- Email: [email protected]
Idiopathic Inflammatory Orbital Pseudotumor and Low-Dose Cyclosporine
LETTERS
TO
THE
Idiopathic Inflammatory Orbital Pseudotumor and Low-Dose Cyclosporine
JOURNAL
thalmos, and choroid and retinal folds in the fundus. Visual acuity was normal and there were no other abnormalities in either eye. Computed tomography demonstrated a diffuse homogeneous infiltration of the right orbit. Results of chest x-ray, thyroid gammagraphy, and serum levels of angiotensin-converting enzyme, lysozyme, and thyroid hormones (T3, T4, and thyroid stimulating hormone) were all normal. The test for thyroid antibodies and an intradermal tuberculin test were negative. Orbital biopsy showed an infiltrate of lymphocytes, plasma cells, and neutrophils without atypical forms or granuloma formation. A diagnosis of idiopathic inflammatory pseudotumor was made and treatment with 60 g of prednisone daily was begun. After 20 days the patient was asymptomatic; however, this therapy severely compromised control of her diabetes. Reduction of the prednisone to 20 mg daily resulted in a reactivation of the orbital pseudotumor. A regimen of cyclosporine was then begun at 5 mg/kg of body weight/day and progressively reduced to a dose of 2 mg/kg of body weight/day, which was then maintained for ten months. The patient remained asymptomatic and her diabetes remained under control during this period. The only toxic effect of the drug was mild hypertrichosis. Subsequent discontinuance of cyclosporine resulted in reactivation of the condition. Radiotherapy (2,000 rad) was given, which prevented any further relapse of the orbital inflammation. The previously unreported efficacy of cyclosporine in the control of inflammatory orbital pseudotumor may help to clarify the pathogenetic mechanism of this condition, which may be mediated by T lymphocytes. The efficacy of such low doses (2 mg/kg of body weight/day) of the drug in maintaining remission means that
Manuel Diaz-Llopis, M.D., and Jose 1. Menezo, M.D. Department of Ophthalmology, La Fe Hospital, University of Valencia.
Inquiries to Manuel Diaz-Llopis, M.D., C/ Cirilo Amoros No.1, Pta. 2,46004 Valencia, Spain.
Orbital pseudotumors account for 5% of orbital disease. They are characterized histopathologically by an infiltrate of lymphocytes, plasma cells, and neutrophils without atypical forms. Their origin is unknown, although an autoimmune mechanism has been postulated. Corticosteroids produce a response in 50% of cases, and the addition of radiotherapy is successful in a further 25% of cases.' The use of cytotoxic drugs (chlorambucil and cyclophosphamide) has been suggested for refractory cases, but the results have been inconclusive. 2 Cyclosporine is an immunosuppressant that is selective for the lymphocyte-mediated immune response. It is of proven efficacy in a number of intraocular conditions! (Behcet's syndrome, sarcoidosis, Harada's syndrome, and rejection of corneal grafts) and in orbital conditions such as Graves' ophthalmopathy.' Its principal advantages with respect to other immunosuppressants are its lack of myelotoxicity and that it does not increase susceptibility to infections. However, its nephrotoxic effect must be closely monitored. A 17-year-old insulin-dependent diabetic girl had a three-week history of acute progressive exophthalmos of the right eye. Examination of the right eye showed chemosis, 6 mm of exoph-
THE JOURNAL welcomes letters that describe unusual clinical or pathologic findings, experimental results, and new instruments or techniques. The title and the names of all authors appear in the Table of Contents and are retrievable through the Index Medicus and other standard indexing services. Letters must not duplicate data previously published or submitted for publication. Each letter must be accompanied by a signed disclosure statement and copyright transfer agreement published in each issue of THE JOURNAL. Letters must be typewritten, double-spaced, on 8 1/2 x J l-inch bond paper with 1112-inch margins on all four sides. (See Instructions to Authors.) They should not exceed 500 words of text. A maximum of two black-and-white figures may be used; they should be cropped to a width of 3 inches (one column). Color figures cannot be used. References should be limited to five. Letters may be referred to outside editorial referees for evaluation or may be reviewed by members of the Editorial Board. All letters are published promptly after acceptance. Authors do not receive galley proofs but if the editorial changes are extensive, the corrected typescript is submitted to them for approval. These instructions markedly limit the opportunity for an extended discussion or review. Therefore, THE JOURNAL does not publish correspondence concerning previously published letters.
