Optic nerve enlargement and chronic visual loss

We present four patients with sarcoidosis of the anterior visual pathways. The first patient presented with unilateral visual loss, a mass lesion at the optic nerve head, and an enlarged orbital optic nerve. The second patient presented with bilateral progressive painless visual loss, associated with optic nerve pallor and visual field loss. In these two patients, optic nerve biopsy was diagnostic of sarcoidosis. The third patient developed optic nerve and chiasmal involvement after sarcoidosis was established by lacrimal gland biopsy. The fourth pateint had optic nerve, pulmonary, and lymph node involvement with sarcoidosis. A conjunctival and lung biopsy were diagnostic. Computed tomography and magnetic resonance imaging have greatly facilitated diagnosis of sarcoidosis of the anterior visual pathways. Sarcoidosis of the anterior visual pathways may occur alone or in association with other ocular or systemic manifestations. A conjunctival or lacrimal gland biopsy may be preferable as the initial diagnostic approach. Treatment of patients with this condition may require systemic immunosuppression, in addition to corticosteroids, to prevent permanent visual loss.     

Neuro-ophthalmological findings in sarcoidosis

PURPOSE: This review emphasizes the importance of neuro-ophthalmological signs and symptoms in sarcoidosis. The presence of ophthalmological and neuro-ophthalmological findings may lead to diagnosis of the disease and the initiation of adequate treatment. MATERIAL AND METHODS: Patients who had been diagnosed with neurosarcoidosis during the period 1990 - 2001 were identified from the departmental diagnostic index. The history, clinical, laboratory and imaging data of patients were analysed. RESULTS: Fifteen patients were identified, four men and 11 women, with a mean age of 44.1 years (range 26-65 years). In six of the 15 (40%), neurological deficits were the initial symptoms. Nine (60%) had known sarcoidosis at the time of presentation. Ten patients (66%) had ophthalmological/neuro-ophthalmological symptoms and signs. CONCLUSION: Neuro-ophthalmological symptoms may develop early in neurosarcoidosis. If neuro-ophthalmological symptoms arise in patients with established biopsy-proven sarcoidosis, the diagnosis is usually easy to make. However, a number of patients with neurosarcoidosis may present with neuro-ophthalmic symptoms before systemic involvement becomes obvious. In this situation the diagnosis is challenging, and the major goal is to establish the presence of systemic sarcoidosis.     

Scleritis associated with sarcoidosis

PURPOSE: To review scleritis cases associated with sarcoidosis and to evaluate clinical features, medical treatment, and clinical course. METHODS: Two cases of diffuse anterior scleritis and a nodular scleritis case associated with systemic sarcoidosis were evaluated. Scleritis was the first manifestation of sarcoidosis in two of the patients and the diagnosis of sarcoidosis was confirmed with a conjunctival biopsy. The third patient had the diagnosis of systemic sarcoidosis when she developed scleritis. RESULTS: Based on the clinical and histological appearance, two patients presented with bilateral diffuse anterior scleritis and the third patient with an anterior nodular scleritis. One patient already had the diagnosis of systemic sarcoidosis at presentation, while ocular findings led to the diagnosis in the other two cases. Conjunctiva or skin biopsies revealed features compatible with the diagnosis of sarcoidosis. Scleritis attacks recurred in two patients. All patients responded well to systemic corticosteroid (prednisone) therapy. CONCLUSIONS: Scleritis associated with sarcoidosis has rarely been described and it may be the presenting sign of systemic sarcoidosis.     

Involvement of orbital tissues by sarcoid

Of 15 biopsy specimens identified as showing sarcoid involving the extraocular orbital tissues, three were from patients known to have systemic sarcoidosis before biopsy. Of the other 12 patients who were not known to have systemic disease at the time of biopsy, 11 were found to have evidence of systemic sarcoidosis after the biopsy. One patient, however, had no evidence of sarcoidosis at the time of the biopsy nor did she develop sarcoidosis during a seven-year follow-up period. Our results suggest that most patients with sarcoid involving the orbit will be found to have evidence of systemic sarcoidosis, but that rare patients exist in whom the orbital lesion appears to represent an isolated process. There was no histologic difference between orbital specimens from patients with systemic sarcoidosis and the specimens from the patient with isolated sarcoid of the orbit.     

