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 170,190 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A 13-year old boy is described with painful joints, papular skinlaesions, malaise and haematuria. Impaired renal function and hypertension were found. In the past he frequently suffered of otitis media. Biopsies of kidney and skin lesions showed a vasculitis, furthermore the C-ANCA-titer (anti-neutrophil cytoplasm antibodies) was strongly elevated which justified the diagnosis Wegener's granulomatosis (WG). This disease is seldom seen in childhood. It is a necrotizing vasculitis with granulomas of mostly the respiratory tract and kidneys. Autoantibodies directed against intracellular antigens of neutrophils were first associated with vasculitis in 1982. Since 1988 two ANCA-subtypes have been recognized: C-ANCA and P-ANCA. The C-ANCA appear to be highly specific for diagnosis and follow-up of WG, and to differentiate of other forms of vasculitis (with P-ANCA). If children present with frequent and serious upper respiratory tract infections, with suspicion of a systemic disease it is important to perform the ANCA-test to differentiate between WG and other forms of vasculitis. The patient was treated with prednisone and cyclophosphamide, after which the symptoms and the ANCA-titer decreased.
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PMID:[A child with Wegener's granulomatosis]. 164 47

The authors present a case report of a 62-year old woman, with hypertension for many years. She suffered from weakness, anorexia and weight loss in the last 6 months. On admission, anemia, elevated ESR, haematuria, proteinuria and renal failure were present. Renal biopsy was compatible with chronic glomerulonephritis. The clinical picture and positivity for P-ANCA suggested systemic vasculitis. Later evidence of maxillary sinusitis and nasal mucosae ulcers as well as pneumonitis, although biopsy did not reveal granulomas, suggested the diagnosis of Wegener Vasculitis. Medicated with Cyclophosphamide and Prednisolone, for a year, with improvement. The authors make a brief discussion of the clinical criteria for classification of ANCA-associated systemic vasculitis.
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PMID:[Vasculitis associated with ANCA]. 794 37

The microscopic polyarteritis is a vasculitis related to necrotizing glomerolunephritis. It always damages at renal and systemic level (a third of the cases presented hemorrhage alveolar). We have showed a case that took place with hemoptysis and renal progressive insufficiency. Among the patient antecedents, we can find arterial hypertension hematuria, rhinitis, epistaxis and artromyalgias. Just before his admittance it showed edemas on lower limbs and eyelids, dysnea, severe hemoptysis, paresthesias and general malaise. The immunologic analysis: Acs. glomerular basal antimembrane: negative, ANCA positive with antimieloperoxidasa specificity. The renal biopsy: focal necrotizing glomerulonephritis with semilunar and negative immunofluorescent. The nasas biopsy: unspecified chronic rhinitis. From the clinic point of view, the patient seemed to have the Wegener granulomatosis apart from the fact that he had hemoptysis which is a rare sign in this cases. However, we could not find any granuloma anatomopatologically, which did not clarify this diagnosis. We diagnosed microscopis Poliarteritis, as a third of the cases presented intrapulmonary haemorrhage. Moreover the renal damage it is identical than in the in Wegener granulomatosis. On the top of that, often we can find p-ANCA, which confirms the diagnosis in 99% of cases. Despite our doubt according to the diagnosis the therapeutical treatment of both illnesses is nowadays identical. This means that we were able to begin a precocious treatment with plasmapheresis, metilprednisolona and ciclofosfamida. After a week treatment there was an evident improvement. Five moth later the illness relapsed.
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PMID:[Microscopic polyarteritis]. 892 53

We report the results of a meta-analysis of 349 patients with Wegener's granulomatosis (WG) that were described in the literature from 1979 onward. We describe the patients in terms of diagnosis (granulomas present or absent in biopsy samples from various organs, results of the anti-neutrophil cytoplasmic antibody [ANCA) test) and of the clinical impact of renal involvement. Furthermore, we report the incidence of histopathologic lesions that were found in 134 renal biopsy samples. Before and after the development of the ANCA test, the percentage of patients in whom WG was diagnosed with histologically proven granulomas is the same. However, after 1987 the diagnosis of the group without granulomas is frequently supported by a positive ANCA test result. For the entire group we found that patients without renal involvement (N = 82) were reported to have lower erythrocyte sedimentation rate (ESR), lower white blood cell count (WBC), less anemia, less hypertension, less occurrence of joint symptoms, and less multi-organ involvement than patients with renal involvement (N = 267). The most frequently reported lesion in the renal biopsy samples was extracapillary proliferation (70%), followed by fibrinoid necrosis of the glomerular tuft (54%). Renal granulomas were reported in only 7 biopsy samples.
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PMID:Wegener's granulomatosis: a meta-analysis of 349 literary case reports. 901 87

