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 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.

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

Objective:To investigate the clinical features of patients with ankylosing spondylitis ( AS) and malignancies. Methods: In the study, 31 AS patients with malignancies in Peking University First Hospital from January 2006 to July 2014 were enrolled, and their clinical features were compared with AS patients without malignancies at the same time in the hospital. Then their general characteristics, clinical and imaging findings of AS, characteristics of malignancies and prognosis were retrospectively analyzed. Results:The 31 cases of AS with malignacies accounted for 11 . 8% of all AS patients admitted to the hos-pital in the same period. They comprised of 27 males (87%) and 4 females (13%) . Their mean ages at diagnosis of AS were 43 ± 17 years ( range:16-76 years) , and their mean ages at diagnosis of malig-nancy were 60 ± 12 years ( range:31-87 years) respectively. Malignancy was diagnosed after the estab-lishment of AS in 27 of our patients. Furthermore, imaging changes typical of AS were found in all the 31 patients at the diagnosis of AS, but the majority of them had never received any proper treatment for AS. The malignancies of 31 AS patients included bladder cancer (7 cases) , hematological malignancy (6 ca-ses), lung cancer (5 cases), renal cancer (5 cases), prostate cancer (2 cases), carcinoma of renal pelvis (2 cases) , breast cancer (2 cases) , gastric carcinoma (2 cases) , rectal cancer (2 cases) , neu-roendocrine carcinoma (2 cases) , colon carcinoma (1 case) , and esophagus cancer (1 case) . Conclu-sion:There is risk of malignancy in AS. Malignancy is not rare in patients with AS. Bladder cancer is the most common one followed by hematological malignancy. In consequence, close attention should be paied to the conditon of AS with malignancy in clinical practice.

SUMMARY Here we reported two patients who presented with panuveitis and were transferred from oph-thalmologists to rheumatologists,for both the patients had oral and genital ulcers.They were misdiag-nosed with Behcet’s disease at first glance.Two young males presented with acute uveitis with history of recurrent oral and genital ulcers.They initially presented with symptoms and signs resembling Behcet’s disease and were treated with systemic steroids with suboptimal responses.Routine laboratory test re-vealed syphilis and human immunodeficiency virus (HIV)infection.After treatment of penicillin and anti HIV virus therapy,the panuveitis was relived.The other patient was lost in the follow up.Recently epi-demiological data indicate that syphilis and HIV infection increase,which can mimic the manifestation of Behcet’s disease.Diagnosis of sexual transmitted diseases,such as HIV or syphilis needs to be ruled out in all cases that mimic the clinical feature of Behcet’s disease,especially for those who had a history of high risk behaviors.Every patient should have history analysis in detail.Screening of sexual transmitted diseases,such as HIV or syphilis is important especially in those rapid progressive panuveitis.Also, other virus infections,such as cytomegalovirus,epstein-barr virus or Herpes simplex virus can cause mucosa ulcers and uveitis.CD4 T cell count is a very important marker to indicate that the patient has immunodeficiency.Erythema nodosa and pseudofolliculitis are the third common clinical manifestation in Chinese Behcet’s disease patients.Rheumatologist should watch out for patients without skin involvement when making the diagnosis of Behcet’s disease.Syphilis-associated uveitis usually has a good prognosis. Treatment of antibiotics can get good response,92% uveitis can be relieved,with 67% improved vision. Acute syphilitic posterior placoid chorioretinitis (ASPPC)is a clinically and angiographically distinct manifestation of ocular syphilis.Systemic glucocorticoid can be used in syphilis induced posterior uveitis, sleritis and optic neuritis,and it can also prevent the Hector’s reaction.However,for patients diagnosed with both HIV and syphilis,regular antibiotic can not prevent relapse.So doctors need to follow up them regularly.Patients who present with uveitis,oral and genital ulcers can be easily diagnosed with Behcet’s disease.Rheumatologists need to be aware of the reemergence of sexual transmitted disease.High degree of clinical suspicion can allow ophthalmologists and rheumatologists to diagnose and treat the disease ear-ly.Correct diagnoses timely can get the good treatment response,and rescue the vision.Treatment with regular antivirus and Penicillin can receive the good response,and moreover glucocorticoid can relieve the inflammation.

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

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.

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.