[Diagnosis and Treatment of 43 Patients with IgG4-related Disease].

OBJECTIVE: To summarize the clinicopathological characteristics, diagnosis and treatment of IgG4-related disease (IgG4-RD). METHODS: The clinical data of 43 cases with IgG4-RD diagnosed from January 2013 to December 2017 were retrospectively analyzed. The clinical data of the patients including clinical characteristics, accessory examinations, diagnosis, and treatment were collected. RESULTS: Among the 43 patients with IgG4-RD, the ratio of male to female was 3∶1, the mean age was (51.3±15.9) years. Eleven patients had gastrointestinal symptoms, including 5 cases of IgG4-related cholangitis with the feature of dilation of the biliary system and narrowing of the lumen in the abdominal enhanced CT scans, and 6 cases of IgG4-related autoimmune pancreatitis with the feature of pancreatic enlargement or soft tissue density shadow in the abdominal enhanced CT scans. There were 10 cases (23.3%) with periorbital involvement, with the feature of intraorbital soft tissue nodule in the CT scan. Besides, 9 cases (20.9%) had lymphadenopathy, 6 cases (14.0%) had fever. The results of autoimmune antibody tests showed that 14 of 42 patients had increased antinuclear antibody (ANA). Among 40 patients who underwent immunoglobulin tests, 25 cases had elevated IgG, 12 cases had increased IgA, and 29 cases had increased IgE. Coombs test were performed for 6 cases and 4 patients were positive. Serum immunoglobulin G4 subtypes showed that the IgG4 levels of 35 patients were higher than 1 350 mg/L. The immunohistochemistry showed that IgG4 (+) cells (3->500/HPF) were infiltrated, with the CD20 (+), CD3ε (+) or CD138 (+). Among the 43 patients, 5 patients underwent operations due to misdiagnosis. All patients were treated with steroid and immunosuppressive agents after diagnosis, and their clinical symptoms were improved. CONCLUSION: The clinical symptoms of IgG4-RD are various, involving multiple organs. Therefore, the standardized diagnosis and treatment of IgG4-RD should be strengthened.

[Comorbidity of Autoimmune Diseases: a Field that Needs Multidisciplinary Attention].

Comorbidity of autoimmune diseases is a very important issue but easily ignored in the clinical practice. The treatment of comorbidity of autoimmune diseases needs cooperation of multiple disciplines,which is totally different from traditional clinical disciplines division and treatment mode. Based on the clinical features of the disease,we will comprehensively look through genetic,environmental,and immune factors involving in molecular and immunological compatibility pathogenesis,and also generalize common pathological features,such as immune complex deposition and accumulation of lymphocytes. We will also investigate the association and differences between the diseases with comorbidity,and explore the outcome and prognosis of comorbidity of autoimmune diseases. With clarify of the concept of autoimmune comorbidities,we hope bring more and more attention on this aspect,so as to improve the diagnosis,treatments as well as the prognosis of these diseases.

[Clinicopathological Analysis of Autoimmune Hepatitis with Sjögren's Syndrome].

OBJECTIVE: To explore the biochemical-immune and pathological characteristics of autoimmune hepatitis (AIH) with Sjögren's syndrome (SS) . METHODS: A total of 76 cases of AIH patients were included from January 2009 to April 2017. Among them,there were 40 cases of AIH with SS and 36 cases without SS. The liver function,immunological index,histological features,length of first diagnosis and treatment costs were compared between the two groups. RESULTS: For AIH+SS group and AIH group,the proportion of women were 97.5% and 77.8%,the proportion of the first diagnosis age less than 60 years were 70% and 47.2%,the median course of disease were 30 months and 9 months,all the difference were statistically significant (P<0.05). The chief complaints in AIH+SS group and AIH group were as follows: cutaneous or scleracterus (52.5% vs. 38.9%),abnormal transaminase (17.5% vs. 44.4%),dryness of mouth and eye (15.0% vs. 2.8%),all the difference were statistically significant (P<0.05). There were no statistically significant difference in hospitalization expenses,and length of stay between the two groups (P>0.05). The median level of total bilirubin (TBIL),direct bilirubin (DBIL) and immunoglobulin (Ig) M of AIH +SS group were higher than those of AIH group,the mean level of albumin (ALB) and complement 3 (C3) of AIH +SS group were lower than those of AIH group,and the positive rate of anti-mitochondrial antibody-M2 (AMA-M2) ,anti-Ro antibody A (SSA),anti-La antibody (SSB) and anti-soluble liver antigen antibody (SLA) of AIH+SS group were higher than those of AIH group (P<0.05). There were no statistically significant difference in histological changes of hepatocytes and bile duct injury rate (P>0.05). CONCLUSION: AIH patients in young and middle-aged women need to be vigilant with SS with main manifestation of skin sclera and high specific autoantibodies positive.

