Bacterial endocarditis mimicking vasculitis with steroid-induced remission.
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Adrenal Cortex Hormones↗
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Results for “Retinal Vasculitis”
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[Retinal lesions in HIV seropositive patients].
118 patients with HIV infection underwent complete ophthalmologic examination to detect the presence of any eye pathology. The study was intended to show a correlation between such pathology and the total CD4 + lymphocyte count considered to be indicative of the immunologic status. The most frequently encountered lesions were cotton-wool patches, vascular congestion, hemorrhages, chorioretinitis, segmental vasculitis and optic disc pallar. Almost all the patients with ocular manifestations, also had a CD4 + count of less than 200/mm3 which suggest that ocular lesions are of bad prognostic significance even in asymptomatic patients.
Acquired Immunodeficiency Syndrome↗
Relationship of extracranial carotid occlusive disease and central retinal artery occlusion.
Central retinal artery occlusion usually results in blindness. The association between central retinal artery occlusion and extracranial carotid disease has not been clearly delineated. We reviewed the case reports of 62 patients with central retinal artery occlusion, 25 of whom underwent carotid angiography as part of the diagnostic evaluation. Fourteen of the 25 (56 percent) were found to have ipsilateral extracranial carotid disease. These patients did not generally have carotid bruits and had normal noninvasive carotid tests. Ten patients underwent ipsilateral carotid endarterectomy; these patients had either embologenic ulcerated plaque or tight stenosis of the carotid artery. There were 11 patients who showed no abnormalities on angiography. Thirteen patients who did not undergo angiography showed clinical evidence of etiologic factors, including vasculitis, an embolism of cardiac origin, and trauma. The remaining 24 patients had no diagnostic workup. Follow-up data were available in six of those patients who underwent carotid endarterectomy for a mean of 34 months; no strokes were reported. In conclusion, over half of patients with central retinal artery occlusion who undergo carotid angiography will have a carotid lesion on the ipsilateral side. This suggests that central retinal artery occlusion is a significant marker for extracranial carotid disease and should be an indication for complete carotid evaluation.
Adolescent↗
Polyarteritis nodosa in hairy cell leukemia: treatment with interferon-alpha.
Polyarteritis nodosa (PAN) is a rare but well described complication of hairy cell leukemia, with 17 cases reported. Treatment has been with prednisone or cyclophosphamide, with variable results. We describe a case of PAN associated with hairy cell leukemia treated with interferon-alpha with autopsy proven resolution of the vasculitis.
Humans↗
Ophthalmological and neuro-ophthalmological involvement in Churg-Strauss syndrome: a case report.
BACKGROUND: It is well known that different types of eye involvement may develop during the course of systemic vasculitides. METHODS: We report here a case of Churg-Strauss syndrome (allergic granulomatous angiitis) characterized by the presence of multiple ophthalmological and neuro-ophthalmological lesions, i.e., mononeuritis of the fourth cranial nerve, multifocal choroidal ischaemia, and bilateral ischaemic optic neuropathy. RESULTS: Ischaemic lesions in the posterior ciliary plexus and chorio-retinal circulation, which appeared simultaneously after a phase of disease activity, were documented. CONCLUSION: The simultaneous occurrence of multiple ocular features in a patient with Churg-Strauss syndrome suggests that regional vasculitis may be the pathological mechanism underlying the multiple ophthalmological lesions in this disorder.
Choroid↗
[Decrease in general health status, febrile state and sinusitis in renal insufficiency].
