia and weight loss, which were thought to be from an ex¬ acerbation of her collagen-vascular dis¬ ease. Systemic steroids produced a remis¬ sion and were given intermittently thereafter. Her current medications were isoxsuprine hydrochloride (Vasodilan) and chlorpheniramine maléate (Teldrin). Results of examination showed a best corrected vision of 20/20 in both eyes. seen
There was extreme diffuse injection of con¬ junctival and episcleral vessels (Fig 1). No nodules or scierai thinning were present. Pupillary reactions and ocular motility were normal. Slit lamp examination showed no keratic precipitates, flare, cells,
other evidence of anterior-chamber in¬ flammation. Applanation tensions were 44 mm Hg in the right eye and 30 mm Hg in the left eye. The facility of outflow (C) was 0.17 in each eye. The angle was wide open, with ciliary body easily visible for 360° in each eye. Goldmann visual fields were en¬ tirely normal. Echothiophate iodide therapy was dis¬ continued and the patient was begun on a regimen of prednisolone acetate 1% (Prednefrin Forte) every hour, for three days, then every two hours; dexametha¬ sone (Decadron) ointment at bedtime and acetazolamide (Diamox), 250 mg three times a day. One week later, there was only minimal ocular injection (Fig 2). Applanation ten¬ sions were 13 mm Hg in the right eye and 14 mm Hg in the left eye, and the facility of outflow was 0.39 in the right eye and 0.36 in the left eye. Acetazolamide therapy or
discontinued; prednisolone acetate was continued, four times a day. One week later, the scieras of the eyes were white, and applanation tensions were 15 mm Hg in each eye. Prednisolone ace¬ was
therapy
was given once daily, with dexa¬ methasone ointment at night. Two weeks later, which was four weeks after beginning therapy, the tensions re¬ mained 15 mm Hg in each eye, and all medications were discontinued. Three months later, there was mild injection, but the intraocular pressure was normal. Case 2.—A 59-year-old woman was seen in May 1973. In 1970, results of ocular ex-
tate
Downloaded From: http://archopht.jamanetwork.com/ by a University of Arizona Health Sciences Library User on 05/28/2015
Fig 1.—Appearance
of eyes of
patient
1
amination elsewhere had showed 20/20 vi¬ sual acuity in both eyes and an intraocular pressure of 22 mm Hg in each eye. In Octo¬ ber 1972, she had episcleritis in the su¬ perior half of her left eye and normal intraocular pressure. Eight weeks of treatment with prednisolone (Inflamase Forte) 1%, every four hours, failed to clear the redness, but the intraocular pressure remained normal. The right eye was not involved. In April 1973, after she had received no steroid therapy for three months, the en¬ tire left globe and part of the right globe were involved with episcleritis. Tensions at this time were 24 mm Hg in the right eye and 45 mm Hg in the left eye. The angles were open; the anterior chamber was quiet, with no ray or cells. Results of conjunctival biopsy on two occasions had showed chronic inflammatory changes. Therapy with a combination of 4% pilocarpine and 1% epinephrine was ineffective and the pa¬ tient was referred here. Past medical history included hyperten¬ sion, which was being treated. She also had arthritis, mainly in her shoulders and in her knees. Medications included raulwolfia serpentina (Rauzide) twice weekly and diazepam (Valium) 2.5 mg three times a
day. Family history was noncontributory. Two days prior to her visit, 1% predniso¬ lone therapy every two hours was started. The corrected visual acuity was 20/20 in both eyes. There was notable episcleral in¬ jection in both eyes, in the left eye more than in the right eye. Slit lamp examina¬ tion was unremarkable for the right eye; however, the left cornea had many fine keratic precipitates and a few larger ones. The left anterior chamber had a trace of flare, but no cells were seen. The angle was wide open in each eye. Goldmann visual
prior
to treatment. Note extreme diffuse
Fig
2.—Left eye of
patient
injection of conjunctiva
episcleral
vessels.