[Correlation of Thyroid Autoantibodies,System Lupus Erythematosus Immunologic Indicators and Disease Activity in SLE with HT].

OBJECTIVE: To investigate the correlation of disease activity and thyroid indicators ,immunologic markers of system lupus erythematosus (SLE) in SLE with Hashimoto's thyroiditis (HT). METHODS: The clinical data of 63 cases of SLE with HT were collected. According to Systemic lupus erythematosus disease activity index 2000 (SLEDAI-2000),we classified the patients into four groups,which were remission group (5 cases),low (19 cases),moderate (12 cases) and high (27 cases) disease activity group. Each patient received the measurement of thyroid function indicators and autoantibodies,SLE immunologic indicators,serum complement (C3,C4),C-reactive protein (CRP),erythrocyte sedimentation rate (ESR), and routine blood test. The correlation of thyroid indicators,immunologic markers and disease activity were analyzed. RESULTS: The difference of free triiodothyronine (FT3) level in the four groups was statistically significant (P<0.05),and FT3 was negatively correlated with SLE disease activity (P=0.007) . There was no significant difference in other thyroid indicators and autoantibodies between the different groups (P>0.05). Negative correlation was found between FT3 level and anti-double-stranded DNA (dsDNA),level of anti-La antibody (SSB) and thyroid stimulating hormone (TSH) or thyroid peroxidase antibody (TPOAb). Thyroglobulin autoantibody (TgAb) was negatively related with C4,and positive correlation between FT3 and C3,FT4 and C4,TgAb and IgA. CONCLUSION: The pathogenesis of HT is associated with the disease activity in the patients of SLE with HT.

[Risk Factors of Respiratory Failure in the Dermatomyositis Patients with Interstitial Lung Disease].

OBJECTIVE: To reveal the clinical features of respiratory failure (RF) in dermatomyositis (DM) patients with interstitial lung disease (ILD),and to explore risk factors of RF in these patients. METHODS: The medical data of 122 DM patients with ILD were retrospectively reviewed: 40 developed RF (RF group),82 did not develope RF (Non RF group). Clinical,laboratory and radiological variables were compared between RF patients and Non RF patients. Multivariate Logistic regression was used to analyze risk factors of RF. RESULTS: In RF patients,the female-male ratio was 3∶1,the median age at DM onset was 49.5 (42.3-58.6) years-old. There were 67.5%,85.0% and 95.0% patients developed RF within 6 months,1 year and 2 years after the onset of DM. The factors significantly associated with RF included DM onset age,clinically amyopathic dermatomyositis (CADM),pneumomediastinum (PNM),aspartate aminotransferase(AST),lactate dehydrogenase (LDH),albumin,neutrophil-lymphocyte ratio,platelet-lymphocyte ratio,anti-Jo-1 antibody presence and ground-glass opacities sign (P<0.05). PMN and anti-Jo-1 antibody failed to be involved in logistic regression model. The regression analysis demonstrated that older DM onset age [odds ratio (OR)=1.791,P=0.025],higher AST level (OR=1.937,P=0.048),CADM diagnosis (OR=3.881,P=0.007) and ground-glass opacities sign (OR=4.187,P=0.014) were independent risk factors of RF in DM patients with ILD. CONCLUSION: RF occurs more often within 2 years of DM onset. The DM patients with older DM onset age,elevated AST level,CADM diagnosis or ground-glass opacities sign took higher risks for RF development.

[The Clinical Significance of Tumor-associated Antigens in Dermatomyositis Patients with Interstitial Lung Disease].

