Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0042384 (vasculitis)
 20,525 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A 42 year old man presented with acute bilateral uveitis and necrotizing retinitis. Systemic investigations including test for AIDS and CMV retinitis were negative. Despite oral Acyclovir, both eyes progressed rapidly to retinal detachment with loss of vision. Early recognition is necessary to diagnose the bilateral acute retinal necrosis syndrome and initiate treatment. Bilateral acute retinal necrosis (BARN) is a term first coined by Young and Bird in 1978 although the syndrome had been originally described by Urayama et al as an unilateral condition. This syndrome is characterized by the triad of acute confluent peripheral necrotizing retinitis, moderate to severe vasculitis and vitritis in an otherwise healthy individual. Rhegmatogenous retinal detachment occurs within two to three months of the onset of the disease and the second eye is involved in 36% of patients, usually within 6 weeks. We herein report a patient who presented with simultaneous BARN leading to retinal detachment in a matter of days. Also, to our knowledge this is the first report of this condition in India.
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PMID:Bilateral acute retinal necrosis--a case report. 130 36

Peripheral nerve lesions observed in the progressive immunodeficiency associated with infection are remarkable for their diversity and their potential severity. At the onset, demyelinating neuropathies predominate, often with signs of atypical polyradiculoneuritis associated with inflammatory lesions or vasculitis sometimes of the necrotizing type, as that observed in periarteritis nodosa. Later on, at the immunosuppression stage, axonal lesions predominate. At the AIDS stage one may find lymphomatous infiltration of nerves and nerve roots and, chiefly, opportunistic cytomegalovirus (CMV) infection. This infection produces meningoradiculitis, mainly in the territory of the cauda equina, or multifocal neuropathy frequently associated with CMV retinitis. Early treatment of CMV neuropathies may stabilize the lesions or even result in functional improvement, but the overall prognosis of late neuropathies remains poor.
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PMID:[Peripheral neurologic manifestations of infection by the human immunodeficiency virus]. 131 15

We examined three patients with acquired immunodeficiency syndrome who had frosted branch angiitis associated with small patches of cytomegalovirus retinitis. Each patient had a low CD4-helper T-lymphocyte count and a T-lymphocyte helper-suppressor ratio of less than 0.1. Treatment with intravenous anticytomegalovirus antibiotics caused the vascular sheathing to resolve within two weeks in all three patients, but each patient continued to have a smoldering retinitis. Retinal biopsy in one of the patients demonstrated virions whose morphologic characteristics were consistent with cytomegalovirus on electron microscopy and the identity of which was confirmed by immunohistochemistry. Although frosted branch angiitis in otherwise healthy patients responds to corticosteroids, similar treatment with corticosteroids for frosted branch angiitis associated with cytomegalovirus retinitis in patients with AIDS does not seem to be indicated. Before corticosteroid treatment is started for a patient with the clinical signs and symptoms of frosted branch angiitis, careful medical examination of the patient is necessary.
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PMID:Frosted branch angiitis associated with cytomegalovirus retinitis. 132 16

The authors report four cases in which retinal biopsy findings yielded unexpected or previously unreported diagnoses in patients with inflammatory retinitis. The tissue diagnoses included Wegener's retinal vasculitis in an immunosuppressed patient with a clinical diagnosis of cytomegalovirus retinitis, a novel viral form in the retina of a patient with cytomegalovirus retinitis, a case of acute retinal necrosis due to cytomegalovirus infection in an immunologically normal adult, and a case of ganciclovir-resistant herpes family viral retinitis. These cases illustrate the use of retinal biopsy in obtaining tissue for diagnosis and guiding treatment in selected cases of retinitis.
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PMID:Pathologic observations made by retinal biopsy. 217 89

The human retroviruses, HTLV-I and HIV, are playing clinically important roles in a variety of ocular disorders, particularly in uveitis. Both viruses are integrated in the genome of infected T cells. HIV-I infection causes the death of the infected T cells, thereby affecting the host defence system and causing AIDS. Subsequent opportunistic infections of ocular tissues, such as CMV retinitis, are a serious problem in clinical ophthalmology all over the world. Another human retrovirus, HTLV-I, has been known as the causative agent of T cell malignancies (ATL and T cell lymphoma) and chronic myelopathy (HAM/TSP), and is now recognised as a causative agent for a specific type of intraocular inflammation characterised by vitreous opacities with mild iritis and mild retinal vasculitis (HTLV-I uveitis). The mechanism by which HTLV-I causes uveitis is still unknown, but our recent data suggest that it is most probably an immune mediated mechanism by activated CD4 T cells infected with the virus. HTLV-I uveitis, therefore, may implicate a significant role of retroviruses in autoimmune diseases and further the pathogenesis of diseases with infection/autoimmune overlap.
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PMID:Human T lymphotropic virus type 1 uveitis. 812 25