1 after one week of treatment.
fields and results of fundus examination entirely normal. Applanation pres¬ sures were 42 mm Hg in the right eye and 40 mm Hg in the left eye, with outflow fa¬ cilities of 0.08 in the right and 0.06 in the left eye. The patient was hospitalized for eval¬ uation of her minimal uveitis in the left eye and her arthritis. She continued to re¬ ceive 1% prednisolone every two hours while awake, and dexamethasone oint¬ ment, at bedtime. A modified uveitis survey disclosed the following: hematocrit reading, white blood cell count, and urinalysis findings were were
normal; erythrocyte sedimentation rate, 25; negative intermediate purified protein derivative; serological tests for brucella,
syphilis, and antinuclear antibodies
and
gave
negative results. Results of blood chem¬ istry studies using a sequential multiple analyzer were within normal limits, except for slightly elevated cholesterol and lactic dehydrogenase levels. Chest x-ray film was unremarkable, except for degenerative ar¬ thritis changes. The initial impression by the rheumatology consultation was degen¬ erative osteoarthritis. When the rheuma¬ toid serology screen gave positive results (tube latex, 1:640; modified Rose titer, 1:160) and hand and foot x-ray films showed erosions typical of rheumatoid ar¬ thritis, the impression was changed to in¬ clude rheumatoid arthritis, although it was largely asymptomatic even in areas where there was radiologie evidence of activity. During the first few days of topical steroid therapy, the intraocular pressure
Downloaded From: http://archopht.jamanetwork.com/ by a University of Arizona Health Sciences Library User on 05/28/2015
were essentially unaffected. Prednisone therapy, 40 mg daily, was started. Four days later, tensions were 30 mm Hg in the right eye and 28 mm Hg in the left eye; one week later, they were 26 mm Hg in the right eye and 22 mm Hg in the left eye. The scieras of the e; as were whiter and the left anterior chamber was
readings
entirely quiet. COMMENT The association of rheumatoid ar¬ thritis with anterior uveitis is well known,3 as is the association of rheu¬ matoid arthritis with episcleritis. But a relationship between episcleritis and glaucoma appears to be less well defined. Watson2 described seven per¬ sons with scleritis and open angle glaucoma whose intraocular pressures responded to treatment of the scle¬ ritis. He found no glaucoma, however, in 192 eyes of 117 patients with episcleritis, four of whom had rheu¬ matoid arthritis. In the two cases presented, only one of the four eyes showed any evi¬ dence of anterior uveitis. It seems reasonable to assume that the three eyes with no cells in the anterior chamber or in the angle had an in¬ flammation that was limited to the trabecular meshwork or to the out¬ flow channels without affecting the uveal vessels to produce the charac¬ teristic picture of anterior uveitis. One can speculate that there may be
edema of the tissues that surround the outflow channels or swelling of the endothelial cells that line the trabeculae so as to compromise aqueous drainage from the eye. A similar mechanism might also be re¬ sponsible for those cases of secondary open angle glaucoma where there is minimal anterior-chamber reaction and an elevated intraocular pressure that responds immediately to topi¬ cally applied steroids. Glaucomatocyclitic crisis may be one such form of uveitis. Increased intraocular pressure in our patients was related to a reduc¬ tion in the facility of aqueous out¬ flow. The first patient showed much improvement in outflow concurrent with her treatment and reduced in¬ traocular pressure. The second pa¬ tient had low facilities of aqueous outflow even after therapy. No at¬ tempt was made to measure the episcleral venous pressure. Increased intraocular pressure that is caused by elevated episcleral venous pressure is usually associated with normal facil¬ ities of aqueous outflow4-5; thus, this is an unlikely cause of the glaucoma. On the other hand, the dramatic decrease in intraocular pressure in both pa¬ tients was closely associated with im¬ provement of the episcleritis. This would suggest an impairment of the angle structures by inflammation as
the mechanism for the glaucoma. As one might expect, there was a substantial improvement in the facil¬ ity of aqueous outflow in the first case after steroid therapy and this oc¬ curred simultaneously with the de¬ creased intraocular pressure and im¬ provement of episcleritis. However, in case 2, the patient continued to have low C values and slightly ele¬ vated intraocular pressure. One must consider the possibility that irrevers¬ ible damage occurred to the outflow mechanism or that the antiinflammatory therapy was still insufficient to eliminate the inflammation. How¬ ever, the fact that the intraocular pressure dropped as much as 14 mm Hg after steroid therapy is certainly consistent with the hypothesis that the patient had a secondary glaucoma that was caused by an inflammatory response in the outflow mechanism. References 1. Lyne AJ, Pitkeathley DA: Episcleritis and scleritis: Association with connective tissue. Arch Ophthalmol 80:171-176, 1968. 2. Watson PG: Management of scleral disease. Trans Ophthalmol Soc UK 86:151-167, 1966. 3. Vail D: Diffuse collagen diseases with ocular complications. Trans Ophthalmol Soc UK 72:155\x=req-\ 169, 1952. 4. Kolker AE, Hetherington J Jr: Becker-Shaffer's Diagnosis and Therapy of the Glaucomas, ed 3. St. Louis, CV Mosby Co, 1970, pp 250-251. 5. Kupfer C: Clinical significance of pseudofacility. Am J Ophthalmol 75:193-205, 1973.
