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Medicina
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Diagnosis and Treatment of Uveitis
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Diagnosis and Treatment of Uveitis

A special issue of Medicina (ISSN 1648-9144). This special issue belongs to the section "Ophthalmology".

Deadline for manuscript submissions: closed (30 January 2023) | Viewed by 22153

Special Issue Editor

Dr. Christine Fardeau

E-Mail Website
Guest Editor
Reference Center for Rare Diseases, Department of Ophthalmology, La Pitié-Salpêtrière Hospital, Paris-Sorbonne University, 75013 Paris, France
Interests: uveitis; macular edema; retinopathy; choroiditis

Special Issue Information

Dear Colleagues,

Uveitis has been linked to a heterogeneous group of diseases and is known as the fifth cause of blindness in western countries. The various causes could be put in main groups: (1) infections, such as toxoplasmosis, the Herpes virus family, and tuberculosis, as well as numerous pathogens more rarely involved, such as Bartonella; (2) auto-immunity disturbances located in the choroid-retina layer, such as acute posterior multifocal placoid pigment epitheliopathy, idiopathic multifocal choroiditis, serpiginous choroiditis, whose pathophysiology is discussed by using OCT and indocyanine green angiographies; (3) auto-immunity disturbances integrated in systemic diseases, such as sarcoidosis, incidence and prevalence of which has recently increased; (4) tumors gathered in masquerade syndrome, with the classical pseudo-uveitis due to intravitreous non-Hodgkin B lymphoma, as well as systemic malignant hemopathies which could induce masquerade syndrome by delaying specific tumor management; and (5) iatrogenic uveitis which could be triggered by the booming and developing anti-cancer immunotherapy, in particular the checkpoints inhibitors. While specific causes need antibiotic and anti-tumor management, the occurrence of secondary inflammatory intraretinal lesions may help to suggest association with immunosuppressive treatments multidisciplinary discussed. In addition to this, the wide use of hydroxychloroquine in Lupus treatment has suggested six stages of toxic HCQ maculopathy which exhibit a potential change over time.

The purpose of the Special Issue is to discuss each of the uveitis causes, using retinal multi-imaging and intra-ocular samplings in order to shorten the time delay for suggesting specific diagnoses of retinochoroidal lesions that need early specific treatment.

Dr. Christine Fardeau
Guest Editor

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Keywords

  • uveitis
  • pseudouveitis
  • masquerade syndrome
  • tuberculosis
  • herpes
  • acute posterior multifocal placoid pigment epitheliopathy
  • choriocapillaries
  • lymphoma
  • hydroxychloroquine maculopathy

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Published Papers (7 papers)

