A case of polyarteritis is reported in an 18-year old woman, occurring 2 years after an allogeneic bone marrow transplant. The clinical manifestations were similar to those of polyarteritis nodosa (PAN) with a wide range of organs involved including life-threatening cardiac and mesenteric problems requiring plasmapheresis and intravenous immunoglobulin (IgIV). (+info)
Correlation between the numbers of gammadelta T cells and CD4+ HLA-DR+ T cells in broncho-alveolar lavage fluid from patients with diffuse lung disease.
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CD4+ HLA-DR+ T cells are known to be increasing in broncho-alveolar lavage fluid (BALF) from patients with sarcoidosis, and related to disease activity. Although there are several reports that the number of gammadelta T cells in peripheral blood from patients with sarcoidosis are increasing, contradictory assertions can be seen about the number of gammadelta T cells in BALF, and the clinical significance on the presence of gammadelta T cells in disease site of patients with diffuse lung disease including sarcoidosis. The absolute number of gammadelta T cells and CD4+ HLA-DR+ T cells in BALF were determined by flow cytometry in 107 patients with diffuse lung diseases; 56 with sarcoidosis, 36 with collagen vascular diseases with lung involvement and 15 with idiopathic pulmonary fibrosis. We also measured the number of the transferrin receptor-positive macrophages in BALF. The correlation between gammadelta T cells and activated (maybe antigen-specific) T cells and macrophages were evaluated. Sarcoidosis patients were also evaluated from the data of the number of gammadelta T cells in peripheral blood by flow cytometry and clinical backgrounds. A significant correlation between the numbers of these two cell types was detected in each of the three patient groups. The percentage of peripheral gammadelta T cells was markedly increased in 7 sarcoidosis patients, each of whom also showed affected organs other than lung, however, 5 individuals did not show an increased number of gammadelta T cells in BALF. The number of gammadelta T cells in BALF did not correlate with the number of transferrin receptor-positive macrophages in all three patient groups. These results suggest that the increased number of gammadelta T cells in diffuse lung diseases likely plays a role in immunosurveillance and contributes to the activation of antigen-specific alphabeta T cell. (+info)
Implications for pathogenesis of patterns of injury in small- and medium-sized-vessel vasculitis.
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The different pathologic features of different types of necrotizing vasculitis indicate that there are different pathogenic mechanisms causing the injury. The pathogenic mechanisms for medium-sized-vessel vasculitis are most effective at causing injury in arteries and are not effective at causing injury in smaller vessels. The predilection of medium-sized-vessel vasculitis for bifurcations may relate to the increased expression of adhesion molecules and increased numbers of intimal macrophages at these sites. The preferential involvement of small vessels by small-vessel vasculitis may relate to the requirement for close apposition between leukocytes and endothelial cells for the pathogenic mechanisms to be operational. The pathology of the necrotizing vasculitis of Kawasaki disease is most consistent with a primary role for monocytes/macrophages and T lymphocytes in the acute injury. The pathology of the necrotizing vasculitis of polyarteritis nodosa and small-vessel vasculitis, including ANCA-vasculitis, is most consistent with a primary role for neutrophils and monocytes in the acute injury. (+info)
The role of the thymus in development of necrotizing arteritis in transgenic rats carrying the env-pX gene of human T-cell leukemia virus type-I.
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Necrotizing arteritis mimicking polyarteritis nodosa occurred in transgenic rats carrying the env-pX gene of human T-cell leukemia virus type I. To investigate the pathogenesis of necrotizing arteritis in these rats (env-pX rats), adoptive transfers of spleen cells and bone marrow cells were done from env-pX rats before they developed arteritis to nontransgenic rats. Necrotizing arteritis occurred in lethally irradiated nontransgenic rats reconstituted by env-pX spleen cells, thus indicating that the env-pX transgene in affected vessels may not be essential for the development of arteritis. In contrast, arteritis was not induced in nontransgenic recipients by adoptive transfers of env-pX bone marrow cells, which suggested that T cells derived from the env-pX thymus may play a role in the development of arteritis. To clarify if the process of differentiation of T cells in the env-pX thymus is crucial to develop necrotizing arteritis, reciprocal exchange of thymus frameworks was done between env-pX and nontransgenic rats. Necrotizing arteritis occurred in nontransgenic rats with an env-pX thymus framework, whereas development of arteritis was suppressed in env-pX rats in which the thymus framework was replaced with a nontransgenic one. This collective evidence shows that the thymus is directly associated with the development of necrotizing arteritis in env-pX rats. (+info)
Arterial distensibility in children and teenagers: normal evolution and the effect of childhood vasculitis.
