A case report of successful treatment with immunoadsorption onto protein A in mixed connective tissue disease in childhood
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Published on: December 15, 2011
Silke Rummler1, Karina Althaus, Bernhard Maak
1Institute of Transfusion Medicine, University Hospital, Jena, Germany. silke.rummler@med.uni-jena.de
Insights
This study shows immunoadsorption therapy effectively treated a young patient with severe mixed connective tissue disease and pulmonary arterial hypertension. The treatment led to significant improvement and sustained remission, allowing the patient to regain health.
Area of Science:
- Immunology
- Cardiology
- Rheumatology
Background:
- Mixed connective tissue disease (MCTD) can lead to severe complications like pulmonary arterial hypertension (PAH) and heart failure.
- Standard immunosuppressive therapies and high-dose steroids were ineffective in this patient.
Observation:
- An 11-year-old male with severe MCTD, PAH (NYHA class III-IV), skin ulcers, Raynaud's phenomenon, and arthritis presented with treatment-resistant disease.
- The patient showed no improvement despite intensive immunosuppressive treatment and high-dose steroids.
Findings:
- Combined therapy including immunoadsorption onto protein A, low-dose cortisone, and bosentan resulted in significant clinical improvement.
- The patient achieved and maintained clinical remission with 22 sessions of immunoadsorption, requiring no further immunosuppression or apheresis.
Implications:
- Immunoadsorption is a promising therapeutic option for severe, refractory MCTD with life-threatening PAH and heart failure.
- This case highlights the potential of targeted immunoadsorption in managing complex autoimmune conditions in pediatric patients.
- Successful treatment led to a complete recovery, with the patient being healthy and constitutionally age-appropriate at follow-up.
Abstract:
An 11-year-old male patient suffering mixed connective tissue disease with life-threatening pulmonary arterial hypertension, progressive heart failure (New York Heart Association class III-IV), skin ulcers, Raynaud's phenomenon and arthritis, showing no improvement after intensive immunosuppressive therapy or high dose steroids, was treated with immunoadsorption onto protein A. With a combined therapy of low-dose cortisone and bosentan and 22 sessions of immunoadsorption, his condition improved significantly and he continues in clinical remission. At the time of writing no further immunosuppressive therapy or immunoadsorption had been necessary. The patient is now 15 years old and healthy with an age-based constitution comparable to the normal population.