Downloaded From: http://archopht.jamanetwork.com/ by a University of Arizona Health Sciences Library User on 05/28/2015
Episcleritis
Harbin, Jr., MD, Irvin P. Pollack,
\s=b\ Two patients had episcleritis and a secondary open angle glaucoma. Ante-
rior uveitis was absent in three of the four eyes. The low facility of outflow suggests that increased episcleral venous pressure was not the mechanism of the glaucoma. The response of the intraocular pressure and, in one case, the outflow facility to steroid therapy suggests that inflammation of angle structures was the pathogenesis of the raised intraocular pressure.
(Arch Ophthalmol 93:948-950, 1975)
An association between episcleritis and open angle glaucoma is known to many clinical investigators; however, a search of the literature failed to disclose documentation of this association or description of the
MD
The collagen-vascular diseases are the most common systemic illnesses that are associated with scleritis and, occasionally, episcleritis. Of these dis¬ eases, rheumatoid arthritis is the
frequent. complications rarely described in
most
Ocular
of episcleritis are the literature and in none of these was there an as¬ sociated glaucoma. The current inves¬ tigation describes two patients, each of whom had rheumatoid arthritis, episcleritis, and open angle glaucoma. REPORT OF CASES
Jr\.
glaucoma. Episcleritis typically ap¬ pears by causing inflammation of the episclera without affecting the vision, and it produces discomfort rather than frank pain. It is distinguished from the more severe scleritis by the absence of scierai thinning, edema, and nodules in episcleritis. Further¬ more, scleritis may be extremely painful and is frequently accom¬ panied by photophobia and tearing.1·2
Submitted for publication July 11, 1974. From the Wilmer Institute, Johns Hopkins University School of Medicine, Baltimore. Read in part before the Wilmer Residents Association meetings, Baltimore, April 18-20,1974. Reprint requests to the Wilmer Institute, Johns Hopkins University School of Medicine, 601 N Broadway, Baltimore, MD 21205 (Dr.
Pollack).
Case L—A 58-year-old woman was first by us in June 1972 because of epi¬ scleritis and glaucoma. She had had excel¬ lent ocular health until six weeks prior to referral here. She had noted mild painless redness of her eyes. When the vascular in¬ jection increased, she consulted her oph¬ thalmologist, who found her intraocular pressure to be 50 mm Hg in the right eye and 35 mm Hg in the left eye. The in¬ traocular pressure did not respond to a therapeutic regimen of pilocarpine or 0.125% echothiophate iodide, and the pa¬ tient was referred to the Wilmer Institute. She had had rheumatoid arthritis for 16 years, and she had been hospitalized two years previously for anemia and weight loss, which were thought to be from an ex¬ acerbation of her collagen-vascular dis¬ ease. Systemic steroids produced a remis¬ sion and were given intermittently thereafter. Her current medications were isoxsuprine hydrochloride (Vasodilan) and chlorpheniramine maléate (Teldrin). Results of examination showed a best corrected vision of 20/20 in both eyes. seen
There was extreme diffuse injection of con¬ junctival and episcleral vessels (Fig 1). No nodules or scierai thinning were present. Pupillary reactions and ocular motility were normal. Slit lamp examination showed no keratic precipitates, flare, cells,
