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ObjectiveAnti-centromere antibody(ACA)-positive primary Sjögren's syndrome (SS) is considered a subtype of SS, and the clinical significance of ACA in SS has not been totally clear. The purpose of this study is to investigate the clinical manifestation and laboratory examination characteristics of primary Sjgren's syndrome(pSS) with anticentromere antibody(ACA). MethodsThe clinical data of 88 pSS patients admitted to the Department of Rheumatology Immunology of the First Affiliated Hospital of Dalian Medical University were collected, including 43 ACA positive patients (ACA positive group) and 45 ACA negative patients (ACA negative group). The clinical characteristics and laboratory indicators of each group were compared.ResultsThe mean age of pSS patients with ACA positive (63.0±11.1) was higher than that of pSS with ACA negative (57.7±12.5), and in the group of ACA (+) pSS, Renand's phenomenon (RP) was more frequent（P0.05).ConclusionThe clinical characteristics and laboratory features of pSS patients with positive ACA differ from those with negative antibody. pSS patients with ACA positive were older, they had more Reynolds phenomenon, liver injury and combined PBC.
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Objective To study the expression of anticentromere antibody (ACA)on multiple diseases to help the clinical diag-nosis and treatment.Methods The clinical and laboratory data of 129 cases with positive ACA were retrospectively ana-lyzed.Results ①116 females and 13 males were found among 129 cases with positive ACA,and serology titer of ACA in fe-male patients was higher than male and more compound antibody were shown in female patients,which was significantly dif-ferent compared with males (χ2=6.28,P=0.01;χ2=6.85,P=0.003).②Among 118 cases which had detailed clinical in-formation,there were 65 patients suffered from autoimmune diseases (55.08%)and 53 cases suffered from non-autoimmune diseases (44.92%).Autoimmune disease cases showed more compound and high serology titer,there were significant differ-ence between the two groups (χ2=21.97,P<0.001;χ2=11.44,P=0.001).Conclusion Though ACA was rarely found,it was shown in autoimmune and non-autoimmune diseases and can be not too severe or fetal and even lead to multi-organ fail-ure.So it should be taken seriously.
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Objective The aim of this study is to analyze the association between IgM anti-mitochon-drial antibody subtypeⅡ(AMA-M2-IgM) and the anti-centromere antibody IgG (ACA-IgG) in primary biliary cirrhosis (PBC) patients, and to investigate the clinical significance of M2-IgM and ACA-IgG in the diagnosis of PBC. Methods We selected 36 cases of PBC patients as research subjects whose AMA-IgG and AMA-M2-IgG were both negative. The M2-IgM positive rate in the ACA-IgG positive group and negative group was compared. We also analyzed the course of disease, pathological changes and the positive conversion rate of M2-IgG between the M2-IgM positive group and negative group. Results There were 9 cases of M2-IgM positive patients (64%) and 5 cases of M2-IgM negative patients (36%) in the ACA-IgG positive group. In the ACA-IgG negative group we found 3 cases of M2-IgM positive patients (14%) and 19 cases of M2-IgM negative patients (86%). So the M2-IgM positive rate in the ACA-IgG positive group was significantly higher than that of the ACA-IgG negative group (P=0.003). According to the tracking detection results, the M2-IgG positive conversion rate in the M2-IgM positive group was 67% (8 patients), which was significantly higher than the M2-IgM negative group 8%, (2 patients) (P=0.001). Conclusion M2-IgM is a specific antibody for PBC in the early stage, which presents earlier than M2-IgG. In the PBC patients whose AMA-IgG and AMA-M2-IgG antibodies are both negative, the M2-IgM positive rate is closely related to the ACA-IgG, so the ACA-IgG detection is very important in the early diagnosis of PBC. Therefore, we speculated that patients with ACA-IgG antibody are more susceptible to PBC.
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Se describe una mujer de 57 años, tabaquista, con necrosis digital, fenómeno de Raynaud y anticuerpo anticentrómero en ausencia de esclerodactilia, esclerodermia proximal y distal y trastornos esofágicos. Fueron descartadas patologías neoplásicas, otras enfermedades del tejido conectivo y enfermedades vasculares, las cuales no fueron desarrolladas durante el tiempo de seguimiento de esta paciente. Se concluye que la paciente tuvo un síndrome RACAND, el cual no evolucionó a otra enfermedad del tejido conectivo hasta el fallecimiento de la misma.
We described a 57-year old woman smoker, with digital necrosis, Raynaud phenomenon and positive anticentromere antibody. Shedidnt show sclerodactily, proximal or distal scleroderma or esophagealdisorders. Malignancies, other connective tissues diseases andvascular diseases were ruled out and were not developed during thefollow up. We conclude that this patient had a RACAND syndrome,which do not evolved into other connective disease until the deathof the patient.
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Humans , Necrosis , Raynaud DiseaseABSTRACT
Objective To investigate the clinical and laboratory characteristics of anticentromere antibody (ACA)and anti-SSA antibody expressions in patients with Primary Sj(o)gren's Syndrome (PSS). Methods Twelve PSS patients with ACA positive but SSA negative(ACA PSS)and 19 PSS patients with SSA positive but ACA negative(SSA PSS)were enrolled into the study and classified into two groups. We compared the age,laboratory data,occurrence of Raynaud's phenomenon(RP),and histological changes in minor labial salivary glands biopsies of the patients from two group. Results The mean age of the ACA PSS group(68.4 ± 7.9)years was significantly higher than that of the SSA PSS(54. 6 ± 16. 2)years group(P ＜ 0. 05). Serum IgG level of ACA PSS group(17. 89 ±4. 08)g/L was close to the normal range,which was significantly lower than that of SSA PSS(27.90 ±6. 72)(P ＜0. 01). Leukocytopenia was less frequently observed in ACA PSS than in SSA PSS(P ＜ 0. 05),the difference between two groups was statistically significant. We also found more frequent RP in the ACA PSS group than SSA PSS group(P ＜ 0. 05). Conclusions Our data confirm that ACA positive PSS differs from SSA positive PSS at several clinical respects and laboratorial examinations.
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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.