Ocular involvement in chronic sarcoidosis

Although spontaneous remissions often occur in sarcoidosis, chronic persistent disabling disease is also observed. Of a series of 183 patients with chronic sarcoidosis, 47 (26%) had ophthalmic involvement. In this series, chronic sarcoid was defined as disease for a minimum of five years. Patients were followed primarily for their systemic disease for a mean of 13 years. Uveitis developed in 35 patients (19%) and was an early manifestation in 32 (91%). The course of the ocular disease did not necessarily parallel that of the systemic disease. Despite the chronic nature of the underlying disease, the anterior uveitis did not pursue a chronic course in 15 of 33 patients (45%) and was generally characterized by a single episode at the onset of disease. Chronic uveitis and secondary glaucoma were poor prognostic signs, as eight of 11 patients with uveitis and glaucoma suffered severe visual loss.     

Ocular manifestations in sarcoidosis]

PURPOSE: The ophthalmic examination of patients with diagnosed systemic sarcoidosis. MATERIAL AND METHODS: 33 patients (17 women, 16 men), aged 22-60 years had ophthalmic examination (visual acuity, anterior and posterior segment evaluation, applanation tonometry). In 8 patients repeated examination was performed (duration of observation: 2-31 months). RESULTS: In 27 patients no characteristic features of ocular sarcoidosis were found. In 6 persons (18.2%) variety of ocular lesions was recognized (nodular infiltrations of the eyelids, chronic uveal inflammation, signs of anterior and posterior uveitis in the past, optic disc oedema). In 3 cases ocular lesions preceded the signs of systemic sarcoidosis. This emphasizes the importance of the routine ophthalmic examination of patients with suspected or proven sarcoidosis.     

Ophthalmologic peculiarities of sarcoidosis

The present paper deals with the frequency of different ophthalmic sarcoid changes and their morphological and photographic characteristics. In addition, the diagnostic value of conjunctival biopsy is evaluated. The findings were based on repeated ophthalmological examinations of an unselected group of 281 patients with histologically confirmed sarcoidosis. Conjunctival (37/218) and lacrimal gland (33/254) changes were encountered more often than sarcoid uveitis (22/281), and a conjunctival granuloma was the most frequent single sarcoid ophthalmic finding. Sarcoid changes in the eyes and the adnexae were often characterized by a scarcity of symptoms and a typical outward appearance. In some cases of sarcoid uveitis fluorescein angiography revealed in the iris and the retina nodules which showed features suggesting the proliferative nature of the disease. The iris infrared transillumination technique illustrated the affinity of sarcoid nodules in the pupillary area. Conjunctival biopsy showed epithelioid cell granulomas compatible with sarcoidosis in nearly half of the patients in whom it was suspected in a slit-lamp examination of the conjunctivae. In the diagnosis of sarcoidosis, conjunctival biopsy is a procedure to be recommended before more demanding methods are tried.     

Pars plana vitrectomy in patients with intermediate uveitis

Objective: To describe the effect of pars plana vitrectomy in patients with intermediate uveitis. Methods: Retrospective analysis of the clinical course and visual outcome following pars plana vitrectomy in patients with intermediate uveitis. Results: Thirty-two patients (43 eyes) were included in the study. Pars plana vitrectomy was combined with cataract surgery in 22 of 43 eyes. The intermediate uveitis was associated with sarcoidosis in 16 eyes and multiple sclerosis in five eyes, and was idiopathic in 22 eyes. The mean (±SD) follow-up was 45.6 (±38) months (range: 6–146 months). In 19 of 43 eyes (44.1%), there was improvement in the course of uveitis, allowing the discontinuation of immunosuppressive treatment in seven patients. Cystoid macular edema resolved in 12 of 37 eyes (32.4%). Forty of 43 eyes achieved a better or retained their initial visual acuity. The remaining three eyes deteriorated by two or more lines in the Snellen chart due to the progression of cataract, chronic cystoid macular edema, and glaucomatous optic atrophy, respectively. Conclusions: The results of this study suggest that pars plana vitrectomy may have a beneficial effect on the course of uveitis and the associated complications of cystoid macular edema, thereby reducing the need for long-term immunosuppression. Pars plana vitrectomy combined with simultaneous cataract surgery can improve the visual outcome in these patients.     

