1/67. Pituitary involvement by Wegener's granulomatosis: a report of two cases.We describe two cases of pituitary involvement by Wegener's granulomatosis. At initial presentation, or during subsequent disease "flares," a pattern of pituitary abnormality was suggested. During periods of remission, we found the pituitary returned to a nearly normal appearance. Loss of the normal posterior pituitary T1 hyper-intensity matched a clinical persistence of diabetes insipidus, suggesting there is permanent damage to this structure by the initial disease process.- - - - - - - - - - ranking = 1keywords = process (Clic here for more details about this article) |
2/67. 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 = process (Clic here for more details about this article) |
3/67. eosinophilia in Wegener's granulomatosis.Significant eosinophilia is a prominent feature in churg-strauss syndrome but has only rarely been described in Wegener's granulomatosis (WG). We describe two Wegener's granulomatosis patients with > 30% eosinophilia on their initial presentations. Other etiologies that could account for their eosinophilia were excluded. Both patients had pulmonary alveolar hemorrhage, sinusitis, arthritis, high-titer cytoplasmic antineutrophil cytoplasmic antibodies (cANCA), and proteinase-3 antibodies, but no evidence of renal disease. Herein we discuss eosinophilia, the differential diagnosis of pulmonary infiltrates and eosinophilia, the role of cANCA in vasculitis and autoimmune disease, compare Wegener's granulomatosis and churg-strauss syndrome, and review possible pathogenic mechanisms.- - - - - - - - - - ranking = 81.551581655029keywords = alveolar (Clic here for more details about this article) |
4/67. Granulomatous pneumocystis carinii pneumonia in Wegener's granulomatosis.This study reports on a first case of granulomatous pneumocystis carinii pneumonia (PCP) in a human immunodeficiency virus-negative patient with antineutrophil cytoplasmic antibody-positive Wegener's granulomatosis whilst receiving immunosuppressive treatment. The patient presented with diffuse alveolar haemorrhage, pauci-immune rapid progressive glomerulonephritis and leukocytoclastic vasculitis of the skin. Granulomatous pneumocystis carinii pneumonia developed under immunosuppressive treatment with cyclophosphamide and prednisone. At the time pneumocystis carinii pneumonia developed, there was a marked lymphopenia with a very low CD8 cell count in the blood. Grocott staining in bronchoalveolar lavage fluid revealed no pneumocystis carinii. The diagnosis was made via a video-assisted thoracoscopic lung biopsy which showed granulomas containing high numbers of pneumocystis carinii cysts.- - - - - - - - - - ranking = 163.10316331006keywords = alveolar (Clic here for more details about this article) |
5/67. Rapidly progressive antineutrophil cytoplasm antibodies associated with pulmonary-renal syndrome in a 10-year-old girl.CONTEXT: The term pulmonary-renal syndrome has been used frequently to describe the clinical manifestations of a great number of diseases in which pulmonary hemorrhage and glomerulonephritis coexist. The classic example of this type of vasculitis is Goodpastures syndrome, a term used to describe the association of pulmonary hemorrhage, glomerulonephritis and the presence of circulating antiglomerular basement membrane antibodies (anti-GBM). Among the several types of systemic vasculitides that can present clinical manifestations of the pulmonary-renal syndrome, we focus the discussion on two types more frequently associated with antineutrophil cytoplasm antibodies (ANCA), microscopic polyangiitis and Wegener's granulomatosis, concerning a 10 year old girl with clinical signs and symptoms of pulmonary-renal syndrome, with positive ANCA and rapidly progressive evolution. CASE REPORT: We describe the case of a 10-year-old girl referred to our hospital for evaluation of profound anemia detected in a primary health center. Five days before entry she had experienced malaise, pallor and began to cough up blood-tinged sputum that was at first attributed to dental bleeding. She was admitted to the infirmary with hemoglobin = 4 mg/dL, hematocrit = 14 %, platelets = 260,000, white blood cells = 8300, 74 % segmented, 4 % eosinophils, 19 % lymphocytes and 3 % monocytes. Radiographs of the chest revealed bilateral diffuse interstitial alveolar infiltrates. There was progressive worsening of cough and respiratory distress during the admission day, when she began to cough up large quantities of blood and hematuria was noted. There was rapid and progressive loss of renal function and massive lung hemorrhage. The antineutrophil cytoplasm antibody (ANCA) test with antigen specificity for myeloperoxidase (anti-MPO) was positive and the circulating anti-GBM showed an indeterminate result.- - - - - - - - - - ranking = 81.551581655029keywords = alveolar (Clic here for more details about this article) |
6/67. May 2001: 32 year old female with dural mass encircling cervical spinal cord.The May COM. A 32-year-old woman with a history of previous mastoid surgery presented with bilateral extremity weakness and ambulatory instability. MRI revealed a dural-based mass completely encircling the upper cervical spinal cord. Workup was significant for an abnormally elevated c-ANCA, positive at a dilution of 1:128. A portion of the lesion was removed by a posterior surgical approach to decompress the cervical cord. Histologic examination of the dura showed a dense granulomatous infiltrate with vasculitis and giant cells; coupled with the positive c-ANCA, the process was felt to be most consistent with Wegener's granulomatosis. Wegener's granulomatosis infrequently involves the dura or meninges and has not previously been reported to affect dura of the cervical cord. Symptomatic improvement followed surgical decompression and high-dose corticosteroid therapy, with resultant resolution of an elevated c-ANCA titer.- - - - - - - - - - ranking = 1keywords = process (Clic here for more details about this article) |
