1/102. Wegener's granulomatosis causing laryngeal and tracheobronchial obstruction in an adolescent girl.A 12-year-old girl presenting with a painful neck mass and ulcerated gingival lesions was found to have Wegener's granulomatosis involving the gingivae, paranasal sinuses, ears, mastoids, larynx, trachea, bronchial tree, lungs, kidneys and joints. The initial illness and two subsequent exacerbations were treated with intravenous and oral cyclophosphamide and prednisone. Secondary infections have been managed with establishment of good drainage and use of appropriate antibiotics. The patient is well with a normal sedimentation rate 4 1/2 years after the onset of her disease.- - - - - - - - - - ranking = 1keywords = kidney (Clic here for more details about this article) |
2/102. kidney disease and silicosis.AIM: To determine the prevalence of kidney disease in a cohort of individuals with silicosis. methods: review of medical records and questionnaires from patients reported to a state surveillance system for silicosis. Reporting of individuals with silicosis is required by state law. All individuals with silicosis reported as required by law to the State of michigan. Individuals included in this article were reported from 1987 to 1995. Cases were reported by hospitals, physicians, the state workers' compensation bureau, or from death certificates. Only individuals who met the criteria for silicosis developed by the National Institute for Occupational safety and health (NIOSH) were included. RESULTS: medical records were reviewed of 583 individuals with confirmed silicosis. This was mainly a population of elderly men. Ten percent of the 583 silicotics were found to have some mention of chronic kidney disease, and 33% of the 283 silicotics who we had laboratory tests on had a serum creatinine level >1.5 mg/dl. An association between kidney disease and age and between kidney disease and race was found among this cohort of 583 silicotics. Individuals with silicosis were more likely to have a serum creatinine level >1.5 mg/dl than age- and race-matched controls. However, no relationship between duration of exposure to silica or profusion of scarring on chest X-ray and prevalence of kidney disease or elevated creatinine levels was found. CONCLUSIONS: This study confirms previous case reports and epidemiologic studies of end-stage renal disease that found an association between kidney disease and exposure to silica. The epidemiologic data are conflicting on the mechanism by which silica causes kidney disease and are compatible with silica being able to cause kidney disease by both an autoimmune and direct nephrotoxic effect. Chronic kidney disease should be considered as a complication of silicosis.- - - - - - - - - - ranking = 28.422287838834keywords = kidney disease, kidney (Clic here for more details about this article) |
3/102. Granulomatous renal pseudotumor in Wegener's granulomatosis: imaging findings in one case.Wegener's granulomatosis is a clinicopathological entity characterized by necrotizing granulomatous angiitis involving the upper- and/or lower respiratory tract and the kidneys. Renal involvement is usually characterized by a rapidly progressive necrotizing glomerulonephritis. A case is presented of a patient who developed renal failure and presented a solitary pseudotumoral lesion in the upper pole of the left kidney. Imaging characteristics on US, CT and MR imaging are discussed. The diagnosis was confirmed with ultrasound-guided needle biopsy.- - - - - - - - - - ranking = 2keywords = kidney (Clic here for more details about this article) |
4/102. Wegener's granulomatosis: pitfalls in the management of pulmonary disease: A case of Wegener's granulomatosis with a hilar mass.Wegener's granulomatosis (WG) is a systemic, granulomatous vasculitis that typically affects the upper airways, lungs, and, in most cases, the kidneys. lung involvement occurs in 85% of patients. A classic feature of WG is multiple pulmonary nodules, which frequently cavitate. Hilar adenopathy or mediastinal masses are rare. These atypical pulmonary findings should raise suspicion of diseases other than WG and lead to biopsy with cultures, even when the diagnosis of WG appears to be certain. These guidelines proved to be reliable in a patient with WG in whom a hilar mass was associated with tuberculosis.- - - - - - - - - - ranking = 1keywords = kidney (Clic here for more details about this article) |
5/102. Wegener's granulomatosis in the elderly.Wegener's granulomatosis classically involves the upper respiratory tract, lungs and kidneys. Rarely, it also affects the skin and heart. Cardiac involvement is uncommon in Wegener's granulomatosis and myocardial infarction is seldom highlighted. It can be a difficult diagnosis to make in the elderly who often have multiple co-existing illnesses. We present a case of a 75-year-old Chinese woman with interesting cardiac and dermatological manifestations of Wegener's granulomatosis.- - - - - - - - - - ranking = 1keywords = kidney (Clic here for more details about this article) |