Clinical course of ocular sarcoidosis in patients with histologically proven systemic sarcoidosis]

PURPOSE: We reviewed the clinical features, natural history and visual prognosis of 9 patients with histologically confirmed ocular and systemic sarcoidosis. PATIENTS: Nine patients underwent a follow-up study between 1993 and 1998. The diagnosis of sarcoidosis was supported by histological evidence of non caseating epithelioid-cell granuloma in tissue biopsy. RESULTS: The mean age was 40.912 years. The mean follow-up was 22.7 months (range 6 - 54 months). The ophthalmic involvement was bilateral in 8 patients. Before treatment, 3 eyes (17.6%) had a visual acuity less than 1/10. Two eyes (11.7%) had a visual acuity less than 3/10 and 10 eyes (58.8%) more than 6/10. The anterior uveitis was granulomatous in 2 eyes (11.7%) and non granulomatous in 8 eyes (47%). Five eyes (29.4%) had a pars planitis. The posterior segment manifestations of sarcoidosis were retinal vasculitis in 7 eyes (41.1%), papillitis in 10 eyes (58.8%) and choroidal granulomas in 2 eyes (11.7%). Seven patients were treated with systemic corticosteroids. One patient was treated with steroid eye-drops. After treatment, one eye (5.8%) had a visual acuity less than 1/10 and 13 eyes more than 6/10. After treatment, the choroidal granulomas disappeared and the intra-ocular inflammation was controlled in all cases. CONCLUSION: Although the number of patients in our series is small, the prognosis for ocular sarcoidosis appears to be good.     

Visual loss in sarcoid-related uveitis

Purpose: To assess the visual acuity outcome after treatment, in patients with differing types of uveitis associated with sarcoidosis, and to determine the types of sight‐threatening complications. Methods: This was an retrospective, non‐comparative, interventional study of patients who had uveitis with a confirmed diagnosis of sarcoidosis and a minimum follow‐up duration of 6 months from presentation. The worst affected eye at presentation was selected for the study. Complications causing impaired vision were documented, changes in visual acuity after completion of treatment were measured, and the risk of ‘poor outcome’ (visual acuity 6/12 and less) was compared in the various types of uveitis, using ‘exact’ multiple logistic regression to control confounding by other prognostic factors. Results: Seventy‐five patients were studied. The spectrum of ocular inflammation in sarcoidosis is wide. Presentation may be acute and non‐granulomatous or chronic and granulomatous with posterior segment involvement being varied. Poor visual outcome was significantly more frequent in multifocal choroiditis (5/7, 71.4%), and in panuveitis without multifocal choroiditis (13/28, 46.4%), compared to only 3/24 (12.5%) patients with anterior uveitis. The excess risks remained significant after adjustment for confounding effects of other prognostic factors including age, sex and manifest systemic sarcoidosis. Causes of visual loss were cataract, glaucoma, macular oedema, vitreous haemorrhage and retinal detachment. Conclusion: Multifocal choroiditis is a less common manifestation of uveitis associated with sarcoidosis but appears to be associated with a worse visual prognosis despite immunosuppressive therapy.     

Aetiology of uveitis in Sierra Leone, west Africa.

BACKGROUND: In 1992, non-onchocercal uveitis caused 9% of blindness, 8% of visual impairment, and 11% of uniocular blindness among patients visiting an eye hospital in Sierra Leone, west Africa. The aim of this study was to determine the aetiology of uveitis in this population. METHODS: General and ophthalmic examination complemented by serum and aqueous humour analyses for various infectious agents was performed for 93 uveitis patients and compared with serum (n = 100) and aqueous humour (n = 9) analysis of endemic controls. RESULTS: At the initial examination, 45 patients (48%) proved to be severely visually handicapped. After clinical and laboratory analyses, an aetiological diagnosis was established for 49 patients (52%). Toxoplasma gondii was the most important cause of uveitis (40/93; 43%). Anti-toxoplasma IgM antibodies were detected in serum samples of seven of 93 patients (8%) compared with one of 100 controls (1%, p < 0.05). At least six patients (15%) with ocular toxoplasmosis had acquired the disease postnatally. Antibodies against Treponema pallidum were detected in 18 of 92 patients (20%) and in 21 controls (21%). Other causes of uveitis were varicella zoster virus (one patient), herpes simplex virus (two patients), and HLA-B27 positive acute anterior uveitis with ankylosing spondylitis (one patient), while one patient had presumed HTLV-I uveitis. CONCLUSIONS: In a hospital population in Sierra Leone, west Africa, uveitis was associated with severe visual handicap and infectious diseases. Toxoplasmosis proved to be the most important cause of the uveitis. Although the distribution of congenital versus acquired toxoplasmosis in this population could not be determined, the results indicate an important role of postnatally acquired disease. The results further suggested minor roles for HIV, tuberculosis, toxocariasis, and sarcoidosis as causes of uveitis in this population.     

