Successful rituximab treatment in a seronegative rheumatoid arthritis patient with concurrent cold agglutinin syndrome and immune thrombocytopenia.

Cold agglutinin disease, a subtype of cold-type autoimmune hemolytic anemia, is referred to as cold agglutinin syndrome (CAS) when it develops secondary to other conditions. Immune thrombocytopenia (ITP) is an autoimmune disorder characterized by the peripheral destruction of platelets. While both CAS and ITP can occur in patients with rheumatoid arthritis (RA), their concurrent manifestation in a single patient has not been reported, leaving the optimal treatment strategy for such a complex case unclear.

Solitary fibrous tumor of the central nervous system with epithelioid neuroendocrine "Transdedifferentiation": A case report and review of the literatures.

Solitary fibrous tumors (SFTs) of the central nervous system (CNS) are rare mesenchymal tumors characterized by a fusion of the NGFI-A-binding protein 2 (NAB2) gene and the signal transducer and activator of transcription 6 (STAT6) gene, immunohistochemically resulting in nuclear expression of STAT6 - an immunohistochemical hallmark essential for diagnosis, as outlined in the fifth edition of the World Health Organization Classification of Tumors. Dedifferentiation, where low-grade tumors transform into high-grade forms, has been observed in SFTs, with documented cases involving sarcomatous or rarely epithelial transformations. We report the first case of a CNS SFT exhibiting "transdedifferentiation" into epithelioid neuroendocrine differentiation.

Efficacy and safety of switching therapy from chenodeoxycholic acid to cholic acid in Japanese patients with bile acid synthesis disorders.

Objectives: This study aimed to assess the safety and efficacy of cholic acid (CA) treatment over 74 weeks in Japanese patients with inherited enzymatic bile acid synthesis disorders (BASD).

Methods: This phase 3, open-label, single-arm study enrolled four Japanese patients diagnosed with BASD, including two with 3β-hydroxy- -C-steroid dehydrogenase/isomerase (HSD3B7) deficiency and two with -3-oxosteroid 5β-reductase (SRD5B1) deficiency. The patients had received chenodeoxycholic acid (CDCA) treatment but were switched to CA treatment.

Interleukin-17A is a potential therapeutic target predicted by proteomics for systemic sclerosis patients at high risk of pulmonary arterial hypertension.

We explored effective therapeutic targets for systemic sclerosis (SSc) patients with high risk for pulmonary arterial hypertension (PAH) by plasma proteomics analysis. A total of fifty-seven patients with SSc were enrolled in the study and the prevalence of PAH was 19.3%.