1/42. Infantile disseminated visceral giant cell arteritis presenting as sudden infant death.The rare clinicopathological entity 'disseminated visceral giant cell arteritis' (DVGCA) was first described in 1978. It is characterized by widespread small-vessel giant cell angitis and extravascular granulomas. A normal and healthy 7-month-old boy who presented unexpectedly with sudden infant death syndrome (SIDS) is reported. Histological examination at autopsy revealed giant cell angitis of the aorta, common carotid, coronary, pulmonary, celiac, mesenteric and common iliac arteries. There were also granulomas in the tracheal wall and liver. To our knowledge, this is the first documented case of DVGCA occurring in an infant younger than 12 months of age. A review of the literature on DVGCA is presented in this report, and the differential diagnosis is discussed.- - - - - - - - - - ranking = 1keywords = giant (Clic here for more details about this article) |
2/42. Pathologic findings in a steroid-responsive optic nerve infarct in giant-cell arteritis.OBJECTIVE: To investigate the pathophysiologic mechanism of optic nerve infarction in giant-cell arteritis (GCA). BACKGROUND: Previous pathologic reports of optic nerve infarction in GCA involved patients who were blind at the time of death. The optic nerve infarcts were primarily retrolaminar in localization. Simultaneous short ciliary and ophthalmic artery vasculitis was found in all patients. methods: Clinical neurologic and ophthalmologic examination, temporal artery biopsy, and neuroimaging tests were performed in a patient with an anterior ischemic optic neuropathy secondary to GCA. Pathologic examination of the viscera, eye, and brain were performed at autopsy 1 month later. RESULTS: A prelaminar/retrolaminar infarct was found in this patient. Subsiding vasculitis was limited to the short ciliary arteries, sparing the central retinal, pial, and ophthalmic arteries. CONCLUSIONS: The authors believe that the visual improvement observed in this patient was the result of preserved, anterior optic nerve collateral circulation, as well as the neuroprotective and anti-inflammatory effect of the corticosteroids.- - - - - - - - - - ranking = 0.71428571428571keywords = giant (Clic here for more details about this article) |
3/42. Giant cell angiitis of the central nervous system with amyloid angiopathy. A case report and review of the literature.We report a new case of giant cell angiitis of the central nervous system associated with cerebral amyloid angiopathy (GA/CAA). A 67-year-old woman was hospitalized with a history of headaches and lapses of consciousness. After improvement with corticosteroidtherpay, treatment was stopped. She relapsed and died 33 days after first admission. Pathological examination showed unusual extension of GA/CAA lesions, in the superficial and deep layer of the cerebral cortex, and in the cerebellum. Simultaneous occurrence of GA and CAA is rare. Histopathologic findings and immunological pathogenesis of the process are discussed: 1) arguments over pre-existence of CAA, responsible for GA; 2) primitive inflammatory process inducing amyloid deposits; 3) GA/CAA may represent an association of histological lesions related to 2 different types of disease: i) neurodegenerative disease with specific lesions (such as presence of diffuse senile plaques and neurofibrillary tangles) inducing inflammatory reaction ii) inflammatory disease, with few or no degenerative lesions, responding to immunotherapy.- - - - - - - - - - ranking = 0.14285714285714keywords = giant (Clic here for more details about this article) |
4/42. systemic vasculitis presenting as a tumorlike lesion. Four case reports and an analysis of 79 reported cases.systemic vasculitis might present as a tumorlike lesion that initially could misdirect the correct diagnosis and the appropriate medical treatment. The aim of the present study is to summarize all reported cases of tumorlike presentation in systemic vasculitides, in order to have comprehensive data on the characteristics of this unusual phenomenon. We report 4 cases of systemic vasculitis presenting as a tumorlike lesion. In addition, we performed a medline search of all English-language papers published from 1966 to 1999, looking for vasculitis presenting as tumorlike lesion. Details were included concerning vasculitis classification, specific characteristics, location of the "tumor," the presence or absence of systemic involvement, and whether surgery was performed before diagnosis. Seventy-nine cases of vasculitis presenting as a tumorlike lesion were found in the literature, in addition to the 4 new cases described. The average age of the reported cases was 50.5 /- 15.8 years, and 51% were female. In 82% of the cases the "tumor" was associated with constitutional symptoms and elevated erythrocyte sedimentation rate (ESR). The most common vasculitis categories with tumorlike presentation were wegener granulomatosis (WG; 28 cases) and giant cell arteritis (GCA; 17 cases). In almost half the patients, surgery was performed before diagnosis. All patients with GCA presented with either a breast or an ovarian tumor. The most common location of a tumorlike lesion was the breast (22%), followed by central nervous system lesions (16%). Other frequent locations were the ovary (10%), caused exclusively by GCA and polyarteritis nodosa (PAN), and the male genitourinary system, almost all caused by PAN. Including vasculitis in the differential diagnosis of a tumorlike lesion might lead to an earlier diagnosis and consequently to prompt and appropriate treatment, avoiding needless operations. Constitutional symptoms and elevated ESR should alert clinicians to the possible diagnosis of vasculitis rather than a tumor. The association of GCA with ovarian pseudotumor is distinct and has not been emphasized before. We therefore suggest that GCA should be included in the list of differential diagnosis of an ovarian or breast tumor in an elderly woman, particularly when systemic symptoms and parameters of inflammation are present.