Systematic review of the presentation of coagulation factor VIII inhibitors in rheumatic diseases: A potential cause of life-threatening hemorrhage.

OBJECTIVES: To provide a comprehensive review regarding the clinical presentation of acquired factor VIII (FVIII) inhibitors, also known as "acquired hemophilia," in patients with rheumatic diseases.

METHODS: A systematic MEDLINE search was conducted to identify English-language articles published from 1993 through January 10, 2012, providing details regarding the clinical presentation, laboratory evaluation, and management of a patient(s) with newly or previously diagnosed autoimmune disease coexistent with an acquired FVIII inhibitor.

RESULTS: In total, 49 patients fulfilled the criteria for inclusion in the review; the greatest percentage (24.5%) had systemic lupus erythematosus, followed by rheumatoid arthritis (16%). The majority (78%) presented with spontaneous mucocutaneous or muscular bleeding. Prolonged activated partial thromboplastin time (aPTT) was identified in all of the 45 patients for whom results were provided. Five patients presented with an asymptomatic prolonged aPTT, which was attributed to a lupus anticoagulant in two patients, only one of whom actually had a coexisting lupus anticoagulant. Invasive procedures led to serious bleeding in both of these patients, one of whom died as a result. The majority (59%) of patients experienced complete or partial remission of their inhibitors, most (96%) after systemic eradicative therapy. A total of three (6%) patients died as a direct result of FVIII inhibitors.

CONCLUSIONS: Although acquired FVIII inhibitors are rare in patients with autoimmune diseases, prompt diagnosis is essential to avoid extensive bleeding, which could be life threatening. Treatment requires eradication of the factor inhibitors. Rheumatologists must be able to distinguish acquired FVIII inhibitors from lupus anticoagulants.