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1/57. methotrexate-induced papular eruption in patients with rheumatic diseases: a distinctive adverse cutaneous reaction produced by methotrexate in patients with collagen vascular diseases.

    BACKGROUND: In the past few years, low doses of methotrexate have been used for treatment of patients with rheumatoid arthritis and other collagen vascular diseases, mainly as an immunosuppressive and corticosteroid-sparing drug. Several cutaneous adverse reactions have been described in association with methotrexate therapy. OBJECTIVE: We describe the clinical and the histopathologic features of distinctive cutaneous lesions that appeared in 4 patients with acute bouts of collagen vascular diseases who were receiving methotrexate therapy. methods: We clinically and histopathologically evaluated cutaneous lesions caused by methotrexate therapy in 4 patients, 2 with systemic lupus erythematosus, 1 with rheumatoid arthritis, and 1 with Sharp syndrome. RESULTS: Clinically, lesions consisted of erythematous indurated papules most commonly located on proximal areas of the extremities. Histopathologic examination of these papules showed an inflammatory infiltrate mainly composed of histiocytes interstitially arranged between collagen bundles of the dermis, intermingled with few neutrophils. In some foci of deeper reticular dermis, small rosettes composed of clusters of histiocytes surrounding a thick central collagen bundle were seen. Cutaneous lesions showed a direct chronologic relationship with methotrexate therapy, and they disappeared when the drug was tapered or withdrawn and corticosteroids were increased. CONCLUSION: patients receiving low doses of methotrexate for acute bouts of collagen vascular diseases may experience characteristic cutaneous lesions with distinctive clinical and histopathologic findings shortly after methotrexate administration. We discuss the differential diagnosis with other dermatoses showing similar histopathologic findings that have been described in patients with collagen vascular diseases.
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keywords = vascular disease
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2/57. association of antiphospholipid antibodies with retinal vascular disease in systemic lupus erythematosus.

    OBJECTIVES: To study the prevalence and characteristics of retinal vascular disease in patients with systemic lupus erythematosus (SLE) and to analyze their relationship with antiphospholipid antibodies (aPL) and other serological markers. patients AND methods: Eighty-two consecutive patients (77 women and 5 men; mean age, 36 years) were studied. All patients fulfilled the 1982 revised criteria of the American College of rheumatology for the classification of SLE. Ophthalmologic examination included assessment of best corrected visual acuity, tonometry, slit-lamp biomicroscopy, and fundus examination. Serologic studies included determination of anticardiolipin antibodies (aCL) (ELISA), lupus anticoagulant (LA) (coagulation tests), antinuclear antibodies (indirect immunofluorescence), anti-dna (Farr's test), and anti-ENA antibodies (counterimmunoelectrophoresis). RESULTS: Retinal vascular disease was detected in 13 (15%) of 82 SLE patients. The retinal lesions consisted of retinal vascular occlusions in six patients (five arterial and one venous), cotton-wool spots in three, optic disc edema in three, retinal hemorrhages in three, and ischemic optic neuropathy in one. Antiphospholipid antibodies were detected in 10 (77%) of these 13 patients: nine had aCL and two had the LA. When compared with patients without retinal vascular disease, patients with retinopathy had a higher prevalence of aPL (77% v. 29%, P = .005). CONCLUSIONS: Retinal vascular disease is frequent in patients with SLE. The presence of aPL is associated with a higher prevalence of retinal abnormalities in SLE patients.
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keywords = vascular disease
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3/57. collagen vascular diseases and radiation therapy: a critical review.

    PURPOSE: Although many oncologists have the impression that patients with collagen vascular disease tolerate radiotherapy less well than other patients, until now this was never described in a review article. methods AND RESULTS: The principal objective was to determine whether patients with collagen vascular diseases have a greater risk of severe radiation therapy complications, than those without a collagen vascular disease. However, most of the publications found on this topic are short anecdotal case reports of patients with increased toxicity after radiation. Consequently, the true incidence of these side effects is unknown. CONCLUSIONS: Unless further studies on this subject are reported, each radiation oncologist should be cautious in treating these patients.
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ranking = 0.875
keywords = vascular disease
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4/57. The histopathology of cutaneous lesions of Kikuchi's disease (necrotizing lymphadenitis): a report of five cases.

    Kikuchi's disease (KD) is an idiopathic, self-limited necrotizing lymphadenitis that can clinically and histologically mimic high-grade lymphoma, including Hodgkin's disease, or can be mistaken for the lymphadenitis of systemic lupus erythematosus (SLE). Involvement of extranodal sites is unusual but well documented, especially in asia, where KD is more common than in north america or europe. The successful distinction of KD from malignant lymphoma and SLE is imperative for the appropriate treatment of affected patients. We describe five patients with cutaneous involvement by KD, all of whom presented with fever, lymphadenopathy, and an eruption on the skin of the upper body, which in one case was clinically suspected to be due to SLE and in another, polymorphous light eruption. The patients ranged in age from 10 months to 42 years (median, 33 years) and included three females and two males. All five patients had negative serologic studies for collagen vascular disease. Each patient had a lymph node biopsy showing the typical necrotizing lymphadenitis of KD. skin biopsies from all five patients shared a specific constellation of histologic features: vacuolar interface change with necrotic keratinocytes, a dense lymphohistiocytic superficial and deep perivascular and interstitial infiltrate, varying amounts of papillary dermal edema, and abundant karyorrhectic debris with a conspicuous absence of neutrophils and a paucity of plasma cells, paralleling the nodal histology in KD. CD68 immunohistochemistry on paraffin-embedded sections showed many histiocytes and plasmacytoid monocytes in all cases, whereas CD3, CD4, and CD8 showed highly variable staining among the cases. There was only rare staining with TIA-1 and CD30. We believe that the papular eruption of KD has recognizable histopathologic features and that a CD68 stain that marks many cells that initially seem to be lymphocytes can be performed to confirm the diagnosis.
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ranking = 0.125
keywords = vascular disease
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5/57. Choroidopathy of systemic lupus erythematosus.