Ocular manifestations in children and adolescents with Lyme arthritis

BACKGROUND: Lyme arthritis is the most frequent late manifestation of Lyme borreliosis and has been associated with ocular inflammation. METHODS: A group of 153 children and adolescents with arthritis, 84 of whom had Lyme arthritis and 69 other causes of arthritis, were followed prospectively for 22-73 (median 44) months in the course of a national study. RESULTS: Three of 84 patients with Lyme arthritis had ocular inflammation (4%), including keratitis, anterior uveitis, and uveitis intermedia. All three had symptoms of decreased visual acuity. Whereas anterior uveitis disappeared without sequelae, a corneal scar and a permanent loss of visual acuity in the patients with keratitis and intermediate uveitis remained. Systematic examination of all patients revealed no further ocular involvement. Of 69 patients with other causes of arthritis who were followed in parallel as a control group, four of 15 patients with early onset pauciarticular juvenile rheumatoid arthritis had chronic anterior uveitis and two of 12 patients with juvenile spondyloarthropathy had acute anterior uveitis. CONCLUSIONS: Ocular involvement with keratitis, anterior uveitis, and intermediate uveitis may occur in children and adolescents with Lyme arthritis. Visual loss appears to be symptomatic, making regular ocular screening of such patients unnecessary.     

Assessment of activity of ocular sarcoidosis by gallium scanning.

Gallium (67Ga) citrate uptake over the orbits, parotid glands, and lungs was examined in six newly detected patients with sarcoidosis and 17 with chronic sarcoidosis. Six of 23 (26%) had uveitis, 18/23 (78%) decreased lacrimal secretion, and 13/16 (81%) epithelioid cell granulomas in conjunctival biopsies. Ten patients with other diseases served as controls. Only five patients had ocular complaints and two had enlarged parotid glands. 67Ga uptake over the orbits and parotids was measured by a quantitative computer based method. Gallium uptake was significantly higher over the orbits (p less than 0.001) and parotids (p less than 0.01) in the newly detected patients and in the parotids (p less than 0.01) in the chronic group than the corresponding uptake in the controls. We suggest that in sarcoidosis an increased 67Ga uptake in the orbital and parotid areas may be a phenomenon comparable to an increased 67Ga uptake in the lungs in active alveolitis. Gallium scan is a good method for revealing even symptomless ophthalmic sarcoid changes. However, in chronic sarcoidosis an equal or only slightly increased gallium uptake over the orbits compared with background activity does not exclude ocular sarcoid disease.     

Incidence of ocular manifestations in patients with histologically confirmed systemic sarcoidosis

BACKGROUND: Intraocular findings in sarcoidosis were described by US studies with 35 % anterior uveitis, 25 % posterior uveitis and 20 - 30 % for late complications in case of first presentation. We report on the spectrum and diagnostic possibilities in 38 German patients. PATIENTS AND METHODS: 38 patients (68 eyes) aged 11 - 58 years (average 42.7 y), who suffered of intraocular manifestations in histological confirmed systemic sarcoidosis (1987 - 1997), were completely evaluated retrospectively. For additional diagnostic tools fluorescein angiography, optical coherence tomography, nuclear magnetic resonance tomography and measurements of angiotensin-converting enzyme, lysozyme and calcium in serum were performed. RESULTS: 15 patients (22 eyes = 32.4 %) had anterior uveitis: granulomatous keratouveitis (10 eyes), granulomas of the iris (6), granulomas of the trabecular meshwork (3) and 22 patients (42 eyes = 61.8 %) an intermediate and posterior uveitis: granulomatous perivasculitis (18 eyes), cystoid maculopathy (8), pars planitis (6), neuroretinitis (4), optic neuritis (3), choroiditis (3). Late complications in case of first presentation were found in 22 patients (39 eyes = 57.4 %): posterior synechiae 14 x, secondary glaucoma 10 x, cataracta complicata 8 x, optic atrophy 6 x, intraocular calcification 1 x. CONCLUSION: Compared to previous studies with heterogeneous ethnic composition, intraocular inflammations with confirmed sarcoidosis in German patients show more than 60 % involvement of the posterior segment. We may speculate on the reasons for these discrepancies as being due to ethnic reasons, age and long-term follow-up. In case of typical ocular signs of sarcoidosis, treatment should be started immediately even without histological confirmation. In some cases histological confirmation is successful 8 years after first presentation.     

