Intraocular Sarcoidosis Research Articles

When and which second-line workup to perform for uveitis—A tertiary care center experience

ObjectiveUveitis are inflammatory disorders of various etiologies. The first-line etiological workup is currently poorly codified, and some patients undergo sequential investigations. However, what leads the clinician to perform subsequent exams and the relevance of such exams remain to be determined. The main objective of the study was to evaluate the relevance and modalities of a second-line workup of patients with uveitis. Materials and MethodsWe performed a monocentric retrospective study in Nancy University Hospital. All adult patients who underwent an etiological workup in the Internal Medicine Department between January 2014 and December 2021 were included. ResultsAmong the 247 patients included, 52 underwent a second-line workup, resulting in a modified diagnosis for 18 of them (34.6%), mainly sarcoidosis, intraocular lymphoma, and Crohn's disease. On multivariate analysis, a follow-up longer than 40 months and idiopathic uveitis were associated with the realization of a second-line workup (OR = 2.97 [1.58 – 5.61]; p = 0.001, and OR = 6.13 [2.3–16.1]; p < 0.01, respectively). The presence of synechia and ocular granuloma were associated with a modification of the diagnosis (OR = 8.03 [1.85–45.48]; p = 0.01, and OR = 5.14 [1.22–24.78]; p = 0.03, respectively). ConclusionThe second-line workup is relevant in up to one-third of patients, mainly if presenting with a modification of ophthalmological examination, synechiae, and a granulomatous feature, and should focus on intraocular lymphoma, sarcoidosis, and Crohn's disease. Larger studies are needed to provide guidelines for second-line workup.

The Role of 18F-FDG PET/CT in Suspected Intraocular Sarcoidosis and Tuberculosis

ABSTRACT Purpose: To determine the utility of fluorine-18 fluorodeoxyglucose Positron Emission Tomography Computed Tomography (FDG PET/CT) in patients with suspected ocular sarcoidosis (OS) or intraocular tuberculosis (IOTB) in a resource-constrained, TB endemic area. Methods: Independent review of the FDG PET/CTs, Computed Tomography (CT) scans and chest radiographs (CXRs) of patients with suspected OS or IOTB and inconclusive conventional workup. Results: Twenty-nine PET/CTs and CXRs were reviewed, with 38% of PET/CTs and CTs demonstrating evidence of TB or sarcoidosis, compared to 21% of CXRs. The sensitivity, specificity, positive and negative predictive values for PET/CT and CT were similar - 85.7%, 95.5%, 85.7% and 95.5% for OS, and 33.3%, 100%, 100% and 68% for IOTB respectively and for CXR, 57.1%, 100%, 100% and 88% for OS, and 16.7%, 100%, 100% and 63% for IOTB. Conclusion: PET/CT added no significant additional benefit over Chest CT in patients with suspected OS or IOTB.

18F-fluorodeoxyglucose positron emission tomography is useful for the diagnosis of intraocular sarcoidosis in patients with a normal CT scan

AimTo assess the usefulness of 18F-fluorodeoxyglucose positron emission tomography (18F-FDG PET/CT) and the predictive factors for the diagnosis of sarcoidosis in patients with uveitis who have normal thoracic tomography.MethodsWe retrospectively...

Utility of routine chest radiography in ocular tuberculosis and sarcoidosis.

Tuberculosis (TB) and sarcoidosis commonly present with pulmonary and ocular involvement. Routine chest radiography (CXR) is recommended in the workup for suspected intraocular TB (IOTB) or intraocular sarcoidosis (IOS); however, data on the utility of CXR in this setting are lacking. A post-hoc analysis was performed of a prospectively collected data set comprising 104 patients with uveitis of unknown cause. A pulmonologist and thoracic radiologist, blinded to the final diagnosis, independently reported these CXRs as being in keeping with TB or sarcoidosis. CXRs were reported as normal/indeterminate (n = 88), probable/previous TB (n = 9) or possible/probable sarcoidosis (n = 8), with a 96% inter-observer concordance. CXRs were more often abnormal in IOS than in IOTB (5/8 vs. 5/34, P = 0.01). CXR had a sensitivity of 14.7%, specificity of 94.3%, positive predictive value (PPV) of 55.6% and negative predictive value (NPV) of 69.5% for IOTB, compared with a sensitivity of 62.5%, specificity of 96.9%, PPV of 62.5% and NPV of 96.9% for IOS. Overall diagnostic accuracy was 54.5% (58.1% in human immunodeficiency virus [HIV] positive participants) in the case of IOTB and 79.9% for IOS. CXR had high specificity and NPV for IOS, and poor overall diagnostic accuracy for IOTB, including in the HIV-positive population.

Diagnosis of intraocular lesions using vitreous humor and intraocular perfusion fluid cytology: Experience with 83 Cases.

