Seronegative arthropathy and associated diseases--a multigenic syndrome?

Abstract

Patients hospitalized for psoriasis, acute anterior uveitis, ankylosing spondylitis or with chronic prostatis were examined. From previous reports and our own results we formulated hypotheses of the genetic mechanisms involved. We then examined all available adult relatives of 75 patients with ankylosing spondylitis. Our previously established genetic hypotheses were tested by segregation analyses in these families. All our results point to the existence of a syndrome of distinct but interacting genetic factors. The main factor is the HLA-B27 associated disease predisposition. Manifestation of this predisposition seems to be influenced by sex and by presence of factors coding for psoriasis and/or psoriatic arthropathy. Our results concerning psoriasis and psoriatic arthropathy supported the previous suggestions that these diseases exhibit genetic heterogeneity. It is known that disease signs may be triggered by infection in genetically predisposed persons. The syndrome therefore has a multifactorial aetiology. Seronegative arthropathy is a term that includes all arthritic components of the syndrome. There is a need for a term that encompasses all clinical signs of the genetic factors involved. We propose 'Hereditary multifocal relapsing inflammation' (HEMRI) as a descriptive term for this syndrome. The disease entities included may be regarded as subtypes of the syndrome.