OBJECTIVE: To investigate the relationship between tumor related markers and the presence of interstitial lung disease (ILD) in dermatomyositis (DM) patients as well as potential serum markers for accompanied ILD. METHODS: Fifty-nine DM patients were included,including 30 patents with ILD. Serum level of anti-transcription intermediary factor1-γ (TIF1-γ) and tumor-associated antigens (TAAs) were detected to analyze the correlation of these markers with ILD. Meanwhile,the diagnostic value of these markers was evaluated by receive operating characteristic (ROC) curve analysis. RESULTS: We found that there were 5 patients bearing malignancies,independent from the presence of ILD (P=0.024). Serum anti-TIF1-γ was positive in 4/5 DM patients with tumor,of which sensitivity and specificity were 36.36% and 97.92%,respectively. In contrast,only one case was found positive anti-TIF1-γ in 30 DM with ILD (P=0.002).Carbohydrate antigen (CA)125,CA153,and cytokeratin-19 fragment (CYFRA21-1) were elevated in DM patients with ILD in compared to those without ILD. The area under curve (AUC) of CYFRA21-1 for the prediction of ILD was 0.745 (P=0.002). The optimal cut-off value was 3.58 ng/mL with a sensitivity of 60.71% and a specificity of 84.00%. The AUC in combination of CA125 and CYFRA21-1 could reach 0.801 (P<0.002) with a sensitivity of 64.29% and a specificity of 82.61%. CONCLUSION: Presence of malignance and positive anti-TIF1γ could be the protective factors for ILD in DM. Elevated serum levels of CA125,CA153,CYFRA21-1 could indicate the accompanying ILD.

[A Retrospective Analysis of Clinical Features of Interstitial Pneumonia with Autoimmune Features].

OBJECTIVE: To analyze the clinical features of interstitial pneumonia with autoimmune features (IPAF) and the correlation between them. METHODS: We respectively analyzed the patients with interstitial lung disease (ILD) admitted in our hospital from January 2014 to January 2017. The patients who met all priori requirements and at least one feature of one domain were recuited, and the clinical features and autoimmune diseases related prognosis were analyzed. RESULTS: There were 90 patients recruited,including 38 patients completely met IPAF classification criteria and 52 patients who incompletely met. The average age was (62.34±14.98) yr.. The pneumonia pattern of complete IPAF patients was non-specific interstitial pneumonia (NSIP), while it was usually interstitial pneumonia (UIP) in the incomplete IPAF patients. During follow-up,11 patients were diagnosed with autoimmune diseases (4 with complete IPAF,and 7 with incomplete IPAF) . According to Cox regression analysis,completely meet the criteria of IPAF was related to the relapse of disease. CONCLUSION: There exist relation between IPAF and autoimmune diseases. The patients with IPAFmay finally develop into autoimmune diseases. The IPAF classification criteria provide basic structure for this disease,but the limitation of the criteria call for revising by more clinical trials.

Lipid peroxidation-mediated inflammation promotes cell apoptosis through activation of NF-κB pathway in rheumatoid arthritis synovial cells.

Rheumatoid arthritis (RA) is a systemic autoimmune disease characterized by chronic inflammation of multiple joints. The central pathogenesis of RA is the proliferation of synovial fibroblasts in response to inflammatory cytokines. However, some of the targeted therapies for inflammation reactions do not display significant clinical improvement after initiation of therapy. Thus, the relationship between inflammatory responses and RA therapy is still incompletely understood. In the present study, we proposed to determine whether enhanced inflammations may lead to cell apoptosis in rheumatoid arthritis synoviocytes. Our results indicated that products of lipid peroxidations, 4-HNE, may induce synovial intrinsic inflammations by activating NF-κB pathways and it may lead to cell apoptosis. Pharmacological inhibition of NF-κB activation may reduce the 4-HNE mediated inflammation responses and subsequent cell apoptosis. Our results may help to clarify the role of inflammations on RA development and imply that blocking NF-κB activation may be partly beneficial for human RA therapy. These findings might provide a mechanism-based rationale for developing new strategy to RA clinical therapy.

[Correlation of RANKL/OPG, dickkopf-1 and bone marrow edema in rheumatoid arthritis with the complaint of knee pain].

