Objective:To investigate the clinical features of ocular involvement and the application of Rose criteria in patients with relapsing polychondritis (RP).Methods:The data from RP patients with ocular involvement were collected and analyzed. Patients included must have at least one major criteria of Michet criteria and the application Rose criteria was also investigated. Demographic data of these patients was presented as percentages. The difference between types of disease onset was tested by Mann-Whitney U and comparison among groups was tested by False Discovery Rate. Results:A total of 192 patients were enrolled 98 males and 94 females. The mean age of disease onset was (42±14) (0.5-79) years old, the median disease duration (DD) was 13(0.5, 600) months. The median RP disease activity index (RPDAI) was 39(9-74) and the median RP organ damage index (RPODI) was 2.5(0.1, 108). The median RP damage index (RPDAM) was 3(1-6). The statistical significant difference was identified in median DD between groups of nose and pinna ( Z=10.775, P<0.01), nose and OEH ( Z=9.277, P<0.01), in RPODI between groups of nose and pinna ( Z=7.999, P=0.031), nose and and extra-cranial organs ( Z=8.115, P=0.030) and eye and airway involvement of RPDAM could be seen between groups ( Z=7.683, P=0.037) respectively. Ocular involvement(50.0%), auricular chondritis(21.4%) and airway chondritis(13.5%) were the top three most common symptoms at disease onset. The ocular involvement(100%), airway chondritis (75.0%) and inner ear involvement(69.3%) were the top three most frequent affected organs. All parts of eye could be involved in RP ocular damage. Single-organ involvement was 59.9%; and multi-organ involvement could be seen in 40.1% patients. Diagnostic strength was enhanced by application of Rose criteria in 171 cases fulfilled Michet criteria and 21(10.9%) cases partially fulfilled Michet criteria fulfilled Rose criteria. Active screening for organ (especially inner ear and airway) involvement would improve the rate of early diagnosis. The pinna and airway involvement suggested nose and middle-ear might be involved. Conclusion:Ocular involvement in RP can involve all parts of the eye ball. Examining the inner ear and airway may help to confirm the diagnosis. It is worthy to apply this to clinical practice.

Wheat grain peroxidase 1 (WP1) belonged to class III plant peroxidase with cofactor heme, which not only has antifungal activity, but also influences the processing quality of flour. In order to enhance functional expression of WP1 in prokaryotic system by increasing endogenous heme synthesis, we constructed a recombinant plasmid pACYC-A-L containing hemA and hemL of Esherichia coli. Then, we co-transformed it into host strain T7 Express with secretive expression vector (pMAL-p4x-WP1) or non-secretive expression vector (pET21a-MBP-WP1), respectively. The MBP-WP1 fusion protein was further purified by amylose affinity chromatography and its peroxidase activity was assayed using 2,2'-azino-bis (3-ethylbenzothiazoline-6-sulfonate) (ABTS) as substrate. At 12 h after induction at 28 degree, the extracellular 5-aminolevulinic acid (5-ALA) production of T7 Express/pACYC-A-L was up to 146.73 mg/L, simultaneously the extracellular porphrins also increased dramatically. The peroxidase activity of functional MBP-WP1 obtained from T7 Express/ (pACYC-A-L + pMAL-p4x-WP1) was 14.6-folds of that purified from T7 Express/ pET21a-MBP-WP1. This study not only successfully enhanced functional expression of wheat peroxidase 1 in Esherichia coli, but also provided beneficial references for other important proteins with cofactor heme.
Escherichia coli ; genetics ; metabolism ; Genetic Vectors ; genetics ; Heme ; biosynthesis ; genetics ; Peroxidases ; biosynthesis ; genetics ; Recombinant Fusion Proteins ; biosynthesis ; genetics ; Transformation, Genetic