Microscopic polyarteritis may involve medium-sized and small blood vessels as well as arterioles, venules and capillaries. We have compared the clinical and laboratory features in patients with microscopic polyarteritis and medium vessel involvement, with the features found in patients with polyarteritis nodosa affecting medium vessels alone. In a 9-year period, 21 patients presented to our hospital with a form of polyarteritis. Seven had microscopic polyarteritis demonstrated histologically (6/7, 86%) and associated with dysmorphic urinary red cells (7/7, 100%), as well as medium vessel vasculitis demonstrated histologically (7/7) or by angiography (1/7, 14%). Five patients had polyarteritis nodosa with medium vessel vasculitis demonstrated histologically (3/5, 60%) or by angiography (2/5, 40%); and no evidence of a glomerular vasculitis on biopsy (2/7, 29%) or in the urinary sediment (0/7, 0%). The remaining 9 patients had microscopic polyarteritis but medium vessel involvement was not excluded by angiography. All patients with microscopic polyarteritis and medium vessel involvement had glomerular hematuria (> 100,000 glomerular RBC/ml), proteinuria > 0.5 g/24 hours), and an elevated serum creatinine (0.166 to 0.811 mmol/l). Other symptoms included fever (6/7, 86%), night sweats (5/7, 71%), gastrointestinal bleeding (4/7, 57%), proximal myopathy (3/7, 43%) and peripheral neuropathy (3/7, 43%). One patient (1/7, 14%) had hypertension. Anemia (6/7, 86%), a raised ESR (6/7, 86%), thrombocytosis (6/7, 86%), hypoalbuminemia (6/7, 86%) and abnormal liver function tests (6/7, 86%) were common. Two patients (29%) had an eosinophilia. All 5 individuals who were tested for ANCA were positive (2cANCA, 2pANCA and one pattern not described). In contrast, in patients with polyarteritis nodosa and medium vessel involvement alone, an elevated ESR was common (4/5, 80%) but fever (1/5, 20%), night sweats (0/5, 0%), proximal myopathy (1/5, 20%) and peripheral neuropathy (1/5, 20%) were seen infrequently; hypertension (1/5, 20%) and eosinophilia (1/5, 20%) were also uncommon; and ANCA were not demonstrated (0/3, 0%). Medium-sized vessel involvement is common in patients with microscopic polyarteristis, and these patients are more likely to have renal involvement and systemic symptoms, and be ANCA-positive, than patients with polyarteritis nodosa alone. Gastrointestinal symptoms are often seen in both groups.
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PMID:Classical polyarteritis nodosa and microscopic polyarteritis with medium vessel involvement--a comparison of the clinical and laboratory features. 910 64

We report a case of a reversible posterior leukoencephalopathy syndrome (RPLS). A 57-year-old male had classical polyarteritis nodosa with mononeuritis multiplex, renal insufficiency, and a high titer of p-ANCA (MPO). He was normotensive. He was treated with high dose methylprednisolone and then with oral prednisolone and cyclophosphamide. Despite the treatment, his renal function rapidly deteriorated and hypertension progressed. He had two generalized seizures; at that time his blood pressure was 200/140 mmHg. CT scan revealed bilaterally symmetric hypodensities in the thalamus, the occipital white matter, and the brainstem. T2-weighted MRI showed increased signal intensities in the temporo-occipital white matter, the thalamus, the posterior limbs of the internal capsules, the external capsules, the midbrain, the pons, and the middle cerebellar peduncles. T1-weighted images showed hypointensities in these areas. Treatment with nifedipine improved his blood pressure; 5 days later he was only moderately disoriented. Follow-up CT demonstrated an ill-defined hypodense area only in the left parietal lobe. To our knowledge, there are only two reported cases of RPLS associated with systemic vasculitides. Interestingly, thalamic lesions are outstanding also in these cases. Under these circumstances, treatment of hypertension is of primary importance, and steroid therapy should not be withdrawn or reduced.
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PMID:[A reversible posterior leukoencephalopathy syndrome in a patient with classical polyarteritis nodosa]. 914 79

