Chronic iridocyclitis is frequently noted coincidentally or because of decreased vision. There is usually little pain. Photophobia and the other symptoms of inflammation are usually absent or minimal. There may be many of the findings seen in acute iridocyclitis, including cells, flare, keratic precipitates, anterior and posterior synechiae, secondary cataract changes, and secondary glaucoma. Band keratopathy, iris nodules, vitreal debris, and macular edema are more characteristically associated with chronic iridocyclitis. These signs may be quantitated and characterized to serve as indicators of inflammatory activity for evaluating levels of activity and effectiveness of management. The characteristics of these findings are useful in the clinical diagnosis of certain conditions.¹

These processes tend to be self-limiting, but the damage done by chronic inflammation can be very destructive to eye tissues, especially if activity is present for months or years. The physical property of ocular transparency, as well as the passage to and focusing of a sharp image on a receptive and functioning retina, is unique to the body, and impairment of this property is a defining characteristic of pathologic process. Ocular transparency is very vulnerable, and systemic disease processes that are minor in terms of their effect on other body processes can have a disastrous effect on this physical property. Diagnostic tests that are used to detect levels of systemic disease producing widespread tissue injury frequently do not have the specificity or sensitivity to detect low levels of injury, and are disregarded or are considered negative when they are not at levels expected in systemic widespread disease. Conventional laboratory testing frequently does not recognize the lower levels of systemic inflammatory disease that also involves the eye and will produce damaging ocular inflammatory injury. The situation is frequently encountered where a specific etiology or mechanism is not identified, or may be only suspected. When a specific treatment is not available, the management is directed at minimizing the damage from the inflammation so that the eye will be capable of the best salvageable function when the inflammation resolves. In chronic processes, the medications used to suppress the inflammatory process can sometimes be more damaging than the disease process itself. An understanding of the disease process and its effect on the eye must be carefully weighed against the long-term effects of medications used in treatment.

Iridocyclitis is basically an anatomic diagnosis, where the iris and ciliary body are the primary areas of inflammatory involvement. As in acute iridocyclitis, signs of chronic iridocyclitis can be present as a spillover from anterior inflammation (e.g., keratitis, primary scleritis), from posterior segment disease (e.g., Toxoplasma retinochoroiditis), or from noninflammatory masquerade syndromes. These other conditions must be considered in the diagnosis of chronic anterior segment inflammatory disease and are covered in other chapters. Multiple causes can produce similar clinical pictures with only subtle differences in the character of the findings. A careful history, review of systems and appropriate laboratory tests, specialty consultations, and repeated observations may be necessary to help clarify the nature of a chronic inflammatory process.

1. Polyarticular JRA is the most common form of the disease (40%). Joint involvement is the major symptom. It is characterized by involvement of five or more joints during the first 6 months of the disease, with a combination of pain, swelling, and limitation of movement. Iridocyclitis develops in approximately 5% of these patients.
   
2. Still's disease, the more severe systemic form of JRA, accounts for 10% of JRA and is associated with fever, leukocytosis, rash, myalgia, splenomegaly, lymphadenopathy, pleuritis, and hepatomegaly. It does not always have joint involvement initially, but frequently goes on to become polyarticular disease. This type is rarely associated with iridocyclitis.
   
3. Oligoarticular and pauciarticular JRA account for 50% of JRA and has two groups that are associated with both immunologic markers and iridocyclitis. One group is HLA-B27-positive patients who tend to have episodes of acute iridocyclitis similar to that seen in the spondyloarthropathies, tend to go on to develop sacroiliitis, and probably represent juvenile onset of these diseases. The other group is antinuclear antibody (ANA)-positive and includes patients who tend to develop the chronic iridocyclitis more commonly associated with JRA.^2–5 There appears to be a genetically associated predisposition. Certain DR, DQ, and DP alleles seem significantly associated with chronic pauciarticular JRA, but not with the appearance of iridocyclitis, whereas DRB1*01 may seem to have a protective effect against the development of iridocyclitis (DRB1*1301, DRB1*801, DRB1*1104, DQA1*0401, DQB1*0402, DPB1*0201).^6,7
   

