Ankylosing Spondylitis with Hypermobility Syndrome – Impossible to diagnose

I once wrote that ankylosing spondylitis (AK) was impossible to diagnose in women:
The Pain Diagnosis that Cannot be Made in Women – Spine Arthritis – Ankylosing Spondylitis
http://painmuse.org/?p=77
Blood work is rarely positive including ESR, CRP, and HLA B27; MRI imaging takes at least 10 years to show positive and radiologist are not very good at reading them. Finally, women have hypermobile SI joints by necessity re pregnancy and rarely show much stiffness. To make things worse, are those cases that have AK and hypermobility syndrome – their chances of diagnosis are low. 4 cases were diagnosed mostly because they were HLA B27 positive (unusual finding) and they had a family history of rheumatic disease

J Clin Rheumatol. 2018 Dec 28. doi: 10.1097/RHU.0000000000000959. [Epub ahead of
print]
Concurrent Joint Hypermobility Syndrome and Spondyloarthropathy: A New Spondyloarthropathy Subgroup With a Less Severe Disease Phenotype?
Shur NF et al
https://www.ncbi.nlm.nih.gov/pubmed/30601197

  • describes 4 cases of ankylosing spondyliltis (AK) with hypermobility
  • all ere female
  • 3/4 were HLA B27 positive and 3/4 gave a family Hx rheumatic disease Hence the women were suspected because they were HLA B27 positive and/or and have a family history of rhenumatologic disease and showed MRI findings.
  • Schober’s test
    – Mark midline at 5 cm below iliac spine
    – Mark midline at 10 cm above iliac spine
    – measure distance upright and bent forward
    – should be more than 20 cm – if not ?AK
  • All were managed without anti-TNF treatments – using just NSAID’s etc

However, women are less likely to show HLA-B27 changes:
Xiong J, Chen J, Tu J, Ye W, Zhang Z, Liu Q, et al.
Association of HLA-B27 status and gender with sacroiliitis in patients with ankylosing spondylitis.
Pak J Med Sci 2014;30(1)http://www.pjms.com.pk/index.php/pjms/article/view/3896/1929

So they were likely exceptionally bad cases with an easy diagnosis. Family history of iritis in 2 cases,  psoriasis in one and AK in one helped.

Comment – make example of a AK case dismissed because she could touch her toes. Part of the disability not talked about is the chronic fatigue associated. I have one patient with chronic depression and fatigue that gives a history of chronic back pain and I suspect the chronic fatigue related to AK augments his depression. A diagnosis of AK is important to patient’s life but will need even more suspicion.

Calin, A., L. Edmunds, and L. Gail Kennedy.
Fatigue in ankylosing spondylitis–why is it ignored?.
The Journal of rheumatology 20.6 (1993): 991-995.
https://europepmc.org/abstract/med/8350337
“Our data reveal that fatigue should be considered a major problem for patients with Ankylosing spondylitis”

However, I suspect women with AK are doomed to obscurity.

 

 

 

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