547
548
AMERICAN JOURNAL OF OPHTHALMOLOGY
there is practically no risk of nephrotoxicity. Cyclosporine is also of proven efficacy in type I diabetes, reducing or even eliminating the need for insulin.' This explains the good diabetic control in our patient, which would be impossible during corticosteroid treatment. Although the anti-inflammatory effect of cyclesporine on the orbital pseudotumor was maintained only during administration of the drug, the low dose used and the resulting absence of nephrotoxicity mean that it may be considered as an alternative to corticosteroid therapy for this condition when use of the latter results in secondary effects such as diabetes, Cushing's syndrome, or osteoporosis.
References 1. Leone, C. R., and Lloyd, W. c.. Treatment protocol for orbital inflammatory disease. Ophthalmology 92:1325, 1985. 2. Garrity, J. A., Kennerdell, J. 5., Johnson, B. L., and Ellis, L. D.: Cyclophosphamide in the treatment of orbital vasculitis. Am. J. Ophthalmol. 102:97, 1986. 3. Nussenblatt, R. B., Rook, A. H., Wacker, W. B., Palestine, A. G., Scher, 1., and Gery, I.: Treatment of intraocular inflammatory disease with cyclesporin A. Lancet 2:235, 1983. 4. Weetman, A. P., McGregor, A. M., Ludgate, M., Beck, L., Mills, P. V., Lazarus, J. H., and Hall, R.: Cyclosporin improves Graves' ophthalmopathy. Lancet 2:486, 1983. 5. Bougneres, P. F., Carel, J. c., Castano, L., Boitard, c., Gardin, J. P., Landais, P., Hors, J., Mihatsch, M. J., Paillard, M., Chaussain, J. L., and Bach, J. F.: Factors associated with early remission of type I diabetes in children treated with cyclosporine. N. Engl. J. Med. 318:663, 1988.
May, 1989
We studied three cases in which corneal abrasions caused by artificial fingernails resulted in severe Pseudomonas corneal ulcers. In two patients, infections were diagnosed after pressure patching of apparent abrasions. Case 1 A 28-year-old woman was hit in her right eye with a piece of debris while manicuring her artificial fingernails. She was treated in an emergency room with scopolamine drops, sulfacetamide ointment, and pressure patching. Approximately 36 hours later, she noted increasing pain and was reexamined. She was then referred to our institution for treatment of a corneal ulcer. Visual acuity was hand motions in the right eye. Examination showed a 6-mm central corneal infiltrate with an adherent fibrin plaque and a hypopyon. A green tint was noted over her nailbeds (Figure). The patient was treated with 40 mg of subconjunctival tobramycin and 100 mg of cefazolin, topical scopolamine 0.25%, and 15 hourly topical fortified tobrarnycin, 15 mg/ml, and cefazolin, 50 mg/ml. Corneal cultures grew P. aeruginosa and the topical cefazolin was discontinued. Six months after the injury, visual acuity was 20/70 secondary to corneal scarring. Case 2 A 37-year-old woman scratched her left eye with a sculptured fingernail and was treated
Pseudomonas Corneal Ulcers After Artificial Fingernail Injuries Anne V. Parker, M.D., Elisabeth J. Cohen, M.D., and Juan J. Arentsen, M.D. Cornea Service, Wills Eye Hospital. This study was supported in part by the Wills Eye Hospital Corneal Research Fund.
Inquiries to Elisabeth f. Cohen, M. D., Cornea Service, Wills Eye Hospital, Ninth and Walnut 515., Philadelphia,
PA 19107.
