Granulomatous Uveitis Research Articles

Early-onset granulomatous arthritis, uveitis and skin rash: characterization of skin involvement in Blau syndrome.

Blau syndrome (BS) is a rare monogenic autoinflammatory disease caused by NOD2 mutations. BS classically presents in early childhood as a triad of granulomatous polyarthritis, uveitis and skin involvement. Joint and ocular involvement have been characterized by several cohort studies but only very little data are available on skin lesions. We aimed to provide a detailed clinical and microscopic analysis of skin manifestations and to study whether they may contribute to an early diagnosis. We conducted a retrospective multicentre study in a French cohort of 21 patients diagnosed with genetically confirmed BS. Skin involvement was the first clinical manifestation of BS in 15/16 patients with dermatological manifestations. The presence of skin lesions was associated with significant shorter age at diagnosis (P=0.03) and diagnostic delay (P=0.04). Dermatological assessment allowed an earlier diagnosis (P=0.001) and reduces the diagnostic delay (P=0.007). Early skin lesions had a homogeneous, stereotypical clinical presentation, namely non-confluent erythematous or pigmented millimetric papules in 13/14(93%) patients. In contrast, skin lesions occurring during later disease stages had a more heterogeneous clinical presentation, including ichthyosiform dermatosis, panniculitis, livedoid lesions and vasculitis. Whatever their time of occurrence and the clinical aspect, all biopsied showed histologically presence of granuloma. Skin involvement in BS is the earliest clinical manifestation of the BS in the large majority of patients. The recognition of dermatological manifestations as granulomatous skin lesions and early dermatological expertise are the key to an early diagnosis of BS. In view of our results, it seems reasonable to propose a simplified view of skin lesions of BS in which the granuloma is the key structure.

Severe Hypotony Maculopathy in Anterior Uveitis Associated with Hodgkin Lymphoma

ABSTRACT Purpose: To describe the clinical course and management of anterior uveitis complicated by ocular hypotony associated with Hodgkin lymphoma. Design: Case report. Methods: Chart and multimodal imaging review, including ultrasound biomicroscopy, widefield fundus pictures, fundus autofluorescence, fluorescein angiography, and indocyanine green angiography. Results: A 44-year-old female with progressive visual deterioration and history of low-grade fever developed bilateral granulomatous anterior uveitis complicated by severe hypotony maculopathy, not improving with systemic and topical steroids. After starting ibopamine 2% eye drops, ocular hypotony progressively resolved with visual recovery. Histologic examination of a biopsied enlarged lymph node of the neck revealed the presence of Hodgkin lymphoma, for which the patient underwent systemic chemotherapy. Conclusion: Severe hypotony maculopathy complicating anterior uveitis can be associated with Hodgkin lymphoma. Topical ipobamine 2% was safe and effective in the treatment of ocular hypotony in this case.