Objective To investigate the clinical and audiological characteristics of non-elder patients with relapsing polychondritis (RP).Methods Clinical and audiological data of patients with RP under 60 years old were collected consecutively and analyzed.The t-test and Mann-Whitney U test were used for statistical analysis between the two groups in quantitative data in normal distribution and non-normal distribution respectively,while Chi-square test was use for qualitative data analysis.Results One hundred and seventy four patients with complete data who fulfilled the Michet criteria were enrolled with a M∶F=1∶1.1.The mean age of disease onset was (39±13) (8-60) years;the median time of disease duration was 12 (1-480) months;the median relapsing polychondritis disease activity index (RPDAI) was 38(10-77) and the median RPODI was 2.4(0.1-56).Auricular chondritis (32.8％,57/174),ocular involvement (24.7％,43/174) and airway chonchritis (21.3％,37/174) were the top three onset-pattern.All parts of external,middle and inner ear were involved in RP.Inner-ear damage was the most common (95.4％,166/174) with insidious cochlea and vestibule equally distributed.Auricular chondritis was predominant in external ear involvement (55.2％,96/174);ET dysfunction was included in eardrum abnormalities of neglected middle-ear involvement (29.9％,52/174).Positive HL by active detection was 71.8％(125/174) with 14.3(25/174) HL fulfilled world health organization (WHO)-2006 criteria,including 52.0％(13/25) disabling HL.Sensorineural hearing loss (SNHL) was predominant in RP after 21 years old while 60.0％(6/10) was seen in childhood RP with non-SNHL.At least two parts of ear involvement were seen in almost all patients with heavy overlap.Conclusion All parts of ear are involved in non-elder RP with age related clinical characteristics.Active detection is a key to find insidious middle-and inner-ear involvement for early RP recognition.RPODI is a potential marker for RP evaluation.

Objective To investigate the clinical characteristics of granulomatosis with polyangiitis (GPA) patients with scleritis.Methods The completed clinical data of 102 GPA patients from March 2012 to July 2016 in Capital Medical University, Tong Ren Hospital were collected. Altogether 43 GPA patients with scleritis were enrolled, and the clinical data were analyzed respectively. The t test, Mann-Whitney U test and x2testwere used to analyze the data. Results The most common ocular manifestation in GPA was scleritis. The incidence of renal damage(37.2% vs 11.9%,x2=9.148,P=0.002),disease activity(BVAS/GPA score 8.9 vs 5.1,Z=7.847, P=0.000),DEI score (7 vs 4,Z=5.919, P=0.000),inflammation index(56 mm/1 h vs 36 mm/1 h, Z=2.456, P=0.016; CRP 51 mg/L vs 8 mg/L, Z=3.748, P=0.000), neutrophil percent (70% vs 61%, t=3.993, P=0.000),complement 3(1.2 g/L vs 1.0 g/L, t=2.652, P=0.009),plasma fibrinogen(3.5 g/L vs 3.3 g/L, t=3.947, P=0.000), D-dimmer (0.5 mg/L FEU vs 0.3 mg/L FEU, Z=2.371, P=0.018) and ANCA positive rate (90.7% vs 57.6%,x2=13.369,P=0.000)were higher in patients with scleritis,as well as a poor prognosis(2009-FFS 0.4 vs 0.1, Z=2.600, P=0.009). However, a lower rate of subglottic involvement was detected in scleritis group (4.7% vs 20.3%, x2=5.169, P=0.023). There were no significant differences between GPA patients with scleritis and episcleritis in clinical feature, disease activity, inflammatory index and prognosis. Aggressive immunosuppres-sive treatment was appropriate for GPA with scleritis. Tumor necrosis factor (TNF)-аinhibitor was an effective treatment. Conclusion GPA with scleritis usually suffered from higher disease activity index,broader DEI,as well as poorer prognosis. Aggressive treatment should be initiated in order to achieve disease remission. Subglottic involvement is less common in patients with scleritis. The phenotype of scleritis is not associated with disease severity.

