Ankylosing spondylitis

Walter G Carlini MD PhD (Dr. Carlini of Providence Medical Group - Medford Neurology received research grants from Acadia, Biogen-Idec, and Eisai as a principal investigator.)
Zachary N London MD, editor. (Dr. London of the University of Michigan has no relevant financial relationships to disclose.)
Originally released June 7, 1999; last updated October 9, 2017; expires October 9, 2020

This article includes discussion of ankylosing spondylitis, Bechterew syndrome, Marie-Strumpell spondylitis, rheumatoid spondylitis, and Von Bechterew disease. The foregoing terms may include synonyms, similar disorders, variations in usage, and abbreviations.

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.

Key points

 

• Ankylosing spondylitis is an autoimmune rheumatologic disorder that may engender a variety of systemic and/or neurologic sequelae.

 

• The pathogenic mechanisms proposed to underlie ankylosing spondylitis may help guide our thinking about autoimmune neurologic disorders.

 

• The modern antitumor necrosis factor therapies for ankylosing spondylitis can cause neurologic complications.

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; Lu and Katz 2005).]

• "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 3 different mammalian orders dating back 30 to 50 million years ago (Rothschild et al 1998). Radiologic studies of Egyptian mummies indicate that some of the pharaohs of ancient Egypt were afflicted with ankylosing spondylitis (Feldtkeller et al 2003). 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 (Leden et al 2009). In 1691, Bernard Connor described a human skeleton with the characteristic features of ankylosing spondylitis (Connor 1691). 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 (van der Linden 1997).

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