OBJECTIVE: To investigate the correlation of the receptor activator of nuclear factor kappa-B ligand (RANKL)/serum osteoprotegerin (OPG) system, Dickkopf-1 (DKK-1) and bone marrow edema (BME) in rheumatoid arthritis (RA) with the complaint of knee pain. METHODS: The clinical data of 50 cases of RA with the complaint of knee pain were collected. According to MRI finding, half of them (25 cases) had bone marrow edema (BME). Each patient received the measurement of serum OPG, RANKL, DKK-1, C-reactive protein (CRP), erythrocyte sedimentation rate (ESR), anti cyclic citrullinated peptide antibody (CCP), rheumatoid factort(RF). The clinical symptoms, disease activity score 28 (DAS28), were compared between BME and non-BME griups, and the correlation between RANKL/OPG system, DKK-1 and BME of RA was analyzed. RESULTS: Compared with non BME group, BME group had shorter course (P=0.000), higher DAS28 score (P=0.009), CRP (P=0:000), RF (P=0.033) and CCP (P=0.012). lower level of serum OPG (P=0.000), higher level of RANKL (P=0.000), RANKL/OPG (P=0.000), and DKK-1 (P=0.001). The severity of bone marrow edema was correlated with the serum RANKL (volume r(s)=0.31, P=0.027; degree r(s)=0.33, P=0.022), RANKL/OPG (volume r(s)=0.29, P=0.039; degree r(s)=0.28, P=0.043), DKK-1 (volume r(s)=0.33, P=0.021; degree r(s)=0.34, P=0.019). CONCLUSION: BME is one of the early signs of bone erosion in RA, there were more active inflammation, autoantibodies, and osteocasts in RA patients with BME.

[Comparison of four assessment criteria for disease activity of rheumatoid arthritis].

OBJECTIVE: To compare of the efficacy of four assessment criteria for evaluating disease activity in rheumatoid arthritis. METHODS: Clinical data were collected from 172 patients ofrheumatoid arthritis. Disease activity was evaluated by four assessment criteria, which are disease activity score 28-C-reactive protein (DAS28-CRP), disease activity score 28-erythrocyte sedimentation rate (DAS28-ESR), simplified disease activity index (SDAI) and clinical disease activity index (CDAI). The patients were divided into four groups which were remission, low, middle and high disease activity. The correlations and consistencies among four methods were compared. RESULTS: Disease activities evaluated by four methods in 172 RA patients were significantly positive correlated (P<0.01). It was significantly consistent between SDAI and CDAI, also between DAS28-ESR and DAS28-CRP, the Kappa values were 0.949 and 0.862 respectively. The differences of the four methods in remission and high disease activity groups were no statistical significance. The proportion of the patients in low disease activity and middle disease activity groups evaluated by DAS28-ESR and DAS28-CRP were statistical different from that evaluated by CDAI and SDAI (P<0.05). CONCLUSION: Strong correlation was observed between the four methods. CDAI could be used to evaluate whether RA is remission or activity. For disease activity assessment, DSA28-ESR and DAS28-CRP seems superior to CDAI and SDAI.

[Clinical value of MDHAQ for disease activity assessment in patients with rheumatoid arthritis].

OBJECTIVE: To evaluate the clinical value of the mutidimensional health assessment questionnaire (MDHAQ) for disease activity assessment in patients with rheumatoid arthritis (RA). METHODS: A total of 100 RA patients were recruited in this study and completed the MDHAQ. Clinical data and involved joint function assessment were also collected. Routine assessment of patient index data 3 (RAPID3) was calculated using the MDHAQ. The correlations between RAPID3 and disease activity score 28 (DAS28), simplified disease activity index (CDAI), simplified disease activity index (SDAI) and clinical characteristics were analyzed. RESULTS: RAPID3 was correlated with erythrocyte sedimentation rate (ESR), C-reactive protein (CRP), swollen joints count, tender joints count and overall physician rating (P<0.05). The MDHAQ had a Cronbach's alpha coefficient of 0.821 and a KMO of 0.576. Bartlett's test of sphericity showed P<0.001. RAPID3 was also correlated with DAS28, CDAI and SDAI, with a coefficient of 0.743, 0.697 and 0.640 (P<0.001), respectively. CONCLUSION: MDHAQ is a reliable instrument for disease activity assessment in patients with RA. It is easy to administer in busy clinical settings.

