1/52. wegener granulomatosis in a child: cutaneous findings as the presenting signs.wegener granulomatosis (WG) is a systemic disease that is particularly unusual in children. A limited form has been described without renal involvement. We report a 14-year-old girl in whom the disease started with acneiform nodular and papular lesions on the forehead. Later necrotic ulcers developed on her forehead, arms, and buttocks. The cutaneous lesions were associated with upper and lower respiratory tract involvement, low-grade fever and arthralgias. Subsequently clinical and laboratory evaluations (increased ESR; leukocytosis and presence of serum IgG antibodies cANCA = 1:160), with chest roentgenograms revealing pulmonary densities and parenchymal infiltration, suggested the diagnosis of WG. The histologic findings of a cutaneous biopsy specimen were ulceration of the epidermis with diffuse neutrophilic inflammatory infiltrate and a late-stage small vessel vasculitis in the dermis. Histopathology of the nasal mucosa was characterized by a granulomatous process with a dense lymphohistiocytic infiltrate with few giant cells, a finding that confirmed the diagnosis of WG. No renal involvement was present. One month of cyclophosphamide (125 mg/day) and prednisone (70 mg/day) therapy markedly improved the patient's clinical condition. At present, 1 year later, she is free from any signs of the disease. According to the literature, the frequency of cutaneous lesions in WG ranges from 16% to 46%. They are the presenting sign only in 6% of patients. Cutaneous lesions are even more uncommon in children. In particular, an "acneiform" presentation is a rare finding in WG.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
2/52. Isolated abdominal vasculitis as an atypical presentation of Wegener's granulomatosis.Although current classifications characterize vasculitic syndromes based upon the size of the vessels involved, the histopathology, and the presence or absence of antineutrophil cytoplasmatic antibodies ANCA (1-3), those occasional patients with vasculitis whose features are not typical may evade diagnosis and effective treatment. We report one such patient who presented with bilateral refractory uveitis and abdominal angina who had a positive C-ANCA. Because of his atypical presentation, this patient's disease progressed over 8 yr despite an extensive gastrointestinal evaluation, before a diagnosis of vasculitis was established angiographically, and immunosuppressive therapy was begun.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
3/52. Periaortitis and aortic dissection due to Wegener's granulomatosis.We describe here a patient with abdominal periaortitis and intramural dissection as early manifestations of Wegener's granulomatosis (WG). Surgical biopsies taken from the retroperitoneal inflammatory tissue surrounding the aorta showed granulomatous vasculitis. The patient had antiproteinase-3 antibodies and suffered from nasal, pulmonary, nervous and renal WG involvement. Although being a vasculitis of medium size and small vessels, WG should be included in the systemic vasculitides which can give rise to (peri)aortic inflammation.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
4/52. Esophageal involvement in Wegener's granulomatosis: a case report and review of the literature.Wegener's granulomatosis is characterized by a granulomatous arteritis involving the upper and lower respiratory tracts, progressive glomerulonephritis and systemic symptoms attributable to small vessel vasculitis. Although multisystemic manifestations are frequent, involvement of the gastrointestinal tract is uncommon. Cases have been reported of intestinal perforation, ulceration and hemorrhage. A patient whose initial presentation of Wegener's granulomatosis was odynophagia secondary to esophageal vasculitis is described. endoscopy revealed multiple punched out ulcerations in the esophagus, which resolved with standard therapy for systemic Wegener's granulomatosis. There are only two previous reports of symptomatic esophageal vasculitis in patients with Wegener's granulomatosis. These reports illustrate the need to consider odynophagia as a reflection of disease activity as opposed to complications of immunosuppressive therapy.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
5/52. Sudden death and Wegener's granulomatosis of the pituitary.Involvement of brain parenchyma or meninges in ANCA-associated small-vessel vasculitis such as Wegener's granulomatosis (WG) is not uncommon. In contrast, involvement of the pituitary is exceedingly rare with only a few cases reported so far. The diagnosis is usually made on the basis of imaging techniques and abnormal pituitary function tests in the setting of active systemic vasculitis. However, histology-proven involvement of the pituitary by WG has not been reported so far. We report a case of WG with histology-proven granulomatous necrotizing inflammation of the pituitary and hypothalamo-pituitary stalk, disclosed at autopsy after the patient had died suddenly and unexpectedly in his sleep. In a setting of histology-proven WG, these findings were regarded as a pituitary manifestation of the disorder. A distinct cause of death could not be found, hence we speculate that hypothalamo-pituitary inflammation due to WG may have caused the sudden death in this patient.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