UNLABELLED: A 59-year old man, dialysed for 9 years because of chronic renal failure of unknown etiology, was also suffering from a hypertensive cardiopathy and anemia. He has received more than 70 units of blood over the past few years. An elevated blood aluminium level prompted treatment with desferrioxamine for 3 years. Following episodes of melena, an intestinal-wall lesion, located 8 cm above the ileo-caecal valve was discovered. Blood serotonin levels were elevated; biopsy of the liver revealed tumor cells with round-shaped nuclei which stained positive for synaptophysin, findings compatible with a carcinoid tumor or a pancreatic islet-cells tumor. Considering the whole clinical situation, aggressive diagnostic of therapeutic measures were not pursued any further. The patient lost 8 kg over the next few months and was finally hospitalised with fever. Physical examination revealed ascites, edema and a gingival abscess which was drained. Blood cultures grew Klebsiella pneumoniae. Antibiotics were prescribed. A month later the patient complained again of fever accompanied by myalgias, edema and pain of the eyelids, mainly on the right side, pain in the maxillary area and on right eye motion. ENT examination revealed a necrotic lesion of the right middle turbinate which on histology was diagnosed as acute purulent rhinitis without granuloma or vasculitis. A CT-scan demonstrated mucosal thickening of the left sphenoidal, ethmoidal, frontal and maxillary sinuses without any osteolytic lesions. The patient suffered a sudden right eye blindness with signs of retinal ischemia accompanied by an elevated sedimentation rate of 130 mm. Steroid treatment for suspicion of a temporal arteritis was introduced. 2 days later the patient was rehospitalized in a semi-comatose condition. No new information was gained from the usual laboratory investigations and the physical examination was comparable to the previous hospital stay. Blood cultures remained sterile. He died a few hours later. DISCUSSION: The discussant, A. de Torrenté MD, accepts the diagnosis of a hypertensive cardiopathy, renal failure of unknown origin and a carcinoid of the terminal ileum with hepatic metastases. The role of iron overload as a contributory factor for the cardiac disease is discussed. More problematic are the manifestations of the terminal disease with involvement of the sinuses, the eyes and the mouth. The "lethal midline granuloma" (a syndrome and not a disease entity) covers many different diseases which can be considered in this case: Wegener's granuloma, various lymphomas, parasitic, bacteria- and fungus-induced diseases.(ABSTRACT TRUNCATED AT 400 WORDS)
Carcinoid Tumor↗
[Papillitis and vasculitis: apropos of a case].
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Female↗
Diffuse necrotizing retinochoroiditis in a child with AIDS and toxoplasmic encephalitis.
We examined a child with a human immunodeficiency virus (HIV) infection who at 15 months of age developed acute encephalitis, followed 1 week later by a diffuse, uniocular retinochoroiditis. The clinical picture in the right eye was characterized by the occurrence of some intraretinal hemorrhages; punctate, yellow-white, outer retinal lesions temporal to the macula; and a quadrantal, white area of necrotic retina located superotemporally. - The vitreous was remarkably clear, and the left eye was normal. Fluorescein angiography revealed small spots of late hyperfluorescence, vasculitis in the posterior pole, and a persistently hypofluorescent quadrantal superotemporal area. Toxoplasma IgM antibodies that were absent 1 week after birth became detectable in the serum and the cerebrospinal fluid. Serological testing for cytomegalovirus was negative. Neurological signs improved on a specific therapy (pyrimethamine and sulfamethopirazine), but the patient died 2 months later of disseminated cytomegalovirus infection.
Acquired Immunodeficiency Syndrome↗
Herpes zoster chorioretinopathy.
Chorioretinitis and subsequent choroidal and retinal pigment epithelial atrophy following herpes zoster ophthalmicus (HZO) have rarely been reported. We report two patients, who several months following attacks of acute HZO, developed posterior fundus features of yellow, non-pigmented, punched-out areas of retinal pigment epithelial and choroidal pigment atrophy, which we have termed herpes zoster chorioretinopathy. An occlusive vasculitic process is proposed as the pathogenesis for this chorioretinopathy, and may be similar to that seen in the delayed cerebral vasculitis following HZO. A previous history of HZO should be sought in patients with a unilateral, multifocal, non-pigmented chorioretinopathy, as this may represent a characteristic delayed feature.
Aged↗
[Vasculitis in ophthalmologic diseases].
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Arterial Occlusive Diseases↗
[Transient trochlear nerve palsy as the presenting neurological sign of panarteritis nodosa].
