Objective To study the clinical features,treatment and prognosis of secondary amyloidosis.Methods All the inpatients diagnosed as secondary amyloidosis were analyzed retrospectively.Results Eight patients were diagnosed as secondary amyloidosis.Their underlying diseases were connective tissue diseases (4 cases) multiple myeloma (3 cases) and chronic hepatitis B (1 case).Average duration from the diagnosis of connective tissue diseases to secondary amyloidosis was 6 2 years.Kidney was the main involved organ with the manifestation of proteinuria (4 cases) and renal failure occurred in 1 case.Their average life span was longer than 10 years after immunosuppressive agents and/or steroid therapy.Secondary amyloidosis occurred after multiple myeloma showed a bad prognosis.Tongue and myocardium were mainly involved.Enlargement of liver and spleen is the prominent manifestation in the patients with amyloidosis secondary to chronic hepatitis.Conclusion Amyloidosis can be evolved from connective tissue diseases,multiple myeloma or chronic inflammatory diseases.There are differences in clinical manifestations and prognosis.The only treatment of secondary amyloidosis is the early management to its underlying disease.

The treat-to-target (T2T) strategies has greatly improved the prognosis of rheumatoid arthritis (RA).However,the emergence of musculoskeletal ultrasound has further brought new challenges and opportunities for RA T2T treatment.It has been revealed the subclinical synovitis generally presented in RA patients who achieved clinical remission.The subclinical synovitis has been confirmed to be associated with subsequent progression of bone erosion and flare.Therefore,clinical remission or low disease activity may be inappropriate or insufficient target.The ultrasound evaluation in addition to clinical assessment can help to control the disease activity better,which may eventually improve the long-term outcomes of RA patients.

Objective To understand the side-effects of cyclophosphamide (CTX) in the treatment of systemic autoimmune diseases and the possible risk factors. Methods Two hundred and forty-one patients with systemic autoimmune diseases were recruited from the Rheumatology Division of Peking University First Hospital during January 1st, 2009 and March 31, 2012. All the patients received oral or intravenous cyclopho-sphamide. The data were collected by medical record review as well as telephone follow-up. Logistic regression analyses were used for statistical analysis. Results Statistical analysis for age, sex, disease, cumulative dose, treatment duration and mode of administration were included in the factor analysis that would impact the CTX related side-effects. Age ( x2=14.8, P=0.002), gender ( x2=11.2, P=0.001), the underlying disease ( x2=26.1, P<0.01), cumulative dose ( x2=9.8, P=0.007) and mode of administration of CTX ( x2=19.5, P<0.01) were all correlated with the incidence of CTX side-effects. Multivariate analysis showed that women [OR=2.32, 95%CI (1.15, 4.70), P=0.02], intravenous-oral sequential use of CTX [OR=5.25, 95%CI (2.30, 11.97), P<0.01] and systemic lupus erythematosus [OR=4.02, 95%CI (2.24, 7.21), P<0.01] as the underlying disease were independent risk factors for CTX side-effects. Conclusion Alopecia, gastrointestinal discomfort and gonads toxicity ware com-monly seen in Chinese patients with systemic autoimmune diseases receiving CTX. Hemor-rhagic cystitis is very rare. Women, intravenous-oral sequential use of CTX and the systemic lupus erythematosus are indepen-dent risk factors for CTX side-effects.

Objective To study the clinical manifestation,treatment and prognosis of patients with primary Sjgren′s syndrome and renal tubular acidosis.Methods Sixty patients who were diagnosed as pSS complicated with RTA during the last 13 years were retrospectively analyzed and followed up for 7 5 years on average.Results Distal renal tubular acidosis ( n =53,83 3%) was much more frequeut than the proximal one ( n =7,11 7%).Hypokalemic paralysis,diabetes insipitus,and calcification of the renal tissue appeared in 49 patients (92 4%).Glomerular lesions were found in 12 of the 15 patients who underwent kidney biopsy.Renal insufficiency was developed subsequently in 4 out of 5 patients with glomerular sclerosis.Abnormal serum creatinine was detected in 11 patients (18 3%),and in 7 of them it persisted 11 5 years on average after the onset of RTA.Two of them died from renal failure and 1 received hemodialysis.Hypergammaglobulinemia was more obvious in the patients with renal insufficiency than those without ( P

Prognosis and treatment of primary Sj?gren′s syndrome with renal tubular acidosis