other evidence of anterior-chamber in¬ flammation. Applanation tensions were 44 mm Hg in the right eye and 30 mm Hg in the left eye. The facility of outflow (C) was 0.17 in each eye. The angle was wide open, with ciliary body easily visible for 360° in each eye. Goldmann visual fields were en¬ tirely normal. Echothiophate iodide therapy was dis¬ continued and the patient was begun on a regimen of prednisolone acetate 1% (Prednefrin Forte) every hour, for three days, then every two hours; dexametha¬ sone (Decadron) ointment at bedtime and acetazolamide (Diamox), 250 mg three times a day. One week later, there was only minimal ocular injection (Fig 2). Applanation ten¬ sions were 13 mm Hg in the right eye and 14 mm Hg in the left eye, and the facility of outflow was 0.39 in the right eye and 0.36 in the left eye. Acetazolamide therapy or
discontinued; prednisolone acetate was continued, four times a day. One week later, the scieras of the eyes were white, and applanation tensions were 15 mm Hg in each eye. Prednisolone ace¬ was
therapy
was given once daily, with dexa¬ methasone ointment at night. Two weeks later, which was four weeks after beginning therapy, the tensions re¬ mained 15 mm Hg in each eye, and all medications were discontinued. Three months later, there was mild injection, but the intraocular pressure was normal. Case 2.—A 59-year-old woman was seen in May 1973. In 1970, results of ocular ex-
tate
Downloaded From: http://archopht.jamanetwork.com/ by a University of Arizona Health Sciences Library User on 05/28/2015
Fig 1.—Appearance
of eyes of
patient
1
amination elsewhere had showed 20/20 vi¬ sual acuity in both eyes and an intraocular pressure of 22 mm Hg in each eye. In Octo¬ ber 1972, she had episcleritis in the su¬ perior half of her left eye and normal intraocular pressure. Eight weeks of treatment with prednisolone (Inflamase Forte) 1%, every four hours, failed to clear the redness, but the intraocular pressure remained normal. The right eye was not involved. In April 1973, after she had received no steroid therapy for three months, the en¬ tire left globe and part of the right globe were involved with episcleritis. Tensions at this time were 24 mm Hg in the right eye and 45 mm Hg in the left eye. The angles were open; the anterior chamber was quiet, with no ray or cells. Results of conjunctival biopsy on two occasions had showed chronic inflammatory changes. Therapy with a combination of 4% pilocarpine and 1% epinephrine was ineffective and the pa¬ tient was referred here. Past medical history included hyperten¬ sion, which was being treated. She also had arthritis, mainly in her shoulders and in her knees. Medications included raulwolfia serpentina (Rauzide) twice weekly and diazepam (Valium) 2.5 mg three times a
day. Family history was noncontributory. Two days prior to her visit, 1% predniso¬ lone therapy every two hours was started. The corrected visual acuity was 20/20 in both eyes. There was notable episcleral in¬ jection in both eyes, in the left eye more than in the right eye. Slit lamp examina¬ tion was unremarkable for the right eye; however, the left cornea had many fine keratic precipitates and a few larger ones. The left anterior chamber had a trace of flare, but no cells were seen. The angle was wide open in each eye. Goldmann visual
prior
to treatment. Note extreme diffuse
Fig
2.—Left eye of
patient
injection of conjunctiva
episcleral
vessels.
1 after one week of treatment.
