Add-on Erythromycin and Tacrolimus Treatment for Primary Sjogren’s Syndrome-Related Immune Thrombocytopenia: A Case Report

A 64-year-old woman with primary Sjogren’s syndrome (SjS) was admitted to our hospital for nasal bleeding, oral bleeding, and purpura on her entire body. On this admission, laboratory findings were as follows: white blood cell counts, 8,910 /μL; hemoglobin, 9.9 g/dL; and platelet (PLT) counts, 0.1×104/μL. Although the anti-PLT antibodies were positive, other autoimmune antibodies including anti-deoxyribonucleic acid antibodies were negative. No immunoglobulin (Ig)M antibodies for cytomegalovirus and Parvovirus B19, and no IgG antibodies for Helicobacter pylori, were detected. No abnormal findings suggestive of infection were identified in the systemic examination. A bone marrow aspiration smear revealed normal bone marrow. Based on these findings, the patient was diagnosed with primary SjS-related immune thrombocytopenia (ITP). The patient initially received intravenous immunoglobulin, methyl prednisolone (PSL) pulse therapy, and subsequent high dose PSL without significant improvement. Therefore, eltrombopag was added. Subsequently, erythromycin (EM) was also added, considering its immunomodulatory effects. After initiating the EM treatment, the PLT counts increased. Therefore, the PSL dosage was reduced. However, the PLT counts gradually decreased. Instead of increasing the PSL dosage again, tacrolimus (TAC) was successfully added. Consequently, the PSL dosage could be successfully reduced. This case suggests that EM and TAC can be effective alternatives when conventional immunosuppressants fail to improve primary SjS-related ITP."