The objective of the study was to evaluate the use of vitreous humor and/or intraocular perfusion fluid (IPF) from pars plana vitrectomy as a diagnostic and therapeutic procedure for intraocular diseases. The cytologic findings with respect to the clinical data, the anatomical findings, and the final diagnosis in 83 cases that underwent intraocular cytologic examinations at the Kansai Medical University Takii Hospital were evaluated. For cytologic examination, the Papanicolaou stain, Giemsa stain, and in some cases, molecular biology and immunocytochemical techniques were used. Most of the clinical diagnoses were uveitis or endophthalmitis. Sixty-eight cases (81.9%) were negative on cytodiagnosis, while 15 cases (18.9%) were positive or suspicious for malignancy. Negative cases included infections and intraocular sarcoidosis (IOS), and all of the positive or suspicious cases were intraocular lymphomas. Some of these latter cases were also diagnosed using immunocytochemical staining or molecular biological procedures as ancillary techniques, performed using vitreous body cytology from IPF. An early diagnosis and treatment of intraocular diseases is necessary to maintain an acceptable degree of quality of life. For an accurate diagnosis, it is necessary to understand the anatomy of the eye. Giemsa staining is recommended in addition to Papanicolaou staining for cytologic diagnostic evaluation of intraocular diseases. Furthermore, for the diagnosis of clonality in intraocular lymphomas, it is often necessary to use ancillary molecular biological procedures, using vitreous fluid.

Intraocular sarcoidosis: association of clinical characteristics of uveitis with findings from 18F-labelled fluorodeoxyglucose positron emission tomography

AimsTo assess specific clinical criteria in patients with uveitis that are related to signs of sarcoidosis on 18F-labelled fluorodeoxyglucose positron emission tomography (18F-FDG-PET).MethodsRetrospective study of 54 consecutive patients with chronic...

Systemic Treatment of Sarcoidosis

Purpose: To compare the evidence base and systemic treatment strategies for sarcoidosis.Methods: Medline and EMBASE literature search on “sarcoidosis AND treatment”, “sarcoidosis AND uveitis AND treatment”, and “sarcoidosis AND eye AND treatment”. The search was limited to randomized controlled trials (RCTs) and meta-analyses.Results: A total of 19 RCTs for the systemic treatment of extraocular sarcoidosis were identified. The majority were on corticosteroid—oral and inhaled. There were two meta-analyses on corticosteroid, including a Cochrane review. Only two RCTs were indentified for the treatment of intraocular sarcoidosis, one on etanercept, and the other from 1967 on prednisolone or oxyphenbutazone vs. placebo. There were no meta-analyses. Due to the paucity of RCTs other treatment studies were included but these were limited to only a few immunosuppressive agents and on small numbers of patients.Conclusion: Limited high-quality evidence exists for the systemic treatment of sarcoidosis, in particular intraocular disease.

Diagnostic utility of vitreous humor fluid cytology for intraocular sarcoidosis: A clinicopathologic study of 7 cases

Sarcoidosis is an enigmatic multisystem sarcoid disease occurring mainly in the lungs, skin, and eyes. Sarcoidosis of the eyes (intraocular sarcoidosis) has been proposed as one of the causes of uveitis. We carried out a clinicopathological study involving seven patients with intraocular sarcoidosis based on vitreous humor fluid cytology. In the seven patients, the sex ratio was 4:3, and the average age at onset was 65.6-years-old. Vitreous opacity was noted in all patients and snowball-like vitreous opacity in 85.7%. Nerve papilloedema was noted in 42.9%. Diagnostic vitrectomy and adrenocortical hormone therapy were performed in all patients. As a result, the symptoms were improved. Vitreous perfusion fluid cytology revealed multinucleate giant cells in 85.7% and lymphocytes and epithelioid cells in all cases. Cytological diagnosis is not included in the current criteria for sarcoidosis, but its value in the diagnosis of intraocular sarcoidosis was suggested based in present study.

Uvéites sarcoïdosiques : actualités diagnostiques et thérapeutiques

Uveitis is a common (20-50%) and early manifestation of sarcoidosis. Typical sarcoid uveitis presents with bilateral mutton fat keratic precipitates, iris nodules, and anterior and posterior synechia. Posterior involvement includes vitreitis, vasculitis and choroidal lesions. Long-term complications are common, and cystoid macular edema is the most important and sight-threatening consequence. Diagnostic work-up of sarcoidosis usually includes chest radiography or computed tomography scan, bronchoscopy with bronchoalveolar lavage, angiotensin converting enzyme, lysozyme, gallium scintigraphy and biopsy. The gold standard for the diagnosis of sarcoidosis should be obtained with histologic examination. However, an international workshop has recently established diagnostic criteria of "intraocular sarcoidosis" (sarcoidosis uveitis) on the basis of a combination of suggestive ophthalmological findings and laboratory tests, when biopsy is not performed or is negative. More recent techniques such as PET-scan and endoscopic ultrasound-guided fine-needle biopsy of intrathoracic nodes should be assessed in future prospective studies. Corticosteroids are the mainstay of treatment. Anterior and unilateral intermediate or posterior uveitis are usually treated with topical corticosteroids. Systemic corticosteroids are indicated in uveitis not responding to topical corticosteroids or in the presence of bilateral posterior involvement, especially with macular edema and occlusive vasculitis. In 5 to 20% of the patients who are corticosteroids resistant or require an unacceptable dose to maintain remission, additional immunosuppression is used, including methotrexate, leflunomide and mycophenolate mofetil. As in systemic sarcoidosis, infliximab has been recently suggested for refractory or sight threatening disease.

