幼年型僵直性脊椎炎－－72病例之臨床觀察

Clinical Observation of Juvenile Ankylosing Spondylitis-- Analysis of 72 cases

三軍總醫院自民國77年1月至83年1月收集門診及住院病人診斷為幼年型僵直性脊椎炎者共72例，其中男性69人，女性3人，平均發病年齡為12.9±2.0歲，初診斷年齡為18.1±2.6歲，發病高峰年齡為15歲，25％有家族史。於診斷時，所有病人皆符合New york criteria的標準。於疾病診斷時，腰椎／薦椎症狀佔100％，頸椎／胸椎症狀佔31.9％，周圍關節病變佔88.9％：依發作形式區分：59.7％為少關節型，40.3％為多關節型；依侵犯部位區分：下肢侵犯者佔87.5％：股關節66.7％，膝關節61.1％，踝關節43.1％，跗關節8.3％，蹠、趾關節5.6％。上肢侵犯佔34.7％：肩關節29.2％，肘關節11.1％，腕關節9.7 ％，掌關節6.9％，指關節4.2％ ；腱鞘病變36.1％。比較發病之初第一年內症狀，腰椎／薦椎症狀佔58.8％，頸椎／胸椎症狀佔8.3％，周圍關節病變佔68.1％。其中90.3％為少關節型，9.7％為多關節型；下肢侵犯者佔65.3％：股關節34.7％，膝關節33.3％，踝關節23.6％，跗關節2.8％，蹠、趾關節1.4 ％。上肢侵犯佔15.3％：肩關節11.1％，肘關節4.2％，腕關節4.2％，掌關節0％，指關節2.8％；腱鞘病變18.1％。薦腸骨關節X光檢查中，第二級佔53％，第三級佔39％，第四級佔8％，放射性骨掃描為陽性者佔92.2％。關節外症狀包括心臟病變16.7％，眼病變25％，及泌尿道病變5.6％。HLA-B27陽性率為88.1％，ANA陽性率為8.1％，RF全為陰性，IgA增高者佔20／42(47.6％)，IgM佔1/42(2.4％)，IgG佔13／41(31.7％)，平均值分別為404.7，165，1576.6 mg/ml。尿液檢查有4例有血尿，2例同意切片，一例為IgM腎病變，一例為間質增生性腎絲球腎炎。

We studied retrospectively the clinical features of 72 Patients (69 males, 3 females) with definite ankylosing spondylitis (A.S) which all fulfilled the New York diagnostic criteria for A.S and had onset before the age of 16 years. HLA-B27 was present in 52 of 59 cases( 88.1%). The mean age at onset was 12.9 ± 2.0 years, and the peak age at onset was at 15 years of age. 18 (25%) patients had family history of A.S and all of them had morning stiffness. 43 (59.7%) cases were pauciarticular type when diagnosed, and 65 (90.3%) cases at onset. By the end of the first year of disease, main features included axial involvement: lumbar/sacroiliac in 42 (58.8%); cervical/thoracic in 6 (8.3%); peripheral arthropathy in 49 (68.1%); and enthesopathy in 13 (18.1%). The frequency of these increased up to 72 (100%); 23 (31.9%); 64 (88.9%); and 26 (36.1%), through the course of the disease. Peripheral arthropathy included (1). Lower limb joint involvement in 47 (65.3%): hip in 25 (34.7%); Knee in 24(33.3%); ankle in 17 (23.6%); tarsal in 2 (2.8%); and MTP/IP in 1(1.4%). (2). Upper limb joint involvement in 11 (15.3%): shoulder in 8 (11.1%); elbow in 3 (4.2%); wrist in 3 (4.2%); MCP in 0; DIP/PIP in 2 (2.8%). the frequency of these increased up to 63 (87.5%): 48(66.7%); 44 (61.1%); 31 (43.1%); 6 (8.3%); 4 (5.6%), and 25 (34.7%): 21 (29.2%); 8 (11.1%); 7 (9.7%); 5 (6.9%); and 3 (4.2%). X-ray examinations of pelvis were performed on all patients and grade II sacroilitis was found in 38 (53%), grade III in 28 (39%), and grage IV in 6 (8%). Tc99m bone scans were positive in 47 (92.2%) of 51 patients tested. IgA was elevated in 20 (47.6%) of 42 patients, IgM 1 (2.4%) in 41, and IgG 13 (31.7%) in 41. Examination of extra-articular manifestotions showed 12 (16.7%) had heart involved; 18(25%) had conjunctivitis/uveitis; and 4 (5.6%) had renal involved.