Juvenile rheumatoid arthritis in two siblings with congenital leucocyte adhesion deficiency

Abstract

A sister and brother with congenital leucocyte adhesion deficiency developed systemic-onset juvenile rheumatoid arthritis (JRA). They showed polyarthritis, spiking fever, reddish eruptions, anaemia, hepatosplenomegaly, increased erythrocyte sedimentation rate, and positive rheumatoid factor. Occurrence of JRA in our patients was thought to be mainly due to a combination of recurrent bacterial infections and abnormal lymphocyte function as a consequence of membrane adhesion-protein deficiency. In view of the familial occurrence, hereditary factors may have played a role in the development of JRA in our patients.