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  • Updated 10.12.2020
  • Released 06.07.1999
  • Expires For CME 10.12.2023

Ankylosing spondylitis

Introduction

Overview

Ankylosing spondylitis is of neurologic interest not only because it may exhibit nervous system manifestations but also because it can be viewed as the articular homologue of multiple sclerosis. It is a chronic, generally progressive autoimmune disease that strikes in the prime of life and gives rise to a varied spectrum of articular and, oftentimes, extraarticular symptoms. What is known of its mechanism of disease could contribute to understanding multiple sclerosis and other autoimmune diseases. In this article, the author discusses the clinical manifestations of ankylosing spondylitis, its diagnostic work-up, and its management, including effective, disease-modifying immunomodulatory therapies.

Historical note and terminology

Etymologically, ankylosing spondylitis derives from the Greek roots ankylos (crooked) and spondylos (joint of the back). The name of the disease is evocative of the severely kyphotic posture exhibited by patients with advanced cases of ankylosing spondylitis. Rheumatoid spondylitis, Bechterew syndrome, and Marie-Strümpell spondylitis are alternate names for ankylosing spondylitis. Ankylosing spondylitis is the principal example of the class of disorders known as the seronegative spondyloarthropathies (See Table 1).

Table 1. The Seronegative Spondyloarthropathies

• Ankylosing spondylitis

• Reactive arthritis (formerly Reiter syndrome) [The name of this syndrome was changed because Hans Reiter, after whom the syndrome was named, was a convicted Nazi war criminal, and use of the term “Reiter syndrome” in the published literature is declining (Panush et al 2003; Wallace and Weisman 2003; 69).]

• "Secondary" spondyloarthropathies

- associated with inflammatory bowel disease
- associated with psoriasis

• Pauciarticular juvenile chronic arthritis

• Undifferentiated spondyloarthropathies

Spondyloarthropathies occur in many mammalian species besides man and have even been identified in fossil specimens from three different mammalian orders dating back 30 to 50 million years ago (89). Radiologic studies of Egyptian mummies indicate that some of the pharaohs of ancient Egypt were afflicted with ankylosing spondylitis (40). DNA sequences coding for HLA-B27, a human leukocyte antigen strongly linked to ankylosing spondylitis, have been isolated from a 1000-year-old ankylosed skeleton unearthed in Sweden (61). In 1691, Bernard Connor described a human skeleton with the characteristic features of ankylosing spondylitis (29). Not until the nineteenth century did clinical reports of patients afflicted with ankylosing spondylitis appear, and it was 1930 before the archetypical radiographic findings in this disease were fully appreciated (116).

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