P42 Delayed diagnosis in paediatric sarcoidosis: lessons we can learn

Abstract Background Sarcoidosis is a rare autoinflammatory systemic granulomatous disease that can affect any organ in the body. Paediatric literature does not give a prevalence of neurosarcoidosis. Clinical features can be tricky to identify and piece together to suggest the diagnosis and biochemical markers such as serum angiotensin converting enzyme lack sensitivity. Methods This case report of a 13 year-old boy demonstrates his journey to diagnosis as he bounced between multiple private and NHS care teams. Referral was to our paediatric rheumatology department with a single oesophageal granuloma and bilateral uveitis. Symptoms include nausea, vomiting, weight loss which was regained, fatigue, disturbed sleep, chronic dry cough, then worsening headaches. He had been treated privately with steroid eye drops for bilateral uveitis. Histology of the oesophageal granuloma showed no acute inflammation. A private paediatric rheumatologist review concluded idiopathic uveitis with no underlying systemic disease that could be delineated at that time. A private haematologist subsequently diagnosed him with a chronic systemic inflammatory process leading to an iron deficient anaemia. Results He represented to his ophthalmologist with a 3 week history of worsening headaches and was found to have papilloedema. He was urgently jointly reviewed in the NHS setting by a paediatric gastroenterologist, neurologist and rheumatologist. Clinically apart from the bilateral pappiloedema there were no signs of disease. Urgent MRI demonstrated widespread inflammation and oedema in both cerebellar hemispheres and within his white matter bilaterally, as well as some cortical patches. Magnetic resonance arteriogram and magnetic resonance venogram were normal. Tonsillar herniation and ventricular tightness due to raised intracranial pressure was noted. He was admitted urgently. Bloods showed normal inflammatory markers, iron deficient anaemia, ANCA negative, ACE 30 iu/L, Quantiferon negative. CXR was normal as were pulmonary function tests. A raised urinary calcium with raised calcium:creatinine ratio led to a renal tract ultrasound study that showed early nephrocalcinosis bilaterally. Ophthalmology review demonstrated granulomatous pan uveitis with macular oedema. NOD2 genetic testing was negative. Conclusion Neurosarcoidosis was diagnosed. He was treated with 2mg/kg IV Methylprednisolone over 3 consecutive days then a slow prednisolone wean starting at 60mg per day orally and 6 times a day Prednisolone eye drops. Weekly methotrexate 15mg subcutaneously and 5mg folic acid were started followed shortly by subcutaneous Adalimumab 40mg fortnightly. Repeated MRI brain scans after treatment commenced showed resolution of cerebral inflammation. Follow up ophthalmology reviews demonstrate resolution of his uveitis. Bilateral nephrocalcinosis has persisted despite normalised serum and urinary calcium. It was not until he was seen by an ophthalmologist with expertise in both areas that the severity and nature of the uveitis including it being granulomatous was picked up. The diagnosis was delayed due to the lack of joined up working between private and NHS sectors. Conflicts of Interest The authors declare no conflicts of interest.

Local Cytokine Expression Profiling in Patients with Specific Autoimmune Uveitic Entities

ABSTRACTPurpose: To evaluate expression of cytokines GM-CSF, IL-11, IL-12p40, IL-12p70, IL-27p28, IL-35, APRIL, BAFF, TWEAK, and LIGHT in uveitis.Methods: Aqueous humor samples from patients with active uveitis associated with Behçet’s disease (BD), sarcoidosis, HLA-B27-related inflammation, and Vogt-Koyanagi-Harada (VKH) disease and control patients were assayed with a multiplex assay.Results: Comparing all patients to controls, GM-CSF, IL-11, IL-12p40, APRIL, and BAFF were significantly increased, whereas LIGHT was significantly decreased. IL-11 and BAFF were the most strongly upregulated, being elevated 19.7-fold and 14.1-fold, respectively, compared with controls. IL-11 was significantly highest in HLA-B27 uveitis. GM-CSF, IL-11, and IL-12p40 were significantly higher in nongranulomatous uveitis (BD and HLA-B27) than in granulomatous uveitis (sarcoidosis and VKH), whereas APRIL and TWEAK were significantly higher in granulomatous uveitis.Conclusions: IL-11-driven immune responses might be more potent in nongranulomatous uveitis, particularly in HLA-B27 uveitis. BAFF and APRIL might contribute to B cell-driven autoimmune response in uveitis.

Granuloma-like formation in deeper retinal plexus in ocular sarcoidosis.

Sarcoidosis is a multi-system disease characterized by the presence of non-caseating epithelioid granulomas in affected tissues and the most frequent cause of noninfectious uveitis in Japan. Ocular manifestations of sarcoid uveitis vary enormously and commonly characterized by granulomatous anterior uveitis, intermediate uveitis, and posterior uveitis such as retinal periphlebitis, choroiditis, optic nerve granuloma and papillitis. However, case reports of multiple granuloma formation related to sarcoid uveitis is uncommon. This report describes the use of optical coherence tomography angiography to visualize changes occurring in granuloma formation in deeper retinal plexus before and after systemic corticosteroid treatment.