    PURPOSE: To describe the ocular and systemic manifestations associated with systemic lupus erythematosus (SLE) choroidopathy. methods: Three new cases of choroidopathy in patients with active SLE were described. Twenty-five published cases of lupus choroidopathy were summarized. RESULTS: There have been 28 cases of lupus choroidopathy (47 involved eyes) that have been reported in the English literature since 1968, including the three current cases. Only two of the patients were male. The choroidopathy was bilateral in 19 patients (68%). All 28 patients (100%) had active systemic vascular disease at the onset of their choroidopathy; 18 (64%) had nephropathy and 10 (36%) had central nervous system (CNS) lupus vasculitis. All but one of the patients had a known diagnosis of SLE at the onset of choroidopathy. 30 of the 47 involved eyes had presenting visual acuity of 20/40 or better; 14 eyes showed improvement in visual acuity with therapy. 23 patients (82%) had resolution of their choroidopathy when their systemic disease was brought under control. Despite treatment, 4 of the 28 patients (14%) died from complications of SLE. CONCLUSIONS: Although less known than retinopathy, lupus choroidopathy may be more common than generally appreciated. It usually serves as a sensitive indicator of lupus activity. The presence of SLE choroidopathy is generally indicative of coexistent (although sometimes occult) nephropathy, CNS vasculitis, and other SLE visceral lesions. immunomodulation of the systemic disease can lead to improvement and resolution of the systemic vasculitis as well as the choroidopathy.
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ranking = 0.125
keywords = vascular disease
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6/57. Red ear(s) syndrome associated with child neuropsychiatric systemic lupus erythematosus.

    This case illustrates that a child having severe neuropsychiatric systemic lupus erythematosus (NPSLE) with seizure and cerebral vascular disease showed excellent clinical outcome in response to intravenous methylprednisolone and cyclophosphamide pulse, and presented unexplained red ears phenomenon.
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ranking = 0.125
keywords = vascular disease
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7/57. Pediatric autoimmune cardiovascular disease.

    Pediatric autoimmune cardiovascular disease can cause serious, sometimes life threatening sequelae on the pediatric population. Valvular, myocardial, and pericardial involvement causing morbidity and mortality can occur in association to rheumatic heart disease, systemic lupus erythematosus, and juvenile rheumatoid arthritis. Serious and potentially life threatening coronary artery involvement can occur in patients with childhood polyarteritis nodosa, takayasu arteritis, and Kawasaki disease.
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ranking = 0.625
keywords = vascular disease
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8/57. Disseminated zygomycosis simulating cerebrovascular disease and pulmonary alveolar haemorrhage in a patient with systemic lupus erythematosus.

    patients with systemic lupus erythematosus (SLE) may be complicated with serious cerebrovascular accidents and pulmonary alveolar haemorrhage. The authors report an autopsy-proven angioinvasive zygomycosis in a patient with SLE. The clinical features of systemic zygomycosis in this patient masqueraded as SLE-related stroke and pulmonary haemorrhage. The case demonstrates that the simultaneous occurrence of complications that clinically suggest pulmonary haemorrhage and multiple brain infarcts in SLE patients should include the rare disseminated zygomycosis in the differential diagnosis.
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ranking = 0.5
keywords = vascular disease
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9/57. Case report. Two cases of pulmonary hypertension with Raynaud's phenomenon. Primary pulmonary hypertension and systemic lupus erythematosus.

    Two cases of hypertensive pulmonary vascular disease with Raynaud's phenomenon are reported. One is a 32-year-old housewife of primary pulmonary hypertension with some immunological disorders and the other is a 28-year-old female of systemic lupus erythematosus. It may be considered that some immunological process could play a role in the etiology of some cases of primary pulmonary hypertension and the relationship of pathogenesis between these 2 cases was discussed.
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ranking = 0.125
keywords = vascular disease
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10/57. Systemic lupus erythematosus presenting earlier as retinal vaso-occlusion.

    Retinal vascular lesions are the most common ophthalmologic manifestation of systemic lupus erythematosus (SLE), occurring in 3% to 29% of cases, generally late in the disease. More rare is the severe vaso-occlusive disease, often termed "retinal vasculitis", which includes central retinal artery occlusion, multifocal arteriolar occlusions, extensive capillary nonperfusion and central venous occlusion. patients with SLE and raised serum concentrations of anticardiolipin antibodies (ACA) have a higher risk of developing occlusive ocular vascular disease. We report a case in which retinal involvement was an earlier manifestation of SLE in a patient without ACA.
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ranking = 0.125
keywords = vascular disease
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