6/52. Cerebral and oculorhinal manifestations of a limited form of Wegener's granulomatosis with c-ANCA-associated vasculitis.The authors report on cerebral and oculorhinal manifestations in a patient with a cytoplasmic pattern of antineutrophil cytoplasmic autoantibody (c-ANCA)-associated vasculitis. Recurrent tolosa-hunt syndrome, cavernous sinus syndrome, Raeder's paratrigeminal neuralgia, and seizures were the major clinical manifestations. brain MRI showed localized enhancing lesions initially in the cavernous sinus and later in the convexity pachymeninges. The lesions disappeared following 9 months of oral prednisolone (15 mg/day) and cyclophosphamide (100 mg/day) therapy. The presence of c-ANCA, demonstration of vasculitis, and depositions of immunoglobulin g (IgG) and fibrinogen in the vessel walls of pachymeninges of the patient confirmed an immune-mediated cause of the vasculitis. Cranial pathology without renal and pulmonary involvement suggests a variant of Wegener's granulomatosis, which is called the "limited" form of Wegener's granulomatosis. MRI, Raeder's paratrigeminal neuralgia, localized pachymeningitis.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
7/52. Vasculitis of the internal carotid artery in Wegener's granulomatosis: comparison of ultrasonography, angiography, and MRI.A 37-year old male with newly diagnosed, untreated Wegener's granulomatosis including glomerulonephritis, sinusitis, conjunctivitis, arthralgias, and positive cANCA, developed a pulsating tumor in the left submandibular region and a reversible ischemic neurologic deficit. ultrasonography revealed both a hyperechoic wall thickening of the left proximal internal carotid artery as is known in Takayasu's arteritis and a surrounding hypoechoic region typical for perivasculitis. The wall thickening and the perivascular infiltrate could be less clearly seen by MRI. ultrasonography, angiography, and MRI demonstrated a 3 cm long, 30%, stenosis. angiography and MRI additionally found a more distally located kinking of the internal carotid artery that was missed by ultrasonography. Carotid artery vasculitis is a rare complication of Wegener's granulomatosis. In this case ultrasonography was superior to angiography and MRI to visualize the artery wall and the surrounding tissue, but it failed to evaluate the whole distance of the vessel.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
8/52. Antigen inhalation as a triggering factor in systemic small-sized-vessel vasculitis. Four cases.In order to identify small-sized-vessel vasculitis occurring immediately after massive inhalation of particles which could be considered antigenic, we performed a retrospective review of patients who consulted our Department of internal medicine between 1980 and 1998 and were diagnosed as having small-vessel vasculitis that developed immediately after massive exposure to inhaled particles. Four patients, 27 to 55 years old, presented with small-sized-vessel vasculitis (one with Wegener's granulomatosis and three with churg-strauss syndrome) which developed after massive inhalation of particles. Clinical manifestations occurred from a few hours to 10 days after inhalation of diesel fumes, cereal dust, flour or cereal dust and/or pigeon droppings. Three patients remain disease-free 8, 9 and 18 years after discontinuing treatment. One patient remains well after 3 years on low-dose corticosteroids and cyclophosphamide therapy. Our data suggest that primary antigens can cause small-sized-vessel vasculitides. Identifying such etiological factors could improve our understanding of the pathogenesis of systemic vasculitides and prevent relapses due to similar antigenic exposure.- - - - - - - - - - ranking = 8keywords = vessel (Clic here for more details about this article) |
9/52. Splenic involvement in Wegener's granulomatosis.OBJECTIVE: A clinicopathologic review of splenic involvement in Wegener's granulomatosis. DESIGN: A retrospective case review, spanning a 10-year period, identified five patients with Wegener's granulomatosis and splenic involvement. SETTING: A large teaching hospital and outpatient clinics. INTERVENTIONS: Cytotoxic therapy (cyclophosphamide and corticosteroids), mechanical ventilation in respiratory failure, renal hemodialysis in renal failure, and general supportive care. RESULTS: Necrotizing granulomatous inflammation and vasculitis with fibrinoid necrosis were found in the spleen in one antemortem case. At autopsy, two cases demonstrated extensive infarction; microscopic examination revealed parenchymal coagulative necrosis, microcalcification, and vascular thrombosis, but no evidence of vasculitis. Two other cases showed nonspecific changes of diffuse hyalinization of blood vessels, vascular congestion, and hemosiderin deposition. CONCLUSIONS: Splenic involvement in Wegener's granulomatosis is rarely diagnosed during life, occurs more frequently than once thought, and can occasionally lead to considerable morbidity.- - - - - - - - - - ranking = 4.7846763929695keywords = blood vessel, vessel (Clic here for more details about this article) |
10/52. Wegener's granulomatosis: angiographic study and case report with prolonged survival.In the limited form of Wegener's granulomatosis pulmonary arteries were pruned and slightly stretched by a cavitating granuloma. Bronchial and renal vasculature were normal. The microscopic focal small vessel changes are not appreciable at angiography. Therefore, angiography is of no value in establishing the diagnosis of Wegener's granulomatosis.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
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