Scleritis associated with sarcoidosis

Purpose: To review scleritis cases associated with sarcoidosis and to evaluate clinical features, medical treatment, and clinical course. Methods: Two cases of diffuse anterior scleritis and a nodular scleritis case associated with systemic sarcoidosis were evaluated. Scleritis was the first manifestation of sarcoidosis in two of the patients and the diagnosis of sarcoidosis was confirmed with a conjunctival biopsy. The third patient had the diagnosis of systemic sarcoidosis when she developed scleritis. Results: Based on the clinical and histological appearance, two patients presented with bilateral diffuse anterior scleritis and the third patient with an anterior nodular scleritis. One patient already had the diagnosis of systemic sarcoidosis at presentation, while ocular findings led to the diagnosis in the other two cases. Conjunctiva or skin biopsies revealed features compatible with the diagnosis of sarcoidosis. Scleritis attacks recurred in two patients. All patients responded well to systemic corticosteroid (prednisone) therapy. Conclusions: Scleritis associated with sarcoidosis has rarely been described and it may be the presenting sign of systemic sarcoidosis.     

Conjunctival deposits as the first sign of systemic sarcoidosis in a pediatric patient

PURPOSE: Sarcoidosis is an inflammatory disease of unknown cause, characterized by noncaseating granulomas. In this study, the authors present a sarcoidosis patient without systemic signs diagnosed by histopathologic analysis of conjunctival deposits. METHODS: A 10-year-old girl had bilateral, focal, multilobular, multiple pale yellow deposits in bulbar conjunctiva for a year. In the patient's biomicroscopic examination, numerous 0.50- to 2-mm diameter pale yellow deposits were determined in both bulbar conjunctivas. The patient had no other systemic or ocular complaints. A conjunctival biopsy was performed. RESULTS: The biopsy specimens showed noncaseating granulomas with prominent asteroid bodies. Serum angiotensin-converting enzyme levels were increased in the patient. Conjunctival deposits were the first manifestation of sarcoidosis in the patient and the diagnosis of sarcoidosis was confirmed with a conjunctival biopsy. CONCLUSIONS: To the authors' knowledge, this is the second study that reports a sarcoidosis patient with non-systemic involvement diagnosed after histopathologic analysis of conjunctival deposits.     

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.     

Retinal cotton-wool spots as the first sign of systemic sarcoidosis.

Isolated retinal cotton-spots have not yet been reported as the sign of ocular sarcoidosis. We describe three patients with retinal cotton-wool spots of initially unknown etiology in whom systemic sarcoidosis was diagnosed 6-16 months later. The diagnosis was confirmed by conjunctival biopsy. These observations indicate that sarcoidosis should be included in the differential diagnosis of retinal cotton-wool spots and patients with these lesions should be followed for the development of this disease.     

Conjunctival non-caseating granulomas in a human immunodeficiency virus (HIV) positive patient attributed to sarcoidosis

PURPOSE: To report a rare case of isolated ocular sarcoidosis in an HIV positive patient. DESIGN: Case report. METHODS: A 47-year-old HIV+ Caucasian male was referred for chronic bilateral follicular conjunctivitis. RESULTS: A conjunctival biopsy that was performed on the right eye showed sarcoidosis. General medical evaluation including a spiral thin cut chest CT scan revealed no systemic involvement. The ocular signs and symptoms resolved completely with topical corticosteroid treatment. CONCLUSIONS: HIV infection and sarcoidosis rarely coexist, presumably because their immunopathogenesis mechanisms diverge. In the absence of systemic involvement, a definite diagnosis can only be made by biopsy of the ocular tissues.     

Conjunctival Biopsy as an Aid in the Evaluation of the Patient with Suspected Sarcoidosis

Conjunctival biopsy is an underused but simple technique in the evaluation of the patient with sarcoidosis and occasionally other systemic diseases. In 55% of patients with biopsy-proven sarcoidosis from other sites, a blind conjunctival biopsy was positive. Bilateral conjunctival biopsies and the examination of multiple sections of each biopsy were essential to obtain this high of a yield. There was no relationship between an anterior uveitis and a positive conjunctival biopsy.     