Clinical and Investigative Profile of Biopsy-Proven Sarcoid Uveitis in India

Purpose: Retrospective analysis of the clinical features, investigative profile, response to treatment, and final visual outcome in histopathologically confirmed cases of sarcoid uveitis. Methods: Retrospective case series analysis was done of 15 eyes of 9 patients seen between July1999 and August 2003 with biopsy-proven sarcoid uveitis. There were 3 were males and 6 females. The mean age at presentation was 44.1 years (range 11–62 years), The mean follow-up was 28.4 months. Results: Six patients had bilateral ocular involvement and 3 had unilateral involvement. Five out of 9 patients had primarily ocular involvement. The most common presentation was intermediate uveitis and granulomatous anterior uveitis in 7 patients. Eight of 9 patients responded well to the medical treatment with systemic and periocular steroids. Conclusions: Ocular lesions can be the primary manifestation of systemic sarcoidosis. Sarcoid uveitis in the Asian Indian population often presents an intermediate uveitis with granulomatous anterior uveitis.     

Anti-TNF-alpha therapy for sight threatening uveitis

AIM: To describe the effect of additional treatment with anti-TNF-alpha therapy in a case series of 13 patients with serious sight threatening uveitis. METHODS: 13 patients with serious sight threatening uveitis were included, of whom six had Beh?et's disease, five had idiopathic posterior uveitis, one had sarcoidosis, and one birdshot retinochoroiditis. Onset and course of ocular inflammation, inflammatory signs, and visual acuity were assessed. Patients were treated with 200 mg (approximately 3 mg/kg) infliximab infusion. Repeat infusions were given based on clinical response. RESULTS: Infliximab treatment resulted in an effective suppression of ocular inflammation in all patients. In patients with non-Behcet's disease uveitis visual acuity in six out of eight improved or was stable. In patients with Behcet's disease visual acuity in five out of six improved or was stable. CONCLUSION: Anti-TNF-alpha treatment may be of value in the treatment of uveitis, and in patients with Beh?et's disease, leading to suppression of ocular inflammation, vasculitis, and improvement of vision in the majority. Based on these results a controlled masked study is warranted.     

Conjunctival biopsy in the diagnosis of sarcoidosis.

We prospectively studied 47 sarcoidosis suspects and compared conjunctival and transbronchial lung biopsies in these patients. Thirty-four patients had positive findings on biopsy by either method. The transbronchial biopsy was positive in 31 patients, and the conjunctival was positive in 19. The transbronchial lung biopsy was more likely to be positive in black patients (p = 0.009) and in patients with pulmonary infiltrates on chest x ray (p = 0.0044). In comparison, the conjunctival biopsy was more likely to be positive in patients with conjunctival follicles (p = 0.036), ocular abnormalities consistent with sarcoidosis (p = 0.02), and in patients with pulmonary infiltrates on chest x ray (p = 0.029). Iritis was present in 12 patients, enlarged lacrimal glands in three, and vitritis in five. We conclude that the conjunctival biopsy is an effective means of diagnosing sarcoidosis and that every sarcoidosis patient should have an ophthalmic examination.     

Immunosuppression in Behçet's disease Clinical management and long-term visual outcome

The authors review the visual prognosis of 44 patients with Beh?et's disease referred to the Ophthalmology and Rheumatology Departments (Hospital S. Jo?o-one of the two major referral centers in Northern Portugal), due to ocular complaints or for routine examination, in the last ten years. All fulfilled the clinical criteria for diagnosis of Beh?et's disease. Twenty-six were male and 18 female, with mean age 37 years (range 23-66). The mean evolution time since the first clinical manifestations was ten years (range 1-21). Aphthous stomatitis (100%) and genital ulcers (77.3%) were the initial manifestations preceding eye involvement. HLA-B(51)(5) was present in 27 of 36 typed patients (75%). Ocular manifestations were present in 33 patients (75%)-22 patients with panuveitis, eight with hypopyon, four with chronic anterior uveitis and three with episcleritis. Mean age of onset of ocular complications was 32 years (range 20-54). Retinal vaso-occlusive vasculitis was diagnosed in 26 patients (22 with panuveitis and four with posterior uveitis). To prevent ocular relapses, all needed immunosuppression with corticosteroids (drops, depo or systemic), combined with cyclosporine A (CsA) (5 mg/kg/day) in 13 patients and with chlorambucil or cyclophosphamide in six patients, when sight threatening uveitis had previously been refractory to treatment with systemic steroids. The 13 patients under CsA were observed for a period ranging from eight months to five years. Lowdose CsA was found to abrogate the intraocular inflammation, was well tolerated and had no major adverse effects. Regardless of the type of treatment, 21% (14/66) of the eyes lost useful vision five to ten years after initial diagnosis.     

