Cases reported "Vasculitis"

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1/18. Pulmonary interstitial fibrosis as a presenting manifestation in perinuclear antineutrophilic cytoplasmic antibody microscopic polyangiitis.

    microscopic polyangiitis (MPA) is one of the vasculitides that is included in the pulmonary renal syndromes. Pathologically, MPA has been defined as necrotizing vasculitis with few or no immune deposits, primarily affecting small vessels including arterioles, venules, or capillaries. Pulmonary interstitial fibrosis (PIF) as an accompanying manifestation in MPA has not been widely appreciated. In the present study, we report six cases of MPA at our institution with radiographic evidence of PIF that was apparent before any treatment was administered. All had biopsy evidence of renal disease that was consistent with MPA as well as positive serum perinuclear antineutrophilic cytoplasmic antibody titers. hemoptysis was observed in approximately one half of the patients. As determined by CT of the chest, PIF was detected in all of the patients and was often present years before a diagnosis of MPA was made. We conclude that PIF may occur as a pulmonary manifestation of MPA. Further appreciation of this finding may lead to more data with respect to the incidence of PIF in MPA, and to a better understanding of the mechanisms that are involved in the development of this finding.
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2/18. MR angiography for the evaluation of non-systemic vasculitic neuropathy.

    Peripheral neuropathy due to vasculitis without any complications of vasculitis in other organs was first reported in 1987. This condition was termed non-systemic vasculitic neuropathy (NSVN). Although vasculitis is believed to develop in small arteries and arterioles in this disease, the level of vascular involvement has not been fully established. We present a case of NSVN followed up by MR angiography, which was thought to be useful to assess the level as well as the state of vascular lesions in this condition.
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3/18. Small vessel vasculitis limited to pleuropulmonary manifestations, possibly induced by endotoxin.

    AIMS: We investigated a rare case of small vessel vasculitis (SVV) limited to pleuropulmonary manifestations, possibly induced by endotoxin, to determine the activation of immuno-mediated cells and endothelia in the pleuropulmonary circulation. methods AND RESULTS: A 44-year-old man with a high fever was X-rayed, revealing bilateral pleural effusion and atelectasis in the chest. His laboratory data were within normal limits except for a high white blood cell count and a high c-reactive protein level. autoantibodies including anti-neutrophil cytoplasmic antibody were negative. Endotoxin was detected in his sera, but repeated cultures of sputa, urine, blood and the pleural effusion were negative for bacteria. Video-assisted thoracic surgery was performed and lung and parietal pleura specimens were obtained. histology showed arterioles or small arteries infiltrated by monocytes or neutrophils with fibrinoid necrosis and acute or chronic venulitis. A diagnosis of SVV in the lung and pleura was made. immunohistochemistry revealed that interleukin (IL)-1beta was expressed in monocytes and vascular cell adhesion molecule (VCAM)-1 on endothelial cells in the vasculitic lesions in the lung. CONCLUSIONS: Endotoxin possibly induced the inflammation in this apparently unique case of pleuropulmonary small vessel vasculitis. immunohistochemistry revealed the expression of IL-1beta and VCAM-1 which may have caused activation of monocytes and endothelial cells within the vasculitic lesions.
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4/18. Unusual morphologic features of uterine leiomyomas treated with gonadotropin-releasing hormone agonists: massive lymphoid infiltration and vasculitis.

    This report describes 2 unusual morphologic features of leiomyomas in patients who had been treated preoperatively with gonadotropin-releasing hormone (GnRH) agonists. In 1 case there was extensive and widespread infiltration of the leiomyoma by numerous small mature lymphocytes, in keeping with a leiomyoma with massive lymphoid infiltration. In the other leiomyoma there were fibrin and foamy histiocytes within the walls of many arterioles, in keeping with a vasculitis. These 2 features, massive lymphoid infiltration and vasculitis, have rarely been described in association with GnRH agonists. Since GnRH agonists are increasingly being used in the management of uterine leiomyomas, pathologists should be aware of these unusual morphologic features in order to avoid diagnostic confusion.
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5/18. Paraneoplastic vasculitis associated with multiple myeloma.

    The association between vasculitis and cancer has been widely reviewed in recent decades. The existence of malignancies in patients with vasculitis has been estimated at about 4.5-8%, haematological neoplasms being the most frequently observed. The haematological malignancies most frequently described are lymphoproliferative diseases such as hairy cell leukaemia and lymphomas. On the contrary, the incidence of paraneoplastic vasculitis in patients with myeloma is low; up to now, we have found nine cases reported on this subject. We report the case of a 73-year-old woman who in 1 year showed three outbreaks of acutely painful, purpuric and ulceronecrotic lesions, localized on the lower extremities. Histopathological study demonstrated thrombosis in the arterioles and leucocytoclastic vasculitis. Complementary tests revealed the presence of multiple myeloma.
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6/18. autopsy case of microscopic polyangiitis with crescentic glomerulonephritis and necrotizing pancreatitis.