Lacrimal bypass surgery in patients with sarcoidosis

PURPOSE: To examine the results of lacrimal bypass surgery in patients with sarcoidosis. METHODS: Patients with sarcoidosis who underwent dacryocystorhinostomy (DCR) or conjunctivodacryocystorhinostomy (CDCR) in two practice settings from 1986 through 1995 were identified and their medical records reviewed. RESULTS: Twelve patients, of whom eight were women, underwent bilateral DCR or CDCR to treat nasolacrimal duct obstruction associated with sarcoidosis. The initial diagnosis of sarcoidosis was established in four patients from a biopsy specimen obtained during DCR. The ages of the patients at diagnosis of sarcoidosis ranged from 39 to 64 years (mean, 49.6 years; median, 45.5 years), whereas their ages at the time of surgery ranged from 42 to 72 years (mean and median, 55 years). The average duration of postoperative follow-up evaluation was 44 months (median, 38.5 months; range, 10 to 82 months). All patients received local corticosteroids postoperatively, and nine patients (75%) were treated with prednisone. Of the 24 lacrimal procedures, 23 (95.8%) were patent to irrigation at the last follow-up examination, and all patients were asymptomatic. CONCLUSION: Lacrimal drainage obstruction may be the initial manifestation of sarcoidosis, and tissue obtained during DCR may help to establish the diagnosis. A successful surgical outcome may require intensive and occasionally long-term therapy with local and systemic corticosteroids.     

Conjunctival biopsy of anophthalmic socket in sarcoidosis

A case of sarcoidosis was diagnosed by a random conjunctival biopsy from an anophthalmic socket. A 22-year-old black female patient presented with anterior and posterior uveitis and subconjunctival nodules in the left eye. Her right eye was enucleated due to a penetrating injury 3 years before admission. Biopsy of subconjunctival nodules in the left eye failed to demonstrate granulomatous inflammation; however, a random conjunctival biopsy from the normal-appearing anophthalmic socket conjunctiva revealed noncaseating granulomas. Conjunctival biopsy is a safe, cost-effective means of diagnosis in sarcoidosis suspects. To our knowledge, this is the first reported case of sarcoidosis confirmed with random conjunctival biopsy from an anophthalmic socket.     

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.     

Conjunctival involvement in familial amyloidotic polyneuropathy

The present study is an evaluation of the possibility of using conjunctival biopsies for establishing the diagnosis of systemic amyloidosis of neuropathic type. Amyloid deposits were demonstrated in conjunctival biopsies from 15 of 16 patients with polyneuropathy and from 1 of 4 patients with vitreous opacities as the only symptom. Thus the sensitivity of conjunctival biopsies for the diagnosis of systemic amyloidosis of this type is similar to that of biopsies from other tissues. It is concluded that a conjunctival biopsy is a useful diagnostic tool since the technique is simple and there is little discomfort for the patient.     

Conjunctival Non-Caseating Granulomas in a Human Immunodeficiency Virus (HIV) Positive Patient Attributed to Sarcoidosis

Purpose: To report a rare case of isolated ocular sarcoidosis in an HIV positive patient. Design: Case report. Methods: A 47-year-old HIV+ Caucasian male was referred for chronic bilateral follicular conjunctivitis. Results: A conjunctival biopsy that was performed on the right eye showed sarcoidosis. General medical evaluation including a spiral thin cut chest CT scan revealed no systemic involvement. The ocular signs and symptoms resolved completely with topical corticosteroid treatment. Conclusions: HIV infection and sarcoidosis rarely coexist, presumably because their immunopathogenesis mechanisms diverge. In the absence of systemic involvement, a definite diagnosis can only be made by biopsy of the ocular tissues.     

Chest computerized tomography in the evaluation of uveitis in elderly women

PURPOSE: To describe the usefulness of chest computerized tomography (CT) in the evaluation of uveitis in elderly women and the clinical characteristics of patients with an abnormal chest CT scan. DESIGN: Prospective noncomparative case series. METHODS: We evaluated 30 elderly women, aged 61-83 years, with chronic iritis, vitritis, or choroiditis and with no definitive cause for their uveitis. All patients underwent a battery of diagnostic laboratory studies and chest CT. RESULTS: The diagnostic examination in most patients included serum angiotensin converting enzyme level, serum lysozyme, rapid plasma reagin level, fluorescent treponemal antibody-absorption test, purified protein derivative skin test, and chest x-rays. Chest CT performed on all patients showed parenchymal, mediastinal, and/or hilar adenopathy in 17 patients (57%). Histopathologic confirmation of sarcoidosis with noncaseating granulomas in the biopsy specimens was obtained in 14 patients: eight by mediastinoscopy, two by bronchoscopy, two by conjunctival biopsy, one by nasal biopsy, and one by vitreous biopsy. CONCLUSIONS: Chest CT can be useful in elderly female patients with chronic uveitis for identifying mediastinal lymphadenopathy and other lesions suggestive of sarcoidosis, as well as to help guide tissue confirmation and to rule out other diagnoses including lymphoma.     