In ANA-positive pauciarticular JRA patients, the age at onset ranges from 6 months to 15 years, but it has occurred in adulthood.⁸ The highest incidence of onset is from 1 to 4 years of age. Systemic symptoms are generally milder. Joint involvement is usually insidious. Weight-bearing joints, particularly the knees, are most commonly involved. This involvement may be asymmetric. An increased incidence of females is seen in the ANA-positive group with iridocyclitis, and an increased incidence of males is seen in the HLA-B27-positive group.⁵

Arthritis must persist for at least 6 weeks in one to four joints when other subtypes are excluded. Other diseases must be excluded, including infectious arthritis due to viral, bacterial, tuberculous, or fungal etiologies. Ankylosing spondylitis, Reiter's syndrome, systemic lupus erythematosus, rheumatic fever, vasculitis, orthopedic disorders, and many other conditions can present in this manner. A rheumatologist may be necessary to clarify the diagnosis, and synovial fluid analysis and biopsy may be needed.⁵

The pauciarticular group has been reported to account for 75% to 95% of all iridocyclitis associated with JRA. The arthritis usually manifests before iridocyclitis is noted. Reports suggest arthritis may be present 1 to 10 years before iridocyclitis; however, iridocyclitis may be the presenting disease.

The HLA-B27-positive group tends to have episodes of acute iridocyclitis with redness, pain, and light sensitivity lasting 3 to 6 weeks and follows the course of the adult iridocyclitis seen with adult spondyloarthropathies.

In the ANA-positive group, the iridocyclitis is usually asymptomatic and insidious in onset. Girls are affected more frequently than boys, with a ratio of about 4:1. Light sensitivity, mild discomfort, redness, tearing, or spots in vision may be the only symptoms. Even when aware of these symptoms, children usually do not complain about them. Evidence of iridocyclitis may be found without symptoms during a routine examination or when the patient has been referred for a periodic examination by the pediatrician, rheumatologist, or other physician aware of this frequent complication association with pauciarticular JRA. A cataract or irregular pupil due to posterior synechiae (Figs. 1 and 2) may be the first sign of ocular involvement.^9–11

There seems to be little correlation between the activity of the joint inflammation and the iridocyclitis. Bilateral involvement has been reported in up to 50% of patients. Band keratopathy (13% to 49%) (see Fig. 2), cataract (22% to 58%), and posterior synechiae (38% to 61%) are frequent complications. Glaucoma is also a serious complication and has been reported in 14% to 22%.^9,12 Vitreal debris, disc edema, and macular edema do occur, but are rarely associated with long-term morbidity. The posterior pole is not usually involved except when there is secondary spillover of severe anterior segment reaction into the vitreous.^10,13–17

When cataracts are severe enough to require surgical removal, newer extracapsular surgical procedures and the use of additional local or systemic corticosteroids at the time of surgery have been reported to improve results. Posterior pars plana lensectomy and removal of any cyclitic membranes present have also improved the prognosis when this complication is involved. Earlier series reported a poor prognosis following surgery, with 48% having a final visual acuity of 20/200 (6/60)* or less.¹⁸ More recent series with improved techniques suggest a better prognosis.¹⁹ Pretreatment and extended postsurgical treatment with topical and oral corticosteroids seems to help control associated inflammatory activity. Surgical procedures for cataract removal are best delayed until the inflammatory process has been inactive for 6 to 12 months. Intraocular lens implants are still of unproved benefit for children; with chronic inflammation, displacement of the lens is a frequently noted complication, along with pigment and cell precipitates on the lens, synechiae of the iris to the lens, increased activity of the inflammation, secondary glaucoma, and vitreoretinal complications.¹⁹ Intraocular lens implantation in these circumstances should probably be avoided.