INTRODUCTION: Panarteritis nodosa (PAN) is a systemic vasculitis affecting small and medium-sized arteries. Neuro-ophthalmological complications of PAN are rare but numerous, and may affect the eye, the visual and the oculomotor pathways. Such complications occur mainly in patients previously diagnosed with PAN. OBSERVATION: A 51-year-old woman presented with an isolated right trochlear (IV) palsy, in the setting of headaches and fluctuating fever of unknown etiology. Erythrocyte sedimentation rate was 13 mm and full blood cell count was normal. Previous chest X-ray and blood studies were negative for an infection or inflammation. Orbital and cerebral CT scan was normal. Spontaneous recovery of diplopia ensued over four days. Two days later, paresthesia and sensory paresis of the dorsal portion of the left foot were present. Lumbar puncture revealed 14 leucocytes (76 percent lymphocytes) with elevated proteins, but blood studies and serologies were negative. A diagnosis of undetermined meningo-myelo-radiculoneuritis was made. Because of a possible tick bite six weeks previously the patient was empirically treated with 2 g intravenous ceftriaxone for 3 weeks. Fever rapidly dropped. Six weeks after the onset of diplopia, acute onset of blindness in her right eye, diffuse arthralgias and fever motivated a new hospitalization. There was a central retinal artery occlusion of the right eye. Blood studies now revealed signs of systemic inflammation (ESR 30 mm, CRP 12 mg/L, ANA 1/80, pANCA 1/40, leucocytosis 12.4 G/L, Hb 111 g/L, Ht 33 percent). Biopsy of the left sural nerve revealed arterial fibrinoid necrosis. A diagnosis of PAN was made. CONCLUSIONS: Transient diplopia can be the heralding symptom of a systemic vasculitis such as PAN, giant cell arteritis and Wegener granulomatosis. In this patient the presence of accompanying systemic symptoms raised a suspicion of systemic inflammation, but the absence of serologic and imaging abnormalities precluded a specific diagnosis initially. A few weeks later, the presence of a second ischemic event (retinal) and positive blood studies led to a further diagnostic procedure. Oculomotor and abducens palsies have rarely been reported in association with PAN. We report the first case of trochlear nerve paresis as the inaugural neurological sign of PAN. This case highlights the importance of considering inflammatory systemic disorders in patients with acute diplopia particularly when they are young, lack vascular risk factors or cause, and complain of associated systemic symptoms.
Diplopia↗
Choroidal infarction, anterior ischemic optic neuropathy, and central retinal artery occlusion from polyarteritis nodosa.
PURPOSE: Ocular ischemia from polyarteritis nodosa (PAN) is rare. The authors present a case of multifocal ocular infarction from PAN. METHODS AND RESULTS: A 70-year-old woman developed hand and foot numbness followed by intermittent blurred vision and binocular horizontal diplopia. Two weeks later, she suddenly lost vision in the right eye from a central retinal artery occlusion and then developed a left anterior ischemic optic neuropathy and bilateral triangular choroidal abnormalities consistent with infarction. Her erythrocyte sedimentation rate and C-reactive protein were elevated. Although giant cell arteritis was suspected, a multiple mononeuropathy was demonstrated by electromyogram and nerve conduction velocity studies. Biopsy specimens from her sural nerve and biceps muscle showed a necrotizing vasculitis with fibrinoid necrosis, consistent with PAN. CONCLUSIONS: Polyarteritis nodosa can produce ischemia of a variety of ocular structures, including the retina, choroid, and optic nerve. In our patient, all three structures were affected. To our knowledge, this is the first reported case of the triangular sign of Amalric in PAN.
Aged↗
Angioimmunoblastic lymphadenopathy with retinitis and drug related exacerbations: a clinicopathological case study.
A patient with angioimmunoblastic lymphadenopathy with dysproteinemia was followed over a three year period from diagnosis to death. He presented with arthralgias, uveitis and respiratory insufficiency and developed hyperuricemic renal failure upon institution of treatment. Aggressive combination chemotherapy was required to reverse progressive thrombocytopenia and pulmonary involvement. A complete remission was achieved twice. There was a striking temporal relationship between the administration of antibiotics or allopurinol and exacerbations of the disease. Hypocomplementemia and transient evidence of vasculitis suggested the presence of immunecomplexes. Serial lymph node biopsies showed the progression of this disorder from a pleomorphic immunoblastic proliferation to a lymphocyte-depleted, fibrotic process, in parallel with a decline from hyper- to hypogammaglobulinemia. This case illustrates the broad clinical spectrum of angioimmunoblastic lymphadenopathy with dysproteinemia and suggests that aggressive treatment is necessary in selected patients.
Agammaglobulinemia↗
Plasma homocysteine level and uveitis in Behçet's disease.
BACKGROUND: Behçet's disease is a chronic relapsing systemic vasculitis of unknown origin in which orogenital ulceration, skin lesions and uveitis are prominent features. Patients with BD have been recognized to be at greater risk for vascular diseases such as thrombosis and aneurysms. Uveitis in BD is usually accompanied by retinal vaso-occlusive disease. OBJECTIVE: To investigate the possible role of hyperhomocysteinemia as a prethrombotic disorder in the development of uveitis. METHODS: The study population consisted of 29 patients with BD (13 males and 16 females with a mean age of 32.3 years, range 21-52) and 24 healthy controls. All patients fulfilled the criteria of the International Study Group for BD. The patients were categorized as having active (n = 8) or inactive disease (n = 11) or only uveitis (n = 10). Plasma fasting homocysteine and C-reactive protein levels were studied in all patients and controls. The fasting homocysteine level was significantly higher in patients with only uveitis than in inactive patients and controls (P = 0.029 and P = 0.26 respectively). It was also higher in patients with active disease compared to healthy controls (P = 0.,001). However, no significant difference was found between homocysteine levels of patients with active or inactive disease (P = 0.69). CRP, as a marker of disease activity, was higher in active BD and in patients with only uveitis than in controls (P = 0.01 and P = 0.001 respectively). CONCLUSION: Homocysteine may play a role in ocular involvement of BD. Chronic inflammation can induce hyperhomocysteinemia, thereby leading to thrombosis in the retinal vascular bed in a way similar to that recently proposed for the pathogenesis of coronary artery disease.