Objective To analyze the clinical features of chronic rhino-sinusitis (CRS) in patients with immunoglobulin G4-related disease (IgG4-RD).Methods A retrospective analysis of the data from March 2013 to July 2015 in our Department of Rheumatology was conducted in patients who were diagnosed as IgG4-RD and were follow-up for over half a year.The clinical features and the results of laboratory tests were compared between of the case group and the control group.The t test,Mann-Whitney U test and Fisher'Exact Test (n ＜40) were used to analyze the data.Results Twenty-four cases in 44 cases of IgG4-RD were complicated with CRS (54％).The mean age was (49±13) years old,with the ratio of male:female was 2∶1;With the longer disease duration [4.0 (1.0,6.3) year vs 0.5 (0.2,4.3) year,U=-2.182,P=0.041],the more the number of organs involved [4.0 (3.8,5.3) vs 3.0 (1.0,4.0),U=-2.827,P=0.005],the higher the ratio of ocular involvement (89％ vs 42％,P=0.013).The higher the percentage of eosinophils in peripheral blood [8.5％(4.0,13.8) vs 3.3％(0.8,8.5),P=0.043],the more common the allergic manifestations (61％ vs 20％,P=0.026),the higher the operation times [1.5(1.0,3.0) vs1.0(0,10),U=2.096,P=0.048] before making the definitive diagnosis than the control group A.The level of ESR/CRP (56％ vs 0,P=0.004) and the number of IgG4 positive plasma cells [57.5(50.0,66.3)/HP vs 10.0(1.8,20.0)/HP,U=4.358,P＜0.01] and the percentage of IgG4/IgG positive plasma cell (40％ vs 10％,P＜0.01) in the nasal mucosal tissues of the IgG4-related chronic rhino-sinusitis were higher than patients with ordinary CRS (the control group B),but there was no difference in the severity of sinusitis manifestations between patients with IgG4-related chronic rhino-sinusitis and the regular CRS.Conclusion IgG4-related chronic rhino-sinusitis is different from regular CRS,and is closely associated with IgG4-related ocular lesion.IgG4-related chronic rhino-sinusitis has some clinical features which are different from other phenotypes of IgG4-related diseases.

Objective To investigate clinical and imaging characteristics of patients with relapsing polychondritis (RP) with insidious airway involvement.Methods Data collected prospectively and consecutively for patients with the diagnosis of RP and their disease activity evaluation was analyzed.The t-test and Mann-Whitney U test were used for statistical analysis between the two groups forquantitative datain normal distri-bution and non-normal distribution respectively,while Chi-square test was use for qualitative data analysis.Results Two hundred and sixteen patients with complete data from Dec 1,2007 to Jul 31,2016 were enrolled with a M:F ratio of 1:1.Mean age of disease onset was (44±16)(8～86) years.The median disease duration was 12 (0.3～480) month.The median relapsing polychondritis disease activity index (RPDAI) was 35 (8～67),the median RPODI was 2.4 (0.1～84).The top three initial presentation were auricular chondritis (28.7％),airway chondritis (24.1％) and ocular involvement (22.2％) respectively.RPODI was significantly higher in auricular chondritis (4.4) (Z=-2.084,P＜0.05) and lower in nose chondritis (0.6) (Z=-2.425,P＜0.05).Up to 81.5％ of the patients were found with airway damage and 52.3％ of them were asymptomatic.Airway damage was mostly located in trachea (79.0％ 139/176) and common features on CT scan werecharacterized by airway wail thickening (72.7％,128/176).Airway narrowing was mostly seen in symptomatic patients while calcification was seen more in asymptomatic patients.Insidious hearing-loss and nose chondritis were found not related to airway damage while obvious auricular chondritis (x2=15.580,P＜0.01),ocular involvement (x2=8.105,P＜0.01) were found to be more in patients with asymptomatic airway damage.All diagnosis before RP was organ-driven.Conclusion Airway involvement in RP is one of the three most common disease initial presentation-sand half of them are asymptomatic.RPODI is a reasonable marker for disease evaluation.Routine follow-up of airway damage (wall thickness,calcification and lumen narrowing) is essential for early RP recognition.