Rituximab as a Treatment Option after Autologous Hematopoietic Stem Cell Transplantation in a Patient with Systemic Sclerosis

Abstract

Systemic sclerosis (SSc) is an intractable autoimmune disease characterized by vasculopathy and organ fibrosis. Autologous hematopoietic stem cell transplantation (AHSCT) should be considered for the treatment of selected patients with rapid progressive SSc at high risk of organ failure. It, however, remains elusive whether immunosuppressive therapies such as rituximab (RTX) are still necessary for such patients after AHSCT, especially in those with bad outcomes. In the present report, a 43-year-old man with diffuse cutaneous SSc received AHSCT. Despite AHSCT, SSc further progressed with progressive symptomatic heart failure with newly developed concomitant mitral and tricuspid valve insufficiency, thus the patient started on RTX 8 months after AHSCT. Shortly after initiation of RTX, clinical symptoms and organ functions ameliorated subsequently. Heart valve regurgitations were reversible after initiation of RTX treatment. Currently, the patient remains in a stable condition with significant improvement of clinical symptoms and organ functions. Reporting about therapies after AHSCT in SSc is a very important issue, as randomized controlled trials are lacking, and therefore this report adds to evidence that RTX can be considered as a treatment option in patients with SSc that do not respond to AHSCT.

Overlapping Case of Advanced Systemic Sclerosis and IgG4-Related Disease after Autologous Hematopoietic Stem Cell Transplantation

“…With steroids being the first-line therapy, RTX or AZA are suggested in case of an inadequate response or relapse [ 14 ]. In addition, RTX could be a therapy of choice for post-transplant systemic sclerosis [ 27 ]. In this respect, if required, RTX could have been proposed for further treatment of the patient.…”

Outcomes of Autologous Hematopoietic Stem Cell Transplantation in an Overlapping Case of Advanced Systemic Sclerosis and IgG4-Related Disease

“…RTX or AZA are suggested in case of inadequate response or relapse [14]. In addition, RTX could be the agent of choice in systemic sclerosis after transplantation [28]. In this regard, the patient might have benefited from the additional RTX after AHSCT.…”

Case report: A successful second autologous hematopoietic stem cell transplantation in refractory systemic sclerosis, with positive effect on skin involvement, pulmonary function and microcirculation

“…In addition, targeted B-cell therapy with rituximab might be considered. Rituximab represents a promising strategy for skin and lung fibrosis in SSc but long term data is missing and in fact, to date, this is an experimental treatment also ( 11 , 14 , 16 ). Furthermore, treatment with Nintedanib was discussed but declined as this has not shown effect on skin involvement.…”

“…NCM images were acquired using an optical probe video capillaroscope equipped with a 200x contact lens and connected to image analysis software (at time of the first HCT VideoCap 3.0, at the time of the second HCT Optilia OP-120 011, Mediscope Digital, videomicroscope (USB interface), OptiPix Capillaroscopy software, clinic 1.7.x with a 200x high resolution objective lens with unpolarized light). Two images per finger were evaluated and consisted of both quantitative and qualitative parameters, as described by the by the EULAR Study Group on Microcirculation in Rheumatic Diseases/Scleroderma Clinical Trials Consortium on Capillaroscopy ( 14 ). Evaluation of images was performed by 2 experienced physicians (MV and BK).…”