Intraocular sarcoidosis: association of clinical characteristics of uveitis with positive chest high-resolution computed tomography findings

AimTo assess specific clinical criteria in patients with uveitis that are related to signs of sarcoidosis on high-resolution computed tomography (HRCT) of the chest.MethodsRetrospective study of 50 consecutive patients with...

International Criteria for the Diagnosis of Ocular Sarcoidosis: Results of the First International Workshop on Ocular Sarcoidosis (IWOS)

Aim: To report criteria for the diagnosis of intraocular sarcoidosis, taking into account suggestive clinical signs and appropriate laboratory investigations and biopsy results. Design: Concensus workshop of an international committee on nomenclature. Methods: An international group of uveitis specialists from Asia, Africa, Europe, and America met in a concensus conference in Shinagawa, Tokyo on October 28–29, 2006. Based on questionnaires that had been sent out prior to the conference, the participants discussed potential intraocular clinical signs eligible for a diagnosis of ocular sarcoidosis. A refined definition of clinical signs, which received two-thirds majority of votes, was included in the list of signs consistent with ocular sarcoidosis. Laboratory investigations were similarly discussed and those tests reaching a two-thirds majority were retained for the diagnosis of ocular sarcoidosis. Finally diagnostic criteria were proposed based on ocular signs, laboratory investigations, and biopsy results. Results: The concensus conference identified seven signs in the diagnosis of intraocular sarcoidosis: (1) mutton-fat keratic precipitates (KPs)/small granulomatous KPs and/or iris nodules (Koeppe/Busacca), (2) trabecular meshwork (TM) nodules and/or tent-shaped peripheral anterior synechiae (PAS), (3) vitreous opacities displaying snowballs/strings of pearls, (4) multiple chorioretinal peripheral lesions (active and/or atrophic), (5) nodular and/or segmental peri-phlebitis (± candlewax drippings) and/or retinal macroaneurism in an inflamed eye, 6) optic disc nodule(s)/granuloma(s) and/or solitary choroidal nodule, and (7) bilaterality. The laboratory investigations or investigational procedures that were judged to provide value in the diagnosis of ocular sarcoidosis in patients having the above intraocular signs included (1) negative tuberculin skin test in a BCG-vaccinated patient or in a patient having had a positive tuberculin skin test previously, (2) elevated serum angiotensin converting enzyme (ACE) levels and/or elevated serum lysozyme, (3) chest x-ray revealing bilateral hilar lymphadenopathy (BHL), (4) abnormal liver enzyme tests, and (5) chest CT scan in patients with a negative chest x-ray result. Four levels of certainty for the diagnosis of ocular sarcoidosis (diagnostic criteria) were recommended in patients in whom other possible causes of uveitis had been excluded: (1) biopsy-supported diagnosis with a compatible uveitis was labeled as definite ocular sarcoidosis; (2) if biopsy was not done but chest x-ray was positive showing BHL associated with a compatible uveitis, the condition was labeled as presumed ocular sarcoidosis; (3) if biopsy was not done and the chest x-ray did not show BHL but there were 3 of the above intraocular signs and 2 positive laboratory tests, the condition was labeled as probable ocular sarcoidosis; and (4) if lung biopsy was done and the result was negative but at least 4 of the above signs and 2 positive laboratory investigations were present, the condition was labeled as possible ocular sarcoidosis. Conclusion: Various clinical signs, laboratory investigations, and biopsy results provided four diagnostic categories of sarcoid uveitis. The categorization allows prospective multinational clinical trials to be conducted using a standardized nomenclature, which serves as a platform for comparison of visual outcomes with various therapeutic modalities.

Sarcoid Cyclitis

Fifty-two patients with ocular sarcoidosis were studied to determine how intraocular tissues are affected in this disease. Of 102 eyes, 36 (35.3%) had iris nodules, 50 (49.0%) demonstrated nodules in the anterior chamber angle, 42 (41.2%) showed ciliary nodules, 37 (36.3%) had snowball vitreous opacities, and 21(20.6%) exhibited candlewax drippings on the retinal vessels. Special note was made of sarcoid changes in the ciliary processes using cycloscopy. Of the total 102 eyes, nodules in the ciliary processes were most predominant in 42 (41.2%), waxy exudates were observed in 25 (24.5%), cyclitic membrane-like exudates were seen in the posterior chamber, in 3 (2.9%), and swelling of the ciliary processes occurred in 12 (11.8%). There were no observable changes in 20 eyes (19.6%). It is remarkable that these changes occur in 80.4% of eyes with intraocular sarcoidosis.