Comparison of Clinical Features and Visual Outcome between Sympathetic Ophthalmia and Vogt–Koyanagi–Harada Disease in Chinese Patients

To characterize the clinical features of sympathetic ophthalmia (SO) and compare SO and Vogt-Koyanagi-Harada (VKH) disease in Chinese patients. Retrospective case series. A total of 131 consecutive SO and 500 VKH disease patients randomly selected from among those referred to our uveitis center from April 2008 through June2018. History, extraocular and ocular findings, best-corrected visual acuity (BCVA), auxiliary examination findings, complications, and therapeutic effects were analyzed retrospectively in SO and VKH disease patients. Visual outcome, extraocular and ocular findings, and therapeutic effects. Sympathetic ophthalmia manifested as posterior uveitis (68.8%) within 2 weeks and equal involvement of anterior and posterior segment (44.4%), respectively, was observed between 2 weeks and 2 months after disease onset. Two months after disease onset, SO patients showed sunset glow fundus (51.2%) and granulomatous anterior uveitis (27.3%). Vogt-Koyanagi-Harada disease patients mainly showed posterior uveitis (100%), anterior segment involvement (92.4%) associated with posterior uveitis (84.9%), and granulomatous anterior uveitis (97.4%) accompanying sunset glow fundus (91.5%) in the 3 periods mentioned above. The frequencies of extraocular manifestations were lower in SO patients (24.4%) as compared with VKH disease patients (84.8%; P < 0.001). Best-corrected visual acuity of SO patients improved from 0.68±0.86 logarithm of the minimum angle of resolution (logMAR) to 0.47±0.78 logMAR (P= 0.01), and BCVA of VKH disease patients improved from 0.67±0.79 logMAR to 0.24±0.53 logMAR (P < 0.001) at 12 months of follow-up. A worse BCVA was noted in SO patients compared with VKH disease patients after treatment (P= 0.003). Kaplan-Meier survival analysis showed that the risk of loss of useful vision in SO patients was significantly higher than that of VKH disease patients (P < 0.001). Chinese SO and VKH disease patients have a different evolutionary process. The frequency of extraocular manifestations in SO patients is much lower as compared with VKH disease patients. Visual outcome is worse in SO as compared with VKH disease.

Sympathetic Ophthalmia after Vitreoretinal Surgeries: Incidence, Clinical Presentations and Outcomes of a Rare Disease

Aim: To evaluate clinical presentation, course and outcomes in patients without a history of penetrating ocular trauma who developed Sympathetic Ophthalmia (SO) following vitreoretinal surgeriesMethods: Retrospective review of clinical records of all patients diagnosed and treated as S.O was done . All cases without a previous history of trauma were included and were analyzed with respect to clinical presentations, anatomic and visual outcomes.Results: 175 cases of sympathetic ophthalmia were diagnosed and treated till June 2017. 16 of these cases had undergone a pars plana vitrecomy (PPV) in the past and had no history of prior ocular trauma. SO after vitreoetinal surgeries accounted for 9.14 percent of all cases of SO .In the same duration, till 2017,a total 41365 PPV were done. Thus 0.038 percent of PPV cases developed a SO . 10 patients were males and 6 were females. The median age at presentation was 45.7 years. The time interval from surgery to diagnosis of sympathetic ophthalmia ranged from 22 days to 4 years after undergoing a surgery. The mean visual acuity in the sympathizing eye was 1.26 logMAR (snellens equivalent of 20/320) which improved to 0.62 logMAR(snellens equivalent of 20/80) after treatment. The most common anterior segment finding was non granulomatous anterior uveitis, seen in 8 cases (50%) while neurosensory detachments were the most common posterior segment presentation (10 cases, 62.5%).12 patients had undergone more than 1 surgery (mean number of surgeries was 1.88). 10 patients had undergone a sutureless PPV (6 cases of 23 gauge and 4 cases of 25 gauge vitrectomy) while 4 patients had undergone a 20 gauge vitrectomy where all sclerotomies were sutured after surgery All patients were treated with systemic steroids and immunosuppresants and 15 out of 16 patients showed significant improvement in the final visual acuity in the sympathizing eyeConclusions: Sympathetic ophthalmia after vitreoretinal surgeries is a rare but potentially sight threatening disease occurring in 0.038 percent of all cases of Pars Plana Vitrectomy. Presence of inflammation in the fellow eye after a vitreoretinal surgery in the other eye should alert the surgeon to possibility of sympathetic ophthalmia

Treatment of presumed trematode-induced granulomatous anterior uveitis among children in rural areas of Egypt.

