Behçet's disease and sacroiliitis in a child

Area of Science:

• Rheumatology
• Immunology
• Pediatrics

Background:

• Behçet's disease (BD) is a multisystem inflammatory disorder.
• Sacroiliitis, inflammation of the sacroiliac joints, can occur in BD but is not always recognized.
• Distinguishing BD-related sacroiliitis from other spondyloarthropathies is crucial for accurate diagnosis and management.

Purpose of the Study:

• To report a case of a pediatric patient with co-occurring Behçet's disease and bilateral sacroiliitis.
• To investigate the relationship between BD and sacroiliitis in this specific case.
• To contribute to understanding the spectrum of musculoskeletal manifestations in Behçet's disease.

Main Methods:

• Case report of a Japanese girl diagnosed with Behçet's disease at age 8.
• Clinical evaluation including assessment of buttock pain onset at age 10.
• Radiographic imaging (pelvic X-rays) and bone scintigraphy to assess sacroiliac joint involvement.
• HLA typing (B44, DR4, B27) to evaluate genetic associations.

Main Results:

• The patient presented with bilateral grade 3 sacroiliitis confirmed by X-ray and increased uptake on bone scintigraphy.
• Spinal mobility and chest expansion were not restricted.
• HLA typing was positive for B44 and DR4, but negative for B27, ruling out seronegative spondyloarthropathy.

Conclusions:

• This case suggests that erosive sacroiliitis can be an intrinsic manifestation of Behçet's disease.
• The findings emphasize the importance of considering sacroiliitis in pediatric patients with BD.
• Further research is warranted to elucidate the pathogenesis and prevalence of sacroiliitis in Behçet's disease.