1/58. lymphomatoid granulomatosis in a child with acute lymphatic leukemia in remission.lymphomatoid granulomatosis, a tumor-like process of unknown etiology, produced progressively destructive disease of the larynx, trachea and bronchi in an eight-year-old girl with acute lymphoblastic leukemia of five years duration. The leukemia had been in remission for 4 1/2 years at the clinical onset of the lymphomatoid granulomatosis. Whether this occurrence suggests that lymphomatoid granulomatosis is a type of neoplasm, or is associated with immunologic depression, cannot be stated. Fortunately rare, and of poor prognosis, the disorder may become more frequent with improved survival rate of patients with leukemia and other neoplasms.- - - - - - - - - - ranking = 1keywords = neoplasm (Clic here for more details about this article) |
2/58. Neurological manifestations of chronic hepatitis c.hepatitis c virus (HCV) infection is often associated with abnormal immunological responses. We describe four patients with vasculitic neurological signs and symptoms following HCV infection. A 56-year-old woman with HCV infection developed peripheral neuropathy characterized by asymmetric distal painful hypesthesia, dysesthesia and moderate motor weakness of the lower limbs. Serological examinations revealed cryoglobulinemia and low levels of complement c4. A biopsy of the sural nerve revealed vasculitic neuropathy. HCV infection associated immunomediated vasculitis was diagnosed. While steroid therapy was ineffective, treatment with interferon-alpha improved the neuropathy considerably without, however, eliminating HCV infection. A 62-year-old man with HCV infection developed peripheral sensory neuropathy. complement c3 was slightly diminished. Nerve biopsy revealed vasculitic neuropathy. A 71-year-old woman developed chronic symmetric sensomotor polyneuropathy. HCV hepatitis followed blood transfusions. cryoglobulins tested positive, consistent with type II cryoglobulinemia. Complements C3 and C4 were diminished. Inflammatory infiltrates in the sural nerve biopsy specimen led to the diagnosis of chronic vasculitic disorder. A 55-year-old woman with HCV infection developed vasculitis of the skin, connective tissue, visceral organs, and kidney, leading to hemodialysis. Neurologically she developed severe apathy and drowsiness, myoclonic jerks, exaggerated deep tendon reflexes, and positive pyramidal signs. magnetic resonance imaging of the brain showed diffuse increased signal abnormalities involving supra- and infratentorial white matter suggesting cerebral vasculitis. cryoglobulins were positive, complements C3 and C4 slightly diminished (54 mg/dl, 4.3 mg/dl). Supportive therapy resulted in neurological improvement. Treatment with interferon-alpha was discontinued because of agranulocytosis. In patients with peripheral neuropathy or signs of leucencephalopathy, a hepatitis c associated vasculitis should be considered in the differential diagnosis.- - - - - - - - - - ranking = 23.884769684889keywords = connective (Clic here for more details about this article) |
3/58. Low-dose cyclosporin for multiple colonic ulcers associated with mixed connective tissue disease.This report describes a patient with multiple colonic ulcers and mixed connective tissue disease. The histological findings of the colonic lesions showed vasculitis with T-cell infiltration, and the peripheral T cells were frequently in the activated phase of the cell cycle. In this patient, low-dose cyclosporin treatment (2.5 mg/kg/day) inhibited the T-cell activation in the peripheral lymphocytes and was very effective in the gastrointestinal disorder, which might be related to T-cell activation. This case suggests the possibility that even low-dose cyclosporin can exert a great influence on peripheral T cells and directly inhibit T-cell activation, thereby improving symptoms related to T-cell activation.- - - - - - - - - - ranking = 119.42384842445keywords = connective (Clic here for more details about this article) |
4/58. De novo ANCA-associated vasculitis occurring 14 years after kidney transplantation.A cadaveric kidney transplant recipient, with no history of a connective tissue disease, was admitted with malaise, arthralgias, diplopia, mild headache, and a painful left eye. The patient was on maintenance immunosuppression for 14 years with cyclosporine and methylprednisolone. Initial laboratory data indicated an elevated serum creatinine from baseline, 2 proteinuria, and 50 to 100 red blood cells (RBCs)/high-power field (HPF) in the urine. Renal biopsy was consistent with necrotizing vasculitis involving glomerular capillaries, with crescent formation and an absence of immune complexes. Perinuclear antineutrophil cytoplasmic autoantibodies (P-ANCA) and anti-myeloperoxidase (MPO) were found to be elevated. To the best of our knowledge, this is the first reported case of an ANCA-associated small vessel vasculitis (SVV) developing in a renal transplant recipient without history of connective tissue disease.- - - - - - - - - - ranking = 47.769539369779keywords = connective (Clic here for more details about this article) |
5/58. hypereosinophilic syndrome presenting as cutaneous necrotizing eosinophilic vasculitis and Raynaud's phenomenon complicated by digital gangrene.Cutaneous necrotizing eosinophilic vasculitis is a recently identified type of vasculitis that is characterized by an eosinophil-predominant necrotizing vasculitis affecting small dermal vessels. Clinically, it presents with pruritic erythematous and purpuric papules and plaques, peripheral eosinophilia and a good response to systemic steroid therapy. This vasculitis can be idiopathic or associated with connective tissue diseases. Although the pathogenic roles of eosinophil-derived granule proteins and interleukins have been documented in diseases associated with eosinophilia, a role of CD40 (a glycoprotein of the tumour necrosis factor receptor superfamily) has rarely been described. We describe two patients with idiopathic hypereosinophilic syndrome (HES) presenting with multiple erythematous patches and plaques on the lower extremities and Raynaud's phenomenon. They satisfied the criteria for the diagnosis of HES by clinical and laboratory investigations. Histopathology of the cutaneous lesions revealed prominent eosinophilic infiltration with local fibrinoid change in vessel walls in the dermis and subcutis. Immunohistochemical detection of CD3, CD4, CD8 and CD40 was performed. Infiltrating eosinophils were strongly stained by anti-CD40 monoclonal antibody. One patient improved with prednisolone, pentoxifylline and nifedipine, without recurrence. The other patient initially improved with steroids, but after self-withdrawal of steroid developed digital ischaemia that evolved to severe necrosis and required amputation. Cutaneous necrotizing eosinophilic vasculitis, Raynaud's phenomenon and digital gangrene may develop as cutaneous manifestations of HES. CD40 may play a part in the pathogenesis of eosinophilic vasculitis in HES.- - - - - - - - - - ranking = 23.884769684889keywords = connective (Clic here for more details about this article) |
