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Herpes Simplex Virus Infections in Immunocompromised Patients
Published in Marie Studahl, Paola Cinque, Tomas Bergström, Herpes Simplex Viruses, 2017
PORN or RPHRN is a fulminant form of retinitis, characterized by rapidly progressive multifocal lesions usually involving both the eyes. Initial lesions consist of multifocal areas of opacification in the outer layers of the retina, often involving also the macula. Lesions coalesce rapidly, resulting in large white-yellowish patches of full-thickness retinal necrosis. Vitreal inflammation is minimal or absent. This syndrome is poorly responsive to systemic antiviral treatments, including aciclovir, foscarnet, and ganciclovir, used as single drugs or in combination. In the majority of the patients, PORN progresses rapidly to retinal detachment and blindness (101). The PORN syndrome differs from ARN for several aspects. In PORN, the initial lesions involve the posterior pole and the periphery and subsequently spread outwards. In addition, retinal vasculitis and optical neuritis are less common. In general, PORN is a more severe disease as it progresses more rapidly and is less responsive to treatment.
Posterior uveitis
Published in Gwyn Samuel Williams, Mark Westcott, Carlos Pavesio, Bushra Thajudeen, Practical Uveitis, 2017
Gwyn Samuel Williams, Mark Westcott
It must be noted that there is no systemic association in the majority of cases of vasculitis though Table 5.4 describes particular conditions that are very strongly linked to particular fundal appearances. There is a huge list of conditions, some not uncommon and some vanishingly rare, that can cause retinal vasculitis. These conditions, including Churg-Strauss syndrome, granulomatosis with polyangiitis (GPA – Wegener's granulomatosis), Whipple's disease and Rift valley fever virus, are so numerous that considering each separately is not useful. A better approach is to see if the appearances fit into Table 5.4 and if so asking appropriate questions about these conditions. If however it is simply a case of posterior uveitis with a vasculitis which is not dramatic then asking about any systemic conditions, skin complaints or recent illnesses is best and if the patient denies any of these then it is mostly pointless to ask the ten thousand questions it would take to drill down into every possible cause of retinal vasculitis that exists. Many textbooks exist with tables of conditions that cause arteritis, phlebitis, ischaemia, occlusive disease and more, with each box containing many conditions and usually the same conditions as in the other boxes.
Case 17: Painful Hands
Published in Iqbal Khan, Medical Histories for the MRCP and Final MB, 2018
Symptoms of SLE can be extremely diverse, affecting many different systems. Hence the condition may present under different specialists, perhaps masquerading as another illness. Systems affected include the following.Musculoskeletal: arthralgia and arthritis, but pain is worse than the physical signs. A deforming arthropathy (described as Jaccoud’s arthropathy) may occur.Renal: disease can range in severity from a benign, focal glomerulitis to a potentially fatal, diffuse membranoproliferative glomerulonephritis.Dermatological: the butterfly rash over the cheeks is well recognised. It may be photosensitive. Other lesions include discoid lupus, scarring alopecia, Raynaud’s disease, livedo reticularis and mucosal (e.g. oral, vaginal) ulcers.Cardiovascular: hypertension, pericarditis, Libman-Sacks endocarditis.Respiratory: cough, dyspnoea, pleuritic chest pain and haemoptysis may all occur.Gastrointestinal: serositis can lead to pain. Impaired motility causes nausea, vomiting and even pseudo-obstruction. Patients may complain of anorexia and weight loss.Neurological: long-standing headaches and memory problems are common. Patients may have a history of seizures. Occasionally, there may be associated psychosis. There may be problems with the vision associated with retinal vasculitis. The most devastating problem is that of strokes.Haematological: Coomb’s positive haemolysis, leucopoenia, thrombocytopaenia.Systemic: fever, lethargy, generalised lymphadenopathy, splenomegaly.