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Research

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23 pages, 59322 KiB  
Article
Acute Posterior Multifocal Placoid Pigment Epitheliopathy (APMPPE): A Comprehensive Approach and Case Series: Systemic Corticosteroid Therapy Is Necessary in a Large Proportion of Cases
by Ioannis Papasavvas, Alessandro Mantovani and Carl P. Herbort, Jr.
Medicina 2022, 58(8), 1070; https://doi.org/10.3390/medicina58081070 - 8 Aug 2022
Cited by 9 | Viewed by 4200
Abstract
Background and objectives: Acute posterior multifocal pigment epitheliopathy/acute multifocal ischaemic choriocapillaritis (APMPPE/AMIC) is part of the group of choriocapillaritis entities. The aim of this article was to report a series of patients with emphasis on the clinical presentation and treatment paradigms. Materials [...] Read more.
Background and objectives: Acute posterior multifocal pigment epitheliopathy/acute multifocal ischaemic choriocapillaritis (APMPPE/AMIC) is part of the group of choriocapillaritis entities. The aim of this article was to report a series of patients with emphasis on the clinical presentation and treatment paradigms. Materials and Methods: Retrospective case series study performed in the Centre for Ophthalmic Specialised care (COS), Lausanne, Switzerland, on patients diagnosed from 2000 to 2021 with APMPPE/AMIC. Procedures performed at presentation and upon follow-up (when available) included best corrected visual acuity (BCVA), routine ocular examination, laser flare photometry (LFP) microperimetry (when available) and visual field testing. Imaging investigations included spectral domain optical coherence tomography (SD-OCT)/enhanced depth imaging OCT (EDI-OCT), OCT angiography (OCT-A) as well as fluorescein and indocyanine green angiography (FA, ICGA). The presence or not of prodromal systemic viral-like symptoms was noted. The localisation of lesions whether foveal or extrafoveal, divided the patients into 2 groups (foveal, peri-or parafoveal). Exclusion criteria were patients diagnosed with APMPPE/AMIC and a positive QuantiFERON test and/or VDRL-TPHA tests. Results: Nineteen (35 eyes) of 1664 new patients (1.14%) were diagnosed with APMPPE/AMIC and included in our study. 13 (68%) were male and 6 (32%) were female. The mean age was 33.1 ± 9.2 years. 16 (84%) patients mentioned a viral prodromal episode or other systemic symptoms, and 3 (16%) did not mention any episode before the onset of ocular symptoms. 15 (39%) out of 38 eyes had foveal localisation of the lesions, 20 (52.6%) had peri- or para-foveal localisations and 3 eyes were normal [3 unilateral cases (15%)]. Mean BCVA at presentation was 0.83 ± 0.24 for the whole group. It was 0.58 ± 0.28 for the group with foveal lesions, increasing to 0.97 ± 0.13 at last follow-up (p = 0.0028). For the group with extrafoveal lesions mean BCVA at presentation was 0.94 ± 0.18, improving to 1.18± 0.10 at last follow-up (p = 0.0039). 13 (68%) patients received prednisone treatment, of whom 2 (10%) received additionally at least one immunosuppressive agent, 4 (20%) patients received no treatment and in 2 patients the information was unavailable. All patients in the foveal lesion group received corticosteroid treatment except one who evolved to bilateral macular atrophy. Conclusions: APMPPE/AMIC is a primary choriocapillaritis. Although it is thought that the disease is self-limited, treatment is necessary in most cases, especially when lesions are located in the fovea. Full article
(This article belongs to the Special Issue Diagnosis and Treatment of Uveitis)
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10 pages, 952 KiB  
Article
Aqueous Humor Analysis in Overlapping Clinical Diagnosis of Cytomegalovirus and Rubella Virus Anterior Uveitis
by Fabrizio Gozzi, Lucia Belloni, Raffaella Aldigeri, Pietro Gentile, Valentina Mastrofilippo, Luca De Simone, Elena Bolletta, Federica Alessandrello, Martina Bonacini, Stefania Croci, Alessandro Zerbini, Gian Maria Cavallini, Carlo Salvarani and Luca Cimino
Medicina 2022, 58(8), 1054; https://doi.org/10.3390/medicina58081054 - 4 Aug 2022
Cited by 3 | Viewed by 1978
Abstract
Background and Objectives: A cross-sectional single-center study was conducted to investigate the etiology in hypertensive anterior uveitis whose clinical features are not fully distinctive from cytomegalovirus or from rubella virus and to demonstrate the possible coexistence of both these viruses in causing anterior [...] Read more.