[Clinical characteristics of rheumatoid arthritis with cold pattern knee pain].

OBJECTIVE: To investigate the clinical characteristic of rheumatoid arthritis associated with cold pattern knee joint pain. METHODS: The study enrolled 60 patients of RA with the complaint of knee pain, 30 cases with cold pattern and 30 cases without cold pattern. The clinical symptoms, DAS28 score, cold knee score were collected, and serum hypoxia inducible factor-1alpha (HIF-1alpha) erythrocyte sedimentation rate (ESR), C-reactive protein (CRP) and thermal texture maps (TTM) of knee temperature were measured. The difference between two groups was compared to analyze the clinical characteristics of RA with cold pattern knee pain. RESULTS: There were differences between the two groups in the course (t=5.932, P=0.000), DAS28 score (t=2.716, P=0.007), knee (delta TTM) (t=7.731, P=0.000), absolute difference of thermal texture maps between popliteal fossia and front district in knee (ATTM) (t=14.295, P=0.000), CRP (t=5.684, P=0.000), ESR (t=4.506, P=0.000), HIF-1alpha (t=4.817, P=0.000). CONCLUSION: RA patients with cold pattern knee pain show the clinical characteristics with longer course of disease, lower level of local inflammation, lower disease activity.

[Disease activity and therapeutic strategy of patients with late-onset rheumatoid arthritis].

OBJECTIVE: To compare the disease activity and therapeutic strategy of late-onset rheumatoid arthritis (LORA) with young-onset rheumatoid arthritis (YORA). METHODS: Medical records of 259 patients with rheumatoid arthritis (RA) were reviewed retrospectively. The cut-off between LORA and YORA was operationally set at 60 years of age at disease onset. Gender ratio, disease duration, feature of involved joint, extra-articular features, laboratory features, disease activity score and treatment strategy were compared between LORA and YORA. RESULTS: The LORA patients had a gender ratio (male/female) of 1/1.88, which was approaching 1/1 in the older age group. It took longer for LORA to be diagnosed than YORA (P<0.001). LORA had more frequent involvement of shoulders (P < 0.001), while elbow, wrist, metacarpophalangeal joint ( MCP), proximal interphalangeal joint (PIP) and ankle joints were more common in YORA (P<0.001). LORA patients were more likely to have anemia than YORA patients (P<0.05). No significant differences were found between LORA and YORA in specific serologic index, including rheumatoid factor and anticyclic citrullinated peptide antibody, and disease activity score 28-C-reactive protein (DAS28-CRP) and clinical disease activity index (CDAI). But LORA had a higher simplified disease activity index (SDAI) (P=0.002). Glucorcoticoid was used in 67.4% LORA patients, compared with 29.3% in YORA patients (P<0.001). In contrast, disease-modifying anti-rheumatic drugs (DMARDs) were used in 73.7% LORA patients, compared with 97.6% in YORA patients (P<0.001). CONCLUSION: Delayed diagnose of LORA is common due to atypical presentation at disease onset. RA should be considered in elderly patients with large joints for differential diagnosis. LORA is more likely to have anemia than YORA, albeit no significant differences in serological index and extraarticular presentations. LORA patients should be treated with DMARDs as aggressively as YORA patients, if their comorbidities allow to do so.

[CCL19/IL-1beta positive feedback loop involved in the progress of inflammation in rheumatoid arthritis].

OBJECTIVE: To explore the function and mechanism of CCL19 in the pathogenesis of rheumatoid arthritis. METHODS: Synovial fibroblasts were collected from 5 cases of rheumatoid arthritis. Peripheral blood mononuclear cells (PBMCs) were obtained from 5 healthy people by Ficoll-Hypaque density gradien centrifugation. The cells were stimulated with IL-1beta, TNF-alpha, IL-17 and other cytokines, and then the expression of CCL19 was detected by RT-PCR. The cells also were treated with different concentration of CCL19, then the expressions of IL-1beta, TNF-alpha were detected by RT-PCR, the expressions of p-ERK, p-p38 were detected by western blot. RESULTS: IL-1beta promoted the CCL19/CCR7 expression in both synovial fibroblasts and PBMCs. CCL19 upregulated the expression of IL-10 in both synovial fibroblasts and PBMCs. The stimulation of CCL19 also increased its receptor CCR7 expression. CCL19 activated p-ERK and p-p38 in PBMCs. CONCLUSION: The positive feedback loop between CCL19 and IL-1 participate in the development of rheumatoid arthritis.