A Curious Case of Occlusive Retinal Vasculitis in a Young Individual Associated with COVID-19 Vaccination
Published in Ocular Immunology and Inflammation, 2023
Retinal vasculitis is a sight-threatening condition that can be associated with infection, a systemic inflammatory disorder, idiopathic, or neoplasia. It includes the clinical presentation of perivascular cuffing or sheathing and vascular leakage with or without occlusion. This includes stages of inflammation, ischemia, and neovascularization. The pathophysiology includes perivascular proliferation of lymphoplasmacytic infiltrates, epithelioid cells as in sarcoidosis, lymphocytic cuffing in lymphoma, direct endothelial injury as an infectious cause, or toxin-related upregulation of immunogenetic mechanisms. Capillary non-perfusion (40%) has been the commonest finding in fundus fluorescein angiography in patients with retinal vasculitis followed by collaterals (19.5%). Neovascularisation, cystoid macular edema, and staining of optic nerve head are other angiographic findings. Abnormality in choroidal blood flow has been demonstrated in patients with retinal vasculitis. Poor prognosis in occlusive vasculitis is associated with cystoid macular edema, epiretinal membrane, and neovascularization and its associated complications like vitreous hemorrhage, fibrovascular proliferation, and tractional retinal detachment.1,2
Ophthalmologic Assesment of Patients with Syphilitic Optic Neuropathy
Published in Ocular Immunology and Inflammation, 2023
Multimodal imaging may help improve the description of intraocular inflammation associated with syphilis and in the assessment and follow-up of vitreoretinal complications. FA characteristics included retinal and capillary leakage, vascular and disc staining, and disc hyperfluorescence.20 The most common finding on FA was optic disc staining in our study, similar to the literature. VF changes are varied and show no specific characteristics and include central, cecocentral, peripheral constriction, and sector-shaped VF defects.21 We found that six eyes had normal VF. Three eyes with normal VF had vitritis only, and those patients had unilateral ON. Three eyes with normal VF had anterior ON. Apinyawasisuk et al. reported 10 eyes of 7 patients with SON. As in our study, they found normal VF in two eyes with SON.15 Patients with normal VF may be diagnosed in the early period before visual field defects occur. We also observed different VF defects in patients with SON, which can be seen in other optic neuropathies. We detected retinal vasculitis in four eyes of two patients. One of these patients had concentric narrowing, and the other had blind spot enlargement. Exudation from retinal vasculitis may have affected the VF results.
A Long-Term Follow-up of Retinal Vasculitis – Do They Develop Systemic Disease?
Published in Ocular Immunology and Inflammation, 2020
Ankush Kawali, Bharathi Bavaharan, Srinivasan Sanjay, Ashwin Mohan, Padmamalini Mahendradas, Bhujang Shetty
Mirjam et al. have elegantly descried role of re-screening of patients diagnosed with PRV and have reported that 54% of their cases were ultimately diagnosed with a specific disease entity known to be associated with retinal vasculitis when they were re-screened in 5 years of follow-up.10 In our patients re-investigations were considered only in selected cases with clinical suspicion. In one of the cases cANCA test turned positive when repeated after 11 months. Although the patient had history of joint pain, diagnosis of ‘granulomatosis polyangiitis’ was not considered by rheumatologist as other signs of the disease were absent. Yet another patient who tested positive for HLA B 5 1 upon reinvestigation, was not labeled Behcet’s disease as clinical criteria remained unfulfilled. This may suggest that mere achieving positive result by repeating laboratory investigations still may not fetch a conclusive diagnosis of systemic underlying disease in PRV. In the absence of ocular diagnostic criteria for retinal vasculitides, classifying them into a specific disease category even in presence of supportive blood investigations remains challenging. It is also possible that treatment commenced for retinal vasculitis may prevent underlying systemic disease to manifest completely. We had 38 cases off medications who had mean 4.5 years of follow-up (range: 0.4–24, median: 3), only one patient (2.6%) developed systemic disease (psoriasis) after 2 years. No recurrence of retinal vasculitis was seen in those cases.