- - - - - - - - - - ranking = 0.14285714285714keywords = giant (Clic here for more details about this article) |
5/42. Necrotizing angiitis of the small intestine related to AA-amyloidosis: a novel association.A 71-year-old man with intestinal pseudo-obstruction was found to have a diffusely thickened adynamic small bowel with AA-amyloid in submucosal vessels and muscularis propria, foreign body giant cell reaction to amyloid, and necrotizing angiitis. The mucosa was unremarkable. Immunostains demonstrated numerous CD68 monocyte/macrophages and CD8 T cells associated with the amyloid deposits. The patient had no evidence of systemic vasculitis and no underlying cause for AA-amyloidosis was identified. Necrotizing angiitis coexistent with amyloid angiopathy has been reported in brain and temporal arteries, but not in the gastrointestinal tract and not with AA-amyloid. The inflammatory cell infiltrates in this case are consistent with a foreign-body and/or cell-mediated immunologic reaction to AA-amyloid, although a role for these cells in amyloid formation cannot be excluded.- - - - - - - - - - ranking = 0.14285714285714keywords = giant (Clic here for more details about this article) |
6/42. Waldenstrom's macroglobulinaemia presenting as reticulate purpura and bullae in a patient with hepatitis b virus infection.Cutaneous manifestations of Waldenstrom's macroglobulinaemia (WM) include purpura, ulcers, urticaria, leukocytoclastic vasculitis, and immunobullous dermatoses. No association has been reported previously of WM and hepatitis b virus (HBV) infection. A 40-year-old female HBV carrier was admitted to hospital because of generalized oedema, oliguria, haematuria, hypertension, fever and blood-tinged sputum. Cutaneous manifestations included generalized petechiae, palpable purpura mainly on the legs, multiple necrotic ulcerations and gangrenous changes on the toes, and necrotic, giant confluent reticulate purpura on the trunk surmounted by several tense bullae. Laboratory investigations revealed monoclonal gammopathy of IgM kappa type (6.7 g/L), membranoproliferative glomerulonephritis associated with HBV infection, Bence Jones proteinuria, and an increased number of abnormal plasmacytoid cells in the bone marrow. Pathologic examination demonstrated immune complex-mediated vasculitis with deposits of IgM in the walls of dermal vessels and secondary subepidermal bulla formation. HBV infection may have caused WM or modified the clinical course in this fatal case.- - - - - - - - - - ranking = 0.14285714285714keywords = giant (Clic here for more details about this article) |
7/42. Serial CT and MRI findings in a patient with isolated angiitis of the central nervous system associated with cerebral amyloid angiopathy.We report serial CT and MRI findings in a biopsy-proven case of cerebral amyloid angiopathy (CAA) with isolated angiitis of the central nervous system (CNS). A 69-year-old man had developed dizziness, dementia, and generalized seizure during the preceding 4 years. An initial examination by brain CT and MRI showed bilateral symmetrical periventricular lesions closely resembling those of Binswanger's disease. Subsequently, the lesions expanded slowly, involving a large area of the right cerebral hemisphere with an obvious mass effect. Since a primary brain tumor was suspected, a brain biopsy was performed, and histopathological examination revealed amyloid beta protein CAA within the meningocortical vessels associated with perivascular monocytic cuffing, indicating the presence of isolated angiitis of the CNS. Multinucleated giant cells containing intracytoplasmic beta protein amyloid around a heavily amyloid-laden cortical vessel were also observed. This is the first case report to show sequential radiographical studies of the leukoencephalopathy associated with CAA and isolated angiitis of the CNS.- - - - - - - - - - ranking = 0.14285714285714keywords = giant (Clic here for more details about this article) |