Adolescent↗
Cerebral infarction associated with vasculitis due to varicella zoster virus in patients infected with the human immunodeficiency virus.
Cases of herpes zoster ophtalmicus (HZO) with delayed contralateral hemiparesis caused by hemispheric stroke secondary to granulomatous angiitis have been reported and are a well-recognized complication of herpes zoster. Similar cases have been reported more recently during infection with human immunodeficiency virus (HIV). We describe two HIV+ patients without any clinical history of zoster dermatitis who developed a sudden hemiparesis followed 2 weeks later for one by an acute retinal necrosis. Computerized tomography (CT) scan, magnetic resonance imaging (MRI), magnetic resonance angiography (MRA), and digital subtraction angiography (DSA) were performed and showed a hemispheric stroke with evidence of a segmental arteritis of the carotid syphon. Varicella zoster virus (VZV) was found in the cerebro spinal fluid (CSF) in the two patients and after puncture of the vitreous fluid of the patient with the acute retinal necrosis. These two cases exemplify the difficulty of diagnosis of stroke in HIV+ patients, which seems to be more frequent than in similarly aged non-infected patients and demonstrates that VZV needs to be taken in consideration and identified even without any past history of zoster dermatitis.
AIDS-Related Opportunistic Infections↗
[Fluorophotometry of changes of hemato-ocular barriers in intermediate uveitis].
PURPOSE: Intermediate uveitis presents a characteristic clinical picture with inflammation in the vitreous and peripheral retina. From a physiological point of view, the disease is characterised by a rupture of blood-ocular barriers. To determine the sites involved, a vitreous fluorophotometry was performed in patients with intermediate uveitis and in a group of healthy patients. METHODS: Seventy-one patients with an intermediate uveitis were studied by vitreous fluorophotometry. Anterior chamber and posterior segment fluorophotometric findings were measured 5 and 60 minutes after injection of fluorescein. Results were compared to a group of healthy patients. RESULTS: Vitreous fluorophotometry demonstrated abnormal leakage of fluorescein in all vitreous sectors of the patients suffering from uveitis compared with the control group (p < 0.001). In the anterior chamber, leakage of fluorescein was not significantly different between the two groups (p > 0.05). CONCLUSION: Results obtained suggest that intermediate uveitis involves a generalized permeability disturbance of the posterior segment secondary to a diffuse vasculitis. However, in the anterior segment this disturbance is not found.
Adolescent↗
Regional ischemic infarcts of the retina.
Macroscopic and light microscopic features of regional ischemic infarcts of retina in autopsy eyes are described. Lesions were found throughout life span, most patients having significant primary or secondary vascular disease (younger had systemic hypertension, rheumatic heart disease, vasculitis or sickle hemoglobinopathy; most older patients had arteriosclerosis). Diabetes mellitus and infarction of other organs (including brain) also were common. Topographically almost all lesions were found in posterior fundus; most were temporal and involved anatomical macula. Microscopically there was destruction of inner retinal layers with preservation of outermost cells of inner nuclear layer; occasionally ganglion cell layer was relatively spared.
Adolescent↗
Microangiopathy with retinopathy, encephalopathy, and deafness (RED-M) and systemic features.
The case of a young woman with a rare syndrome of acute encephalopathy followed by deafness and retinopathy developing over 1 year is reported. Unlike previously described similar cases, she had considerable systemic symptoms and signs including polyarthralgia-arthritis, diffuse myalgia, malar rash, livedo reticularis, night sweats, and fatigue suggestive of systemic lupus erythematosus. However, results of most immunological investigations were repeatedly normal, including antinuclear antibodies. Anticardiolipin antibodies were elevated on one occasion. Cyclophosphamide has been the most effective treatment for exacerbations of the disease, which have continued to occur over 6 years. This microangiopathic syndrome more likely relates to an immunologically mediated vasculitis of small blood vessels than to a thromboembolic etiology.
Adult↗