Preliminary results on long-term follow-up of systemic sclerosis patients under extracorporeal photopheresis

Abstract

Background

Extracorporeal photopheresis (ECP) has been considered for treatment of patients with systemic sclerosis (SSc).

Objectives

To study the 12-month effects of ECP on laboratory parameters and evaluate the SSc-related long-term survival.

Methods

59 SSc patients who had received at least 6 ECP cycles were included. Lab parameters were assessed at baseline (ECP naïve), after 6 months, and after 12 months. 20-year follow-up data were collected for all patients.

Results

31 (59/52.5%) patients presented with elevated serum III procollagen (sPIIINP) levels at baseline which significantly declined after 6- and 12-month ECP. Total lymphocyte counts as well as circulating immune complexes (CICs) significantly decreased after 12-months ECP. On long-term follow-up, patients had received a median of 37.5 (6-167) ECP cycles over a median period of 64 (6-281) months. 20-year follow-up revealed only 8 (59/13.6%) SSc-related deaths and 51 (59/86.4%) survivors.

Conclusions

One-year ECP induces changes in lab parameters, such as sPIIINP, CICs, and lymphocyte counts, which have previously been implicated in the pathogenesis of SSc. More importantly, our data reveal, for the first time, that ECP-treated SSc patients appear to have extremely favorable 20-year survival rates compared to other SSc cohorts reported in the literature.

Keywords:

• Extracorporeal photopheresis
• extracorporeal photochemotherapy
• scleroderma
• systemic sclerosis
• survival

Acknowledgment

This work is part of the doctoral thesis of Olcay Özsoy.

Disclosure statement

T.G. has received speakers and/or advisory board honoraria from BMS, Sanofi-Genzyme, MSD, Novartis Pharma, Roche, Abbvie, Almirall, Janssen, Lilly, Pfizer, Pierre Fabre, Merck-Serono, outside the submitted work. L.S. has received speakers and/or advisory board honoraria from BMS, Sun-Pharma, MSD, and Novartis. OÖ, DB, and C.H.S. have no conflict of interest to declare.

Figure 1. Kaplan-Meier curve showing 20-year systemic sclerosis (SSc)-related survival (n = 59) in patients who had received extracorporeal photopheresis. 51 patients (86.4%) survived within a 20-year follow-up period. Only in the dcSSc subgroup, eight patients (13.6%) died.

Correction Statement

This article has been republished with minor changes. These changes do not impact the academic content of the article.