Figure (Parker, Cohen, and Arentsen). Case 1. Pseudomonas chromonychia.
TO
THE
Idiopathic Inflammatory Orbital Pseudotumor and Low-Dose Cyclosporine
JOURNAL
thalmos, and choroid and retinal folds in the fundus. Visual acuity was normal and there were no other abnormalities in either eye. Computed tomography demonstrated a diffuse homogeneous infiltration of the right orbit. Results of chest x-ray, thyroid gammagraphy, and serum levels of angiotensin-converting enzyme, lysozyme, and thyroid hormones (T3, T4, and thyroid stimulating hormone) were all normal. The test for thyroid antibodies and an intradermal tuberculin test were negative. Orbital biopsy showed an infiltrate of lymphocytes, plasma cells, and neutrophils without atypical forms or granuloma formation. A diagnosis of idiopathic inflammatory pseudotumor was made and treatment with 60 g of prednisone daily was begun. After 20 days the patient was asymptomatic; however, this therapy severely compromised control of her diabetes. Reduction of the prednisone to 20 mg daily resulted in a reactivation of the orbital pseudotumor. A regimen of cyclosporine was then begun at 5 mg/kg of body weight/day and progressively reduced to a dose of 2 mg/kg of body weight/day, which was then maintained for ten months. The patient remained asymptomatic and her diabetes remained under control during this period. The only toxic effect of the drug was mild hypertrichosis. Subsequent discontinuance of cyclosporine resulted in reactivation of the condition. Radiotherapy (2,000 rad) was given, which prevented any further relapse of the orbital inflammation. The previously unreported efficacy of cyclosporine in the control of inflammatory orbital pseudotumor may help to clarify the pathogenetic mechanism of this condition, which may be mediated by T lymphocytes. The efficacy of such low doses (2 mg/kg of body weight/day) of the drug in maintaining remission means that
Manuel Diaz-Llopis, M.D., and Jose 1. Menezo, M.D. Department of Ophthalmology, La Fe Hospital, University of Valencia.
Inquiries to Manuel Diaz-Llopis, M.D., C/ Cirilo Amoros No.1, Pta. 2,46004 Valencia, Spain.
Orbital pseudotumors account for 5% of orbital disease. They are characterized histopathologically by an infiltrate of lymphocytes, plasma cells, and neutrophils without atypical forms. Their origin is unknown, although an autoimmune mechanism has been postulated. Corticosteroids produce a response in 50% of cases, and the addition of radiotherapy is successful in a further 25% of cases.' The use of cytotoxic drugs (chlorambucil and cyclophosphamide) has been suggested for refractory cases, but the results have been inconclusive. 2 Cyclosporine is an immunosuppressant that is selective for the lymphocyte-mediated immune response. It is of proven efficacy in a number of intraocular conditions! (Behcet's syndrome, sarcoidosis, Harada's syndrome, and rejection of corneal grafts) and in orbital conditions such as Graves' ophthalmopathy.' Its principal advantages with respect to other immunosuppressants are its lack of myelotoxicity and that it does not increase susceptibility to infections. However, its nephrotoxic effect must be closely monitored. A 17-year-old insulin-dependent diabetic girl had a three-week history of acute progressive exophthalmos of the right eye. Examination of the right eye showed chemosis, 6 mm of exoph-
THE JOURNAL welcomes letters that describe unusual clinical or pathologic findings, experimental results, and new instruments or techniques. The title and the names of all authors appear in the Table of Contents and are retrievable through the Index Medicus and other standard indexing services. Letters must not duplicate data previously published or submitted for publication. Each letter must be accompanied by a signed disclosure statement and copyright transfer agreement published in each issue of THE JOURNAL. Letters must be typewritten, double-spaced, on 8 1/2 x J l-inch bond paper with 1112-inch margins on all four sides. (See Instructions to Authors.) They should not exceed 500 words of text. A maximum of two black-and-white figures may be used; they should be cropped to a width of 3 inches (one column). Color figures cannot be used. References should be limited to five. Letters may be referred to outside editorial referees for evaluation or may be reviewed by members of the Editorial Board. All letters are published promptly after acceptance. Authors do not receive galley proofs but if the editorial changes are extensive, the corrected typescript is submitted to them for approval. These instructions markedly limit the opportunity for an extended discussion or review. Therefore, THE JOURNAL does not publish correspondence concerning previously published letters.