Diagnosis of ocular sarcoidosis by diagnostic criteria for systemic sarcoidosis

PURPOSE: To evaluate the diagnostic criteria for systemic sarcoidosis in diagnosis of ocular sarcoidosis. SUBJECTS AND METHODS: Subjects were 105 ocular sarcoidosis suspects and 37 patients with other uveitis. We diagnosed ocular sarcoidosis suspects using the diagnostic criteria for systemic sarcoidosis proposed by the Japanese Committee for Diffuse Lung Diseases. The criteria included histological and clinical diagnosis, and the clinical diagnosis required 5 systemic tests: 1) tuberculin skin test, 2) serum gamma-globulin(gamma-gl), 3) serum angiotensin converting enzyme(ACE), 4) serum lysozyme, and 5) 67Ga scintigraphy. Three positive findings including either 1) or 3) fulfilled the clinical diagnosis. RESULTS: Sixty-two patients were histologically and/or clinically diagnosed, and 43 patients remained undiagnosed. The histological and clinical diagnosis did not produce the same diagnostic yields. The sensitivity of ACE and gamma-gl was low. The percentage of patients showing increased lymphocytosis and/or CD 4/8 in bronchoalveolar lavage was similarly high in the diagnosed and undiagnosed, suggesting the presence of definitive ocular sarcoidosis in the undiagnosed. CONCLUSIONS: The diagnostic criteria for systemic sarcoidosis yielded false negative results in diagnosing ocular sarcoidosis. The selection and combination of systemic tests for clinical diagnosis should be further studied.     

Conjunctival biopsy in the diagnosis of ocular sarcoidosis

Sarcoidosis is a multisystem granulomatous disease of unknown etiology that can affect almost every organ in the body, particularly the lungs, skin, eyes, and thoracic lymph nodes. A definitive diagnosis of sarcoidosis requires that a biopsy be performed. A specimen can be obtained from any affected ocular structure, including conjunctiva, lacrimal gland, eyelid skin, and orbit. Among them, conjunctival biopsy has been suggested as a sensible and safe procedure for confirming suspected sarcoidosis. We describe three patients in whom ocular symptoms were the sole initial manifestations of sarcoidosis and who were diagnosed based on the results of a conjunctival biopsy. We also discuss the efficacy of and indications for conjunctival biopsy.     

Conjunctival biopsy in sarcoidosis

In a prospective study of systemic sarcoidosis, 28 patients underwent 55 conjunctival biopsies. The incidences of positive granulomatous lesions in proven and suspected sarcoidosis cases were 71.4% (ten of 14 patients) and 28.5% (four of 14 patients), respectively. Thus, random, bilateral conjunctival biopsies should be performed as soon as possible in all cases of presumed sarcoidosis.     

Eleven cases of sarcoidosis of the optic nerve

Of 11 patients (eight women and three men, ranging in age from 16 to 48 years) who had sarcoidosis of the optic nerve that caused decreased visual acuity and visual field abnormalities, only two were known to have sarcoidosis at the time the visual impairment developed. Four patients had granulomas involving the optic nerve head, four had granulomatous inflammation of the orbital or intracranial optic nerve or chiasm, and three had retrobulbar neuritis. All 11 patients had histologically confirmed idiopathic noncaseating granulomatous inflammation and eight of the 11 had abnormalities compatible with sarcoidosis in chest roentgenograms. In the three patients in whom the serum level of angiotensin-converting enzyme was determined, it was increased in one and normal in the other two. Computed tomography of the anterior visual pathways was the single most useful neurodiagnostic study. Treatment with corticosteroids was beneficial in six of the 11 cases. These cases demonstrated that sarcoidosis should be included in the differential diagnosis of any inflammatory or compressive lesion involving the anterior visual pathways.