[Controlled clinical study on compound Decumbent Corydalis Rhizome and diclofenac in treatment of knee osteoarthritis].

To evaluate the efficacy and safety of compound Decumbent Corydalis Rhizome (DCR) in treating patients with knee osteoarthritis (OA). Totally 79 patients with knee osteoarthritis were selected from out-patient and inpatient departments of West China Hospital and randomly divided into the test group and the control group. The test group (n = 41) was given Compound DCR with the dosage of 1.8 g · d(-1), while the control group (n = 38) was administered with diclofenac sodium with the dosage of 75 mg · d(-1). After 12 weeks of treatment, the total efficacy rates based on patients/physicians evaluation for experimental and control groups were 68.29%, 63.41% and 71.05%, 63.16%, respectively, without significant difference between the two groups. Both of the two groups showed significant improvements in the main efficacy indexes (pain on walking 20 m) and minor indexes (tenderness on palpation, Western Ontario and McMaster Universities OA index (WOMAC) and Short-Form Health Survey (SF-36 ), but without significant difference in efficacy between them. The incidence of related adverse events was 24.39% in the test group and 47.37% in the control group, respectively, with significant differences between the two groups (P < 0.05). In the controlled study, compound DCR is as efficient as diclofenac sodium but more tolerable, with a good clinical application prospect.

[Comprehensive understanding and study on idiopathic inflammatory myopathies to improve its diagnosis and treatment].

Idiopathic inflammatory myopathies(IIM) are a group of autoimmune diseases characterized by muscle inflammation and progressive weakness. Diagnosis of IIM relies on the combination of clinical manifestation, blood biochemical indicators, myositis specific antibodies, electromyogram and pathology of muscle. Classification criteria of IIM keeps developing, and different criteria has different value on clinical application. IIM may involve important organs such as skin, joint, cardiac muscle and lung, except for skeletal muscle. Internal organs involvement should be evaluated and predicted, which could help to improve the prognosis of IIM. It is necessary to evaluate the disease activity objectively and accurately, which now could be accomplished with uniform and standardized clinical assessment system established by international organizations as the International Myositis Assessment and Clinical Studies Group. The immune pathological progress of IIM has already been well-known but the pathogenesis mechanism needs further study. Comprehensive evaluation of disease activity and organ damage need be emphasized before the beginning of treatments. Treatment strategies should be individualized, and the side effects of therapeutic drugs should be monitored. Multidisciplinary collaboration contributes to the improvement of diagnosis and therapy for IIM.

[Comparison of four diagnostic criteria for idiopathic inflammatory myopathy].

OBJECTIVE: To compare the clinical values of four different criteria for diagnosing idiopathic inflammatory myopathy (IIM). METHODS: The four different criteria published in 1975, 1995, 1997 and 2004 were applied to 94 IIM patients and 98 patients with other myopathies in West China Hospital. RESULTS: The sensitivity of the four criteria for diagnosing IIM was 56. 4% (1975), 87. 2% (1995), 61.7 % (1997), and 52. 1% (2004) respectively. The specificity of the four criteria for diagnosing IIM was 78. 6% (1975), 20. 4% (1995), 78. 6% (1997), and 90. 8% (2004) respectively. The Youden index was 35.0% (1975), 7.6% (1995), 40. 3% (1997), and 42.9% (2004), with an odd product of 4. 74 (1975), 1. 75 (1995), 5. 91 (1997) and 10. 77 (2004) respectively. The Kappa value of the four criteria was 0. 351 (1975, P<0.05), 0. 075 (1995, P>0. 05), 0. 404 (1997, P<0.05 ) and 0. 433 (2004, P<0.05), and their area under the ROC curve was 0. 675 (1975, P=0. 00), 0. 538 (1995, P=0.36),0. 701 (1997, P=0.00) and 0. 715 (2004, P=0.00) respectively. CONCLUSION: The 2004 criteria have a better value in diagnosing IIM.

[Clinical features and risk factors associated with interstitial lung disease in patients with classic dermatomyositis or clinical amyopathic dermatomyositis].