The purpose of this study is to evaluate the efficacy of systemic antiparasitic medications alone or in combination with surgical aspiration in management of presumed trematode-induced anterior uveitis in children. Prospective case series. Children who presented with anterior chamber (AC) granuloma were included in the study. All patients received antiparasitic treatment and after 2 weeks; patients were divided based on their clinical improvement in terms of the baseline granuloma area into two groups: group A (<2.5 mm2) who continued on antiparasitic medications only (n = 15) and group B (≥2.5 mm2) who underwent surgical aspiration (n = 15). Basic demographics data, visual acuity (VA), corneal thickness, granuloma area and AC activity (cells and flare) were recorded and analysed. Systemic work-up including stool and urine analysis, full blood count, chest X-ray and schistosomiasis titre were performed. Thirty eyes of 30 patients were included in the study with a mean age of 13.4 ± 2.42 years. All patients were male. Patients were examined and followed at Mansoura Ophthalmic Center, Mansoura University. Both groups showed statistically significant improvement in VA, AC activity, corneal thickness and granuloma area (p-value < 0.001), which was achieved with medical treatment only in group A. However, in group B granuloma required aspiration and did not recur after that. Presumed trematode-induced AC granuloma is common among children living in the rural areas of Egypt. Antiparasitic medication alone was found to be effective for small-sized granulomas. Surgical aspiration is an effective adjuvant procedure to treat large-sized ones.

Revised criteria of International Workshop on Ocular Sarcoidosis (IWOS) for the diagnosis of ocular sarcoidosis

AimsInternational criteria for the diagnosis of ocular sarcoidosis (OS) was established by the first International Workshop on Ocular Sarcoidosis (IWOS) and validations studies revealed certain limitations of the criteria. To...

Evaluation of changes in retinal and choroidal thickness using spectral domain optical coherence tomography in unilateral non granulomatous acute anterior uveitis

To assess retinal and choroidal thickness changes in eyes with a first episode of unilateral non granulomatous acute anterior uveitis (AU). Thirty-one patients with acute unilateral non granulomatous AU who had undergone spectral domain optical coherence tomography (SD-OCT) of both eyes were included in this retrospective study. Retinal thickness (RT) as defined by the Early Treatment Diabetic Retinopathy Study (ETDRS) and subfoveal choroidal thickness (SFCT) obtained by SD-OCT were recorded. Healthy fellow eyes of the patients served as the control. A significant thickening in 1mm of ETDRS subfield and in subfoveal choroid was observed in eyes with acute AU when compared with the unaffected fellow eyes (P=0.005 and P<0.00001, respectively). RT and SFCT of affected eyes did not show a significant difference in terms of HLA-B27 antigen presence or absence (P>0.05, for all). Significant posterior segment changes were detected in eyes with acute unilateral non granulomatous AU.

Initial misdiagnosis of Vogt-Koyanagi-Harada disease

PurposeTo report the initial misdiagnosis of patients with Vogt–Koyanagi–Harada (VKH) disease. MethodsThe medical records of 76 consecutive patients diagnosed with VKH disease were reviewed retrospectively at The Eye Center, Riyadh, Saudi Arabia. Patients were referred to The Eye Center from Saudi Arabia and other Middle Eastern countries. The initial diagnosis was made by an ophthalmologist or neurologist. The main outcome measure was to evaluate cases with VKH disease who were initially misdiagnosed. ResultsIn 7 (9.2%) out of the 76 patients the initial diagnosis was incorrect. Patients were initially misdiagnosed as optic neuritis (1.3%), intracranial hypertension (1.3%), brain tumor (1.3%), Susac disease (1.3%), migraine (1.3%), rhegmatogenous retinal detachment (1.3%) or anterior granulomatous uveitis of unknown etiology (1.3%). Patients underwent unnecessary tests including MRI and invasive procedures including CSF analysis and anterior chamber paracentesis. ConclusionThe initial diagnosis of patients with VKH disease was incorrect in 9 % of the cases. Delay in the diagnosis of VKH disease may lead to delay in management and may cause irreversible damage to the photoreceptors with poor visual outcome.