6/58. Juvenile neutrophilic eccrine hidradenitis: a vasculitis-like plantar dermatosis.Neutrophilic eccrine hidradenitis is a variety of neutrophilic dermatosis described in patients with different neoplasms, most often leukaemia, and different chemotherapy regimens. It is characterised by neutrophilic infiltration of the eccrine coils of sweat glands. Recently it has been described in healthy juveniles, involving primarily the soles of the feet. We describe five new cases of juvenile neutrophilic eccrine hidradenitis all showing a good prognosis or a self-limiting course.- - - - - - - - - - ranking = 0.5keywords = neoplasm (Clic here for more details about this article) |
7/58. February 2001: A 74 year old man with a history over 3 months of increasing dyspnea and malaise.The February COM: A man of 78 years with idiopathic late-onset cerebellar ataxia developed renal failure in association with a high ESR and positive pANCA. This was complicated by a subclinical spinal subarachnoid hemorrhage which was related to necrotizing inflammation of small leptomeningeal vessels. Renal cortical infarcts were due to similar inflammation in arcuate and interlobular arteries. Spinal subarachnoid hemorrhage is rare and usually due to rupture of an arteriovenous malformation. However, an immunogenic connective tissue disorder should be considered in the differential diagnosis. In this case, the histology and results of an autoantibody screen support a diagnosis of microscopic polyangiitis.- - - - - - - - - - ranking = 23.884769684889keywords = connective (Clic here for more details about this article) |
8/58. ANCA-positive periaortic vasculitis: does it fall within the spectrum of vasculitis?retroperitoneal fibrosis (RPF) is a disease of unknown aetiology that has sometimes been reported in association with connective tissue disorders and systemic vasculitis. We report here two cases of antineutrophil cytoplasmic antibody (ANCA)-positive RPF showing clinical evidence of rapidly progressive glomerulonephritis. Although treatment with prednisone and cyclophosphamide led to a remission of RPF in both cases, renal function was restored in only one patient and the other progressed to chronic renal failure. The paper reviews the literature concerning ANCA-positive RPF and discusses the relationship between ANCA-positive vasculitis and RPF.- - - - - - - - - - ranking = 23.884769684889keywords = connective (Clic here for more details about this article) |
9/58. Eales' disease with neurological manifestations.Eales' disease is a primary retinal perivasculitis of an undetermined etiology seen predominantly in the Indian subcontinent. However, neurological involvement is rare. We report here a patient of retinal perivasculitis with neurological dysfunction. Our patient is a 39 years male who developed acute diminished vision right eye in March 99, which progressed for four days and remained static. In April 99 he developed acute diminished vision left eye, which progressed to near total blindness in 48 hours. He was undergoing ophthalmic evaluation. Fourty five days later he developed incoordination and weakness left half of body. The examination revealed bilateral retinal perivasculitis with pyramidal signs and left sided cerebellar signs. Investigations revealed an ESR of 40 mm at the end of first hour. His CT head revealed bilateral basal ganglionic infarcts. MRI head revealed enhancing lesions both basal ganglia and right parietal region. cerebrospinal fluid examination showed xanthochromic fluid with markedly elevated protein and lymphocytic pleocytosis. His workup for connective tissue disorders was negative. He was put on ATT with steroids. Eales' disease is presumed allergy to tuberculoprotein. A trial of ATT with steroids has been tried with varying results. Our patient had bilateral retinal perivasculitis and neurological dysfunction. He had lymphocytic pleocytosis with markedly elevated protein in the CSF.- - - - - - - - - - ranking = 23.884769684889keywords = connective (Clic here for more details about this article) |
10/58. Concomitant intramyocardial and epicardial vasculitis in an autopsied heart allograft for cardiac rhabdomyosarcoma.Primary cardiac tumours are rare, with only one quarter of the patients being malignant. The vast majority of malignant neoplasms of the heart are sarcomas. We describe a patient of primary cardiac rhabdomyosarcoma presented as coronary artery disease and recurrent myocardial infarction. Histopathologic finding of the excised native heart revealed a high grade pleomorphic rhabdomyosarcoma in the myoepicardial portion of the anterior wall with rupture. The accompanying unusual feature was myocardial infarction because of tumour emboli of the left anterior descending and left circumflex coronary arteries. After transplantation, the patient developed mild to moderate acute cellular rejection of the transplanted heart on post-transplantation day 1, 8, and 44, respectively. Unfortunately, he expired on the post-transplantation day 47 because of acute rejection, presenting as concomitant intramyocardial and epicardial lymphocytic vasculitis and multifocal myocardial ischaemia. We found that this uncommon medial lymphocytic vasculitis lesion was mediated by T cells and also by antibody directly against smooth muscle cells of small arteries. The consequence of such immune response would be compromised myocardial oxygenation resulting in allograft failure.- - - - - - - - - - ranking = 0.5keywords = neoplasm (Clic here for more details about this article) |
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