Chronic Cyclitis Research Articles

Editorial: Fuchs’ uveitis: from Imperial Vienna to global appraisal

At the beginning of the last century, a uveitis entitylinked to heterochromia was identified. It was firstdescribed in 1902 by Ernst Fuchs, working inHabsburg Imperial Vienna, in these terms: ‘‘Whenthe colours of both eyes are different, it can happenthat in one of the two eyes, always in the eye with thebrighter iris, a cataract develops. In addition thesebrighter eyes present also a chronic cyclitis withcorneal keratic precipitates’’ [1]. This observationwas published in 1902 in the 9th edition of ErnstFuchs’ ophthalmology textbook (Fig. 1).Of course heterochromia, a very obvious clinicalsign, had been described much earlier. Aristotlenamed it heteroglaucos. A famous heterochromic inantiquity was Alexander the Great, who rode a horse,‘‘Bukephalos’’, which was itself heterochromic.In 1904, Georges Weill from Strassburg, at thetime in German-ruled Alsace, described seven caseswhere ‘‘heterophthalmus’’ was associated with cata-ract and cyclitis with keratic precipitates (KPs). Hesaid that he had performed a widespread literaturesearch and had not found any previous report of thisparticular association, and therefore claimed that hewas the first to have mentioned the association of‘‘heterophthalmus, cataract and cyclitis’’ [2] (Fig. 2).Weill had not found any mention of this triad, asFuchs’ first observation was not published in ajournal but in his textbook (Fig. 1). In response toWeill’s article, Ernst Fuchs published an article in1906 that is now considered the primary article onthis entity (Fig. 3). The title, ‘‘About complicationsof heterochromia’’, shows that heterochromia was atthe center of interest [3]. In the 15th edition of hisophthalmology textbook published in 1926 and editedby his pupil Salzmann, Fuchs gave a completedescription of the features of the entity that came tobe known under the eponym of ‘‘Fuchs’ heterochro-mic cyclitis’’, including heterochromia, cataract,cyclitis with KPs and, most importantly, vitreousinvolvement (Fig. 4). He also mentioned that theseeyes tolerated surgery very well [4].Ernst Fuchs (Fig. 5) had already retired, in 1915, asDirector of the Second Vienna Eye Hospital [5]. HebecameDirectorofthisinstitutionin1885,1 yearafterthe Austrian ophthalmologist, Karl Koller, working atthe Vienna General Hospital, had introduced cocaineas a topical anesthetic in ophthalmology. This discov-ery represented a tremendous leap forward for oph-thalmology as it came at the start of a marvelousexpansion in ocular surgery. Ophthalmology inVienna, thanks to people like Ernst Fuchs, was then,

Intermediate uveitis pars planitis

Uveitis, an inflammation of the uveal tract of the eye, anatomically may be classified as (1) anterior uveitis predominantly involving the iris and the anterior part of the ciliary body (pars plicata) (2) intermediate uveitis involving posterior part of the cilliary body (pars plana) and the extreme periphery of the retina (3) posterior uveitis involving choroid and retina posterior to the vitreous base. Intermediate uveitis consists of two syndromes (1) pars planitis and (2) chronic cyclitis. Both of these are characterised by vitritis and absent or minimal anterior uveitis. The only feature differentiating the two is absence of clinically detectable deposition of exudate over pars plana (snow-banks) in cases of chronic cyclitis. The clinical pattern of these syndromes have evolved under various names such as “cyclitis” by Fuchs in 1908 [1], “peripheral uveitis” by Schepens in 1958 [2], “pars planitis” by Welch and Wehlan in 1960 [3] and finally “Intermediate uveitis” by International Uveitis Study Group in 1987 [4].

