ABSTRACT
Objective To investigate clinical characteristics of relapsing polychondritis(RP)and to improve early recognition for it.Methods Clinical and laboratory data of 56 patients with RP were analyzed retrospectively.Results Ratio of number of male patients to female ones was 1.2.Age at onset was(46?11)years(ranging from 27 to 71)and average interval between onset and diagnosis was(21? 35)months,(8?6),(16?31)and(29?37)months for patients initial onset with auricle,respiratory tract and joints involved,respectively.Site involved included airway in 40 patients(71.4%),auricle in 32 (57.1%),joints in 32(57.1%),eyes in 27(48.2%),nasal chondritis in 25(44.6%)and inner ear in 13(23.2%).At initial stage of the course,17 patients were misdiagnosed as respiratory infection (30.4%),nine as perichondritis(16.1%),six as pulmonary tuberculosis(10.7%),five as rheumatoid arthritis(8.9%).Seven of 40 patients with airway involvement received metallic stents for their tracheobronchial stenosis.Four patients whose condition never improved after regular therapy all had respiratory involvement.Conclusions Patients of RP with initial onset at non-auricle,non-nasal sites tended to be misdiagnosed.Prevalence of airway involvement was not so low with a poor prognosis in patients of RP.
ABSTRACT
Objective To investigate the significance of anticentromere antibody(ACA)in patients with primary biliary cirrhosis(PBC).Method ACA was detected using indirect immunofluorescence(IIF)in 99 patients with PBC,and the difference was compared between patients with and without ACA.Results Fifty-three patients(53.5%)had ACA in serum.The average age of onset was elder in patients with ACA than in patients without ACA[(52.6?1.5)vs(46.2?2.0),P=0.012].There was no significant difference in sex ratio between the two groups.Incidence of gastrointestinal bleeding was higher in patients with than without ACA (13.2% vs 0,P=0.014).Difference was not significant in symptoms such as fatigue,pruritus,icterus,etc. Though it didn't reach statistically significant,the incidence of esophageal varices was higher in patients with ACA than without ACA(45.8% vs 10.0% respectively,P=0.061).There was no significant difference in diam- eter of portal vein,splenomegaly,ascites.Biochemically only serum total protein reached statistical significance between the two groups[(73.3+1.1)g/L vs(78.1+l.7)g/L respectively,P=0.017].Patients with ACA had low- er serum IgG than ACA negative patients[(15.1?0.6)g/L vs(18.4?1.0)g/L respectively,P=0.006].Nuclear envelope pattern(NE),one of the patterns of ANA,was rare in ACA positive group than ACA negative group (16.7% vs 50.0% respectively,P=0.002).Conclusion The prevalence of ACA is high in patients with PBC. Patients with ACA have high risk of oesophageal varices and gastrointestinal bleeding.Nuclear envelope pat- tern of ANA is rare in patients with ACA.