류마티스관절염에서 후천성 혈우병이 동반되는 경우는 드물지만 빠른 진단과 치료가 예후에 가장 중요하기 때문에 류마티스관절염의 환자에서 출혈성 경향을 보이는 경우 반드시 혈액 응고 검사를 시행하여 후천성 혈우병의 가능성을 배제해야 한다. 저자들은 류마티스관절염과 후천성 혈우병이 동반된 환자에서 스테로이드와 cyclophosphamide로 호전된 1예를 경험하였기에 보고하는 바이다.

Acquired hemophilia is a rare disease caused by an autoimmune reaction to coagulation factor VIII, The mortality rate of this disease is very high (8∼22%). Clinical manifestations are different from congenital hemophilia. Various diseases are associated with acquired hemophilia, including autoimmune diseases such as systemic lupus erythematosus or rheumatoid arthritis (RA), tumors, inflammatory bowel disease, psoriasis, asthma, diabetes, acute hepatitis B or C, and drug reactions. However, the underlying cause is unknown in approximately 50% of cases. A few cases of acquired hemophilia with RA have been published. However, no cases have been reported in Korea. We had a patient with longstanding RA and acquired hemophilia who was suffering from upper and lower extremity purpura with a deep intramuscular hematoma. The patient was successfully treated using cyclophosphamide combined with steroid.