Consultation withthe Specialist

Introduction Inflammation involving any part of the uveal tract (iris, ciliary body, and choroid) is described as uveitis. Adjacent ocular structures, including the retina, vitreous, and optic nerve, may be affected as well. The term uveitis is derived from the Latin uva, or grape, due to the similarity in appearance between the bluish vein structure visible in a peeled blue grape and the middle vascular layer of the eye, the uvea. Within the broader term of uveitis are more specific designations, depending on the anatomic area involved. Thus, inflammation of the iris is called iritis, inflammation of the ciliary body is called cyclitis, and both occurring together is termed iridocyclitis. These latter structures, along with the anterior choroid, share a common blood supply and, if affected, tend to be inflamed together. Such inflammation can be referred to as anterior uveitis. Posterior uveitis describes inflammation of the posterior choroid (choroiditis), the retina (retinitis), or both (chorioretinitis). When the middle portion of the eye is involved (primarily retinal vessels and the peripheral portions of the retina and uveal tract), the condition is described as intermediate uveitis. This term has replaced other labels that appear in the literature, including chronic cyclitis, peripheral uveitis, and pars planitis.

Consultation with the Specialist

Introduction Inflammation involving any part of the uveal tract (iris, ciliary body, and choroid) is described as uveitis. Adjacent ocular structures, including the retina, vitreous, and optic nerve, may be affected as well. The term uveitis is derived from the Latin uva, or "grape," due to the similarity in appearance between the bluish vein structure visible in a peeled blue grape and the middle vascular layer of the eye, the uvea. Within the broader term of uveitis are more specific designations, depending on the anatomic area involved. Thus, inflammation of the iris is called iritis, inflammation of the ciliary body is called cyclitis, and both occurring together is termed iridocyclitis. These latter structures, along with the anterior choroid, share a common blood supply and, if affected, tend to be inflamed together. Such inflammation can be referred to as anterior uveitis. Posterior uveitis describes inflammation of the posterior choroid (choroiditis), the retina (retinitis), or both (chorioretinitis). When the middle portion of the eye is involved (primarily retinal vessels and the peripheral portions of the retina and uveal tract), the condition is described as intermediate uveitis. This term has replaced other labels that appear in the literature, including chronic cyclitis, peripheral uveitis, and pars planitis.

Cyclitic glaucoma

A series of patients is presented showing the widely variable clinical behaviour of different groups of patients who share the common features of cyclitic glaucoma. These cyclitic glaucomas may be classified: 1. Glaucomatocyclitic crises: unilateral or bilateral. This condition presents acutely, almost always in males with a strong tendency to spontaneous resolution but a liability to subsequent recurrence. Some cases are slow to resolve and may recur frequently. 2. Heterochromic cyclitis: glaucoma infrequently complicates heterochromic cyclitis and then with only a mild rise in intraocular pressure (IOP). 3. Chronic cyclitis: unilateral or bilateral. This is more common in females often presenting with a high rise in IOP which may be difficult to control medically. An insidious form of chronic cyclitis occurs in middle-aged, blue-eyed females, often with sarcoid, and is likely to progress to profound visual loss if unrecognised.

Fluorescein Angiography of the Iris and Specular Microscopy of the Corneal Endothelium in Some Cases of Glaucoma Secondary to Chronic Cyclitis

A series is presented of middle- to old-aged female patients with gray irides and mild bilateral cyclitis accompanied by severe glaucoma with advanced cupping and field loss in the late stages. The cyclitis is accompanied by edema of the corneal endothelium, with the presence of inflammatory cells on the posterior surface of the endothelium; these changes were reversible with topical corticosteroids. There also was hypoperfusion of the iris with microneovascular changes, but these changes were not reversible. The rise in intraocular pressure (IOP) was associated with changes in the endothelial cells of the posterior cornea that may be accompanied by similar changes in the endothelium of the trabecular meshwork. A large proportion of these patients had sarcoidosis, but the more florid ocular appearances of sarcoid were not present although two patients had chorioretinal scarring. The use of topical corticosteroids was important in helping to control the elevated IOP in these cases. Recognition and treatment of the condition is important, because cupping and field loss were extreme in long-standing cases.

