Diagnosis Of Ocular Sarcoidosis Research Articles

The Inflammatory Dilemma: A Unique Case of Ocular Sarcoidosis and The Road to Diagnosis and Management

Introduction Sarcoidosis is an inflammatory condition that affects several organ systems. The eye is affected in approximately 30-60% of people with sarcoidosis, with uveitis being the most frequent ocular manifestation.1 The diagnosis of ocular sarcoidosis in the absence of systemic manifestations is challenging due to it mimicking other uveitic conditions, and lab work often being non-specific. Case Report A 38-year-old African American male presented with a chief complaint of blurry vision and a sensation of pressure behind his left eye, denying pain. Bilateral anterior non-granulomatous uveitis was diagnosed and treated accordingly. The patient had several episodes of recurrent uveitis prompting a full infectious and inflammatory work-up. He subsequently suffered from systemic-related symptoms and was eventually diagnosed with sarcoidosis based on lung biopsy and neuroimaging. Ocular history, imaging, lab-work, and a multidisciplinary approach was used to confirm the diagnosis of sarcoidosis. Conclusion Sarcoidosis is a condition which predominantly affects the lungs and can have extrapulmonary manifestations. Any part of the eye and adnexa can be involved either in isolation or simultaneously, with uveitis being the most common manifestation. The goal of treatment of sarcoid associated uveitis is controlling the inflammation to prevent permanent ocular damage and vision reduction sequelae. Patients with ocular sarcoidosis should be monitored by their primary care doctor and referred to the appropriate specialties for possible systemic manifestations.

Ocular Sarcoidosis and Autoimmune Polyglandular Syndrome Type 2: A Case Report

ABSTRACT Purpose to describe a clinical case of ocular sarcoidosis in a patient with Autoimmune Polyglandular Syndrome Type 2 (APS-2). Methods an 86-year-old female diagnosed with APS-2 was referred to our uveitis department with rapid visual loss in her left eye during a 3-month period. Her best-corrected visual acuity (BCVA) was counting fingers in her left eye (OS) and 20/40 in her right eye (OD). Slit-lamp biomicroscopy was unremarkable OD but revealed granulomatous keratic precipitates OS. Fundoscopy revealed bilateral optic disc oedema and +2 and 4+ vitritis (SUN classification) in her OD and OS, respectively. Results the patient underwent chest X-Ray which revealed bilateral hilar lymphadenopathy and fibrosis. On high-resolution computed tomography of the lungs, ground-glass opacities were visible, and a diagnosis of ocular sarcoidosis was presumed. After exclusion of infectious diseases, the patient was treated with methotrexate and oral corticosteroids and there was substantial improvement of the optic nerve oedema and vitritis. At the most recent visit, 2 years later, OS BCVA was 20/50. Conclusion There may be an association between ocular sarcoidosis and APS or other autoimmune disorders.

Unusual Corneal Sarcoidosis Manifestations

ABSTRACT Aim To document atypical presenting forms of ocular sarcoidosis at the corneal level. Methods Case report. Results A 63-year-old woman presented multiple uncommon unilateral primary corneal conditions as manifestation of ocular sarcoidosis, including peripheral ulcerative keratitis, sterile corneal infiltrate (corneal granuloma), and sterile infiltrates related to a corneal foreign body, requiring medical and surgical management to control the inflammatory symptoms and to preserve the integrity of the eyeball. An excisional biopsy of a nodule in the temporal conjunctiva was performed under topical anesthesia. Histological analysis revealed a non-caseating granuloma, confirming the diagnosis of ocular sarcoidosis. Conclusion When thinking of ocular involvement in patients with ocular sarcoidosis, it is essential to remember that manifestations such as peripheral ulcerative keratitis, sterile corneal infiltrate, and sterile foreign body-related infiltrates may be presentations of this disease.

Progress in the diagnosis of ocular sarcoidosis.

Sarcoidosis is a multisystem granulomatous inflammation that affects multiple organ systems. The spectrum of extraocular and ocular involvement is wide and may precede systemic involvement. The diagnosis of ocular sarcoidosis relies on a combination of clinical findings, laboratory investigations, and radiographic findings. These include but are not limited to serum angiotensin-converting enzyme (ACE), lysozyme, plain-film radiographs of the chest, computed tomography (CT) scans of the chest, pulmonary function testing, bronchoalveolar lavage, and retinal imaging among others. In this review, we highlight current and evolving systemic investigations and approaches to ophthalmic imaging when considering the diagnosis of ocular sarcoidosis.

