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)

Concentration of von Willebrand's factor antigen (WFAg) was measured by solid phase enzyme immunoassay in the blood serum of 17 female patients (mean age 56.7 +/- 12.1 years) with primary Sjogren's syndrome (PSS) lasting, on the average, 4.1 +/- 1.5 years. Mean WFAg level in patients with PSS was equal to 2.56 +/- 1.4 IU/ml and exceeded significantly that of donors' (1.06 +/- 0.34, p < 0.001). In 9(53%) of 17 patients WFAg concentration was higher than normal (> 2.1 IU/ml). WFAg mean levels and frequency of its elevation did not differ significantly in patients with chronic parotitis, enlarged salivary glands, arthralgia/arthritis, lymphadenopathy, lung lesions, polyneuropathy and patients free of the above symptoms. In patients with myalgia, Raynaud's syndrome, skin vasculitis and vascular diseases WFAg concentrations were higher than in patients without them. There was no significant correlation between WFAg level and ESR, concentration of C-reactive protein, presence of rheumatoid and antinuclear factors.
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PMID:[Clinical significance of Willebrand's factor antigen in patients with primary Sjogren's syndrome]. 950 26

Sneddon was the first one to describe the association of cerebrovascular insult (CVI) and skin changes by livedo reticularis type. Angiography of this patient revealed normal brain blood vessels, occlusive changes or the changes similar to those in moya-moya disease. Computed tomography (CT) of the brain most frequently revealed ischemic lesions and/or diffuse atrophy of the brain, although the normal finding was also possible. Considering the frequent finding of the increased titer of anticardiolipin antibodies (ACA) in those patients, those antibodies could be the most important in the pathogenesis of Sneddon's syndrome. A case of female patient, aged 66 years, with livedo reticularis and recurrent CVI was presented in this study. By panangiography were revealed normal blood vessels of the brain, by scintigraphy two ischemic lesions and by CT ischemic lesions with pronounced cortical and subcortical reductive changes. In laboratory findings were observed increased immunocomplexes and the presence of lupus-like anticoagulants, increased ACA titer, the increase in fibrinogen value associated with increased ESR. The result of bone marrow examination was normal, while the borreliosis test was negative. Vasculitis was excluded by skin biopsy. Solid improvement was noticed after the immunosuppresive therapy was administered, but in the last disease exacerbation with new CVI, the outcome was lethal 3 years since the disease onset.
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PMID:[Antiphospholipid antibodies in a female patient with the Sneddon syndrome]. 976 46

Twenty-nine patients with clinically defined Takayasu arteritis and 26 healthy control volunteers were recruited by INSSYS investigators from their clinical practices. Patients with Takayasu arteritis were divided into those with clear-cut clinically active or inactive disease based on Birmingham Vasculitis Activity Scores. Multiple serological tests were performed including ESR, C-reactive protein, tissue factor, von Willebrand factor, thrombomodulin, tissue plasminogen activator, ICAM-1, VCAM-1, E-selectin and PECAM-1. No test was reliably able to distinguish between healthy volunteers and patients with active Takayasu arteritis. At present there is no known serological test which can consistently supplant vascular histopathology in determining the activity of Takayasu arteritis.
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PMID:Surrogate markers of disease activity in patients with Takayasu arteritis. A preliminary report from The International Network for the Study of the Systemic Vasculitides (INSSYS). 995 19

We report a 59-year-old man with chronic sensorimotor polyneuropathy mimicking chronic inflammatory demyelinating polyneuropathy. Sural nerve biopsy revealed marked loss of myelinated fibers and myelin ovoid formation with vasculitis in the epineurium. Renal biopsy was performed subsequently because of the laboratory findings suspecting systemic vasculitis of which consisted CRP positivity, ESR elevation, perinuclear antineutrophil cytoplasmic antibodies (pANCA), and creatinine clearance reduction. The biopsy specimen revealed segmental necrotizing glomerulonephritis with frequent formation of a crescent. These findings confirmed the diagnosis of microscopic polyangitis. Oral administration of prednisolone with additional azathioprine following ethylprednisolone pulse therapy was effective, and pANCA was not detected anymore. We conclude that vasculitic neuropathy has to be considered as a possible cause of polyneuropathy.
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PMID:[A case of polyneuropathy by microscopic polyarteritis nodosa]. 998 56