PROGRESSIVE CORNEAL ENDOTHELIAL CELL CHANGES IN ANTERIOR SEGMENT DISEASE

AbstractWe have demonstrated marked changes in the corneal endothelium in some groups of patients with ocular anterior segment disease including chronic cyclitis, both heterochromic an! classical pseudoexfoliation of the lens capsule, and anterior segment pigment dispersal syndrome.The most striking changes occurred in heterchromic cyclitis with reduced endothelial cell counts (p>0.01) and altered cellular morphology. In almost all eyes with cyclitis endothellal belbs were seen. In eyes with unilateral pseudoexfoliation of the lens capsule there was a lower endothelial cell count with pleomorphisrn and polymegathism, the changes being somewhat more marked with raised introcular pressure (p>0.07). In anterior segment pigment dispersal syndrome there was an even smaller reduction in count (p>0.05)with mild changes in morphology.Factors producing endothelial cell changes included hypoperfusion of the iris with microneovascularisation of the iris stroma, intercellular bleb formation with oedema of the corneal endothelium. and raised intraocular pressure.A normal iris circulation is important to the integrity of the corneal endothelium.

PROGRESSIVE CORNEAL ENDOTHELIAL CELL CHANGES IN ANTERIOR SEGMENT DISEASE

We have demonstrated marked changes in the corneal endothelium in some groups of patients with ocular anterior segment disease including chronic cyclitis, both heterochromic and classical, pseudoexfoliation of the lens capsule, and anterior segment pigment dispersal syndrome. The most striking changes occurred in heterochromic cyclitis with reduced endothelial cell counts (p less than 0.01) and altered cellular morphology. In almost all eyes with cyclitis endothelial belbs were seen. In eyes with unilateral pseudoexfoliation of the lens capsule there was a lower endothelial cell count with pleomorphism and polymegathism, the changes being somewhat more marked with raised intraocular pressure (p less than 0.01). In anterior segment pigment dispersal syndrome there was an even smaller reduction in count (p less than 0.05) with mild changes in morphology. Factors producing endothelial cell changes included hypoperfusion of the iris with microneovascularization of the iris stroma, intercellular bleb formation with oedema of the corneal endothelium, and raised intraocular pressure. A normal iris circulation is important to the integrity of the corneal endothelium.

Changes in the iris vasculature and corneal endothelium in chronic cyclitis.

Fluorescein angiography of the iris vasculature and specular microscopy of the corneal endothelium were used to examine two important anterior chamber tissues in cases of heterochromic cyclitis, classical chronic cyclitis and some cases of iridocyclitis. The 13 cases of heterochromic cyclitis and eight cases of classical chronic cyclitis all showed vascular hypoperfusion of the iris, except for two young females with heterochromia. In these cyclitic eyes there was pupillary leakage of dye and all but one young female with heterochromia showed evidence of microneovascularization of the iris. Most patients showed evidence of abnormalities of the corneal endothelium, but this was most marked in elderly patients with heterochromic cyclitis, probably due to the long duration of the condition. Six of the patients with chronic cyclitis had bilateral disease and five of these were elderly women with glaucoma and grey irides, the group being distinct enough to form a discrete clinical entity. Seventeen of the patients with chronic cyclitis were females with grey irides suggesting that this type of patient is more susceptible to cyclitis. Patients with acute iridocyclitis showed little abnormality on fluorescein angiography, while those with recurrent or chronic disease showed vascular hypoperfusion and microneovascularization suggesting that these changes in the iris contribute to the chronicity of the condition. It is suggested that hypoperfusion and microneovascularization of the iris and abnormal permeability are important factors in the production of chronic cyclitis, and that the integrity of the corneal endothelium is affected by these changes if they operate over a long period of time.

Secondary Fuchs's heterochromic cyclitis: a new approach to an old disease.