547
548
AMERICAN JOURNAL OF OPHTHALMOLOGY
there is practically no risk of nephrotoxicity. Cyclosporine is also of proven efficacy in type I diabetes, reducing or even eliminating the need for insulin.' This explains the good diabetic control in our patient, which would be impossible during corticosteroid treatment. Although the anti-inflammatory effect of cyclesporine on the orbital pseudotumor was maintained only during administration of the drug, the low dose used and the resulting absence of nephrotoxicity mean that it may be considered as an alternative to corticosteroid therapy for this condition when use of the latter results in secondary effects such as diabetes, Cushing's syndrome, or osteoporosis.
References 1. Leone, C. R., and Lloyd, W. c.. Treatment protocol for orbital inflammatory disease. Ophthalmology 92:1325, 1985. 2. Garrity, J. A., Kennerdell, J. 5., Johnson, B. L., and Ellis, L. D.: Cyclophosphamide in the treatment of orbital vasculitis. Am. J. Ophthalmol. 102:97, 1986. 3. Nussenblatt, R. B., Rook, A. H., Wacker, W. B., Palestine, A. G., Scher, 1., and Gery, I.: Treatment of intraocular inflammatory disease with cyclesporin A. Lancet 2:235, 1983. 4. Weetman, A. P., McGregor, A. M., Ludgate, M., Beck, L., Mills, P. V., Lazarus, J. H., and Hall, R.: Cyclosporin improves Graves' ophthalmopathy. Lancet 2:486, 1983. 5. Bougneres, P. F., Carel, J. c., Castano, L., Boitard, c., Gardin, J. P., Landais, P., Hors, J., Mihatsch, M. J., Paillard, M., Chaussain, J. L., and Bach, J. F.: Factors associated with early remission of type I diabetes in children treated with cyclosporine. N. Engl. J. Med. 318:663, 1988.
May, 1989
We studied three cases in which corneal abrasions caused by artificial fingernails resulted in severe Pseudomonas corneal ulcers. In two patients, infections were diagnosed after pressure patching of apparent abrasions. Case 1 A 28-year-old woman was hit in her right eye with a piece of debris while manicuring her artificial fingernails. She was treated in an emergency room with scopolamine drops, sulfacetamide ointment, and pressure patching. Approximately 36 hours later, she noted increasing pain and was reexamined. She was then referred to our institution for treatment of a corneal ulcer. Visual acuity was hand motions in the right eye. Examination showed a 6-mm central corneal infiltrate with an adherent fibrin plaque and a hypopyon. A green tint was noted over her nailbeds (Figure). The patient was treated with 40 mg of subconjunctival tobramycin and 100 mg of cefazolin, topical scopolamine 0.25%, and 15 hourly topical fortified tobrarnycin, 15 mg/ml, and cefazolin, 50 mg/ml. Corneal cultures grew P. aeruginosa and the topical cefazolin was discontinued. Six months after the injury, visual acuity was 20/70 secondary to corneal scarring. Case 2 A 37-year-old woman scratched her left eye with a sculptured fingernail and was treated
Pseudomonas Corneal Ulcers After Artificial Fingernail Injuries Anne V. Parker, M.D., Elisabeth J. Cohen, M.D., and Juan J. Arentsen, M.D. Cornea Service, Wills Eye Hospital. This study was supported in part by the Wills Eye Hospital Corneal Research Fund.
Inquiries to Elisabeth f. Cohen, M. D., Cornea Service, Wills Eye Hospital, Ninth and Walnut 515., Philadelphia,
PA 19107.
Figure (Parker, Cohen, and Arentsen). Case 1. Pseudomonas chromonychia.