Lymphocyte Immunophenotyping and CD4/CD8 Ratio in Cerebrospinal Fluid for the Diagnosis of Sarcoidosis-related Uveitis

ABSTRACT Background: This study aimed to assess the diagnostic relevance of CD4/CD8 ratio in cerebrospinal fluid (CSF) for the etiological diagnosis work-up of uveitis. Methods: We consecutively included patients who were referred to our department for the diagnostic workup of intermediate and/or posterior uveitis. Etiological diagnoses were established in a blind manner regarding CD4/CD8 ratio. Results: Fifty-two patients were included. A diagnosis of ocular sarcoidosis was made in 15 (29%) patients, 21% had another determined diagnosis while 50% remained of undetermined origin. Median CD4/CD8 ratio in CSF was 4.57 (IQR 3.39–5.47) in ocular sarcoidosis, 1.74 (1.60–3.18) in uveitis due to other determined cause (P = .008), and 2.83 (2.34–3.54) in those with uveitis of undetermined origin (P = .007). CD4/CD8 ratio >3.23 was associated with a diagnosis of ocular sarcoidosis. Conclusion: Determination of CD4/CD8 ratio in CSF can be useful for diagnosis work-up since a CD4/CD8 ratio >3.23 in CSF is associated with ocular sarcoidosis.

Ophthalmic and neuro-ophthalmic manifestations of sarcoidosis.

Sarcoidosis is a multisystemic inflammatory disease that commonly affects the eye and less often the neuro-ophthalmic pathways. The manifestations can be quite variable but can have characteristic signs and clinical features. This review provides a comprehensive overview of the various ocular and neuro-ophthalmic manifestations of sarcoidosis, emerging diagnostic measures and approach to treatment. Particular focus is given to recent advances in diagnostic approach and available treatment options. Laboratory investigations, chest and nuclear medicine imaging remain important techniques for helping to diagnose sarcoidosis. Recent evidence suggests a role for aqueous humor analysis in the diagnosis of ocular sarcoidosis. Characteristic neuroimaging may help differentiate neurosarcoidosis from other causes. The role of blind conjunctival biopsy for suspected neurosarcoidosis is discussed. The emerging role and use of biologics is delineated for the treatment of both ocular and neuro-ophthalmic sarcoidosis. Sarcoidosis can affect any part of the visual system: the most common ocular manifestation is uveitis and the most common neuro-ophthalmic manifestation is optic neuropathy. Although diagnosis remains challenging, recent advancements in diagnosis are promising. Emerging biologics with particular efficacy for ocular and neuro-ophthalmic disease provide expanding treatment options for sight-threatening disease.

High Resolution Chest Computerized Tomography in the Diagnosis of Ocular Sarcoidosis in a High TB Endemic Population

ABSTRACTPurpose: To review the role of high resolution chest computed tomography (HRCT) in ocular sarcoidosis in a high TB endemic population.Methods: This was a retrospective study.Results: Out of 140 cases, 54 had ocular sarcoidosis, while 86 cases had ocular tuberculosis. Abnormal HRCT findings was noted in 52 cases (96.3%) of ocular sarcoidosis compared with 55 cases (64.7%) of ocular tuberculosis (p = 0.001). Mediastinal lymphadenopathy was the most common finding in both groups (p = 0.544). Hilar lymphadenopathy and fissural nodules were significantly seen in ocular sarcoidosis (p = 0.001). Necrosis was seen in three cases of ocular sarcoidosis. In nearly half of the cases, it was not possible to differentiate between sarcoidosis and tuberculosis on HRCT.Conclusions: HRCT is a useful diagnostic tool in ocular sarcoidosis. Bilateral hilar lymphadenopathy and fissural nodules are significant findings in ocular sarcoidosis. A confident diagnosis of ocular sarcoidosis is made by the amalgamation of results of clinical, radiologic, and other laboratory investigations.

Directed conjunctival biopsy and impact of histologic sectioning methodology on the diagnosis of ocular sarcoidosis

BackgroundSarcoidosis is an idiopathic, multi-system, granulomatous disease with well-described ocular manifestations. However, other uveitic etiologies can manifest in a similar fashion, and ocular disease may precede systemic manifestations. Definitive diagnosis requires histologic confirmation of non-caseating granulomatous inflammation. This study reports the diagnostic yield of directed biopsy of conjunctival follicles in patients with uveitis suspected to be secondary to sarcoidosis, and compares an institutional standard tissue sectioning method to a multi-plane technique.ResultsA retrospective analysis was performed of all patients who underwent directed conjunctival biopsy for suspected ocular sarcoidosis. A total of eight patients were identified; all were females. Directed conjunctival biopsy was positive in three of seven patients using standard histologic processing method, a yield of 43%. Using the multi-plane technique increased the cumulative yield to 63%.ConclusionsDirected conjunctival biopsy is a minimally invasive, cost-effective, and moderately high yield method of diagnosing ocular sarcoidosis. Using a multi-plane sectioning method may increase biopsy yield when standard sectioning techniques are negative.