Aqueous tap and rapid diagnosis of cytomegalovirus anterior uveitis: the Reggio Emilia experience.

The diagnosis of cytomegalovirus (CMV) anterior uveitis in immunocompetent patients requires confirmation by polymerase chain reaction (PCR) analysis and/or intraocular antibody index (AI) assay. In this study, we analyzed the different contributions of PCR and AI to CMV diagnosis by performing one single aqueous tap. A retrospective chart review was conducted of HIV-negative patients attending the Ocular Immunology Unit of Azienda Unità Sanitaria Locale - IRCCS, Reggio Emilia, Italy, from March 2015 to April 2018 with a diagnosis of hypertensive anterior granulomatous uveitis compatible with suspected CMV etiology. Diagnosis was confirmed by real-time PCR (RT-PCR) and intraocular antibody production against CMV on aqueous humor samples. Clinical features were compared to antibody titer and diagnostic delay. Twenty-three patients with suspected CMV uveitis (13 males, 10 females, mean age 48 ± 16years) were included in the analysis. AI was positive in 20/23 (87%) samples, and PCR tested positive in 9/23 (39%). By combining both tests, the sensitivity was 100%. Median diagnostic delay was 29months (IQR 9-107). Diagnostic delay and antibody titer were significantly associated with glaucoma (r = 0.714, p < 0.0001; r = 0.476, p = 0.02, respectively). Our data suggest that to improve the diagnostic accuracy of CMV anterior uveitis, PCR and AI are both useful and complimentary. In our series, AI was the most sensitive diagnostic tool. One single aqueous tap is sufficient to achieve 100% sensitivity in CMV diagnosis. Early diagnosis is necessary to prevent the development of glaucoma.

Clinical Characteristics and Treatment of 308 Panuveitis Patients over 10 Years: Results from the KKESH Uveitis Survey Study Group

ABSTRACTPurpose: To describe the clinical characteristics and surgical outcomes of panuveitis over a 10-year period.Methods: Data were collected on panuveitis etiology, treatment, surgery and change in vision.Results: Overall, 308 patients (594 eyes) were evaluated, 54.9% with non-granulomatous and 45.1% with granulomatous uveitis. Fifty-four patients had infectious and 254 had non-infectious uveitis. In infectious panuveitis, vision remained unchanged at last visit. The most frequent diagnosis was presumed intraocular tuberculosis (PIOTB) uveitis (48.1%). In the non-infectious subgroup vision increased significantly by 2 lines at last visit (p = 0.020). The most common diagnose was Vogt–Koyanagi–Harada (VKH) disease (38.6%). Initial therapy was oral prednisone in 86.6% with non-infectious etiology followed by immunosuppressive agents. Surgery secondary to complications (mainly cataracts) was performed in 46.7% patients.Conclusion: In Saudi Arabia, panuveitis was mainly due to VHK. PIOTB was the most common etiology for infectious panuveitis. Immunosuppressive therapy and surgery maintained BCVA in non-infectious panuveitis.

The CC chemokines CCL8, CCL13 and CCL20 are local inflammatory biomarkers of HLA-B27-associated uveitis.

To determine the concentrations of the CC chemokines CCL2, CCL7, CCL8, CCL11, CCL13, CCL20, CCL24 and CCL26 in aqueous humour (AH) samples from patients with specific uveitic entities. Aqueous humour samples from patients with active uveitis associated with Behçet's disease (BD) (n=13), sarcoidosis (n=8), HLA-B27-related inflammation (n=12), Vogt-Koyanagi-Harada (VKH) disease (n=12) and control patients (n=9) were assayed with the use of a multiplex assay. When considering all uveitis patients as one group, all chemokine levels except CCL2 were significantly increased compared to controls. CCL8, CCL13 and CCL20 were the most strongly upregulated, 48-fold, 118-fold and 173-fold, respectively, above control AH levels. CCL8 and CCL13 levels were significantly higher in HLA-B27-associated uveitis than in sarcoidosis and VKH disease. CCL20 levels were significantly higher in HLA-B27-associated uveitis than in BD, sarcoidosis and VKH disease. In addition, CCL20 levels were significantly higher in BD than in VKH disease. In HLA-B27-associated uveitis, CCL8, CCL13 and CCL20 were upregulated 111-fold, 255-fold and 465-fold, respectively, compared with controls. CCL8, CCL13 and CCL20 levels were significantly higher in nongranulomatous uveitis (BD and HLA-B27-associated uveitis) than in granulomatous uveitis (sarcoidosis and VKH disease). Immune responses mediated by CCL8, CCL13 and CCL20 appear to be more potent in nongranulomatous uveitis, particularly in HLA-B27-associated uveitis.