Objective To promote preventive and therapeutic measures for osteoporosis by investigating the prevalence of low bone mineral density (BMD) in connective tissue disease patients who were taking corticosteroid and identify the associated risk factors of osteoporosis.Methods Thirty-eight female patients who had connective tissue diseases and were taking corticosteroid in PU MCH rheumatology outpatient clinic were studied and followed up from Oct 2006 to April 2007.Lumber spine and right femoral bone mineral density were determined bv dual energy X ray absortiometry (DEXA).Clinical information was obtained from a questionnaire of history and medical records.Duration and accumulative dosage of glucocorticoid intaking.menopause time were obtained retrospectively.Correlation analysis between BMD and clinical information was conducted.Results ① In 38 patients,23 (61%) patients showed a normal BMD,10 (26%) were osteopenia,3 (8%) were iu the osteoporotic range,while 2 patienLs (53%) had fragile fracture.② Compared with patients with normal BMD,subjects with low BMD had significantly older age,longer period after menopause and higher accumulated dose of corticosteroids.③ Postmenopausal women had significantly lower BMD in lumber and hip than premenopausal women.④ Either of vertebral and right hip BMD correlated negatively with the accumulated dosage of cortieosteroids by simple linear regression.⑤ The correlation between BMD and accumulated dosage of corticosteroids improved after correcting for the effect of age by partial correlation analysis (Pearson partial vertebral r=-0.8,P=0.009;right hip r=-0.3,P=0.010).⑥ A stepwise multivariate linear regression model was constructed to explore the relationship between the different clinicsl factors studied and a low BMD.Two statistically significant variables were menopause status (P=0.0000) and a higher steroid accumulated dosage (P=0.008).Conclusion Low BMD is common in connective tissue disease patients receiving corticosteroid.Risk factors for low BMD are postmenopause.duration and the accumulated dosage of glucocorticosteroid.The high prevalence of low BMD implies that more attention should be paid to the prevention and treatment of osteoporosis and fractures in connective tissue disease patients who are taking corticosteroids.

Objective:Anti-cyclic citrullinated peptide (CCP) antibody is an important biomarker as-sociated with the diagnosis and prognosis of rheumatoid arthritis (RA). Different studies showed inconsistency in the relationship between anti-CCP antibody titers and RA disease activity. Therefore, we investigated the association between anti-CCP antibody with the possibility of achieving treatment target and flare.Methods:The enrolled RA patients must be anti-CCP antibody positive at baseline, and had at least one test result of anti-CCP antibody during follow-up at least one year after the baseline. The patients were divided into declined group and non-declined group according to the decrease of anti-CCP antibody titer over 10% or not during follow-up from the baseline. Single factor comparison, Pearson correlation, Spearman correlation and Kendall correlation analysis were used.Results:A total of 124 patients were included in this study. Sixty-five and 59 patients were in anti-CCP antibody declined and non-declined groups, respectively. At the end of the follow-up, the proportion of patients who achieved clinical remission or low disease activity were 78%(51/65) and 68% (40/59)in the declined and the non-declined groups, respectively ( P=0.181). The changes of Disease Activity Score with 28 joint (DAS28)-C-reaction protein (CRP), DAS28-erythrocyte sedimentation rate (ESR), tender joint count (TJC) and CRP in the declined group were significantly greater than those of the non-declined group ( P values <0.05). There was no positive correlation between anti-CCP antibody titer and several disease activity indicators at baseline ( r values <0.3, P values >0.05). The changes of anti-CCP antibody titers during the follow-up were also not correlated with changes in disease activity (but r values <0.3, P values <0.05). Meanwhile, both the baseline anti-CCP antibody titers and the changes of the anti-CCP antibody titers during follow-up were neither correlated with whether the patient achieved clinical remission or low disease activity at the end of the follow-up nor whether relapse happened. Conclusion:There is no significant correlation between anti-CCP antibody levels at baseline and disease activity, achievement of treatment target, or recurrence after treatment. The value of anti-CCP antibody in assessing disease activity, predicting treatment response, and predicting relapse needs to be confirmed in further large-scale prospective studies.