Gallium67 citrate scanning in patients with lacrimal gland and conjunctival sarcoidosis. A report on three cases

Gallium citrate Ga67 scanning of the head and thorax of 3 patients with sarcoidosis is presented. Two patients had a progressive pulmonary sarcoidosis of recent onset and in one patient, with a chronic course of the disease, the lung changes had resolved. All 3 patients had a conjunctival sarcoid change confirmed by biopsy and a lacrimal gland affection suggested by clinical examination. A highly increased Ga67 uptake in the lacrimal and parotid glands as well as in the nasopharynx was found in all patients, and an increased lung uptake was found in the newly detected cases. It is concluded that gallium scanning is a sensitive method for detecting minute ophthalmic changes in sarcoidosis. It also reveals chronic localized ocular affection in cases in which the lung changes are no longer detectable. The mechanism and specificity of gallium uptake in sarcoidosis is also discussed.     

Sarcoidosis

Sarcoidosis commonly involves the eye, causing uveitis, the lacrimal gland, and the cranial nerves, including the optic nerve itself. Several microorganism types have recently been located in sarcoid granulomas, suggesting an infective cause in predisposed individuals. The approach to diagnosis has been refined, including the use of high-resolution computed tomography of the chest. New ophthalmic manifestations have been described, suggesting a wider role for sarcoidosis in intraocular inflammation, and indocyanine green angiography has clarified choroidal involvement. Immunosuppressive and anticytokine treatments can be effective in severe systemic sarcoidosis and should be considered in sight-threatening disease.     

Angiotensin-converting enzyme in uveitis and sarcoidosis

Serum angiotensin-converting enzyme (SACE) was studied in a group of 29 sarcoidosis patients (among whom five had uveitis) and in 22 patients with non-sarcoid uveitis. SACE was normal in all non-sarcoid patients and elevated in 62 per cent of the patients with sarcoidosis. Thus an elevated SACE is closely linked to sarcoidosis and may be a valuable tool in evaluating patients with uveitis in search of a sarcoid origin.     

Uveitis in childhood sarcoidosis

BACKGROUND: Sarcoidosis is a rare cause of uveitis in childhood. Differentiation from other rheumatic diseases may be difficult. We report on five children with uveitis in childhood sarcoidosis and review the previous literature. RESULTS: Children were aged between seven month and 13 years at the time of diagnosis of uveitis. Four of the five patients had panuveitis, the fifth one had intermediate uveitis. In addition, three children had systemic disease with skin, joint and central nervous system involvement. The two children younger than 5 years showed the clinical triad of eye, joint and skin disease. Uveitic complications included corneal band keratopathy, cataract, posterior synechiae, macular scars and choroidal neovascularisation. Vision-limiting complications were found in six of nine eyes at the first visit. CONCLUSION: In childhood sarcoidosis two clinical courses can be differentiated. Younger children aged up to five years express a clinical triad of arthritis, skin lesions and uveitis. In the older group of patients, the clinical course is more similar to that of adults. Rapid diagnosis and quiescence of disease are of particular importance to improve the long-term visual prognosis.     

Presumed ocular sarcoidosis.

BACKGROUND: Sarcoidosis is a multisystemic granulomatous disease of unknown etiology. Ocular manifestations commonly occur in patients with sarcoidosis, with a granulomatous anterior uveitis as the most-prevalent ocular sign. Acute symptoms of uveitis, such as pain, photophobia, lacrimation, or redness, may be absent. Without early detection and timely treatment, this "silent uveitis" may cause permanent ocular damage. CASE REPORTS: Two patients came in for routine eye examinations, with no symptoms of anterior uveitis. The first, a 36-year-old man, had a bilateral granulomatous anterior uveitis. The uveitis was treated with topical corticosteroids, with no complications. The second case involved a 44-year-old woman with chronic, recurrent, bilateral, non-granulomatous anterior uveitis. Despite treatment with topical and oral corticosteroids. the patient had a prolonged course with recurrent episodes leading to secondary sequelae, including glaucoma and cataract. Both patients were co-managed with internal medicine, and in each case, systemic workups were consistent with sarcoidosis. CONCLUSION: Anterior uveitis often manifests as the initial presentation of sarcoidosis. Without acute symptoms, the detection and diagnosis may be delayed, leading to visual deterioration. The diagnosis of sarcoidosis may be difficult, owing to the lack of definitive diagnostic criteria and a variety of presentations. Histologic confirmation may not always be possible or practical. However, a range of serological and radiological tests, when combined with physical and ophthalmic evaluation, can lead to the presumed diagnosis of sarcoidosis. Aggressive treatment is imperative in order to prevent permanent structural damage to the eye resulting from this idiopathic inflammation.