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BACKGROUND: Polyarteritis nodosa is a necrotising vasculitis of the medium sized and small muscular arteries. The inflammatory and subsequent reparative processes may alter the arterial mechanical properties. The effect of vasculitic damage on arterial distensibility has never been explored however. AIM: To determine the normal values and the effect of childhood vasculitis on arterial distensibility in children and teenagers. METHODS: Distensibility of the brachioradial arterial segment was studied using pulse wave velocity (PWV proportional, variant 1/ radical distensibility), in 13 children with polyarteritis nodosa at a median age of 11.8 (range 4.9-16) years. As a control group, 155 healthy schoolchildren (6-18 years, 81 boys) were studied. PWV was assessed using a photoplethysmographic technique; blood pressure was measured by an automatic sphygmomanometer (Dinamap). Data from patients were expressed as z scores adjusted for age and compared to a population mean of 0 by a single sample t test. Determinants of PWV in normal children were assessed by univariate and multivariate linear regression analyses. RESULTS: Age, height, weight, and systolic blood pressure correlated individually with the brachioradial PWV. Multivariate analysis identified age as the only independent determinant. Ten of the patients were in clinical remission, while three had evidence of disease activity at the time of study. The PWV in the patient group as a whole was significantly greater than those in healthy children (mean z score +0.99). Raised C reactive protein concentration (>2 mg/dl) in the three patients with active disease was associated with a higher PWV when compared to those in remission (z score +2.78 v +0.45). The diastolic blood pressure of the patients was higher than those of the controls (z score +1.04) while the systolic pressure was similar (z score -0.36). CONCLUSIONS: PWV in the brachioradial arterial segment increases gradually during childhood independent of body weight, height, mass, and blood pressure. Increased PWV, and hence decreased distensibility, in this peripheral arterial segment occurs in polyarteritis nodosa and is amplified during acute inflammatory exacerbation. (+info)
Evaluation of the Sorensen diagnostic criteria in the classification of systemic vasculitis.
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OBJECTIVES: To evaluate the use of the diagnostic criteria for Wegener's granulomatosis (WG) and microscopic polyangiitis (mPA) proposed by Sorensen et al. in the classification of primary systemic vasculitis (PSV). METHODS: We applied to our cohort of PSV patients the American College of Rheumatology (ACR) criteria for WG, Churg-Strauss syndrome (CSS) and polyarteritis nodosa (PAN), the Chapel Hill Consensus Conference (CHCC) definitions for WG, mPA and CSS, the Hammersmith criteria for CSS and the Sorensen criteria for WG and mPA. RESULTS: Ninety-nine PSV cases were identified. Fifty-six fulfilled criteria for WG (ACR), 60 for PAN (ACR) and 15 for CSS (ACR). Four fulfilled the Hammersmith criteria for CSS. Thirty-nine were defined as mPA (CHCC). Fifty-three patients fulfilled the Sorensen criteria for WG and three for mPA. Five of six patients classified as WG (ACR) who did not meet the Sorensen criteria were excluded by eosinophilia. Six patients who did not fulfil WG (ACR) met the Sorensen criteria for WG. CONCLUSION: The classification of systemic vasculitis is complicated and many cases fulfil more than one set of criteria. The Sorensen criteria for WG is limited by its exclusion of eosinophilia despite reports of an association. We recommend that tissue eosinophilia or peripheral eosinophilia of <1.5x10(9)/l should not exclude a diagnosis of WG. With this modification, the Sorensen criteria for WG may be a useful method of classification, especially in confirming the classification of WG in patients who fulfil both WG (ACR) and mPA (CHCC). Few patients fulfilled the Sorensen criteria for mPA which suggests that they are not of value in classification. (+info)
Polyarteritis associated with hypopharyngeal carcinoma.
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A rare case of polyarteritis associated with a solid tumor is presented. A 66-year-old man was referred to our hospital, because of gangrene in the bilateral fingers and toes, right pleural effusion, and an abnormal sensation in the throat. A diagnosis of polyarteritis was made based on pleuritis, digital gangrene and the arteriography findings. He also had a hypopharyngeal carcinoma. After being treated with intermittent intravenous cyclophosphamide, oral corticosteroid, alprostadil and aspirin, the pleural effusion rapidly disappeared, while the digital gangrene gradually improved. For the treatment of hypopharyngeal carcinoma, radiation therapy was initiated and resulted in complete disappearance. (+info)
Magnetic resonance imaging of skeletal muscle involvement in limb restricted vasculitis.
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BACKGROUND: Limb restricted polyarteritis nodosa (PAN) and PAN-type diseases such as isolated vasculitis of skeletal muscle are localised vasculitides affecting the skin, muscles, or peripheral nerves, usually of the lower limbs. These diseases often present with non-specific clinical symptoms and normal laboratory values and electromyograms. The usefulness of magnetic resonance imaging (MRI) of skeletal muscle has been poorly investigated to date. OBJECTIVE: To describe the MRI findings in the legs of three patients with limb restricted vasculitides (two PAN, one isolated vasculitis of the skeletal muscle) with histologically established muscle involvement. METHODS: MRI was carried out on calf muscles and T(2) weighted images, unenhanced T(1) weighted images, and STIR sequences were obtained. RESULTS: Muscle damage resulted in oedema-like changes on MRI characterised by hyperintense signals in T(2) weighted and slow tau inversion recovery (STIR) sequences and normal unenhanced T(1) weighted sequences of one or several leg muscles. CONCLUSIONS: MRI should be considered a useful complementary examination that might facilitate the recognition of limb restricted vasculitides, and possibly indicate the site for muscle biopsy. It could also be useful in monitoring the course of the disease. Future studies should also evaluate MRI for systemic PAN or other systemic vasculitides with muscle involvement. (+info)