Human T-lymphotropic virus type 1 (HTLV-I) has an etiologic role in adult T-cell leukemia (ATL) and HTLV-I associated myelopathy (HAM). This paper reviews the ophthalmic literature relevant to the retrovirus. Patients with ATL may have intraocular invasion of proliferated T lymphocytes, lymphomatous lesion in the ocular adnexa, or cytomegalovirus retinitis due to immunocompromisation. Patients with the chronic neurological disease HAM may present with retinal vasculitis, isolated cotton-wool spots, probably immune-mediated uveitis, and/or retinochoroidal degenerative change. Similar retinal vascular or uveal inflammatory disease is occasionally seen in otherwise healthy HTLV-I carriers. Clinical and epidemiologic data suggest the existence of HTLV-I associated uveitis (HAU) that is assumed to be associated with the retrovirus. Further ophthalmic research is encouraged to elucidate the clinical significance of the worldwide HTLV-I infection.
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PMID:[HTLV-I and ocular disease]. 831 63

Two vaso-occlusive events, branch retinal artery occlusion (BRAO) and branch retinal vein occlusion (BRVO), were observed in the retina of an HIV-infected patient with cytomegalovirus (CMV) retinitis who developed neovascularization of the disc (NVD). Although BRVO and reversible NVD have been reported in association with CMV retinitis, we have seen no reports of concomitant BRAO. CMV damages endothelial cells and causes an occlusive vasculitis. In HIV-infected individuals, damaged endothelial cells and rheologic problems result in increased blood viscosity. HIV infection has also been associated systemically with elevated levels of cytokines, including tumor necrosis factor alpha (TNF-alpha). In vitro, TNF-alpha exerts effects that decrease fibrinolytic potential; this activity in the circulation of a patient with AIDS may lead to vascular occlusive events. In the patient reported here, the retinal changes were not reversed by induction therapy with ganciclovir and the NVD did not regress.
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PMID:Branch retinal artery occlusion (BRAO) combined with branch retinal vein occlusion (BRVO) and optic disc neovascularization associated with HIV and CMV retinitis. 873 6

The purpose of this study is to present the first report of a case of primary frosted branch angiitis from the UK and to review the characteristics of this rare disease. Primary frosted branch angiitis causes characteristic florid translucent retinal perivascular sheathing of both arterioles and venules in association with variable uveitis, retinal oedema and visual loss, normally with good recovery. A total of 57 cases have been reported in the world literature. Atypical, typically focal frosted branch angiitis may also occur secondary to other causes of intraocular inflammation, especially cytomegalovirus retinitis. Primary frosted branch angiitis has a characteristic presentation but a variable course, typically affecting children or young adults. The disease is likely to represent a common immune pathway in response to multiple infective agents. The optimal treatment is unclear.
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PMID:Frosted branch angiitis: a review. 1537 57

An unusual case of CMV retinitis and AIDS who developed immune recovery uveitis (IRU) despite a low CD4+ T cell count achieved during HAART is reported. A 36-year-old female complained of blurred vision in both eyes six months after initiation of anti-CMV retinitis therapy and HAART. Ocular examination revealed a substantial intraocular inflammation causing a dense vitreous haze and frosted branch angiitis. Consecutive CD4+ T cell counts were 20 cells/mm(3) or less and plasma HIV mRNA was undetectable. The laboratory test for Cytomegalovirus was positive whereas those for infections known to cause uveitis were negative. The inflammatory reaction resolved with treatment, but she developed retinal detachment just before she died of pulmonary complications. A review of the literature led us to propose that our patient developed an intraocular inflammation which may probably be a form of IRU and it might be appropriate to employ additional criteria in the definition and the diagnosis of IRU.
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PMID:Immune recovery uveitis associated with highly active antiretroviral therapy in a patient with CMV retinitis and AIDS despite a low CD4+ T cell count: case report and a review of the literature. 1919 61

Hyper-immunoglobulin E recurrent infection syndromes (HIES) have distinct features, with identified associated mutations of STAT3, TYK2, and DOCK8. Among 197 Taiwanese patients with primary immunodeficiency on a referral-base of over 23 million inhabitants, STAT3 (R382W and Q469R) and DOCK8 mutations (exon 1-9 deletion) were identified in two patients each from six AD-HIES and five AR-HIES patients, respectively. Aside from decreased Th17 and memory B cells, characteristic facies and pneumatocele were not mutually exclusive regardless of STAT3 and DOCK8 mutations. One with novel DOCK8 deletion had notable cytomegalovirus retinitis, cerebral vasculitis, lead deposition, and amenorrhea. In adolescence, three AD-HIES patients without STAT3 mutation died of myocardial infarction, staphylococcus sepsis, and proteus sepsis while receiving chemotherapy for lymphoma. Close follow-up of the HIES phenotype rather than identifying genetic mutations should be the cornerstone of intervention at this juncture because of relatively lower percentage of identifying mutations in Taiwanese HIES (4/11; 36.5%).
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PMID:Clinical, immunological and genetic features in Taiwanese patients with the phenotype of hyper-immunoglobulin E recurrent infection syndromes (HIES). 2132 46


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