A study of 17 cases of Fuchs's heterochromia. Among these patients 10 had chorioretinal scar. In 3 cases the disease was seen after chronic cyclitis in a case after traumatism. Fuchs's heterochromia seems be in almost all cases a secondary disease. We do not know how a primary ocular disease can lead to Fuchs's syndrome. it is possible that this mechanism is autoimmune.

Circulating immune complexes in uveitis

The serum of 63 patients suffering from various clinical types of endogenous uveitis with unknown etiology was investigated for the presence of circulating immune complexes (CIC). None of the patients was suffering from a systemic disease usually associated with CIC. Significantly elevated CIC levels were detected in 2 women with chronic cyclitis. One of them was also suffering from paresis of the facial nerve. The investigation reported here indicates that C1q-binding CIC are of no importance in the pathogenesis of endogenous uveitis. On the one hand one should consider the possibility of local IC formation, on the other hand one should be aware of the fact that IC does not always represent a pathogenic factor but that it may also occur as a secondary phenomenon after non-immunologic tissue destruction.

T and B lymphocytes in the aqueous humor of patients with uveitis.

Sixteen patients with different types of uveitis (ocular toxoplasmosis, chronic cyclitis, Vogt-Koyanagi-Harada syndrome, Behçet's syndrome, ocular herpes, and a probable nematode) had the aqueous humor withdrawn and had the number of T, B, and null lymphocytes assessed. T and B lymphocytes were identified by rosette formation with sheep erythrocytes and with human erythrocytes sensitized with IgM antibody and complement or zymosan-C3 complexes, respectively. A much higher number of T lymphocytes than B lymphocytes was observed in all cases. The results were not influenced by treatment with steroids or immunosuppressive drugs for local, periocular, or systemic effects.

Failures of cytostatic treatment in ophthalmology (author's transl)

At the Department of Ophthalmology of the Univeristy of Zurich, cytostatic drugs have been used since 1962 for the treatment of various eye conditions: 16 keratoplasties, 11 cases of degenerative or proliferative eye disease, six cases of retinal vasculitis and 147 of uveitis (sympathetic ophthalmia, Behcet, chronic cyclitis, rheumatoid infantile uveitis etc. After a few rather unsatisfactory trials with methotrexate and azathioprine, a routine therapy combining cyclophosphamide and procarbazine was established. Of the 29 failures (16.1%) six were in the group of keratoplasties (37.5%!), three in cases of degenerative disease, three in the six vasculitis cases, and only 17 in the 147 cases of uveitis. The main cause of failure in corneal grafts was pre-existent vascularization, and in uveitis belated commencement of therapy. In cases where blindness threatens immunodepressors should be administered as early as possible. In the 180 cases reported here, these drugs did not produce any serious complications.

Delayed Hypersensitivity to Retrobulbar Injections of Methylprednisolone Acetate

A patient with chronic cyclitis developed hyperemia of the episcleral and bulbar conjunctival vessels after retrobulbar injections of methylprednisolone acetate suspension. Intradermal testing revealed the responsible allergen to be methylprednisolone acetate; hypersensitivity could not be demonstrated by patch testing, despite use of increased concentrations.

HLA antigens and uveitis.

We have studied the antigen frequencies in a group of adult patients with acute and chronic iridocyclitis and chronic, cyclitis. In patients with iridocyclitis a statistically significant increased incidence of HLA-B27 was noted, even in those patients without joint or systemic disease. Patients with chronic iridocyclitis also were noted to have an increased incidence of B27; however, those patients with chronic cyclitis did not. On the basis of HLA-B27 studies there appears to be a fundamental difference between adult and juvenile iridocyclitis.

