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Rituximab in life threatening antisynthetase syndrome.

E Vandenbroucke¹, J C Grutters, J Altenburg

¹Department of Pulmonology, University Hospital Ghent, 9000 Ghent, Belgium. elkevandenbroucke@hotmail.com

Rheumatology International

|February 3, 2009

Summary

Antisynthetase syndrome, characterized by interstitial lung disease and muscle inflammation, can be severe. This case study shows rituximab effectively treated a patient refractory to other therapies, improving respiratory function.

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Area of Science:

Rheumatology
Pulmonology
Immunology

Background:

Antisynthetase syndrome is an autoimmune disorder associated with interstitial lung disease (ILD) and muscle inflammation.
The presence of anti-Jo-1 and anti-Ro/SSA antibodies can indicate a more severe ILD presentation.
This patient presented with life-threatening respiratory failure refractory to standard treatments.

Observation:

The patient exhibited severe interstitial pneumonitis, polyarthritis, and polymyositis.
Initial treatment with glucocorticoids and cyclophosphamide was ineffective.
The patient's condition rapidly improved following two sequential rituximab infusions.

Findings:

Rituximab treatment led to rapid clinical improvement in a patient with severe, refractory antisynthetase syndrome.
Treatment response correlated with a decrease in serum soluble IL2-receptor levels.
Positron emission tomography (PET) scans confirmed reduced pulmonary disease activity post-rituximab.

Implications:

Rituximab represents a potential therapeutic option for severe antisynthetase syndrome refractory to conventional treatments.
Monitoring soluble IL2-receptor levels may serve as a biomarker for treatment response.
PET-scans can effectively assess treatment efficacy in ILD associated with autoimmune conditions.