The chronic persistence of inflammation makes this iridocyclitis particularly difficult to control. Cycloplegia and topical corticosteroids are sometimes sufficient in mild disease and may be necessary on a long-term basis. Constant reevaluation and adjustment of treatment is necessary in active disease. Oral corticosteroids may be needed in severe disease. The long-term use of oral corticosteroids can produce severe side effects, especially in children, and their use must be seriously weighed in terms of therapeutic benefits versus disability produced by the treatment. Systemic nonsteroidal anti-inflammatory agents, such as tolmetin and naproxen, seem to have a significant place in the treatment of more severe childhood intraocular inflammation, if they are well tolerated. They must be used on a longer term basis, however, and response to treatment takes months to assess. The currently understood side effects, however, make their long-term use appear safer than alternative long-term treatments.^2,20 Naproxen has been noted to cause a condition called pseudoporphyria, and was associated with significant facial scarring in 12% in one study.^21–23 Renal side effects, including glomerular microproteinuria, have been reported in association with the use of nonsteroidal anti-inflammatory agents in JRA treatment.²⁴

The presence of antinuclear antibodies in patients with JRA allows the separation of a group particularly prone to the development of iridocyclitis. An 88% incidence of positive antinuclear antibodies in patients with iridocyclitis suggests that this group has a significantly increased risk of iridocyclitis.¹⁰ These patients should be examined regularly with the slit lamp for signs of iridocyclitis. When iridocyclitis has been present, but in remission, slit-lamp examinations are suggested every 1 to 3 months. When there are no signs of inflammatory activity, a slit-lamp examination approximately every 3 months is appropriate. Children with polyarticular JRA who are ANA-negative and have no ocular inflammation should be examined at least yearly.¹² Adult-onset JRA is occasionally seen, and the extension of active disease into adulthood is also noted in the pauciarticular group with eye involvement.

Heterochromic iridocyclitis is a frequently undiagnosed condition, especially in brown-eyed persons in whom the brown pigment may obscure the iris stromal changes. Loss of the sharpness of detail in the iris rugae is observed.^36,37 The loss of stromal substance and thinning of the iris is usually diffuse, involving the entire iris, and does not involve sectors as in herpes simplex uveitis or herpes zoster uveitis. Appearances may resemble iris atrophy seen in other conditions. This is usually easily differentiated by the somewhat unique distribution 360° over the endothelium of the keratic precipitates and their appearance along with the fibrin stellate flecks between precipitates. Its frequency has been reported to be as high as 2% to 3% of patients seen in several uveitis series,²⁷ and it has been reported in identical twins.³⁸

Neovascularization of the anterior chamber angle and of the iris is seen. Although its cause is unknown, abnormalities in the iris circulation in fluorescein angiography suggest a poor arterial circulation and would support hypoxia as a possible underlying cause for the neovascularization and iris stromal changes.^39,40

Posterior synechiae are extremely rare but have been reported when iris cellular nodules are present.³⁶ Rubeosis has been noted,⁴¹ and neovascular changes have also been associated with the recognized appearance of a filiform anterior chamber hemorrhage following paracentesis of the anterior chamber. In one study this occurred in 22 of 23 patients, starting usually in the angle 180° from the site of paracentesis and producing a small hyphema in the affected eye.⁴²

Glaucoma occurs in approximately 15% to 59% of patients with heterochromic iridocyclitis and is believed to be due to trabecular sclerosis, owing to plasma cell obstruction of the angle, or to neovascularization.^28,29,43 The glaucoma is sometimes very difficult to control; if due to plasma cell infiltration, treatment with corticosteroids may be helpful. Usual medical management with timolol, miotics, and carbonic anhydrase inhibitors may be effective, but varied responses are encountered and control of pressure by these means may be temporary. An abnormal membrane in the angle has been reported visible in advanced glaucoma poorly responsive to medical management. This glaucoma seems poorly responsive to laser trabeculoplasty and usually requires some type of filtration procedure.⁴⁴ Trabeculectomy and trephining have been reported as successful in controlling the pressure.^44,45

Cataract surgery has been reported to have a good prognosis in a retrospective study of 29 patients reported by Smith and O'Connor⁴⁶ and in a series by Franceschetti.⁴⁷ Liesegang⁴⁴ reported on a series of 17 patients undergoing cataract surgery. Multiple complications were experienced, and only 8 of the 17 obtained 20/40 (6/12) vision or better. Extracapsular cataract extraction and pseudophakic implantation within the capsule have been reported in a few patients with good short-term results; when successful, these procedures have provided much improved functional vision.^30,48–51

Most patients with heterochromic iridocyclitis can be managed best with observation alone. Topical corticosteroids may decrease the anterior chamber and anterior vitreous cells and inflammatory debris, but when the topical corticosteroids are discontinued, the inflammatory products usually return to pretreatment levels. If there are keratic precipitates and opacities in the visual axis, vision may be temporarily improved by the use of corticosteroids. Usually, cataract is the cause of the visual loss, and prolonged use of corticosteroids may cause more problems with cataract formation and aggravate glaucoma in corticosteroid-sensitive patients. Mydriasis may be necessary if iris nodules are present and if formation of synechiae appears likely, although this is rare in this condition.

