ABSTRACT
Objective:To evaluate the efficacy and safety of eltrombopag standard therapy in the treatment of patients with connective tissue disease (CTD) associated with thrombocytopenia who were non-responder to recombinant human thrombopoietin (rhTPO) or relapsed after discontinuation of rhTPO.Methods:Retrospectively analysis of clinical data of 4 cases presented with CTD associated with thrombocytopenia who were non-responder to rhTPO or relapsed after discontinuation of rhTPO, and then treated with eltrombopag from November 2017 to June 2022. The literature were reviewed on eltrombopag in the treatment of CTD associated with thrombocytopenia.Results:The platelet count of 4 patients increased to more than 30×10 9/L after 2 weeks administration of eltrombopag. The median time was 32 (14, 41) days for platelet counts returning to normal level. The dosage of eltrombopag was gradually reduced to maintain normal platelet count, which was helpful for tapering of glucocorticoid and other immunosuppressant. No adverse event was observed during the treatment. Conclusion:Eltrombopag is safe and effective for CTD associated with thrombocytopenia, for patients who are non-responders to rhTPO or relapsed after discontinuation of rhTPO treatment in particular.
ABSTRACT
Objective@#To assess the association between lupus nephritis disease activity and anti-C1q antibodies.@*Methods@#The study analyzed the medical records of 98 patients with lupus nephritis (LN), 35 patients without lupus nephritis. LN disease activity was measured by the systemic lupus international collaborating clinics (SLICC) renal activity score of 2008. All biopsied tissues were scored based on the International society of nephrology/Renal pathology society (ISN/RPS) 2003 LN pathological typing standards, acute and chronic index scores were used to evaluate the activities of lupus. All patients were test for the levels of anti-dsDNA and anti-C1q antibodies using the enzyme-linked immuno sorbent assay (ELISA), C3, C4, 24-hour urinary protein performed in parallel. For normally distributed quantitative parameters, the differences between groups were assessed by t test. Mann-Whitney U test was performed for non-normally distributed data. The cut-off values were evaluated by using receiver operating characteristic (ROC). The Spearman methods were used to test correlations.@*Results@#Patients with LN had a higher levels of anti-C1q antibodies than patients without lupus nephritis [3.94 (10.2, 91.3) AU/ml与6.9 (2.0, 15.4) AU/ml; Z=-4.299, P<0.01]. Patients with inactive lupus nephritis had higher levels of C1q, C3, C4 than active LN (t=2.393, 3.777, 2.557; P<0.05). Patients with active lupus nephritis had higher levels of anti-C1q antibodies than inactive LN (Z=-4.632, P<0.01). Anti-C1q antibody levels were positively correlated with levels of 24-hour urinary protein, AI score (r=0.327, P<0.01) and SLICC score (r=0.493, P<0.01), and were negatively correlated with serum C1q (r=-0.373, P<0.01), C3 (r=-0.532, P<0.01) and C4 (r=-0.463, P<0.01). The optimal cutoff value of Anti-C1q for a diagnosis of active LN was 48.9 RU/ml, and the sensitivity and specificity were 62.5% and 80%, respectively. The area under the curve (AUC) was 0.771.@*Conclusion@#Anti-C1q antibodies are more closely correlated with renal disease activity, and anti-C1q antibody is an important serum marker for monitoring LN activity, but its pathological mechanism in the pathogenesis of LN still needs to be further explored.