Cryoglobulinemic vasculitis in systemic sclerosis successfully treated with mycophenolate mofetil

Area of Science:

• Rheumatology
• Immunology
• Internal Medicine

Background:

• Systemic sclerosis (SSc) is a rare autoimmune disease characterized by fibrosis and vascular abnormalities.
• Cryoglobulinemic vasculitis is an uncommon complication, with only two prior cases reported in SSc patients.
• This case highlights the diagnostic and therapeutic challenges of this rare comorbidity.

Observation:

• A patient with SSc and secondary Sjőgren's syndrome presented with small-vessel vasculitis.
• Clinical manifestations included arthritis, purpura, microhaematuria, digital gangrene, and myocardial ischemia.
• Laboratory findings revealed mixed cryoglobulinemia, antinuclear antibodies (ANA), rheumatoid factor, and anti-SSA/Ro antibodies.

Findings:

• Initial treatment with corticosteroids and cyclophosphamide failed to control vasculitis symptoms.
• Switching to mycophenolate mofetil resulted in significant clinical improvement.
• This suggests mycophenolate mofetil may be an effective therapeutic option for cryoglobulinemic vasculitis in SSc.

Implications:

• This case expands the understanding of cryoglobulinemic vasculitis in SSc.
• It underscores the importance of considering vasculitis in SSc patients with relevant symptoms.
• Mycophenolate mofetil offers a promising alternative treatment strategy for this rare condition.