Background and Objectives: A cross-sectional single-center study was conducted to investigate the etiology in hypertensive anterior uveitis whose clinical features are not fully distinctive from cytomegalovirus or from rubella virus and to demonstrate the possible coexistence of both these viruses in causing anterior uveitis. Materials and Methods: The clinical charts of a cohort of patients with hypertensive viral anterior uveitis of uncertain origin consecutively seen in a single center from 2019 to 2022 were retrospectively reviewed; data on the clinical features, aqueous polymerase chain reaction, and antibody response to cytomegalovirus and rubella virus were collected. Results: Forty-three eyes of as many subjects with viral anterior uveitis of uncertain origin were included. Thirty-two patients had an aqueous polymerase chain reaction or antibody index positive to cytomegalovirus only, while 11 cases had an aqueous antibody response to both cytomegalovirus and rubella virus. This latter overlapping group had a statistically significant higher rate of hypochromia and anterior vitritis (p-value: 0.02 and < 0.001, respectively). Conclusions: The simultaneous presence of intraocular antibodies against cytomegalovirus and rubella virus could redefine the differential diagnosis of hypertensive viral anterior uveitis, demonstrating a possible “converged” immune pathway consisting in a variety of stimuli. Full article
(This article belongs to the Special Issue Diagnosis and Treatment of Uveitis)
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11 pages, 4781 KiB  
Article
Clinical Manifestations and Outcomes of Tubercular Uveitis in Taiwan—A Ten-Year Multicenter Retrospective Study
by Chun-Ju Lin, Ning-Yi Hsia, De-Kuang Hwang, Yih-Shiou Hwang, Yo-Chen Chang, Yueh-Chang Lee, Yung-Ray Hsu, Po-Ting Yeh, Chang-Ping Lin, Hsi-Fu Chen, Wei-Chun Jan, Wei-Yu Chiang and Ming-Ling Tsai
Medicina 2022, 58(3), 376; https://doi.org/10.3390/medicina58030376 - 3 Mar 2022
Cited by 5 | Viewed by 2467
Abstract
Background andObjectives: This 10-year multicenter retrospective study reviewed the clinical manifestations, diagnostic tests, and treatment modalities of tubercular uveitis (TBU), including direct infection and indirect immune-mediated hypersensitivity to mycobacterial antigens in Taiwan. Materials and Methods: This retrospective chart review of [...] Read more.
Background andObjectives: This 10-year multicenter retrospective study reviewed the clinical manifestations, diagnostic tests, and treatment modalities of tubercular uveitis (TBU), including direct infection and indirect immune-mediated hypersensitivity to mycobacterial antigens in Taiwan. Materials and Methods: This retrospective chart review of patients with TBU was conducted at 11 centers from 1 January 2008 to 31 December 2017. We used a multiple regression model to analyze which factors influenced best-corrected visual acuity (BCVA) improvement. Results: A total of 79 eyes from 51 patients were included in the study. The mean age was 48.9 ± 16.4 years. The mean change of LogMAR BCVA at last visit was −0.21 ± 0.45. Diagnostic tools used include chest X-ray, chest computed tomography, Mantoux test, interferon gamma release test (QuantiFERON-TB Gold test), intraocular fluid tuberculosis polymerase chain reaction, and bronchial alveolar lavage. The clinical manifestations included 48% posterior uveitis and 37% panuveitis. In the sample, 55% of the cases were bilateral and 45% unilateral. There was 60.76% retinal vasculitis, 35.44% choroiditis, 21.52% serpiginous-like choroiditis, 17.72% vitreous hemorrhage, 12.66% posterior synechiae, 6.33% retinal detachment, and 3.80% choroidal granuloma. Treatment modalities included rifampicin, isoniazid, pyrazinamide, ethambutol, oral steroid, posterior triamcinolone, non-steroidal anti-inflammatory drugs, vitrectomy, and immunosuppressants. BCVA improved in 53.2% of eyes and remained stable in 32.9% of eyes. In the final model of multiple regression, worse initial BCVA, pyrazinamide, and receiving vitrectomy predicted better BCVA improvement. Ethambutol was associated with worse visual outcomes. Seven eyes experienced recurrence. Conclusions: This is the largest 10-year multicenter retrospective study of TBU in Taiwan to date, demonstrating the distribution of clinical manifestations and clinical associations with better treatment outcomes. The study provides a comprehensive description of TBU phenotypes in Taiwan and highlights considerations for the design of further prospective studies to reliably assess the role of ATT and vitrectomy in patients with TBU. Full article
(This article belongs to the Special Issue Diagnosis and Treatment of Uveitis)
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Review