    Herein is reported the case of an 84-year-old woman who initially manifested rapidly progressive glomerulonephritis following a urinary tract infection. Laboratory findings showed a high titer of myeloperoxidase-antineutrophil cytoplasmic antibody (MPO-ANCA). Treatment with high-dose i.v. steroids resulted in clinical recovery and an undetectable MPO-ANCA titer. Two months later the patient was readmitted in a state of severe shock. Laboratory examination showed the deterioration of renal function, leukocytosis, and coagulation abnormalities consistent with disseminated intravascular coagulation (DIC). The patient died 12 days later. The post-mortem examination revealed necrotizing pancreatitis due to acute-stage vasculitis typified by fibrinoid necrosis of the arterioles and venules, and crescentic glomerulonephritis with healed-stage vasculitis. In the lungs, capillaritis with diffuse alveolar hemorrhage was not evident, but arteriolitis and phlebitis were occasionally seen. This case represents an unusual complication of necrotizing pancreatitis in the setting of microscopic polyangiitis. Thus, it is important to consider reactivation independent of the titer of ANCA in the course of the disease.
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7/18. Systemic AA-amyloidosis related to MPO-ANCA microscopic polyangiitis: a case report.

    We report autopsy findings in an 83-year-old woman with myeloperoxidase-type anti-neutrophil cytoplasmic antibody (MPO-ANCA)-positive microscopic polyangiitis and systemic AA amyloidosis. With a diagnosis of MPO-ANCA-related microscopic polyangiitis, the patient was treated with corticosteroids, but she died of intractable enteritis. autopsy showed inactive vasculitis affecting small arteries in kidney, lung, intestinal tract, and skeletal muscle. Gastrointestinal viscera were thickened, and AA-amyloid was demonstrated in arterioles and surrounding tissues. amyloidosis also involved heart, kidney, gallbladder, pancreas, salivary gland, and subcutis. ANCA-positive microscopic polyangiitis appears to have been the likely cause of this patient's AA-amyloidosis.
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8/18. Severe cerebral and systemic necrotizing vasculitis developing during pregnancy in a case of systemic lupus erythematosus.

    We describe a fatal case of systemic lupus erythematosus (SLE) developing cerebral and systemic necrotizing vasculitis during pregnancy. The patient was discovered to have SLE at 14 weeks' gestation. Although the symptoms disappeared without treatment with corticosteroid in the 2nd trimester, she presented with meningoencephalitis due to vasculitis in the 3rd trimester. polyarteritis nodosa (PAN)-like necrotizing vasculitis of the small muscular arteries and arterioles, with acute and healing lesions in the leptomeninges, brain parenchyma and visceral organs was observed at postpartum autopsy. PAN-like vasculitis in the central nervous system is quite rare in SLE. This case is also suggestive in terms of the influence of pregnancy on the activity of SLE.
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keywords = arteriole
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9/18. Reversal of rejection-induced coronary vasculitis detected early after heart transplantation with increased immunosuppression.

    Four patients who underwent heart transplantation, in whom coronary obstruction was seen early after transplantation, are described. Repeated acute rejection episodes were detected within the first 2 months in each patient. Coronary obstruction or ischemia was shown through a combination of T1-201 isotopic study findings, evidence of vasculitis of a small coronary arteriole seen at endomyocardial biopsy, or coronary angiographic results. Vigorous treatment for rejection (antithymocyte globulin and bolus methylprednisolone) was given, and coronary artery lesions or myocardial ischemia resolved after treatment. Rejection-induced coronary obstruction should be considered in patients with repeated acute rejection episodes who are predisposed to the development of vascular rejection. Early after transplantation such obstruction is caused by diffuse vasculitis of small and medium-sized vessels and may be reversed with increased immunosuppression.
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keywords = arteriole
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10/18. Systemic lupus erythematosus presenting with hyporeninemic hypoaldosteronism in a 10-year-old girl.

    hyperkalemia has been noted to occur spontaneously in patients with long-standing systemic lupus erythematosus who did not have advanced renal insufficiency. The patients previously described all had relatively normal renin-aldosterone systems, and the hyperkalemia was thus presumed to be secondary to a primary defect in renal tubular potassium secretion. We describe at 10-year-old girl with lupus nephritis, without significant renal insufficiency, who had hyperkalemia from hyporeninemic hypoaldosteronism postulated to be due to vasculitis involving the afferent/efferent arterioles and juxtaglomerular apparatus.
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