fields and results of fundus examination entirely normal. Applanation pres¬ sures were 42 mm Hg in the right eye and 40 mm Hg in the left eye, with outflow fa¬ cilities of 0.08 in the right and 0.06 in the left eye. The patient was hospitalized for eval¬ uation of her minimal uveitis in the left eye and her arthritis. She continued to re¬ ceive 1% prednisolone every two hours while awake, and dexamethasone oint¬ ment, at bedtime. A modified uveitis survey disclosed the following: hematocrit reading, white blood cell count, and urinalysis findings were were
normal; erythrocyte sedimentation rate, 25; negative intermediate purified protein derivative; serological tests for brucella,
syphilis, and antinuclear antibodies
and
gave
negative results. Results of blood chem¬ istry studies using a sequential multiple analyzer were within normal limits, except for slightly elevated cholesterol and lactic dehydrogenase levels. Chest x-ray film was unremarkable, except for degenerative ar¬ thritis changes. The initial impression by the rheumatology consultation was degen¬ erative osteoarthritis. When the rheuma¬ toid serology screen gave positive results (tube latex, 1:640; modified Rose titer, 1:160) and hand and foot x-ray films showed erosions typical of rheumatoid ar¬ thritis, the impression was changed to in¬ clude rheumatoid arthritis, although it was largely asymptomatic even in areas where there was radiologie evidence of activity. During the first few days of topical steroid therapy, the intraocular pressure
Downloaded From: http://archopht.jamanetwork.com/ by a University of Arizona Health Sciences Library User on 05/28/2015
were essentially unaffected. Prednisone therapy, 40 mg daily, was started. Four days later, tensions were 30 mm Hg in the right eye and 28 mm Hg in the left eye; one week later, they were 26 mm Hg in the right eye and 22 mm Hg in the left eye. The scieras of the e; as were whiter and the left anterior chamber was
readings
entirely quiet. COMMENT The association of rheumatoid ar¬ thritis with anterior uveitis is well known,3 as is the association of rheu¬ matoid arthritis with episcleritis. But a relationship between episcleritis and glaucoma appears to be less well defined. Watson2 described seven per¬ sons with scleritis and open angle glaucoma whose intraocular pressures responded to treatment of the scle¬ ritis. He found no glaucoma, however, in 192 eyes of 117 patients with episcleritis, four of whom had rheu¬ matoid arthritis. In the two cases presented, only one of the four eyes showed any evi¬ dence of anterior uveitis. It seems reasonable to assume that the three eyes with no cells in the anterior chamber or in the angle had an in¬ flammation that was limited to the trabecular meshwork or to the out¬ flow channels without affecting the uveal vessels to produce the charac¬ teristic picture of anterior uveitis. One can speculate that there may be
edema of the tissues that surround the outflow channels or swelling of the endothelial cells that line the trabeculae so as to compromise aqueous drainage from the eye. A similar mechanism might also be re¬ sponsible for those cases of secondary open angle glaucoma where there is minimal anterior-chamber reaction and an elevated intraocular pressure that responds immediately to topi¬ cally applied steroids. Glaucomatocyclitic crisis may be one such form of uveitis. Increased intraocular pressure in our patients was related to a reduc¬ tion in the facility of aqueous out¬ flow. The first patient showed much improvement in outflow concurrent with her treatment and reduced in¬ traocular pressure. The second pa¬ tient had low facilities of aqueous outflow even after therapy. No at¬ tempt was made to measure the episcleral venous pressure. Increased intraocular pressure that is caused by elevated episcleral venous pressure is usually associated with normal facil¬ ities of aqueous outflow4-5; thus, this is an unlikely cause of the glaucoma. On the other hand, the dramatic decrease in intraocular pressure in both pa¬ tients was closely associated with im¬ provement of the episcleritis. This would suggest an impairment of the angle structures by inflammation as
the mechanism for the glaucoma. As one might expect, there was a substantial improvement in the facil¬ ity of aqueous outflow in the first case after steroid therapy and this oc¬ curred simultaneously with the de¬ creased intraocular pressure and im¬ provement of episcleritis. However, in case 2, the patient continued to have low C values and slightly ele¬ vated intraocular pressure. One must consider the possibility that irrevers¬ ible damage occurred to the outflow mechanism or that the antiinflammatory therapy was still insufficient to eliminate the inflammation. How¬ ever, the fact that the intraocular pressure dropped as much as 14 mm Hg after steroid therapy is certainly consistent with the hypothesis that the patient had a secondary glaucoma that was caused by an inflammatory response in the outflow mechanism. References 1. Lyne AJ, Pitkeathley DA: Episcleritis and scleritis: Association with connective tissue. Arch Ophthalmol 80:171-176, 1968. 2. Watson PG: Management of scleral disease. Trans Ophthalmol Soc UK 86:151-167, 1966. 3. Vail D: Diffuse collagen diseases with ocular complications. Trans Ophthalmol Soc UK 72:155\x=req-\ 169, 1952. 4. Kolker AE, Hetherington J Jr: Becker-Shaffer's Diagnosis and Therapy of the Glaucomas, ed 3. St. Louis, CV Mosby Co, 1970, pp 250-251. 5. Kupfer C: Clinical significance of pseudofacility. Am J Ophthalmol 75:193-205, 1973.
Downloaded From: http://archopht.jamanetwork.com/ by a University of Arizona Health Sciences Library User on 05/28/2015