The etiology of heterochromic iridocyclitis remains obscure. Its relation to other types of heterochromia seems unclear. Damage to the sympathetic nervous system and heterochromic iridocyclitis together was substantiated in only 12 of the 1771 cases reviewed.²⁸ Reports of histologic changes with lymphocytes, plasma cells, and Russell bodies infiltrating the iris support an etiology of active local immunoglobulin production. It has been suggested that an immunologic etiology may be present in cases involving a local defect in B-cell regulation and formation of local immune complexes producing vascular disease, seen as chronic vascular occlusive changes and subsequent infarction, hypoxia, neovascularization, and vascular leakage. This hypothesis remains to be confirmed, but it is consistent with many observations seen in the condition, both clinically and histopathologically.^30,52–55

Ocular involvement is seen in approximately 27% of patients with systemically diagnosed sarcoidosis, and anterior uveitis occurs in 66% of this ocular involvement. Eye disease may be the major clinically visible sign of activity of the disease.⁵⁸ Serum lysozyme,⁶² angiotensin-converting enzyme levels,⁶³ gallium scanning, and conjunctival or lacrimal gland and lip biopsies⁶⁴ have been recommended to help substantiate the clinical diagnosis.

Minimal inflammatory changes, such as even a few cells in the anterior chamber, are detectable in the eye. These changes elsewhere in the body might not be recognized. Diagnosis and management of this inflammatory process requires an awareness of its ocular and systemic multiple clinical presentations and variable course.^58,65

The intraocular inflammation may be acute, but more characteristically it is chronic and usually has few symptoms until vision is affected, or until significant structural changes have occurred. It may affect all parts of the eye. The chronic iridocyclitis is characterized by keratic precipitates that are well defined at the edges and tend to be more concentrated in the peripheral cornea (Fig. 8). The anterior chamber contains cells and flare, and the cells have a great tendency to form cellular nodules, either as keratic precipitates, precipitates on the iris, or at the pupil margin as Koeppe nodules (Fig. 9). The nodules tend to become the site of both peripheral anterior synechiae and posterior synechiae. The cellular reaction is usually sensitive to corticosteroids, but may recur as the corticosteroids are withdrawn.^58,65 Treatment responses are further obscured by the frequent tendency for sarcoidosis to undergo spontaneous remission with time, and then recur at a later date. If oral steroids are indicated, moderate to low alternate-day dosages usually control the inflammatory process and produce less systemic side effects.

Vitreous opacities frequently cause decreased vision, but usually resolve without causing permanent vision loss. Visual loss is usually due to persistent macular edema with subsequent macular injury, preretinal membrane formation, or cataract formation.^69,70 Diffuse retinal vascular leakage is usually seen on fluorescein angiography.⁷¹ The peripheral membrane over the pars plana and peripheral retinal area may play a role in the pathogenesis of this disease.⁷² A specific diagnostic test for this condition is not available, and other conditions characterized by low-grade chronic inflammation may sometimes appear similar clinically (e.g., nematode over the ciliary body, multiple sclerosis, sarcoidosis, atypical retinitis pigmentosa).⁷⁰

Topical corticosteroids, as well as periocular injections or a short-term oral dosage, are usually effective in management when the disease course is limited. For long-term management, especially for prolonged macular edema and/or neovascularization of the vitreous base or peripheral membrane, more aggressive management is necessary. Reports indicate that cryotherapy has been helpful.^70,73,74

