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Wegener's granulomatosis in two sisters.

作者信息

Muniain M A, Moreno J C, Gonzalez Cámpora R

出版信息

Ann Rheum Dis. 1986 May;45(5):417-21. doi: 10.1136/ard.45.5.417.

DOI:10.1136/ard.45.5.417
PMID:3718017
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC1001903/
Abstract

We describe two sisters with necrotising granulomas. The intervals between the onset of symptoms and diagnosis were eight and nine years. The pathological findings showed necrotising granulomas in both patients, this being consistent with a diagnosis of pathergic (Wegener's) granulomatosis. The one patient that accepted treatment responded well to cyclophosphamide, though the facial lesions responded only to radiation therapy. Since the second was almost asymptomatic we conclude that relatives of patients with Wegener's disease should be investigated.

摘要
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/f2ad/1001903/6e212fe7c59a/annrheumd00272-0068-a.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/f2ad/1001903/29f24681b128/annrheumd00272-0066-a.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/f2ad/1001903/523408bbf01f/annrheumd00272-0066-b.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/f2ad/1001903/f9488e080bf5/annrheumd00272-0067-a.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/f2ad/1001903/6e212fe7c59a/annrheumd00272-0068-a.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/f2ad/1001903/29f24681b128/annrheumd00272-0066-a.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/f2ad/1001903/523408bbf01f/annrheumd00272-0066-b.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/f2ad/1001903/f9488e080bf5/annrheumd00272-0067-a.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/f2ad/1001903/6e212fe7c59a/annrheumd00272-0068-a.jpg

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Wegener's granulomatosis in two sisters.
Ann Rheum Dis. 1986 May;45(5):417-21. doi: 10.1136/ard.45.5.417.
2
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本文引用的文献

1
Pathergic granulomatosis.
Am J Med. 1955 Dec;19(6):829-31. doi: 10.1016/0002-9343(55)90150-9.
2
The protracted superficial phenomenon in pathergic (Wegener's) granulomatosis.坏疽性(韦格纳氏)肉芽肿病中的迁延性浅表现象。
Hum Pathol. 1981 May;12(5):458-67. doi: 10.1016/s0046-8177(81)80027-5.
3
Indolent Wegener's granulomatosis.惰性韦格纳肉芽肿
Ann Rheum Dis. 1983 Aug;42(4):398-407. doi: 10.1136/ard.42.4.398.
肉芽肿性多血管炎(韦格纳氏)与HLA - DPB1*04及SEMA6A基因变异的关联:全基因组分析证据
Arthritis Rheum. 2013 Sep;65(9):2457-68. doi: 10.1002/art.38036.
4
Genetically determined severity of anti-myeloperoxidase glomerulonephritis.遗传决定抗髓过氧化物酶肾小球肾炎的严重程度。
Am J Pathol. 2013 Apr;182(4):1219-26. doi: 10.1016/j.ajpath.2012.12.006. Epub 2013 Feb 4.
5
Familial granulomatosis with polyangiitis: three cases of this rare disorder in one Indoasian family carrying an identical HLA DPB1 allele.家族性肉芽肿性多血管炎:一个携带相同 HLA DPB1 等位基因的印度裔亚洲家庭中出现三例这种罕见疾病。
BMJ Case Rep. 2012 Nov 9;2012:bcr0120125502. doi: 10.1136/bcr.01.2012.5502.
6
Association of HLA genes with clinical outcomes of ANCA-associated vasculitis.HLA 基因与抗中性粒细胞胞质抗体相关性血管炎临床结局的关联。
Clin J Am Soc Nephrol. 2012 Aug;7(8):1293-9. doi: 10.2215/CJN.13071211. Epub 2012 May 17.
7
DRB1*15 allele is a risk factor for PR3-ANCA disease in African Americans.DRB1*15 等位基因是非洲裔美国人中 PR3-ANCA 疾病的风险因素。
J Am Soc Nephrol. 2011 Jun;22(6):1161-7. doi: 10.1681/ASN.2010101058. Epub 2011 May 26.
8
The pulmonary physician in critical care * illustrative case 3: pulmonary vasculitis.重症监护领域的肺科医生 * 示例病例3:肺血管炎
Thorax. 2003 Jun;58(6):543-6. doi: 10.1136/thorax.58.6.543.
9
An unusual case of multiple cranial nerve palsies in Wegener's granulomatosis.韦格纳肉芽肿病中多颅神经麻痹的一例罕见病例。
J Natl Med Assoc. 2000 Sep;92(9):455-7.
4
Pulmonary angiitis and granulomatosis revisited.
Hum Pathol. 1983 Oct;14(10):868-83. doi: 10.1016/s0046-8177(83)80162-2.
5
Wegener's granulomatosis: prospective clinical and therapeutic experience with 85 patients for 21 years.韦格纳肉芽肿病:85例患者21年的前瞻性临床与治疗经验
Ann Intern Med. 1983 Jan;98(1):76-85. doi: 10.7326/0003-4819-98-1-76.
6
HLA antigen frequencies in systemic vasculitis: increase in HLA-DR2 in Wegener's granulomatosis.
Arthritis Rheum. 1983 Jan;26(1):102-5. doi: 10.1002/art.1780260118.
7
Limited forms of angiitis and granulomatosis of Wegener's type.局限性韦格纳型血管炎和肉芽肿病
Am J Med. 1966 Oct;41(4):497-527. doi: 10.1016/0002-9343(66)90214-2.
8
Wegener's granulomatosis: studies in eighteen patients and a review of the literature.韦格纳肉芽肿病:18例患者的研究及文献综述
Medicine (Baltimore). 1973 Nov;52(6):535-61. doi: 10.1097/00005792-197311000-00002.
9
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