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36 pages, 6903 KiB  
Review
Sarcoid Uveitis: An Intriguing Challenger
by Pia Allegri, Sara Olivari, Federico Rissotto and Roberta Rissotto
Medicina 2022, 58(7), 898; https://doi.org/10.3390/medicina58070898 - 4 Jul 2022
Cited by 9 | Viewed by 4314
Abstract
The purpose of our work is to describe the actual knowledge concerning etiopathogenesis, clinical manifestations, diagnostic procedures, complications and therapy of ocular sarcoidosis (OS). The study is based on a recent literature review and on the experience of our tertiary referral center. Data [...] Read more.
The purpose of our work is to describe the actual knowledge concerning etiopathogenesis, clinical manifestations, diagnostic procedures, complications and therapy of ocular sarcoidosis (OS). The study is based on a recent literature review and on the experience of our tertiary referral center. Data were retrospectively analyzed from the electronic medical records of 235 patients (461 eyes) suffering from a biopsy-proven ocular sarcoidosis. Middle-aged females presenting bilateral ocular involvement are mainly affected; eye involvement at onset is present in one-third of subjects. Uveitis subtype presentation ranges widely among different studies: panuveitis and multiple chorioretinal granulomas, retinal segmental vasculitis, intermediate uveitis and vitreitis, anterior uveitis with granulomatous mutton-fat keratic precipitates, iris nodules, and synechiae are the main ocular features. The most important complications are cataract, glaucoma, cystoid macular edema (CME), and epiretinal membrane. Therapy is based on the disease localization and the severity of systemic or ocular involvement. Local, intravitreal, or systemic steroids are the mainstay of treatment; refractory or partially responsive disease has to be treated with conventional and biologic immunosuppressants. In conclusion, we summarize the current knowledge and assessment of ophthalmological inflammatory manifestations (mainly uveitis) of OS, which permit an early diagnostic assay and a prompt treatment. Full article
(This article belongs to the Special Issue Diagnosis and Treatment of Uveitis)
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20 pages, 6011 KiB  
Review
Hidden in the Eyes—Recurrence of Systemic Hemopathies Reportedly “In Remission”: Six Cases and Review of Literature
by Margot Denier, Sarah Tick, Romain Dubois, Remy Dulery, Andrew W. Eller, Felipe Suarez, Barbara Burroni, Claude-Alain Maurage, Claire Bories, Johanna Konopacki, Michel Puech, Didier Bouscary, Alberte Cantalloube, Emmanuel Héron, Ambroise Marçais, Christophe Habas, Vincent Theillac, Chafik Keilani, Gabrielle R. Bonhomme, Denise S. Gallagher, Julien Boumendil, Wajed Abarah, Neila Sedira, Stéphane Bertin, Sylvain Choquet, José-Alain Sahel, Lilia Merabet, Françoise Brignole-Baudouin, Marc Putterman and Marie-Hélène Erreraadd Show full author list remove Hide full author list
Medicina 2022, 58(3), 456; https://doi.org/10.3390/medicina58030456 - 21 Mar 2022
Cited by 5 | Viewed by 2865
Abstract
Background and Objectives: Secondary ocular localizations of hematological malignancies are blinding conditions with a poor prognosis, and often result in a delay in the diagnosis. Materials and Methods: We describe a series of rare cases of ocular involvement in six patients [...] Read more.
Background and Objectives: Secondary ocular localizations of hematological malignancies are blinding conditions with a poor prognosis, and often result in a delay in the diagnosis. Materials and Methods: We describe a series of rare cases of ocular involvement in six patients with hematological malignancies, reportedly in remission, who presented secondary ocular localizations, challenging to diagnose. Two patients had an acute lymphoblastic leukemia (ALL) and developed either a posterior scleritis or a pseudo-panuveitis with ciliary process infiltration. One patient had iris plasmacytoma and developed an anterior uveitis as a secondary presentation. Two patients had a current systemic diffuse large B-cell lymphoma (DLBCL) and were referred either for intermediate uveitis or for papilledema and vitritis with secondary retinitis. Finally, one patient with an acute myeloid leukemia (AML) presented a conjunctival localization of a myeloid sarcoma. We herein summarize the current knowledge of ophthalmologic manifestations of extramedullary hematopathies. Results: Inflammatory signs were associated with symptomatic infiltrative lesions well displayed in either the iris, the retina, the choroid, or the cavernous sinus, from the admission of the patients in the ophthalmological department. These findings suggest that patients with ALL, AML, systemic DLBCL, and myeloma can present with ophthalmic involvement, even after having been reported as in remission following an effective systemic treatment and/or allograft. Conclusions: Early detection of hidden recurrence in the eyes may permit effective treatment. Furthermore, oncologists and ophthalmologists should be aware of those rare ocular malignant locations when monitoring patient’s progression after initial treatment, and close ophthalmologic examinations should be recommended when detecting patient’s ocular symptoms after treatment. Full article
(This article belongs to the Special Issue Diagnosis and Treatment of Uveitis)
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32 pages, 16771 KiB  
Review
Diagnosis and Treatment of Primary Inflammatory Choriocapillaropathies (PICCPs): A Comprehensive Overview
by Ioannis Papasavvas and Carl P. Herbort, Jr.
Medicina 2022, 58(2), 165; https://doi.org/10.3390/medicina58020165 - 21 Jan 2022
Cited by 10 | Viewed by 3816
Abstract
Purpose: Primary inflammatory choriocapillaropathies (PICCPs) belong to a group of intraocular inflammatory diseases with the common characteristic of inflammatory choriocapillaris hypo- or non-perfusion as the main clinicopathological mechanism. The purpose of our article is to describe clinical characteristics and multimodal imaging, that can [...] Read more.
Purpose: Primary inflammatory choriocapillaropathies (PICCPs) belong to a group of intraocular inflammatory diseases with the common characteristic of inflammatory choriocapillaris hypo- or non-perfusion as the main clinicopathological mechanism. The purpose of our article is to describe clinical characteristics and multimodal imaging, that can help the diagnosis and treatment of PICCPs. Methods: Narrative review with multimodal imaging analysis. Results: Choriocapillaris non-perfusion can affect the end-choriocappilaries, at the benign end of the PICCP spectrum (MEWDS), to larger choriocapillaris vessels or precapillary vessels at the origin of more severe forms such as acute posterior multifocal placoid pigment epitheliopathy (APMPPE), idiopathic multifocal choroiditis (MFC) and Serpiginous Choroiditis (SC). Diagnosis is mostly based on multimodal imaging and especially on indocyanine green angiography (ICGA), fundus autofluorescence (FAF) and spectral-domain optical coherence tomography (SD-OCT)/OCT-angiography (OCT-A). ICGA shows the typical pattern of patchy lobular hypofluorescence reflecting hypo- or non-perfusion of the choriocapillaris that can also take the aspect of geographic areas in the more severe forms. Treatment depends on the severity of the disease and goes from observation in MEWDS and some mild cases of APMPPE, to oral corticosteroid and/or immunomodulator agents in the more severe conditions of APMPPE and MFC and SC cases. Close multimodal monitoring is crucial in order to introduce or adjust treatment. Conclusion: PICCPs are resulting from one common clinicopathological mechanism, inflammatory choriocapillaris hypo- or non-perfusion. ICGA findings are essential for the diagnosis and follow-up of PICCPs, but non-invasive methods such as FAF and SD-OCT/OCT-A also have their role especially in follow-up of the diseases. Treatment should be individualized according to the pathology and the evolution of lesions. Full article
(This article belongs to the Special Issue Diagnosis and Treatment of Uveitis)
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Other