A 46-year-old woman was referred to our department in July 1996 with complaints of fever and myalgia in her calves. She had a 20-year history of purulent sputum; diffuse panbronchiolitis had been diagnosed in 1983. Physical examination revealed low-pithed rhonchi over the lung fieldis and hypesthesia of the right leg. She had a white blood cell count of 16,100/mm3, including 4% eosinophils, and a platelet count of 80.0 x 10(4)/mm3. The serum IgE level was 2,200 U/ml, and the cold hemagglutinin titer was high. Pulmonary-function tests showed mixed ventilatory dysfunction, and arterial blood gas analysis revealed a PaO2 of 55.8 Torr on room air. Pseudomonas aeruginosa was cultured from her sputum. A chest X-ray film and CT scan showed diffuse nodular shadows and bronchiectatic changes with mild hyperinflation. An infiltrative lesion in right S6 area could also be seen. Administration of broad-spectrum antibiotics did not alleviate her symptoms. The level of myeloperoxidase-specific antineutrophil cytoplasmic antibody (MPO-ANCA) in serum was 245 EU/ml, and 67Ga scintigraphy showed marked accumulation in the abdomen. Abdominal angiography demonstrated a bead-like appearance and irregularities in the peripheral branches of the hapatic artery, the splenic artery, the cystic artery, and the superior mesenteric artery. Because of the high MPO-ANCA level and the angiographic abnormalities, MPO-ANCA-related vasculitis was diagnosed. She was treated with 1 g of methylprednisolone daily for 3 days, followed by 60 mg of prednisolone and 50 mg of cyclophosphamide daily. Her condition improved dramatically, and the MPO-ANCA level became almost normal. During treatment, her blood pressure rose markedly with a normal serum creatinine level and normal urinalysis. Plasma renin activity was 13.3 ng/ml/hr. Renal angiography showed stenoses and irregularities in the peripheral branches of renal arteries bilaterally. These findings led to a diagnosis of renovascular hypertension due to vasculitis. Her blood pressure was controlled with an angiotensin-converting enzyme inhibitor and a calcium antagonist. Vasculitis associated with chronic supportive lung disease has occasionally been reported, which suggests a casual relation between chronic respiratory infection and ANCA-related vasculitis. Systemic vasculitis should be taken into account as a potential complication of chronic suppurative lung disease.
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PMID:[Diffuse panbronchiolitis with myeloperoxidase-specific antineutrophil cytoplasmic antibody-related vasculitis]. 974 63

Scleroderma renal crisis is characterized by intimal thickening of the afferent glomerular arterioles resulting in hypertension and fibrinoid necrosis of the capillary tuff. We report a 67-year-old man with long-standing systemic sclerosis who developed normotensive progressive renal failure, proteinuria, and a nephritic urinary sediment with serum myeloperoxidase-antineutrophil cytoplasmatic antibodies (MPO-ANCA). Renal biopsy showed pauci-immune crescentic glomerulonephritis but none of the typical vascular changes of scleroderma renal crisis. Because comparable cases have recently been reported from Japan, normotensive MPO-ANCA-positive crescentic glomerulonephritis may form an entity of progressive renal failure in scleroderma.
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PMID:MPO-ANCA-Positive crescentic glomerulonephritis: a distinct entity of scleroderma renal disease? 1019 34

A 74-year-old male was referred for the sudden onset of bilateral sudden deafness. The patient had no history of any disease or trauma to the head. Pure tone audiometry revealed bilateral moderate, to severe, sensorineural hearing loss. Auditory brain stem responses (ABRs) showed normal peak and interpeak latencies. These audiological findings suggested that his hearing loss could be attributed to inner ear lesions. However, we felt an alternative explanation for this sudden deafness was likely to exist because the patient also had a month-long fever of unknown origin (FUO) and weight loss of 5 kg/month. Using the criteria of The American College of Rheumatology, we made the diagnosis of polyarteritis nodosa (PAN). Serum MPO-ANCA was positive (x 661). For treatment, the patient was begun on prednisolone and cyclophosphamide. Nine months later, fever, hypertension, nephritis, pneumonitis, and arthritis had completely resolved, the MPO-ANCA became negative (MPO-ANCA < x 10). Furthermore, his hearing improved.
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PMID:Sensorineural hearing loss as the initial manifestation of polyarteritis nodosa. 1127 37

A Japanese girl aged 13 years with myeloperoxidase anti-neutrophil cytoplasmic antibodies(MPO-ANCA)-associated glomerulonephritis(GN) progressed to end-stage renal failure after 7 years' clinical observation. She had been suffering from recurrent disease flare associated with serum MPO-ANCA elevation(i.e. 153 EU/ml, 208 EU/ml and 358 EU/ml, maximum at each of the episodes, normal < 10 EU/ml). Each flare was treated successfully with prednisolone combined with cyclophosphamide and azathioprine. However, her renal function gradually deteriorated, and peritoneal dialysis(PD) was initiated 7 years after the onset of the disease. During the clinical course, no extrarenal manifestations were observed. Due to subsidence of the serum MPO-ANCA titer(10 EU/ml) after starting PD, prednisolone and azathioprine were tapered thereafter. Her daily urine volume was preserved at approximately 600 ml at that time. She suddenly developed fatigue with severe anemia, oliguria and hypertension 4 months after discontinuation of immunosuppressive therapy. The serum titer of MPO-ANCA increased to 100 EU/ml. These clinical observation suggests that disease flare may occur in selected patients with MPO-ANCA-associated GN, who develop end-stage renal failure requiring PD. Although recurrent flare associated with an increased serological activity in a proportion of patients with lupus nephritis who have received dialysis has been reported to date, to our knowledge, a similar clinical observation in the MPO-ANCA-associated GN has not been reported. Selected patients with the disease should be followed with close observation after undergoing dialysis.
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PMID:[A case of ANCA-associated glomerulonephritis without extrarenal symptoms with disease flare after starting dialysis]. 1221 82


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