It is important to consider ischemia as a cause of anterior segment changes resembling other causes of inflammation. If ischemic inflammation is suspected, a thorough workup should be carried out immediately for the underlying cause and should be done by an internist, rheumatologist, or physician experienced in these systemic diseases. Evaluation of the erythrocyte sedimentation rate should be performed on an emergency basis for anyone having suspected giant cell arteritis (cranial arteritis or temporal arteritis) or polymyalgia rheumatica. If the diagnosis can be substantiated, systemic corticosteroids should probably be started without delay. The diagnosis should be made as quickly and as firmly as possible, with a tissue diagnosis if indicated, as well as an elevated sedimentation rate, compatible clinical course, and associated findings to confirm the diagnosis.^77,78

Additional consideration should be given for other causes of vasculitis or vascular occlusive disease and should include evaluations for elevated blood pressure, diabetes, hemoglobinopathy (e.g., sickle cell disease), hyperviscosity syndromes, aortic arch syndromes (e.g., Takayasu's arteritis), vasculitis of other types, and other causes of carotid and smaller vessel vascular occlusive disease.^79,80

Normal T-cell tolerance of lens protein is circumvented in some situations, leading to the production of antibodies, but usually tolerance is maintained.¹⁰¹ Additional studies suggest that different types of immune complexes may produce different reactions, and the immune complex that stimulates oxygen-derived free radicals may play an important role in tissue destruction in immune complex disease. This appears to be a mechanism for inflammation, at least partially independent from that of polymorphonuclear proteolytic enzyme release or arachidonic acid products. These and other factors probably help to account for the variability that is observed clinically.¹⁰² The role of the anterior chamber-associated immune deviation in an intact ocular-splenic axis and subsequent spleen-generated suppressor cell population in this process remains unclear.

The iridocyclitis is usually characterized by lid and conjunctival edema; corneal edema; large, well-formed keratic precipitates, cells, and flare in the anterior chamber; iris hyperemia; and synechiae. The inflammation typically occurs 1 to 14 days after lens injury. Delayed onset may occur weeks to months later. It is usually unilateral unless the lens capsule has been ruptured in the second eye. The symptoms may be acute or chronic.¹⁰³ A unilateral process may occur with cataract surgery in the second eye. Iridocyclitis may occur occasionally without capsule injury in a hypermature lens.¹⁰⁴

The clinical appearance of lens-induced iridocyclitis is so similar to that of sympathetic ophthalmia that they are difficult to tell apart, and both require previous injury. Blodi¹⁰⁵ found both conditions present in 23% of his cases of sympathetic ophthalmia in a retrospective histologic study. Phacotoxic effect of lens material is difficult to evaluate, and evidence of toxic effects is unproven.¹⁰⁶ Phacotoxic uveitis is the term used for the low-grade inflammation sometimes seen after extracapsular cataract extraction. It may occur as a result of surgical trauma, or it may be a low-grade form of phacoanaphylaxis. Treatment of lens-induced inflammation with corticosteroids is helpful, but removal of the remaining lens material may be needed. Lens-induced iridocyclitis may appear similar to a low-grade postsurgical endophthalmitis, such as that caused by Propionibacterium acnes.¹⁰⁷ Prolonged and unresponsive inflammation may suggest a need for aqueous cultures and appropriate antibiotic therapy. Reduction of toxic reaction to intraocular lenses and improved lens design and proficiency in the capsular placement of intraocular lenses have reduced the incidence of associated iridocyclitis. Pseudophakic associated inflammations are less likely to have large, well-formed keratic precipitates.^108–111

Phacolytic glaucoma appears to be a glaucoma secondary to the macrophage removal of lens material from a hypermature lens. The glaucoma results from trabecular obstruction by enlarged lens-protein-filled macrophages. These do not form keratic precipitates, and iris changes of inflammatory disease are not present. This does not appear to be an inflammatory process.^106,107

Ocular findings include keratitis, recurrent iridocyclitis, nodules and hyperemia, extensive synechiae, thickening of the iris, and clouding of the lens. When the posterior pole is involved, papilledema is seen, along with choroiditis, which is multifocal, elevated, hemorrhagic, and circumscribed, with minimal surrounding reaction or subretinal edema. Brucellosis choroiditis is self-limiting and resolves, leaving pigmented scars. Recurrences are common. Histopathology of lymphoid nodules in the iris or other ocular tissue contains no visible organisms.¹¹⁷ In acute disease, one can culture organisms from blood; in chronic disease, serologic testing is widely available but difficult to interpret, especially in relationship to eye disease in the absence of systemic disease. Treatment with tetracycline is effective.¹¹⁸

Brucellosis is a rarely encountered cause of chronic iridocyclitis, as long as the time-honored techniques of pasteurization, animal inspection, and cooking of meats are used and enforced.