7/67. Periaortitis heralding Wegener's granulomatosis.We describe a 47-year-old man who successively presented atheromatous coronary artery disease, cholesterol embolism after angioplasty, periaortitis with presence of c-ANCA, and finally typical pulmonary lesions caused by Wegener's granulomatosis. This case illustrates the link between atheromatous and inflammatory process and emphasizes that periaortitis may be a feature of Wegener's granulomatosis.- - - - - - - - - - ranking = 1keywords = process (Clic here for more details about this article) |
8/67. Atypical squamous cells as a diagnostic pitfall in pulmonary Wegener's granulomatosis. A case report.BACKGROUND: Wegener's granulomatosis (WG) is characterized by systemic, necrotizing, granulomatous inflammation accompanied by vasculitis. It classically involves the triad of the upper respiratory tract, lungs and kidneys. Isolated pulmonary lesions of WG may present in some patients as pulmonary masses, simulating neoplasms. The features of WG can be suggested by cytologic study. Atypical epithelial cells associated with WG have previously been reported as a cause of a false positive diagnosis of bronchoalveolar carcinoma. CASE: In this case the cytologic findings included atypical squamous cells in a background of acute, chronic and granulomatous inflammation. In several respiratory specimens the atypical squamous cells were incorrectly interpreted as diagnostic of squamous cell carcinoma. The correct diagnosis of WG was confirmed with open lung biopsy, which demonstrated necrotizing granulomatous inflammation with geographic necrosis and associated vasculitis. CONCLUSION: Markedly atypical squamous cells mimicking squamous cell carcinoma can be found accompanying the inflammatory process associated with WG and are a possible diagnostic pitfall. The possibility of WG as well as other inflammatory processes should always be considered in the differential diagnosis of squamous cell carcinoma of the lung. This case is the only reported case of WG in which atypical squamous cells were a diagnostic pitfall, initially suggesting a diagnosis of squamous cell carcinoma.- - - - - - - - - - ranking = 83.551581655029keywords = alveolar, process (Clic here for more details about this article) |
9/67. Zonal distribution of glomerular collapse in renal allografts: possible role of vascular changes.Collapsing glomerulopathy (CG), an aggressive variant of focal segmental glomerular sclerosis, is a renal disease with severe proteinuria and rapidly progressive renal failure. The pathogenesis of CG is unknown. It strongly resembles human immunodeficiency virus (hiv)-associated nephropathy, but the patients are hiv negative. The characteristic glomerular lesion is capillary loop collapse with prominent podocytes filling Bowman's space. Interestingly, these glomerular changes are usually associated with severe tubulointerstitial injury, including tubular epithelial degenerative changes, microcystic dilation of several tubules, and interstitial inflammatory cell infiltrate. Recently, it became evident that the morphologic pattern of CG may appear not only in native kidneys, but also de novo in renal allografts, and that the pattern of CG in renal transplants is not always associated with severe proteinuria. Studies describing CG in renal allografts are all based on biopsies. We report 3 allograft nephrectomy specimens that showed a zonal distribution of the characteristic collapsing glomerular changes with associated tubulointerstitial injury. All 3 kidneys had obliterative vascular changes. One nephrectomy specimen had chronic obliterative transplant arteriopathy, 1 had acute vascular rejection, and 1 had thrombotic microangiopathy. None of the patients had severe proteinuria. Our cases suggest that the morphologic pattern of CG in renal allografts may not represent the same disease process as CG in native kidneys and provide further evidence that collapsing glomerular changes do not define the disease entity of CG, but rather represent a pattern of renal injury. Among other factors, hemodynamic disturbance may play a role in the development of the pattern of CG in renal allografts.- - - - - - - - - - ranking = 1keywords = process (Clic here for more details about this article) |
10/67. Treatment of scleral melt associated with uveal prolapse by pericardium grafting in Wegener's granulomatosis.PURPOSE: To report the use of preserved pericardium tissue in the treatment of scleral melt associated with uveal prolapse secondary to Wegener's granulomatosis. Wegener's granulomatosis may initially present with ophthalmic findings including scleral melting with uveal prolapse. In order to preserve the globe and treat this potentially fatal disease, prompt diagnosis, surgical correction of uveal prolapse, and subsequent immunosuppressive therapy are imperative. If advanced scleral melt associated with uveal prolapse is present, pericardium grafting with conjunctival advancement is a useful technique. methods: Case report of a 70-year-old white female who initially presented with a unilateral sderal melt associated with uveal prolapse involving 6 clock hours of the surgical limbus. Emergent pericardium grafting of the involved limbus with conjunctival advancement performed to correct the endangered globe. Laboratory data including sedimentation rate, cANCA, computerized tomography of the chest, and urinalysis confirmed the diagnosis of Wegener's granutomatosis. RESULTS: After pericardium grafting and immunosuppressive therapy the patient returned to baseline visual acuity, the sedimentation rate normalized, and the pulmonary lesions and renal failure resolved. The patient has been followed for 3 years since the initial diagnosis of Wegener's granulomatosis and there has been no recurrence. CONCLUSIONS: To the best of the author's knowledge, this is the first report using processed pericardium to emergently treat scleral melt associated with uveal prolapse, thereby allowing preservation of the globe. In addition to management of the scleral melt, systemic evaluation and treatment with immunosuppressive therapy are necessary.- - - - - - - - - - ranking = 1keywords = process (Clic here for more details about this article) |
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