Uveitis and serous retinal detachment secondary to systemic dabrafenib and trametinib

Abstract Case report The case is presented of a 39-year-old woman with metastatic melanoma treated with dabrafenib and trametinib. She presented with a severe acute panuveitis with granulomatous anterior uveitis, vitritis, and multiple serous retinal detachments. Dabrafenib and trametinib were suspended, and treatment with a systemic and topical corticosteroid was started. A good response was obtained, with a recovery of visual acuity of 1.0 in both eyes within two weeks. Discussion Dabrafenib and trametinib can lead to severe uveitis. Treatment with corticosteroids and discontinuation of therapy with dabrafenib and trametinib led to an anatomical and functional improvement, and resolved the episode rapidly. Ophthalmologists must be aware of this toxicity, given the increasing use of those drugs.

P165 New relevant associations with vogt-koyanagi-disease (VKH) were found in Mexicans, using NGS technology. A new allele at DPA1 locus was detected in a VKH patient

Aim VKH is a systemic autoimmune disorder affecting tissues containing melanin, mainly the eyes. It is characterized by severe bilateral granulomatous uveitis. VKH is a T cell mediated disease vs. specific auto-antigens relevant in its pathogenesis. We and others showed that a shared epitope in the first DRB1 domain is clearly associated with VKH. DRB1*04:05 and 04:04 are strongly involved in Mexicans and other contributing DRB1 alleles, were also published by us: DRB1*04:05> *04:04 > *04:07 > *01:01 > *01:02. We now explored the whole MHC region, to unravel the complete MHC genetics of VKH. Methods We selected 77 unrelated donors from Mexico City and 49 VKH patients from the APEC. DNA samples were extracted in the Maxwell 16 instrument. For HLA typing, the MIA FORA NGS FLEX HLA Kit (Immucor, Inc) and The Illumina MiSeq, were used. Sequence analysis was performed with the Mia Fora software. Genotypes were assigned at all loci unambiguously, except for DPB1. Allele and haplotype frequencies (AF/HF) were done with the Arlequin, PyPop and Hapl-o-Mat v.1.1 software. Results A hierarchy of Class I/class II associations were found with deep sequencing: DQB1*03:02:01 (p = 0.000007); DQA1*03:01:01(p = 0.0002); C*03:04:01 (p = 0.007); DRB1*04:04:01(p = 0.005); DRB1 *04:07:01 (p = 0.01); DRB1*04:05:01; (p = 0.02); B*48:01:01 (p = 0.04); A*68:01:01(p = 0.02); DPB1 *03:01:01(p = 0.02). The OR was between 2.9 and 7.3. Amerindian (AMI), Hispanic and Asian alleles are evident at all loci. The most frequent haplotype in VKH is of Mexican AMI descent : A*02:01:01 B*39:05:01 C*07:02:01 DRB1*04:07:01

Optic Neuropathy and Macular Ischemia Associated with Neurosarcoidosis: A Case Report.