We analyzed the clinical and pathological characteristics of patients with idiopathic rapidly progressive glomerulonephritis (idiopathic RPGN), who had been hospitalized in recent three years. Five out of 8 patients with idiopathic RPGN were positive for anti-neutrophil cytoplasmic autoantibodies (ANCA), most of them showed pANCA by indirect immunofluorescence technique. Compared with those who were ANCA negative, ANCA positive patients had an elder onset age; most of them had fever, arthragia, myalgia, skin rash, and some patients also had deafness, eye and nasal disorders. ANCA positive patients had a positive CRP, elevated gammaglobulins and accelerated ESR (> 100 mm/1 h). Typical pathological findings for ANCA positive patients were focal segmental fibroid necrosis of the glomerular capillary tufts and diffuse crescents formation. After an intensive therapy, the renal functions of most ANCA positive patients were obviously improved and most of them became hemodialysis independent. The results suggested that idiopathic RPGN might be a renal limited form of small vessel vasculitis and the patients with idiopathic RPGN had a better prognosis. Early diagnosis and appropriate intensive therapy are critical for patients with idiopathic RPGN.
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PMID:[Idiopathic rapidly progressive glomerulonephritis and small vessel vasculitis]. 1043 70

Wegener granulomatosis (WG) is a systemic disease that is particularly unusual in children. A limited form has been described without renal involvement. We report a 14-year-old girl in whom the disease started with acneiform nodular and papular lesions on the forehead. Later necrotic ulcers developed on her forehead, arms, and buttocks. The cutaneous lesions were associated with upper and lower respiratory tract involvement, low-grade fever and arthralgias. Subsequently clinical and laboratory evaluations (increased ESR; leukocytosis and presence of serum IgG antibodies cANCA = 1:160), with chest roentgenograms revealing pulmonary densities and parenchymal infiltration, suggested the diagnosis of WG. The histologic findings of a cutaneous biopsy specimen were ulceration of the epidermis with diffuse neutrophilic inflammatory infiltrate and a late-stage small vessel vasculitis in the dermis. Histopathology of the nasal mucosa was characterized by a granulomatous process with a dense lymphohistiocytic infiltrate with few giant cells, a finding that confirmed the diagnosis of WG. No renal involvement was present. One month of cyclophosphamide (125 mg/day) and prednisone (70 mg/day) therapy markedly improved the patient's clinical condition. At present, 1 year later, she is free from any signs of the disease. According to the literature, the frequency of cutaneous lesions in WG ranges from 16% to 46%. They are the presenting sign only in 6% of patients. Cutaneous lesions are even more uncommon in children. In particular, an "acneiform" presentation is a rare finding in WG.
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PMID:Wegener granulomatosis in a child: cutaneous findings as the presenting signs. 1046 11