OBJECTIVE: To investigate the clinical features and risk factors associated with interstitial lung disease (ILD) in patients with classic dermatomyositis (classic DM) or clinical amyopathic dermatomyositis (CADM). METHODS: Medical records of 121 DM patients (77 with classic DM and 44 with CADM) were reviewed retrospectively. The risk factors associated with ILD were identified through Binary Logistic Regression analyses. RESULTS: ILD presented in 40. 3% (31/77) of patients with classic DM and 50. 0% (22/44) of patients with CADM. Overall, the DM patients had a prevalence of ILD of 43. 8%. Univariate analyses showed that arthralgia, cough, dyspnea, anti-Jo-1 antibody, malignant tumors, and the levels of Erythrocyte sedimentation rate (ESR) and Albumin (ALB) were associated with ILD. The Multivariate Logistic Regression analysis identified arthralgia (beta= 1. 119, P= 0. 023), cough (beta= 2. 423, P= 0. 003) and ESR (beta= 0. 041, P= 0. 009) as risk factors of ILD. CONCLUSION: ILD is a common complication of classic DM/CADM and arthralgia, cough and high level of ESR are independent risk factors of ILD.

[Idiopathic inflammatory myopathies complicated with pneumonias: clinical characteristics and pathogen analysis].

OBJECTIVE: To investigate the clinical characteristics, pathogen constitution and their tolerance to antibiotics in idiopathic inflammatory myopathies (IIM) patients complicated with pneumonia and the associated risk factors. METHODS: The clinical data and pathogen test results of 93 IIM patients with pneumonia and 52 IIM patients without pneumonia (control group) were retrieved and compared. RESULTS: Age of onset and interstitial lung disease were identified as risk factors associated with IIM complicated with pneumonia. Gram negative bacteria were the most common pathogen. Klebsiella pneumoniae was the most common bacteria and Candida albicans was the most common fungus causing infections in the IIM patients, which were relatively sensitive to the third- and fourth-generation of antibiotics such as cephalosporin, fluoroquinolones and aminoglycoside. CONCLUSION: Older patients with interstitial lung disease are prone to having pneumonia, especially those caused by gram negative bacteria and other atypical pathogens. Timely and reasonable anti-infection treatment is essential.

[Levels of acute-phase-reactants in patient with dermatomysitis and its correlations with IL-6 and dermatomyositis disease activity].

OBJECTIVE: To determine the association between acute-phase-reactants (APR) and interleukin-6 (IL-6) in patient with dermatomysitis (DM). METHODS: The levels of C-reactive protein (CRP), serum amyloid A protein(SAA) and serum ferritin (SF) in peripheral blood of 31 adult DM patients were determined by chemiluminescence immunoassay, and compared with those of 23 patients with systemic lupus erythematosus (SLE), 22 patients with rheumatoid arthritis (RA), and 18 patients with Sjagren syndrome (SS). The correlations between the levels of those APR and IL-6 were examined. We also measured dermatomyositis disease activity using myositis disease activity assessment tool (MDAAT), and examined its association with APR levels. RESULTS: DM patients had significantly lower level of CRP [(17. 08 +/- 17. 18) mg/L] than those patients with RA [(85. 95 +/-60.62) mg/L, P<0. 000 1], SLE [(51. 34+/-52. 98) mg/L, P=0. 006] and SS [(47. 00+/-47. 24) mg/L, P= 0.018]. DM patients had significantly lower level of SAA [(92. 04 +/- 98. 93) mg/L] than those patients with RA [(311.30 +/- 292. 45) mg/L, P= 0. 002] and SS [(284. 31 +/- 325. 30) mg/L, P= 0. 025]. DM patients had significantly higher level of SF [(510. 10 +/- 610. 73) ng/mL] than those patients with SS [(220. 33 +/- 164. 07) ng/ mL, P=0. 02], as well as those with RA and SLE albeit without statistical significance. All of the three APRs were positive correlated with IL-6 level. No significant associations between APR and systemic or global disease activities were found, although CRP was associated with constitutional disease activity and SF was associated with pulmonary disease activity. CONCLUSION: DM patients have lower levels of elevated APR than the other three common connective tissue diseases, which is associated with IL-6 but not with global disease activity.