Polymyositis and pemphigus vulgaris in a patient: successful treatment with rituximab.

Abstract

Rituximab has been documented to be an effective treatment for autoimmune diseases with contribution of B cells. We report a case of antisynthetase syndrome with a history of EBV-induced lymphoma which developed a pemphigus vulgaris. Rituximab was effective both on polymyositis and on pemphigus. Fifteen months later, the patient died from a septic shock after the first cyclophosphamide infusion for amyloidosis while the B cell population remained depleted. Rituximab may be a good alternative to immunosuppressive drugs in polymyositis and pemphigus especially in lymphoma-risk patients. However, it did not prevent progression to secondary amyloidosis and the fatal infection developed in this patient raises the question of whether a prolonged B cell depletion with rituximab contributes to a greater risk of infection.

• Authors: Anne Tournadre, Stéphanie Amarger, Pascal Joly, Michel d'Incan, Jean-Michel Ristori, Martin Soubrier
• Journal: Joint bone spine (Joint Bone Spine) Vol. 75 Issue 6 Pg. 728-9 (Dec 2008) ISSN: 1778-7254 [Electronic] France
• PMID: 18455465 (Publication Type: Case Reports, Journal Article)

Chemical References

• Antibodies, Antinuclear
• Antibodies, Monoclonal
• Antibodies, Monoclonal, Murine-Derived
• Immunosuppressive Agents
• Rituximab
• Ligases
• Prednisone

Topics

• Amyloidosis (complications, pathology)
• Antibodies, Antinuclear (immunology)
• Antibodies, Monoclonal (therapeutic use)
• Antibodies, Monoclonal, Murine-Derived
• Drug Therapy, Combination
• Fatal Outcome
• Female
• Humans
• Immunosuppressive Agents (therapeutic use)
• Kidney Diseases (complications, pathology)
• Ligases (immunology)
• Middle Aged
• Pemphigus (drug therapy, immunology, pathology)
• Polymyositis (drug therapy, immunology, pathology)
• Prednisone (therapeutic use)
• Rituximab
• Shock, Septic
• Syndrome