Complications of Chronic Cyclitis

Follow-up examinations, ranging from four to more than 20 years, were performed on 100 patients with chronic cyclitis whose ages at onset were from 4 to 58 years. Cataracts were found in 42% of eyes and macular disease secondary to macular edema in 28% of eyes. Band keratopathy, glaucoma, retinal detachment, retinoschisis, vitreous hemorrhage, retinal hemorrhage, and vessels leaving the disk margin were also noted. The complications resulting in decreased vision in chronic cyclitis were macular edema in active cases and macular degenerative changes in the late inactive stages. Of all eyes with final visual acuity of 6/12 (20/40) or less, 74% had permanent, late macular changes secondary to earlier cystoid macular edema. Vitreous opacities or cells, or both, caused decreased visual acuity in the early active stages of chronic cyclitis but were not major factors in the ultimate visual prognosis in the late inactive stages. At the final examination, vitreous opacities caused a visual loss in only 9% of the eyes that had visual acuity of 6/12 (20/40) or less. It was difficult to determine whether corticosteroids caused cataract formation and glaucoma.

Optic Disk Neovascularization Associated with Chronic Uveitis

Disk neovascularization associated with chronic uvetitis occurred in five patients. The uveitis was most frequently diagnosed as chronic cyclitis, with a marked inflammatory cell response in the vitreous body. Using direct and indirect ophthalmoscopy, slit-lamp biomicroscopy with the Goldmann contact lens, perimetry, and fluorescein angiography and angioscopy, we found no areas of vascular occlusion or nonperfusion. Inflammation probably produced chemical mediators capable of traveling to the optic disk and inducing neovascular proliferation, in the absence of retinal vascular occlusion.

Transactions of the American Ophthalmological Society: 99th Annual Meeting.

<i>The Transactions of the 99th Annual Meeting of the American Ophthalmological Society</i>, 1963, includes many reports that are significant to ophthalmologists. The standard of writing is excellent, and a range of subjects is discussed. The papers presented by members of the society deal with studies of iridectomy in advanced angle closure glaucoma, di-isopropyl fluorophosphate in the treatment of esotropia, scleral cautery with iridectomy, vitelline macular degeneration, orbital reconstruction with polyvinyl sponge, chronic cyclitis, and other topics of similar concern. The theses submitted for membership at this meeting were, for the most part, well-organized documents, entailing much work and considerable thought. The theses presented are the following: Metabolism of the Alloxan Diabetic Rat Retina, Andrew de Roetth, Jr., MD. Ocular Changes Due to Contact Lenses, Joseph M. Dixon, MD. The Role of Cholesterol in the Pathogenesis of Coats' Disease, James R. Duke, MD. The Ultrastructure of the Retinal Pigment Epithelium With

PSEUDO-GLIOMA, RETINITIS AND RETRO-LENTAL FIBROPLASIA.

Summary The clinical features of many apparently widely differing syndromes may be explained on common grounds. It is not suggested that they have a common cause. The vascular malformation of the retina present in Von Hippel's disease is responsible for changes in the fundus which resembles those seen in Coate's disease. In early childhood they lead to the so-called infantile form of that disease and, in infancy, to one of the forms of pseudo-glioma. When the inflammation involves the ciliary body and iris, the picture of a chronic cyclitis with the formation of a membrane, and its harmful effect on the lens, is superadded. This is the picture which is seen to appear in the premature infant born with a normal fundus and which gradually develops a “retro-lental fibroplasia.” Retrolental fibroplasia is a retinitis associated with an irido-cyclitis occurring in the immature eye.

The Removal of Foreign Bodies from the Eye.

Burlington, Iowa, Aug. 25, 1902. To the Editor: —I would like to add a brief supplement to my remarks in the discussion of Dr. Sweet's paper at the Saratoga meeting. By reference to these remarks it will be seen that I reported a case of foreign body in the eye in which I felt that the x -ray photography was of the character criticised by Dr. Lippincott in the same discussion. My suspicion of failure in the x -ray work was borne out by the subsequent history of the case, which is as follows: On my return home the 1st of July I found that the eye was becoming soft and shrunken and had every sign of chronic cyclitis. I thereupon enucleated it. The foreign body, which was shown in the picture exhibited, as apparently high up in the globe or in the orbit (as the photographer said) was really