In this study, we present a case of bilateral optic neuropathy and macular ischemia in the right eye associated with neurosarcoidosis. A 26-year-old woman presented to our clinic with complaints of bilateral blurred vision. Bilateral granulomatous anterior uveitis, vitritis, optic neuropathy, and macular ischemia were detected in the right eye in slit-lamp examination. She also reported complaints of fever, weakness, sweating, arthralgia, and headache for 2 months. She was referred to the pulmonary diseases unit of our hospital due to hilar lymphadenopathy seen in her chest x-ray, and biopsies were taken for diagnostic purposes. Histological analysis of the mediastinal lymph node biopsies revealed chronic, non-caseating, granulomatous inflammation. Furthermore, the patient was referred to a neurologist due to concomitant complaint of intense headaches. She was diagnosed with neurosarcoidosis supported by findings on cranial magnetic resonance imaging and lumbar puncture. She received a 3-day course of high-dose (1 g/day) intravenous steroid treatment (methylprednisolone) followed by a tapering dose of oral prednisone. The patient began receiving oral methotrexate 15 mg/week as a steroid-sparing agent. Significant improvement in neurological and ophthalmological symptoms occurred in the first week of treatment. In this case report, we emphasized that neurosarcoidosis should be included in the differential diagnosis of patients with both bilateral optic neuropathy and macular ischemia. Furthermore, early diagnosis and timely treatment of neurosarcoidosis are important for favorable visual outcomes.

Tuberculous Uveitis Presenting with a Bullous Exudative Retinal Detachment: A Case Report and Systematic Literature Review

ABSTRACTPurpose: To describe tuberculous uveitis (TU) presenting as a bullous retinal detachment (RD) and to perform a comprehensive literature review on TU with similar features.Methods: Observational case report and systematic literature review.Results: An 84-year-old woman presented with bilateral granulomatous uveitis and bullous RD in the left eye. The interferon gamma release assay was strongly positive, but all other tests were unremarkable. The patient was diagnosed with TU and started on anti-tubercular therapy (ATT) and systemic steroids with excellent treatment response. Twenty-six articles (32 cases) reported TU with exudative RD. Choroidal tuberculoma was the most common clinical manifestation, followed by optic disc edema and retinal exudate. Systemic steroids with ATT improved vision in more patients (78.6%) than ATT alone (50.0%) or oral steroids followed by ATT (50.0%).Conclusion: Atypical presentations of TU make diagnosis and treatment difficult. A high level of suspicion for TU is needed to minimize ocular morbidity.

Uveítis anterior aguda hipertensiva granulomatosa bilateral como efecto adverso a brimonidina tópica

Clinical caseThe case concerns an 81-year-old woman on treatment with a topical fixed combination of timolol and brimonidine who was diagnosed in the Emergency Department with acute anterior granulomatous hypertensive uveitis. The patient responded favourably to the withdrawal of the eye drops without showing any subsequent relapse. DiscussionUveitis due to brimonidine is a rare adverse effect, but it must be known. Once the diagnosis is suspected, the effective treatment is the withdrawal of brimonidine, with or without the addition of topical corticosteroids to control inflammation depending on the severity of the condition. It is a process with an excellent prognosis.

Differential CXC and CX3C Chemokine Expression Profiles in Aqueous Humor of Patients With Specific Endogenous Uveitic Entities.

To determine the levels of the neutrophil chemoattractants CXCL1, CXCL2, CXCL5, CXCL6, and CXCL8, the T helper 1 chemoattractants CXCL9, CXCL10 and CXCL11, the lymphoid chemokines CXCL12 and CXCL13 and the soluble form of the transmembrane chemokines CXCL16 and CX3CL1, in aqueous humor samples from patients with specific uveitic entities. Aqueous humor samples from patients with active uveitis associated with Behçet's disease (n = 13), sarcoidosis (n = 8), HLA-B27-related inflammation (n = 12), Vogt-Koyanagi-Harada (VKH) disease (n = 12), and healthy controls (n = 9) were assayed with the use of a multiplex assay. All chemoattractant levels were significantly higher in all patients than in the controls. The levels of all neutrophil chemoattractants and CXCL10, CXCL16, and CX3CL1 were significantly higher in nongranulomatous uveitis (Behçet's disease and HLA-B27-associated uveitis) than in granulomatous uveitis (sarcoidosis and VKH disease), whereas the levels of the B cell chemoattractant CXCL13 were significantly higher in granulomatous uveitis than in nongranulomatous uveitis. CXCL13 levels were highest in the patients with VKH disease. CXCL9, CXCL11, and CXCL12 levels did not differ significantly. Inflammation in nongranulomatous uveitis appears to be driven by neutrophils and T helper 1 lymphocytes, whereas B lymphocytes may contribute to the inflammatory process in granulomatous uveitis, particularly in VKH disease.