Objective:To analyze the characteristics of various clinical parameter reflecting the di-sease activity of patients with rheumatoid arthritis (RA) and explore their objectivity and different clinical values.Methods:The clinical data and ultrasonic data of 28 joints of RA patients diagnosed between 2014 and 2018 were collected. The characteristics of clinical indicators were analyzed, and their correlation with total grey scale (GS)/power Doppler (PD) scores of 28 joints was explored. Semi-quantitative score (0-3 points) of GS and PD for synovial hyperplasia was performed on 28 joints of selected patients by ultrasound. Total GS/PD scores include 28 joints. The characteristics of clinical parameters were analyzed, and their correlation with total GS/PD scores of 28 joints was explored. The normal distribution data was represented by mean SD, while the non-normal distribution data was represented by median (interquad interval, IQR). Correlation analysis was performed using 95% Spearman nonparametric correlation coefficient. All statistical tests were bilateral, with a significance of P<0.01. Results:163 RA patients were enrolled. 85% of them were female, with an average age of (52.0±13.0) years and a median course of disease 34(24, 45) months. The disease activity score in 28 joints C-reactive protein (DAS28-CRP), simplified disease activity index (SDAI) and clinical disease activity index (CDAI) were 4.2(2.4, 5.4), 17.9 (5.7, 33.3) and 16.0 (5.0, 28.5), respectively. There was discordance between tender joint count (TJC) and swollen joint count (SJC) in some patients. Eighty-nine (54.6%) patients had higher TJC than SJC, while 19(11.7%) patients had fewer TJC than SJC. The accordance between physician's global assessment (PGA) and evaluator Sglobalassessmentofdiseaseactivity (EGA) was observed in only 61 cases (37.4%). Eighty-nine patients (54.6%) had a higher PGA than EGA. Overall, all the parameters [TJC, SJC, PGA, PGA, erythrocyte sedimentation rate (ESR) and CRP] were positively correlated with the total GS/PD scores ( r>0.50, P<0.01). Composite disease activity scores, DAS28, SDAI and CDAI, were also significantly correlated with total GS/PD scores ( r>0.59, P<0.01). But compared with TJC, the correlation between SJC and GS/PD was better ( r=0.59/0.60, P<0.01; r=0.50/0.51, P<0.01). Similarly, compared with GPA, the correlation between EGA and GS/PD was better ( r=0.66/0.67, P<0.01; r=0.55/0.58, P<0.01). Conclusion:The composite disease activity scores and all their components are significantly correlated with ultrasonic synovitis. Compared with TJC and PGA, SJC, EGA, CRP and ESR show a higher correlation with joint ultrasonic synovitis, and are more objective and meaningful in the evaluation of RA disease activity.

Objective To investigate the ultrasound features of gouty joints during acute and past attacks. Methods Clinical data and ultrasound features of joints during the acute and past attacks of gouty patients who were experiencing an acute attack were collected. The differences of ultrasound features between two episodes, as well as the relationship with clinical characteristics were analyzed. Results Sixty-four patients were enrolled with 21 (33%) patients at their first attack. The first metatarsophalangeal (MTP1) joints were most frequently involved, meanwhile, 9.4% patients had two or more joints attacked during one episode. The most prevalent feature was synovitis at the acute phase, followed by double contour (DC) sign (18 cases, 28%), bone erosion (12 cases, 19%) and tophi (10 cases, 16%), while 23 patients had two or more pathological changes. Whereas, the DC sign was found most in previously attacked joints (10 cases, 33%), followed by tophi (8 cases, 27%), bone erosion (7 cases, 23%) and synovitis (4 cases, 13.3%). No positive pathological changes were found in asymptomatic joints. Synovitis was more common in joints during their acute attacks (80% vs 13%, χ2=20.475, P<0.01), however, DC sign and tophi were more common in previous attacked joints (10% vs 33% and 0 vs 27%, χ2=3.892, 6.642, P<0.05, respectively). Both the DC sign and tophi were positively correlated with the disease duration. Conclusion Synovitis is the most prevalent feature in gouty joints during acute episodes; even bone erosion can be found at the first attack. The DC sign, tophi and bone erosion are common in past attacked joints. The prevalence of both DC sign and tophi are increased along with disease durations. Subclinical synovitis can be detected in a small past of joints which are not at acute attack.

Objective To evaluate the clinical and image characteristics of rheumatoid arthritis (RA) patients accompanied with bronchiectasis (BR).Methods By retrospectively analyzing 67 patients admitted to our department,we divided RA patients into RA-BR group and non-BR group,according to the presence of BR or not.For those with interstitial lung disease (ILD),the ILD lesions were classified by inflammatory,fibrosis and mixed pattern.The clinical and image characteristics,as well as the correlation between BR patients with clinical/other lung changes were analyzed.Independent sample t test was used to compare the difference between the two groups if the data was normally distributed and those data that were not distributed normally were analyzed by Mann-Whitney U-test.Spearman correlation analysis was used for variables related analysis.Logistic regression analysis was used to identify independent risk factors for the presence of respiratory symptoms.Results Twenty-nine patients (43％) showed BR in CT scan.There was no statistically significant difference between BR and uon-BR group in age,gender,disease course of RA,smoking history and rheumatoid serology;respiratory symptom was presented in 6 pts in both groups;bronchiectasis involved unilaterallung in 10 patients (34％) and bilaterally in 19 (66％),26 patients (90％) were found to have bronchiectasis in their lower lobe.ILD was presented in 12 (41.4％) and 13 (34％) cases in BR and non-BR group,respectively (P＞0.05).BR in RA was not correlated with clinical parameters and presence of ILD.Logistic regression showed BR [OR:1.201,95％ CI.(1.023-1.411),P＜0.05] and ILD [OR:10.646,95％CI:(1.944-58.305),P＜0.01] were independent risk factors associated with respiratory symptoms in RA pts.Conclusion BR is a common lung lesion in pts with RA in China,and the dose was not correlated with ILD,the number of segment involved by BR is a risk factor for respiratory symptoms.