8/42. cerebral amyloid angiopathy with granulomatous angiitis ameliorated by steroid-cytoxan treatment.We report a case of a 62-year-old black woman who, 8 months prior to death, developed confusion, apraxias, disorientation, and difficulties with her vision. There was no dementia. Computed tomography (CT) scan and magnetic resonance imaging (MRI) suggested a tumor in the right posterior parietal white matter. A biopsy of the lesion displayed granulomatous angiitis and severe cerebrovascular amyloidosis, but no tumor was identified. Chronic inflammation with an occasional multinucleated giant cell was seen about the amyloid-infiltrated vessels. The cortex demonstrated gliosis but no plaques or tangles. Subsequently, the patient was treated with steroids and Cytoxan, with an improvement in her neurologic status. She died of opportunistic bronchopneumonia 8 months after the initial onset of her symptoms. On postmortem examination, the biopsied area of the brain showed atrophy with gliosis. Amyloid angiopathy was present but in much lesser degree than in the biopsy. Scant perivascular inflammatory infiltrates were seen only focally, and no giant cells were observed. The amyloid, both in the biopsy and autopsy material, was of the Alzheimer A4 type. This case suggests that steroid and cytoxan treatment ameliorated the angiitis and the amyloid angiopathy as well. The pertinent literature is discussed.- - - - - - - - - - ranking = 0.28571428571429keywords = giant (Clic here for more details about this article) |
9/42. myocardial infarction caused by rheumatoid vasculitis: histological evidence of the involvement of T lymphocytes.Rheumatoid arthritis (RA) is a chronic joint disease that can be complicated with extra-articular manifestations due to vasculitis. We describe a patient with RA who developed systemic vasculitis and died of myocardial infarction. autopsy demonstrated vasculitis of the left anterior descendent and circumflex coronary arteries, which were narrowed or occluded with organizing thrombosis. Formation of granuloma with multinucleated giant cells was also observed in media of the circumflex artery. There was no microscopic evidence of atheroma formation in the coronary arteries. Of note, there was a follicle-like infiltration of CD45RO-positive T lymphocytes in interna of the left coronary arteries and the right renal artery. Although not frequently reported, coronary vasculitis as a cause of myocardial infarction should be considered in patients with RA. Moreover, our results suggest that infiltration of T lymphocytes might be involved in the development of rheumatoid vasculitis.- - - - - - - - - - ranking = 0.14285714285714keywords = giant (Clic here for more details about this article) |
10/42. Temporal concurrence of vasculitis and cancer: a report of 12 cases.OBJECTIVE: Vasculitis has been associated with solid organ and hematologic cancer. The rarity of these associations, and in many reports the lack of temporal relationships, has led to skepticism about vasculitis being a paraneoplastic syndrome. The objective of the present study was to review cases of concurrent vasculitis and cancer at the Cleveland Clinic Foundation over an 18.5-year period and explore evidence that would support the notion of vasculitis being a type of paraneoplastic disease. methods: Retrospective review of the records of all patients diagnosed with vasculitis and cancer within 12 months of each other was performed using an ICD-9 diagnostic data base at the Cleveland Clinic Foundation. Patients with known chronic autoimmune disease or serologic evidence of hepatitis B or C infection were excluded. A standardized data collection instrument was used to document information about presentation, treatment, and course of illness. RESULTS: During the 18.5 years of our study, more than 15 million inpatients and outpatients were seen at the Cleveland Clinic. Of these, 2,800 patients had vasculitis independent of cancer, more than 69,000 patients had cancer, and 69 patients had been identified who had both malignancies and systemic vasculitis. Only 12 patients were identified in whom both vasculitis and cancer occurred within the same 12 months. Mean age was 65 years (range 45-79). There was no gender preference (M = F). In 8 of the 12 cases, diagnoses were made within 3 months of each other. In 6 of the patients, the diagnoses of both processes were made within 1 month. Ten of the 12 patients had vasculitis 1 to 3 months prior to or concurrent with the diagnosis of cancer. Six of the 12 patients had solid organ tumors, 4 had lymphoma, 1 had leukemia, and 1 had multiple myeloma. The most common vasculitis was cutaneous leukocytoclastic vasculitis (LCV), which occurred in 7 cases. Four cases of LCV were associated with solid organ tumors. Other vasculitides included giant cell arteritis (n = 2), polyarteritis nodosa (n = 2), and Wegener's granulomatosis (n = 1). The response of the vasculitis to glucocorticoid and cytotoxic therapy varied. Complete remission of vasculitis occurred in 4 of the patients, partial improvement occurred in 4 patients, and no improvement was noted in 4 patients. Complete remission occurred in 3 of the 4 patients in whom vasculitis and cancer were treated concurrently. Eight of 10 patients in whom followup was greater than 2 months demonstrated concordance of disease activity and treatment response for both cancer and vasculitis. CONCLUSION: The close temporal relationship of cancer and vasculitis in our patients adds to circumstantial evidence of vasculitis at times being a paraneoplastic condition. Failure of a vasculitis to respond to conventional therapy should raise questions about underlying malignancy. Effective treatment of the cancer enhances the likelihood of improvement in vasculitis.- - - - - - - - - - ranking = 0.14285714285714keywords = giant (Clic here for more details about this article) |
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