McMenemy A
University of Texas Medical School, Houston.
Curr Opin Rheumatol. 1992 Aug;4(4):479-83.
Whipple's disease is a rare multisystem disorder of infectious etiology. Efforts to culture the responsible organism have been unsuccessful. Nucleotide sequencing and amplification of bacterial 16S ribosomal DNA revealed the organism to be most similar to bacteria of the Rhodococcus, Streptomyces, and Arthrobacter genera. Several clinical studies of the long-term use of colchicine for the treatment of familial Mediterranean fever demonstrate its utility for symptom control and prevention of complications by amyloidosis in both adults and children. Normal growth, development, and subsequent fertility were seen in children treated with colchicine. Adult-onset Still's disease has previously been thought to have a generally good outcome, although some patients develop chronic arthritis and disability. No markers have been available for prognosis. A study of 62 patients revealed the presence of polyarthritis, root joint involvement, and rash at initial presentation to be associated with a poorer outcome. Enteropathic arthritis may be seen as a complication of both Crohn's disease and ulcerative colitis. The onset of peripheral arthritis coincides with or follows the onset of bowel symptoms in most cases, whereas spondylitis may precede the onset of inflammatory bowel disease by years. HLA-B27 is present in 50% to 75% of cases of spondylitis. No HLA association with inflammatory bowel disease or peripheral arthritis has been consistently found.
惠普尔病是一种病因不明的罕见多系统疾病。培养致病微生物的努力一直未成功。对细菌16S核糖体DNA进行核苷酸测序和扩增后发现，该微生物与红球菌属、链霉菌属和节杆菌属的细菌最为相似。几项关于长期使用秋水仙碱治疗家族性地中海热的临床研究表明，它在控制症状和预防成人及儿童淀粉样变性并发症方面具有效用。接受秋水仙碱治疗的儿童生长、发育正常，随后生育能力也正常。成人斯蒂尔病以前被认为总体预后良好，尽管一些患者会发展为慢性关节炎和残疾。以前没有可用的预后标志物。一项对62名患者的研究发现，初次就诊时出现多关节炎、累及根部关节和皮疹与预后较差有关。肠病性关节炎可能是克罗恩病和溃疡性结肠炎的并发症。在大多数情况下，外周关节炎的发作与肠道症状的发作同时出现或在其后出现，而脊柱炎可能比炎症性肠病的发作提前数年出现。50%至75%的脊柱炎患者存在HLA - B27。尚未始终发现HLA与炎症性肠病或外周关节炎有关联。