An 84-year old man was admitted to Mitoyo General Hospital because of progressive malaise and edematous erythema on both eyelids (Heliotrope erythema). He also noted blister on his neck as well as erythema on the extensor surface of finger joints (Gottron's sign), elbows and knees. Neither weakness nor pain of his proximal muscle was elicited on physical examination on admission. His blood test disclosed positive inflammatory signs (i.e., mild elevation of ESR and positive CRP) without elevated value of muscle enzymes. Electromyogram showed normal pattern. Infiltration of inflammatory cells was not revealed by histological examination of biopsied muscle. A diagnosis of 'amyopathic dermatomyositis' was made based on these observations. Computed tomography of his chest disclosed interstitial pneumonia spreading over both lower lung fields. Colon fiberscopy revealed a polyp in his descending colon, which was classified into group I on cytological examination. He was treated with two sets of methylprednisolone (mPSL) pulse therapy (500 mg/day, 3 consecutive days, intravenously) followed by 30 mg/day of oral prednisolone (PSL). His skin lesions responded well to the above treatment and the dose of oral PSL was tapered. One month after the initiation of treatment, severe stomatitis as well as a large ulcer beneath his tongue developed accompanying an intractable pain. Mucosal biopsy revealed necrotizing vasculitis in medium-sizedartery at the bottom of ulcer. Another set of mPSL pulse therapy brought a prompt relief of his symptom and prohibited the recurrence of oral lesion. It should be noted that our patient did not fulfill the diagnostic criteria for DM because of the lack of muscular symptoms whereas he had characteristic skin lesions. Regarding the frustration possibly encountered at the time of diagnosing amyopathic DM, both sensitivity and specificity of the skin lesion for the diagnosis of DM were investigated. Moreover, the rarity of blister as a skin manifestation of DM was discussed as well.
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PMID:[A case of amyopathic dermatomyositis presenting blister and oral ulcer]. 1069 7

Primary angiitis of CNS(PACNS) or granulomatous angiitis of CNS is a rare inflammatory disease of small blood vessels mostly confined to the CNS. The clinical and pathological features of 3 autopsied cases are described. Clinically all the three PACNS patients were young males, age ranging from 19 to 31 years. All presented with varied neurological manifestations. There was no evidence of systemic disease in any of the cases. The ESR was normal and CSF analysis showed chronic meningitic pattern. The cerebral angiogram in one case was normal and the CT scan done in another case showed multiple intracerebral haematoma due to vasculitis. Brain biopsy was not done. Diagnosis was made at post-mortem examination. Histology showed characteristic but variable degree of granulomatous and non-granulomatous angiitis of small vessels. Venulitis with parenchymal haemorrhages was the predominant feature and in one case phlebitis with thrombosis was noted. Since the disease responds to steroids and immunosuppressive therapy, establishing antemortem diagnosis is important. In view of the association of angiitis of CNS with bacteria and viral infections, their role in the evolution of the disease needs to be investigated.
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PMID:Primary angiitis of CNS : neuropathological study of three autopsied cases with brief review of literature. 1087 79

We recently experienced three cases of aortitis syndrome that were associated with perinuclear antineutrophil cytoplasmic antibody (ANCA). In the three cases, roentgenographic examination revealed the typical appearance of stenosis or occlusive subclavian arteries. In addition, two cases showed a thickened thoracic aorta wall and the remaining case had irregular stenosis of both common iliac arteries. All three cases had persistently increased ESR and CRP over the years. These findings suggested the diagnosis of aortitis syndrome. ANCA tests were performed because of rapidly progressive glomerulonephritis symptoms in two patients and marked excretion of beta(2)-microglobulin in urine in one patient. The test showed P-ANCA in all three patients, with two patients identified as anti-MPO antibody and the third patient as non-MPO antibody. The implication of ANCA in the pathogenesis of aortitis syndrome is presumed to be: ANCA, which plays an important role in the pathogenesis of small vessel vasculitis, induces vasculitis of the vasa vasorum in the aorta or main branches (or both) and this pathologic process results in the pathogenesis of aortitis syndrome.
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PMID:Aortitis syndrome associated with positive perinuclear antineutrophil cytoplasmic antibody: report of three cases. 1098 Mar 43

We report the first occurrence of gemcitabine-induced vasculitis. It concerns a 45-year-old man diagnosed with non-small lung cancer since 2 months. After the first cycle of chemotherapy, consisting of gemcitabine and cisplatin, he developed myalgia and swelling of arms and legs with impairment of movement. This re-occurred during the second cycle of chemotherapy. Further anemia, elevated ESR and increased creatininephosphokinase. A surgical biopsy showed leucocytoclastic vasculitis and necrosis of muscle tissue. The chemotherapy was stopped and the complaints disappeared and did not return.
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PMID:Vasculitis due to gemcitabine. 1214 Jan 48


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