Intravenous cyclophosphamidevsrituximab for the treatment of early diffuse scleroderma lung disease: open label, randomized, controlled trial

Abstract

Clinical Trials Registry - India, www.ctri.nic.in, CTRI/2017/07/009152.

Cytotoxic CD4+ T lymphocytes may induce endothelial cell apoptosis in systemic sclerosis

“…Consistent with previous reports, Th2 cell numbers were greatly increased in bullous pemphigoid tissues and outnumbered those in SSc and healthy control skin ( Figure 2C and Supplemental Figure 2). Despite the 5-to 10-fold greater number of CD4 + T cells in skin biopsies of bullous pemphigoid patients compared with SSc biopsies, we found the absolute numbers of tissue-infiltrating T lymphocytes have not been systematically interrogated at the tissue level (22)(23)(24).…”

“…Although prominent numbers of both B and T cells were observed, the absolute number of infiltrating T cells was greater than those of infiltrating B cells ( Figure 1, A and B). Although randomized double-blind clinical trials examining the utility of B cell depletion therapy in SSc have not been performed, some studies have reported clinical improvement with rituximab, especially in the context of early diffuse SSc (22,23). Additionally, activated B cells have been observed in the circulation of SSc patients (24,36).…”

Management of systemic sclerosis‐associated interstitial lung disease in the current era

“…Recent data from a Phase 3 trial showed that the treatment of early ILD (mean FVC% of 82% with mild ILD on HRCT) in high‐risk populations (early diffuse cutaneous SSc and elevated C‐reactive protein [CRP] with 50% having positive anti‐SCL‐70 antibodies) with tocilizumab led to stabilization of FVC% vs a decline of 6.5% in the placebo group at 48 weeks . Similarly, an open‐label trial of patients with early diffuse cutaneous SSc and positive anti‐SCL‐70 found improvement in FVC% at 24 weeks with the use of two courses of rituximab (1000 mg × 2 doses) vs monthly pulse CYC . We offer treatment to patients with subclinical ILD and elevated CRP and/or positive anti‐SCL‐70.…”

Progressive fibrosing interstitial lung disease associated with systemic autoimmune diseases

“…However, MMF is now the most widely used therapy for SSc-ILD and has been endorsed as firstline therapy by consensus panels of experts [46,53,54]. Rituximab and azathioprine are also used in the treatment of SSc-ILD, but the evidence that these drugs may preserve or improve lung function in these patients comes solely from retrospective or open-label studies [55][56][57][58].…”