眼サルコイドーシス国際診断基準−眼科医による眼所見からのサルコイドーシスの診断

近年，全国の大学付属病院におけるぶどう膜炎（内眼炎）の統計（2003 年）では，サルコイドーシスは原因疾患として約13％を占め，以前より増加していることが明らかになった1）．眼科医のあいだでのサルコイドーシス診断基準の浸透とともに，眼科で検査をオーダーして診断をつけることが増えたためと考えられる．2006 年に診断基準の改訂が行われ，「サルコイドーシスの診断基準と診断の手引き−2006 」が作成された2）．同年，眼科医がサルコイドーシスを診断できるような国際診断基準を定めるためのワークショップが東京で開かれた．北米，ヨーロッパ，アジア各国のぶどう膜炎を専門とする眼科医が集まり，各国のサルコイドーシス事情を紹介したのち，日本の診断基準をたたき台にして，出席者（内科医も参加）の話し合いにより眼所見，検査所見，診断法を決め，「International Criteria for the Diagnosis of Ocular Sarcoidosis」（以下，国際診断基準）を作成した3）．

Review for Disease of the Year: Differential Diagnosis of Ocular Sarcoidosis

Sarcoid is characterized by the heterogeneity of its clinical expression as well as the silent nature and nonspecificity of many of its clinical findings. Diagnosis is often difficult due to its ability to mimic other diseases. In the absence of a highly specific confirmatory test, one must be cognizant of the features that distinguish these simulating conditions from sarcoidosis. Establishing an appropriate differential for patients suspected of having sarcoid requires one to consider the entire clinical presentation, the presence or absence of risk factors for masquerading disorders, patterns of resolution or progression, and response to treatment. This paper provides an overview of ocular conditions that may share part of the spectrum of clinical manifestations associated with sarcoid.

Ocular Sarcoidosis

Ocular disease occurs in approximately a third of sarcoidosis patients. The rate of disease varies around the world, with Japanese sarcoidosis patients having ocular disease in more than 70% of cases. If untreated, ocular disease can lead to permanent visual impairment, including blindness. The most common manifestation is uveitis, with anterior involvement often being self-limiting, whereas posterior involvement can be chronic. The diagnosis of ocular sarcoidosis in patients with known sarcoidosis usually requires a specific examination by an ophthalmologist. For patients presenting with uveitis of unknown etiology, criteria have been proposed for diagnosing ocular sarcoidosis. The treatment of ocular disease ranges from topical therapy to systemic treatments such as methotrexate. Recent reports have demonstrated that monoclonal antibodies blocking tumor necrosis factor can be quite effective for chronic refractory ocular sarcoidosis.

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.

Ocular sarcoidosis in Kuwait with a review of literature.

To study 18 cases of sarcoidosis, the hall mark of which was uveitis. Referrals from peripheral eye clinics. 18 patients with bilateral almost symmetric uveitis, negative Mantoux test and positive gallium scan were enrolled in the study. Preliminary bronchoscopy and bronchial lavage were not done due to social habits. Age ranged between 7-48 years with median 15 and mean 19 years. Although the patients were multinational, yet all of them were residents of Kuwait. A father and son were affected within 8 months period. 78% were strictly ocular, associated pulmonary and salivary gland affection 11% each. Sole anterior uveitis was found in 28%, associated with intermediate uveitis in 55% and with posterior in 16.6% of cases. Clinically detectable dry eyes associated 33% of cases. All our patients developed glaucoma which resolved with treatment of uveitis in 88% of them. Gallium uptake of the eye balls was found in 22%, of the lacrimal glands (panda sign) in 67%, and of the salivary glands or chest 11% each. Positive biopsy was found in 72%, 22% of which was conjunctival. Chest X-ray and SACE were positive in 11%. 61% had hypergammaglobulinaemia and all had negative ANA and RF. (1) 61% of ocular sarcoidosis presented below sarcoid age. (2) Multinationality together with father's and son's affection indicate a climatic or environmental insult in an already predisposed person. (3) Routine chest X-ray and SACE may not be adequate for diagnosis of ocular sarcoidosis. Gallium should be done in suspected cases. (4) Follow-up for prospects is emphasized.

Bronchoalveolar lavage studies in uveitis patients without radiological intrathoracic involvement of sarcoidosis.

In the present study, we investigated thirty uveitis patients who had a possible diagnosis of ocular sarcoidosis, no respiratory symptoms, and normal chest X-rays, in order to determine whether bronchoalveolar lavage was useful for establishing a diagnosis of subclinical pulmonary involvement of sarcoidosis. Sixteen of 30 uveitis patients showed bronchoalveolar lymphocytosis (39 +/- 11%) as compared to normal controls (14 +/- 3%). Ten of these 16 patients were associated with significantly increased proportions of CD3+ cells with elevated CD4+/CD8+ ratios (P less than 0.01). These data were consistent with those of patients with active pulmonary sarcoidosis (stage 1 and 2) except for total cell count in lavage fluid, but different from those of patients with hypersensitivity pneumonitis. Transbronchial lung biopsy showed noncaseating epithelioid cell granuloma in 4 of 10 uveitis patients with bronchoalveolar T-lymphocytosis and increased CD4+/CD8+ ratios. Immunohistochemical studies of biopsied lung tissues showed many CD3+ cells and CD4+ cells with very few CD8+ cells in the granuloma. These results suggest that T-lymphocytosis with elevated CD4+/CD8+ ratios in bronchoalveolar lavage fluid could be a good marker in predicting the pulmonary involvement of sarcoidosis in patients with suspected ocular sarcoidosis and normal chest X-rays.