Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0262471 (ENT)
 5,307 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

In this uncontrolled study 15 patients with ANCA-associated systemic vasculitis, who were poor responders to conventional therapy, were treated with single or multiple courses of intravenous immunoglobulin (IVIG), 30 g/day over 5 days. Clinical and serological evaluation was performed before and 4 weeks after IVIG. Six of the 15 patients experienced clinically significant benefit from IVIG. Improvement was confined to single organ manifestations (skin, ENT findings), no improvement was seen with conjunctivitis and scleritis, pericarditis or nephritis. No patient experienced complete remission after IVIG. Repeated courses of IVIG at 4-week intervals were no more effective than single courses. In six anti-proteinase 3 (PR3)-positive patients pretreatment sera were incubated with F(ab')2 fragments of the IVIG preparation in vitro to measure the inhibitory effect of IVIG on anti-PR3 activity. An inhibition of anti-PR3 activity by 25-70% was observed; this did not correlate with clinical effects. Approximately 40% of patients benefited from IVIG treatment, though complete remission of disease activity did not occur. Neither clinical characteristics nor the inhibitory effect of the IVIG preparation on serum anti-PR3 activity in vitro predicted clinical response to this treatment modality.
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PMID:Treatment of anti-neutrophil cytoplasmic antibody (ANCA)-associated systemic vasculitis with high-dose intravenous immunoglobulin. 762 88

The primary ANCA associated vasculitides, Wegener's granulomatosis (WG), Churg Strauss syndrome (CSS) and microscopic polyangiitis (MPA), frequently affect the ENT region. For several reasons WG is of special significance for the otorhinolaryngologist. First, disease activity limited to the upper respiratory tract (localized WG) often proceeds the systemic vasculitis (generalized WG). The early diagnosis therefore has decisive consequences for stage adapted therapy. Second, in most cases (nearly 80%) WG is diagnosed histologically on biopsy specimens from the ENT region. During the initial phase of WG this is of diagnostic relevance, because at this stage the serologic parameters (acute-phase proteins) usually have a normal value and PR3-ANCA is (still) negative in 2/3 of the patients. Third, in many cases recurrences reveal increased activity in the ENT region, or start in this area. Clinically in most cases chronic rhinosinusitis with crusting and epistaxis is seen, sometimes with septal perforation and/or saddle nose. Apart from this there are often unclear middle ear symptoms with recurrent effusions and the inner ear is sometimes also affected. Laryngeal manifestations are typically located in the subglottic area and lead to subglottic stenosis. In the differential diagnosis, diseases in which epitheloid cell granulomas occur, such as sarcoidosis and TBC, need to be considered, but also foreign body granulomas and fungus diseases. Finally malignant tumours, especially malignant lymphomas, have to be ruled out.
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PMID:[Manifestations of primary vasculitis in the ENT region]. 1158 19

A 49 year old woman was admitted because of general poor health condition having lasted for 15 days and been associated for 6 months with sinusitis. During hospitalisation, she developed thrombosis of the retina central artery and signs of cerebral vasculitis. A diagnosis of Wegener disease was suggested based on one side on the presence of multiple excavated pulmonary macronodules and, on the other side, on the signs of cerebral vasculitis. No renal dysfunction was documented in our patient. The detection of serum antineutrophil antibodies (c-ANCA and anti-PR3) confirmed the diagnosis of Wegener disease without the need of surgical pulmonary biopsy. In addition to the typical ENT and pulmonary symptoms, this particular clinical presentation of unusual ocular and cerebral symptoms, reminds us that Wegener disease is a vasculitis that can give multisystemic disorders.
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PMID:[Case of the month. Wegener's disease]. 1264 92

A case of Wegener's granulomatosis in female aged 47 is described. Otological symptoms and signs connected with middle ear inflammation and masked mastoiditis about two months outstriped other signs particular from lower respiratory tract. The patient was surgically treated--antromastoidectomy was performed. After few days rapid worsening of patient's general state followed with hectic fever and inflammatory pulmonary changes. The patient was next treated in the Clinic of Pulmonary Diseases and Tuberculosis where the diagnosis of Wegener's granulomatosis was established on the basis of immunological findings (antibodies c-ANCA and PR3). The patient is treated from 9 months with cyclophosphamide and prednisone with improvement. The inability of ENT-surgeon in proper diagnosis and treatment in initial stage of the disease is stressed.
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PMID:[Otologic signs and symptoms as first manifestations of Wegener's granulomatosis with very severe clinical course--review of the literature and case report]. 1531 97

Wegener's granulomatosis (WG) is rarely diagnosed during the reproductive years and uncommonly manifests for the first time during pregnancy. We report a case of de novo WG presenting at 30 weeks gestation with classical symptoms of WG (ENT, pulmonary). The diagnosis was confirmed by radiological, laboratory, and histological investigations. With a multidisciplinary approach, she had a successful vaginal delivery of a healthy baby. She was treated successfully by a combination of steroids, azathioprine and intravenous immunoglobulin in the active phase of disease for induction of remission and by azathioprine and steroids for maintenance of remission. The significant improvement in her symptoms allowed us to continue her pregnancy to 37 weeks when delivery was electively induced. Transplacental transmission of PR3-ANCA occurred but the neonate remained well. This case of de novo WG during pregnancy highlights the seriousness of this disease and the challenge in management of such patients.
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PMID:Wegener's granulomatosis occurring de novo during pregnancy. 1964 53

Granulomatosis with polyangiitis (GPA) (Wegener) is a necrotizing vasculitis combining inflammation of the vascular wall and peri- and extravascular granulomatosis. Clinically, GPA is characterized in its full form by ENT signs, lung, and kidney involvement. One of the key features of GPA is the presence of ANCAs-cytoplasmic in approximately 90% of systemic forms and in 50% of localized forms-directed against proteinase 3 in most cases. PR3-ANCAs are highly specific to GPA and therefore have a high diagnostic value. Treatment of GPA is based on a combination of corticosteroids and cyclophosphamide or rituximab, which allows remission to be achieved in more than 80% of cases. Azathioprine was the most widely used maintenance treatment, but low-dose semi-annual rituximab maintenance infusions further decrease relapses with acceptable safety. Nevertheless, relapses occur in more than 50% of cases. One of the biggest treatment challenges is the occurrence of side effects, the severity and frequency of which are often linked to the prolonged treatment course, which is difficult to avoid.
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PMID:Granulomatosis with polyangiitis (Wegener's). 3256 97