She complained of bleeding from the sclera of her left eye, nose, and gingiva and reported dyspnoea. best of our knowledge, this is the first case report of concomitant TTP and asymptomatic Sj?grens syndrome in a pregnant woman. It highlights the association between pregnancy, autoimmune disease, and TTP. It also emphasizes the importance of an enzyme-linked immunosorbent assay in the diagnosis and rituximab in the treatment of patients with acquired TTP. Supplementary Information The online version contains supplementary material available at 10.1186/s12884-021-04167-9. Keywords: Thrombotic thrombocytopenic purpura, Pregnancy, Rituximab, Sj?grens syndrome Background Thrombotic thrombocytopenic purpura (TTP) is a rare life-threatening disease with an untreated mortality rate of 90% [1]. TTP is characterised by extensive platelet thrombus in the microvasculature [2], thrombocytopenia, mechanical haemolysis, Edivoxetine HCl injury, and dysfunction of involved tissues and organs [3]. Typical clinical manifestations of TTP include thrombocytopenia, microangiopathy haemolytic anaemia (MAHA), neuropsychiatric symptoms, renal function damage and fever [4]. According to the Oklahoma TTP-HUS Registry, 70% of cases of TTP occur in women, 45% of whom are of child-bearing age [5]. Moreover, TTP occurs in one out of 25,000C100,000 pregnancies, mostly in the late third trimester or during the puerperium [6]. Due to its heterogeneous clinical presentation, the phenotype of TTP during pregnancy and its management have not been well documented [6, 7]. Pregnancy-associated TTP can be divided into congenital TTP (cTTP) and acquired TTP (aTTP) according to the patients genetic background and antibody detection. One study showed that 66% of women presenting with acute TTP during pregnancy or in the immediate postpartum period had late-onset previously undiagnosed congenital disease [6]. Pregnancy-related aTTP may be associated with autoimmune disease [8]. There have been several reports of aTTP presenting secondary to a connective tissue disease, such as systemic lupus erythematosus, mixed connective tissue disease, rheumatoid arthritis, scleroderma, or dermatomyositis. Compared to other connective tissue diseases, primary Sj?grens syndrome (pSS) combined with TTP is quite rare [9]. Most patients with aTTP harbour ADAMTS13 inhibitors, while 11.5C17% of patients have non-neutralising antibodies [10]. Non-neutralising antibodies can only be detected by enzyme-linked immunosorbent assay-based detection methods [11]. We have encountered a case of pregnancy-associated TTP complicated by pSS with non-neutralising antibodies that was successfully treated with rituximab in addition to plasma exchange and pulse corticosteroid therapy. Case presentation A 25-year-old woman who was in week 36 of her first pregnancy was admitted to our obstetrics department on October Edivoxetine HCl 18, 2019. She complained of bleeding from the sclera of her left eye, nose, and gingiva and reported dyspnoea. She also had a 2-day history of diarrhoea and blurred vision in the left eye. There was no medical history or family history of abnormal bleeding. The foetal heart rate was normal (140 beats per minute) and there were no uterine Edivoxetine HCl contractions. Obstetric ultrasonography revealed that the foetus had an appropriate gestational age. An initial complete blood cell analysis BMPR2 indicated a platelet count of 7.0??109/L, a haemoglobin level of 66?g/L, a reticulocyte count of 5.71%, and a white blood cell count within the normal range. The unbound bilirubin level was elevated to 57.83?mol/L and the serum lactate dehydrogenase (LDH) level to 4886?U/L. Direct and indirect Coombs Edivoxetine HCl tests were negative. Anti-SS-A/60KD (+), anti-SS-A/52KD (++), and anti-nuclear antibody (ANA) (+) were detected and anti-double-stranded DNA was negative on regular screening. TTP was suspected. However, she had no neurological symptoms or fever. No schistocytes were identified in her blood smear. Given that her blood pressure was increased to 140/80?mmHg, her renal function tests showed slight proteinuria (+++), and her aspartate aminotransferase (AST) was increased to 52?U/L, a diagnosis of pregnancy-related thrombotic microangiopathy was made. However, in view of the difficulty in distinguishing between TTP, HELLP (Haemolysis Elevated Liver enzymes Low Platelets) syndrome, pre-eclampsia, and haemolytic uraemic syndrome, an ADAMTS13 test was requested. While waiting for the ADAMTS13 test result, the patient progressed to hypoxaemia, bilateral hydrothorax, and heart failure. Given that the patient was nearly full-term and had a significantly decreased platelet count, a caesarean section was performed immediately. This resulted in delivery of a healthy female baby weighing 2100?g (with Apgar scores of 9 after 1?min and 10 after 5?min) who was transferred to the neonatal care unit. The operation went well, and 100?mL of bloody ascites was noted during surgery. However, the patients platelet count was still very low (approximately 30??109/L) after delivery. According to the PLASMIC scoring system [12], the PLASMIC score.