Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0042384 (vasculitis)
 20,525 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

In summary, PGWG corresponds to an early phase of WG, presenting only with extravascular granuloma. Primarily, ENT, eye, or lung tissues are involved, but any organ may be affected. The diagnosis is made by typical palisading granuloma associated with elevated c-ANCA levels in the patients' serum. The concept of an early granulomatous lesion (PGWG) facilitates the early diagnosis of WG and leads to classification of different forms of disease according to the multistep evolution hypothesis: first PGWG, then localized or limited forms of the disease, and ultimately generalized vasculitis with renal involvement (classical WG). The concept of PGWG forms the basis for future therapeutic trials where folate antagonists are restricted to PGWG, while immunosuppressive treatment is required for the other stages of WG.
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PMID:Purely granulomatous Wegener's granulomatosis: a new concept for an old disease. 219 77

Kawasaki disease (mucocutaneous lymph node syndrome) is an acute vasculitis of childhood carrying a 1-2 per cent mortality from cardiovascular complications. Despite the extensive literature on Kawasaki disease in paediatric journals, there has been a paucity of documentation in the otolaryngology literature. This is despite the fact that Kawasaki disease may present as an otolaryngological emergency before the diagnosis is established. We describe three cases of Kawasaki disease, all of which presented to the ENT department of this hospital within a period of two months. These cases illustrate the slow evolution characteristic of the disease and highlight the difficulties of diagnosis in the initial febrile stage. We emphasize the importance of considering the diagnosis when treating a young child with a pyrexia resistant to antibiotics, as prompt introduction of therapy may decrease the risk of fatal coronary artery or cardiac involvement.
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PMID:Acute ENT admission in Kawasaki disease. 238 17

In a prospective multicentre study on the clinical significance of ANCA in renal diseases, sera from 920 patients with rapidly progressive renal failure and/or renal disease in association with extrarenal signs suggestive of a systemic vasculitis were tested for the presence of ANCA by indirect immunofluorescence (IIF) and ELISA. 193 of 920 cases (20.9%) were positive by IIF and 180 (19.5%) by ELISA, using a 'crude' cytoplasmic extract as substrate. The sensitivity and specificity of IIF for 'pauci-immune' cresentic necrotizing GN (CNGN), in association or not with systemic vasculitis, was 87.5 and 95.6% respectively. The IIF pattern and antigen specificity (alpha granules and MPO) correlated well with the clinical features: a cANCA pattern (alpha granules) was associated with ENT involvement (probable Wegener's granulomatosis); a pANCA pattern (MPO) with 'idiopathic' CNGN and small-vessel vasculitis without respiratory tract disease (microscopic polyarteritis); patients with a pulmonary-renal syndrome had either c or pANCA in a similar proportion. Our study confirms a high sensitivity and specificity of ANCA for patients with CNGN. ANCA should be considered an important diagnostic test in patients with renal diseases, especially in the presence of rapidly progressive renal failure.
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PMID:Diagnostic significance and antigen specificity of antineutrophil cytoplasmic antibodies in renal diseases. A prospective multicentre study. Italian Group of Renal Immunopathology. 752 6

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

We report five cases of seronegative inflammatory rheumatism associated with nasal polyposis. The patients were four women and one man, mean age 49.5 years (range 42-59 yrs.). Two patients had polyarthralgia predominating in the hands, wrists and knees and two patients had symmetrical acromelic polyarthritis. The fifth patient, a woman, had oligoarticular arthritis. In a single female patient, X-rays showed moderate erosions of both tarsometatarsal bones. The inflammatory syndrome was moderate with mean ESR 23 (12-38) and immunological investigations were negative except for the presence of pANCA (50-200 U) in three patients. HLA-A1, B8, and Bw35 antigens were found in three of the five patients. In all cases, nasal and sinus polyposis (NSP) preceded rheumatism and the joint symptoms were accompanied by worsening of the ENT symptoms. NSP was confirmed by CT scan of the nasal fossae and sinuses. Polyps were surgically removed in four patients and the histology showed neither granuloma nor vasculitis. In four patients the joint symptoms, which responded poorly to nonsteroidal anti-inflammatory drugs (NSAIDs), improved markedly after ENT treatment (surgery and topical steroids) and synthetic antimalarials. The concomitant course of the joint and ENT symptoms suggests there may be a link between inflammatory rheumatism and NSP.
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PMID:Inflammatory rheumatism and nasal polyposis. 909 5

Report of one case of Vernet's syndrome (involving the IX, X and XIth cranial nerves) in a young woman, as early sing of SEL. The patient presented the 4 criteria suggested by the American Society in order to diagnose SEL: arthritis, serositis, positive anti-nuclear antibodies and anemia. The AA. carry out a study in search of other cases sitting in the larynx and a perusal about etiopathogenical theories as well. Hinting, for the clinical picture, of being it due to a localised vasculitis of vasa nervorum, a treatment with corticoids and pentoxifylline was ordered, being the outcome, after 3 weeks, the eradication of ENT syndrome.
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PMID:[Vernet's syndrome as an early manifestation of systemic erythematous lupus]. 919 9

Relapsing polychondritis is a rare disease probably of auto-immune etiology comprising inflammatory involvement of cartilage as well as phenomena of vasculitis. ENT manifestations are frequent and the authors present a case involving chondritis of the auricle, the nasal septum and affecting the cochleo-vestibular system as well. The diagnosis is based on the evocative clinical picture and on histological confirmation. Treatment consists of corticosteroids and immunosuppressive agents.
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PMID:ENT manifestations of relapsing polychondritis. 924 71

Wegener's granulomatosis (WG) is a necrotising granulomatous disease affecting the upper and/or lower respiratory tracts and is associated with focal glomerulonephritis. Formerly believed to be a multisystem disease, a localised form (LWG) is now recognised as a distinct subtype. We describe 6 cases of LWG with no renal or pulmonary involvement detected at the presentation or during follow-up. The total follow-up period ranged from 3 to 7 years in 5 cases. The diagnosis was based on clinical features, antineutrophil cytoplasmic antibody test and histological findings (necrotising granulomatous vasculitis, epithelioid granulomas with varying degrees of chronic inflammatory cells). All patients responded to standard immunosuppressive treatment. Our cases highlight the predilection of LWG for the head and neck region and hence these patients frequently present in the ENT departments. LWG has been discussed as a subtype of WG with a better prognosis and the previous literature has been reviewed on this subject. A high index of suspicion helped by serology and histology enables an early diagnosis, and commencement of proper treatment can prevent the irreversible destructive lesions.
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PMID:Localised Wegener's granulomatosis in otolaryngology: a review of six cases. 1081 Feb 60

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 53-year-old man was referred to the ENT department with a large mass in the left supraclavicular fossa. The histological diagnosis showed the mass to have arisen due to a granulomatous vasculitis consistent with Churg-Strauss syndrome (CSS). CSS usually comprises asthma, eosinophilia and systemic vasculitis although limited forms of the disease exist where one of these diagnostic criteria is missing. This is one such case as the patient was non-asthmatic.
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PMID:Limited form of Churg-Strauss syndrome presenting as a mass in the neck. 1248 82


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