Clinical characteristics of juvenile-onset ankylosing spondylitis / 中华全科医师杂志

Objective:To explore the clinical characteristics of juvenile-onset ankylosing spondylitis.Methods:Clinical data of 350 cases of ankylosing spondylitis diagnosed in Beijing Jishuitan Hospital from January 2014 to December 2019 were collected. There were 75 cases with the symptom onset in age ≤16 years (juvenile-onset ankylosing spondylitis, JoAS), and 275 cases with the symptom onset in age>16 years (adult-onset ankylosing spondylitis, AoAS). The clinical characteristics of two groups were analyzed.Results:Compared with AoAS, JoAS had a higher proportion of males [98.7% (74/75) vs. 79.6% (219/275); χ 2=15.65, P<0.01] and longer course of disease [11(8,15) vs. 8(4,15) years; Z=-3.09, P<0.01]. Compared with AoAS, JoAS was more prone to have peripheral joint swelling and pain [45.3%(34/75) vs. 18.9%(52/275), χ 2=22.20, P<0.01], hip pain [26.7%(20/75) vs. 15.3%(42/275), χ 2=5.25, P=0.03] or heel pain [9.3%(7/75) vs. 2.9%(8/275), χ 2=5.93, P=0.02] as the first clinical manifestation. Compared with AoAS, JoAS had a higher incidence of radiological hip involvement [77.3%(58/75) vs. 43.3%(119/275), OR=4.71, Wald=25.60, P<0.01], lower bone mineral density than peers [34.7%(26/75) vs. 23.3%(64/275), OR=2.23, Wald=7.20, P<0.01], higher incidence of malnutrition [25.3%(19/75) vs. 13.8%(38/275), OR=2.16, Wald=5.84, P=0.02] and higher incidence of acute uveitis [17.3%(13/75) vs. 6.5%(18/275), OR=2.72, Wald=6.24, P=0.01] after adjusting the course of disease. Conclusion:Compared with adult-onset ankylosing spondylitis, juvenile-onset ankylosing spondylitis is more prone to have peripheral joint swelling or hip pain as the first clinical manifestation; the radiological hip involvement, lower bone mineral density than peers, malnutrition and uveitis are more likely to occur.

Clinical analysis of 20 cases of paraneoplastic syndrome with prominent osteoarticular involvement / 中华风湿病学杂志

Objective To investigate the clinical characteristics of paraneoplastic syndrome with prominent osteoarticular involvement. Methods The clinical materials of 20 patients with paraneoplastic syndrome with prominent osteoarticular involvement were collected. The characteristics of clinical manifest-ations, laboratory tests and imagines were analyzed. Results Among the 20 patients, 16 were male and 4 were female, with a mean age of 44.5 years and a median course of 6 months. Ten cases were associated with hematological tumor and 10 cases were associated with solid tumor. Eleven cases presented as peripheral arthritis (7 cases of polyarthritis, 4 cases of oligoarthritis/monoarthritis), 5 cases presented with hypertrophic osteoarthropathy (HOA) and 4 cases presented with tumor-induced osteomalacia (TIO). Three cases were acute lymphocytic leukemia, 2 cases were multiple myeloma, 1 case was lymphoma, and 1 case was bone tumor in polyarthritis. Four oligoarthritis cases were all associated with acute lymphocytic leukemia. All 5 cases of HOA were associated with lung cancer. All 4 cases of TIO were associated with tumor of mesenchymal tissue. Extra-articular manifestations presented in 14 cases and inflammatory markers increased in 15 cases. anti-cyclic cirullinated peptide (anti-CCP) antibodies was low titer positive in only 1 case and other parameters including rheumatoid factor (RF), anti-CCP antibodies, antinuclear antibodies spectrum (ANAs) and human leukocyte antigen (HLA)-B27 were negative. Multiple bone imaging abnormalities appeared in 15 cases. Conclusion Osteoarticular manifestations may be the first symptom of malignancy and difficult to diagnose. It is necessary to be highly aware of potential malignancy.

The clinical analysis of patients with Turner syndrome and spondyloarthropathy / 中华风湿病学杂志

Objective To investigate the clinical characteristics of Turner syndrome in patients with spondyloarthropathy.MethodsSix patients from our hospital were included and the clinical manifestations,laboratory parameters and imaging presentations were analyzed retrospectively and the relevant literature were reviewed.ResultsThe mean age of these 6 cases was (27±5) years.Two cases were spondyloarthropathy,3 cases were ankylosing spondylitis,1 was psoriatic arthritis.Five cases were complicated with peripheral arthriris,2 cases were complicated with inflammatory back pain,4 cases had vertebral activity limitation,3 cases had joint deformity.The karyotype of 6 cases was 45,XO.Six cases were complicated with osteoporosis.Conclusion Turner syndrome can be presented with spondyloarthropathy,arthritis,osteoporosis.Dysplasia of bone is common.

Twenty-nine cases of primary Sjogren's syndrome complicated with malignancies / 中华风湿病学杂志

Objective To investigate the clinical features and risk factors of primary Sjogren's syndrome (pSS) patients with malignancies.Methods The clinical features of 29 pSS patients with malignancies from January 1985 to March 2008 were retrospectively analyzed.Results The ratio of pSS with malignancies to all hospitalized pSS patients from January 1985 to March 2008 was 2.2%.In these patients,8 were lymphoma,4 were breast cancers,2 were myeloma,2 were thymoma,2 were lung cancer,2 were gastric carcinoma,2 were hepatoma,2 were colon carcinoma and 2 were tongue cancer.Uterine cervix cancer,renal carcinoma,thyroid carcinoma and carcinoma of parotid gland each were fund in this series of patients.Conclusion The most common malignancy in pSS is lymphoma and the major of pathological type is B cell non-Hodgkin's lymphoma.

A clinical analysis of primary Sj(o)gren's syndrome with anticentromere antibodies / 中华内科杂志

Objective To investigate the clinical manifestations, immunological features and prognosis of primary Sj(o)gren's syndrome(pSS) with anticentromere antibodies(ACA). Methods Sixty pSS patients with ACA in our hospital between 1985 and 2006 were screened retrospectively and compared with those without ACA. Results The mean age at the onset of pSS with ACA was higher than that of those without ACA[(48±11)yr vs(41±12)yr,P=0.000].There was no difference in sex ratio,dry mouth.dry eyes and positive salivary gland biopsy between the two groups(P＞0.05).Compared with those without ACA,patients with ACA presented a higher prevalence of liver involvement(68.3%vs 37.0%,P=0.000),while a lower prevalence of renal involvement(13.3%vs 30.9%,P=0.009), neuropathy(1.7% vs 11.5%,P=0.025)and hypergammaglobulinemia(20.8%vs 45.7%,P=0.002). The difference was not significant between the two groups in Raynaud's phenomenon,articular involvement,myositis,hematologic involvement,lung involvement,and thyroiditis. While both groups showed the same prevalence of antinuclear antibody(ANA),the patterns of ANA-IF were different and the discrete speckled pattern was the most freqent in patients with ACA and occurred in 61.7%. Different from those without ACA,patients with ACA presented a lower prevalence of anti-SSA,anti-SSB, rheumatoid factor, and antiU1 RNP,while showed a higher prevalence of antimitochondrial antibodies(AMA) and AMA-M2. The most frequent cause of death was the complications associated with cirrhosis,notably bleeding varices(3/5 cases). Conclusion Patients with ACA present a high risk of liver involvement. Because of the remarkable difference in the mean age of disease onset and also differences in systemic damage, immunological and antibody features, pSS with ACA may be a special subtype of pSS.