Systemic lupus erythematosus with acquired hemophilia A: A case report and literature review / 中南大学学报(医学版)

Systemic lupus erythematosus (SLE) complicated with acquired hemophilia A (AHA) is a rare condition with frequently delayed diagnosis and a high mortality rate, so it is necessary to strengthen the understanding of this disease. In this study, the characteristics and treatment in 1 case of SLE complicated by AHA is reported and analyzed, and a literature review is conducted. The patient was a 29-year-old young female with a 10-year history of SLE, the main clinical manifestation was severe abdominal bleeding. Laboratory tests revealed that the activated partial thromboplastin time (APTT) was notably prolonged (118.20 s), and the coagulation factor VIII activity (FVIII꞉C) was extremely decreased (0.20%) with high-titer of factor VIII (FVIII) inhibitor (31.2 BU/mL). After treating with high-dose glucocorticoid, immunoglobulin, cyclophosphamide, rituximab, blood transfusion, and intravenous infusion of human coagulation FVIII, the coagulation function and coagulation FVIII꞉C were improved, and FVIII inhibitor was negative without serious adverse reactions. During the next 5-year follow-up, the patient's condition was stable and no bleeding occurred. In the case of coagulation dysfunction in SLE, especially with isolated APTT prolongation, AHA should be screened. When the therapeutic effects of glucocorticoid combined with immunosuppressants are not desirable, rituximab could be introduced.

Differences between Chinese men and women with adult-onset non-radiographic axial spondyloarthritis / 中华风湿病学杂志

Objective:To explore the clinical characteristics of adult-onset non-radiographic axial spondyloarthritis (nr-axSpA) in different genders.Methods:A total of 662 patients with adult-onset nr-axSpA (age at disease onset ≥16 years) who visited the Rheumatology Department of the First Affiliated Hospital of Shantou University Medical College from 1999 to 2020 were included in the study. Comparisons of baseline demographic and clinical characteristics between different genders were performed.Results:Overall, the male-to-female ratio was 1.17∶1, and the prevalence of human leukocyte antigen-B27 (HLA-B27) positivity was 71.8%(475/662). The median baseline disease duration and age at diagnosis was 1.6 (0.5, 4.0) years and 25.0 (21.0, 33.0) years respectively. The males had a significantly earlier age at disease onset and diagnosis [21.0 (18.0, 28.0) vs 25.0 (21.0, 30.0), Z=5.63, P<0.001; 24.0 (19.0, 32.0) vs 27.0 (23.0, 34.5), Z=4.90, P<0.001, respectively] than females. HLA-B27 positivity was more frequent in males than in females [78.4% (280/357) vs 63.9%(195/305), χ2=17.06, P<0.001]. The prevalence of inflammatory back pain (IBP), morning stiffness, nocturnal pain, enthesitis, hip and groin pain were higher in males, whereas females showed a higher prevalence of small joint involvement of the hands. At baseline, males had higher median ankylosing spondylitis disease activity score (ASDAS)-C-reaction protein (CRP) [3.0(2.3, 3.8) vs 2.4(2.0, 3.0), Z=5.59, P<0.001] and a greater prevalence of high disease activity ASDAS-CRP>2.1 [81.9%(185/227) vs 67.9%(133/195), χ2=11.08, P=0.001] than females. The proportions of male patients with elevated CRP levels and erythrocyte sedimentation rate (ESR) were also higher than those of female patients [49.0%(175/357) vs 27.9%(85/305), χ2=30.85, P<0.001; 49.3%(176/357) vs 33.4%(102/305), χ2=16.98, P<0.001, respectively]. Conclusion:The adult-onset nr-axSpA in China is characterized by a comparable sex ratio. Males have an earlier age at disease onset and are higher HLA-B27 positivity with higher prevalence of IBP, enthesitis, hip and groin pain, as well as high disease activity.

Characteristics and clinical significance of nailfold capillaroscopy in patients with systemic lupus erythematosus / 中华风湿病学杂志

Objective:To summarize the characteristics of nailfold capillaroscopy (NC) in patients with systemic lupus erythematosus (SLE) and explore its clinical significance.Methods:NC examination was performed in 162 SLE patients. The clinical data of SLE patients was collected. Tianniu NC scoring standard was used. The t test was applied to analyze the measurement data, the χ2 test was applied to analyze the counting data. the Pearson or Spearman test was used to evaluate the correlative factors of NC in patients with SLE. Results:NC abnormalities were seen in 87.7%(142/162) of SLE patients, and the incidence of mild, moderate and severe abnormalities was 29.0%(47 cases), 45.1%(73 cases) and 13.6%(22 cases) respectively. The most common NC abnormal manifestation in SLE patients was decreased blood flow velocity (86.4%). In patients with moderate to severe NC abnormalities, the proportions of patients with Raynaud's phenomenon (37.9% vs 23.9%, χ2=2.955, P=0.043) and interstitial lung disease (8.0% vs 0, χ2=5.213, P=0.023), and the level of D-Dimer [(1 992±2 279) μg/L vs (1 248±1 721) μg/L, t=-1.624, P=0.013] were significantly higher than those in the groups with normal/mild NC abnormalities. Correlation analysis demonstrated that Raynaud's phenomena, interstitial lung disease, pulmonary hypertension and D-Dimer were positively correlated with the NC abnormality. Conclusion:NC abnormalities are common in SLE patients. Decreased blood flow velocity is the most frequent manifestation. SLE patients with moderate to severe NC abnormalities should be actively screened for pulmonary hypertension and interstitial lung disease.