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7 pages, 29896 KiB  
Case Report
Diagnosis and Treatment of Central Serous Chorioretinopathy in Patients with Scleritis
by Yu-Chien Tsai, Yann-Guang Chen, Yueh-Chang Lee, Yih-Shiou Hwang and Yun-Hsiu Hsieh
Medicina 2023, 59(5), 949; https://doi.org/10.3390/medicina59050949 - 15 May 2023
Viewed by 1584
Abstract
Central serous chorioretinopathy (CSCR) is characterized by central neurosensory retinal detachment from the retinal pigment epithelium. While the association between CSCR and steroid use is widely recognized, it is difficult to distinguish whether the subretinal fluid (SRF) in ocular inflammatory disease results from [...] Read more.
Central serous chorioretinopathy (CSCR) is characterized by central neurosensory retinal detachment from the retinal pigment epithelium. While the association between CSCR and steroid use is widely recognized, it is difficult to distinguish whether the subretinal fluid (SRF) in ocular inflammatory disease results from steroid use or an inflammation-related uveal effusion. We report the case of a 40-year-old man who presented to our department with intermittent redness and dull pain in both eyes that had persisted for three months. He was diagnosed with scleritis with SRF in both eyes and steroid therapy was started. Inflammation improved with steroid use, but SRF increased. This indicated that the fluid was not caused by the posterior scleritis-related uveal effusion but by steroid use. SRF and clinical symptoms subsided after steroids were discontinued completely and immunomodulatory therapy was initiated. Our study highlights that steroid-associated CSCR must be considered in the differential diagnosis of patients with scleritis, and prompt diagnosis with an immediate shift from steroids to immunomodulatory therapy can resolve SRF and clinical symptoms. Full article
(This article belongs to the Special Issue Diagnosis and Treatment of Uveitis)
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