[HLA B27-negative forms of Bechterew's disease].

Area of Science:

• Immunogenetics
• Rheumatology
• Clinical Medicine

Background:

• Ankylosing spondylitis, also known as Bechterew's disease, is strongly associated with the HLA-B27 antigen.
• However, a subset of patients with Bechterew's disease are negative for HLA-B27, and their clinical characteristics are less understood.

Purpose of the Study:

• To investigate the clinical features and genetic associations in patients with Bechterew's disease who are negative for the HLA-B27 antigen.
• To identify potential alternative genetic markers or clinical patterns in this patient group.

Main Methods:

• Retrospective analysis of 45 patients diagnosed with Bechterew's disease over an 8-year period who tested negative for HLA-B27.
• HLA-A, -B, and -C antigen typing was performed on 35 of these patients.
• Clinical data, including disease onset, course, and specific manifestations, were collected and analyzed.

Main Results:

• Among the HLA-B27 negative patients, 12 carried antigens from the B27 cross-reacting group (B7, Bw22, B40).
• Patients without psoriasis exhibited antigens B13, B16, and B17, which are linked to psoriasis vulgaris.
• Nine patients were carriers of the Cw2 antigen, which is in unbalanced linkage with B27.
• The B27-negative Bechterew's disease cohort presented with late onset, a slow and favorable disease course, exclusively spinal involvement, and a lack of iridocyclitis and cardiac lesions.

Conclusions:

• Bechterew's disease can occur in the absence of the HLA-B27 antigen, presenting distinct clinical phenotypes.
• The identified clinical features in B27-negative patients suggest a potentially milder or different disease pathway.
• Further research into alternative genetic markers and the specific pathophysiology of B27-negative Bechterew's disease is warranted.