This is not always the case, however, and chronic iridocyclitis has occurred. One documented case of apparent chronic iridocyclitis was shown to be due to Staphylococcus aureus. The infection worsened with corticosteroid therapy and cleared with appropriate antibiotic therapy.¹²⁷ Patients with fungal infections can present with chronic iridocyclitis, especially after intraocular surgery.^128,129

Any such suspicious intraocular inflammation warrants a search for a source of infectious emboli. If an infectious process is seriously suspected, aqueous and vitreous fluids should be obtained for diagnosis and appropriate antibiotic therapy should be instituted.^130–133 Systemic as well as topical antibiotics are usually indicated, and vitrectomy with intravitreal antibiotics may be needed.

Close attention must be paid to the stability and level of inflammation of the eye. Low intraocular pressure and a significant presence of cells or flare of the anterior segment indicate chronic low-grade anterior segment inflammation that may not easily be controlled medically. This suggests that postoperatively there may be a greater tendency for increased inflammation, fibrin, and synechiae formation. Chronic low-grade inflammation may be associated with a decreased corneal endothelial cell count. Preoperative specular microscopy gives the appropriate assessment. Although pachymetry readings may be helpful, these readings tend to fluctuate somewhat in patients with uveitis. It is best to wait until the inflammation can be better controlled before proceeding with surgery.

It is very important to pay attention to the presence of anterior and posterior synechiae. Anterior and posterior synechiae can be broken as part of the surgical procedure. The use of viscoelastic material facilitates the release of synechiae, and may reduce the likelihood of bleeding. A thinned and atrophic iris may require an iridotomy or multiple iridotomies, and possibly even pupil reconstruction. Recurrent inflammation with acute episodes may lead to an occluded pupil. In that situation, sector iridectomies or radial iridotomies are best left unclosed. A generous peripheral iridectomy is appropriate in patients with chronic inflammation.

Evaluation of the status of the zonules is important. Patients with chronic uveitis tend to have some weakening of the zonules and have less stability of the lens capsule during the cataract procedure. Attention to this is necessary, because the technique may need to be modified. Modern extracapsular phacoemulsification techniques using hydrodissection will loosen the nucleus and reduce the trauma to weakened zonules. Preservation of the capsular bag is helped by capsulorhexis and hydrodissection. Modern viscoelastics facilitate not only cataract removal, but also lens implantation. In the presence of weak zonules or a tear in the capsule, the trauma during the operation and postoperative capsular shrinkage can lead to significant difficulties, including intraoperative or postoperative subluxation or dislocation of the lens implant along with the capsular bag.

A small, sutureless incision with appropriate tunneling technique is of great advantage. Sutures tend to aggravate inflammation, create residual astigmatism, and provide limited long-term closure if healing does not occur. Long-term postoperative corticosteroid treatment, as well as preoperative intensive corticosteroid treatment, is usually necessary; this seriously affects wound healing, so a small, self-closing incision is of great advantage. The skilled use of iris hooks or retractors makes access to the lens capsule more possible. A surgical peripheral iridectomy large enough to remain open, even with considerable inflammation, is needed. If a lens implant is to be considered, it must be confidently placed within the lens capsule, because any other placement will result in mechanical factors that are likely to aggravate any existing inflammatory process.

Cataract surgery in this situation requires experience with many difficult techniques, the ability to change the surgical approach to fit each patient's problems, and if an implant is indicated, the selection of an appropriate type of lens implant for the situation. Cataract surgery in patients with chronic iridocyclitis remains one of the major surgical challenges. It should be considered only after the surgeon has seriously evaluated the patient's situation, calculated the risks involved, and obtained the patient's informed consent.^{48–51,134–144}

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