Intravenous immunoglobulin may be an effective therapy for refractory, active diffuse cutaneous systemic sclerosis

Area of Science:

• Rheumatology
• Immunology
• Dermatology

Background:

• Diffuse cutaneous systemic sclerosis (dcSSc) is a severe autoimmune disease characterized by ত্বক thickening and organ involvement.
• Treatment options for refractory dcSSc are limited, necessitating exploration of alternative therapeutic strategies.

Purpose of the Study:

• To evaluate the efficacy of intravenous immunoglobulin (IVIG) as an adjunctive therapy for patients with active, refractory diffuse cutaneous systemic sclerosis (dcSSc).

Main Methods:

• A retrospective review of a single-center experience using IVIG in 30 patients with active dcSSc.
• Comparison of modified Rodnan Skin score (mRSS) at baseline and at 6, 12, 18, and 24 months post-IVIG initiation.
• Statistical comparison of mRSS changes with historical control data from clinical trials of D-penicillamine, relaxin, collagen, and mycophenolate mofetil (MMF).

Main Results:

• Adjunctive IVIG treatment led to a significant decrease in mean mRSS from baseline (29.6) to 24 months (15.3) in patients with active dcSSc.
• At 12 months, the mean change in mRSS was significantly better with IVIG compared to D-penicillamine and collagen treatments.
• IVIG's 12-month efficacy was comparable to that of mycophenolate mofetil (MMF) in primary responders.

Conclusions:

• Intravenous immunoglobulin (IVIG) may serve as an effective adjunctive therapy for active dcSSc in patients who have not responded to other treatments.
• Further prospective studies are warranted to confirm the role of IVIG in managing refractory dcSSc.