6/102. diabetes insipidus and anterior pituitary insufficiency as presenting features of Wegener's granulomatosis.Central diabetes insipidus (DI) is a rare complication of Wegener's granulomatosis (WG), which usually presents after pulmonary or kidney involvement. Anterior pituitary dysfunction secondary to WG has been extremely rare, documented in only three cases. We report a case of a 47-year-old postmenopausal woman who was diagnosed with hypopituitarism in November 1999 and started on vasopressin, thyroxine, and hydrocortisone. She sought treatment at the Mayo Clinic in February 2000 with a purpuric rash, fever, cough, shortness of breath, and blood in the sputum. Computed tomography of the chest showed a 6-cm irregular mass in the right lower lobe, and a biopsy of the mass showed marked reactive atypia and necrosis. Positive C-antineutrophil cytoplasmic antibodies (ANCA) and skin biopsy of a purpuric lesion showing leukocytoclastic vasculitis confirmed the diagnosis of WG. Hormonal studies showed low gonadotropins, thyroid-stimulating hormone (TSH), and prolactin. magnetic resonance imaging (MRI) of the head showed cystic enlargement of the pituitary gland that did not enhance with gadolinium. Two months into the treatment with cyclophosphamide and prednisone, she had persistent pituitary dysfunction, despite the normal appearance of the pituitary gland on repeat MRI. We conclude that WG should be included in the differential diagnosis of DI and anterior pituitary dysfunction in the proper clinical setting. early diagnosis and treatment may be crucial in preventing pituitary gland destruction and long-term endocrine sequelae. We suggest screening for anterior pituitary failure in the presence of the WG-associated DI.- - - - - - - - - - ranking = 1keywords = kidney (Clic here for more details about this article) |
7/102. Wegener's granulomatosis presenting as mediastinal soft tissue mass invading the tracheal wall.Wegener's granulomatosis (WG) is a clinicopathologic entity of unknown cause characterised by a necrotising granulomatous vasculitis affecting multiple organs, especially the upper and lower respiratory tracts, lung and kidney. The lung is the most frequently, and sometimes the only involved organ. Single or multiple nodules, with or without cavitation, are the most common pulmonary manifestations in WG, but mediastinal involvement is atypical. The sole tracheal involvement is rare and hilar and mediastinal involvement has been thought not to be part of the clinical feature. We experienced a rare case of WG presenting as paratracheal mediastinal lesions with tracheal wall invasion, which responded dramatically to corticosteroid treatment. We present this case with a review of the literature.- - - - - - - - - - ranking = 1keywords = kidney (Clic here for more details about this article) |
8/102. Wegener's granulomatosis: presentation as an otologic problem.Presentation of Wegener's granulomatosis as an otologic problem is unusual and leads to incorrect diagnosis and unsuccessful therapy. In seven years, five patients with a diagnosis of otitis media failed to respond to the usual therapeutic regimens. All had surgical manipulations on the middle ear or mastoid, or both. Wegener's granulomatosis was suspected because of persistence of the otologic problem. All patients developed pulmonary lesions and had positive diagnoses by biopsy of either the lung, kidney, or upper respiratory mucosa. Withe timely treatment, all patients have survived.- - - - - - - - - - ranking = 1keywords = kidney (Clic here for more details about this article) |
9/102. Wegener's granulomatosis triggered by infection?Wegener's granulomatosis is a systemic disease of unknown origin, although recent studies suggest that auto-immune mechanisms and infection play a role in the pathogenesis of this disease. Wegener is characterized by a necrotizing vasculitis involving the lungs (pulmonary infiltrates), the upper airways and the kidneys (rapidly progressive glomerulonephritis). We present a case of a male patient admitted because of progressive deterioration of the general condition with weight loss, a unilateral neck mass, unilateral purulent rhinorrea and fever. CT-scan evaluation demonstrated a unilateral expanding mass in the sing-nasal cavity, obliterating the ethmoid complex. MRI revealed signs of intracranial inflammatory reaction and onset of absedation. A malignancy was suspected but a diagnosis of Wegener's granulomatosis was established based on histologic criteria (nasal biopsy) and a positive titer for anti-cytoplasmic antibodies (cANCA). During follow-up, nasal carriage of Staphyloccocus Aureus could be documented. An overview of Wegener's granulomatosis will be provided with emphasis on the potential role of acute infections as a trigger for Wegener's granulomatosis and the head and neck manifestations.- - - - - - - - - - ranking = 1keywords = kidney (Clic here for more details about this article) |
10/102. Three cases of C-ANCA-positive vasculitis treated with immunoadsorption: possible benefit in early treatment.Wegener's granulomatosis is a vasculitic disease predominantly affecting the upper respiratory tract, lungs, and kidneys. Three patients with Wegener's granulomatosis and rapidly progressive glomerulonephritis were treated with an intensified regimen of immunoadsorption (IA) (Excorim or Therasorb) in addition to cyclophosphamide (CYC) and methylprednisolone (PRE). Patient A had been in remission under oral CYC/PRE. The first exacerbation was treated successfully with 4 IA treatments without changing medication. Patient B experienced 3 flares within 1 year, which were treated with 28 IA (3-7 IAs/course), intravenous CYC after each course, and PRE. A fall of creatinine levels from 120 to 190 micromol/L to 100 micromol/L was noted after IA and before administration of CYC. Patient C presented in uremia. autoantibodies were eliminated by 11 IA treatments parallel to CYC/PRE therapy. They remained within a normal range for >1 year's follow-up; however, kidney function did not return. In conclusion, the observations in patients A and B suggest a beneficial therapeutic effect of early IA in WG.- - - - - - - - - - ranking = 2keywords = kidney (Clic here for more details about this article) |
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