A study of ten Japanese patients with seronegative spondylarthropathy: a tentative proposal

Abstract

We reviewed ten patients with seronegative spondylarthropathy (SNSA), who all fulfilled the European Spondylarthropathy Study Group criteria for spondylarthropathy (SpA); seven patients also met the Amor criteria for SpA. Seronegative spondylarthropathy was not a uniform syndrome but rather a wide spectrum of complex disease with characteristics of sacroiliitis and enthesopathy. The most frequent symptom at diagnosis of SNSA was inflammatory low back pain, followed by asymmetric oligoarthralgia and Achilles tendonitis and/or plantar fasciitis. Systemic complications were revealed as eye and skin involvement. Imaging methods including pelvic radiography, scintigraphy, and computed tomography scanning were useful in detecting spondylarthropathic changes, which were characteristic of SNSA. Human leukocyte antigen (HLA) typing showed various patterns among patients, in which HLA-B27 was positive in three patients with ankylosing spondylitis. HLA-B51, which is a well-known genetic factor associated with Behçet's disease (BD), was positive in two patients who were apparently distinct from BD. Two patients with palmoplantar pustulosis showed symptoms and signs characteristic of SNSA. Although we have few SNSA patients in the present study, we would like to propose that HLA-B51 positive SpA would be considered as a subset of SNSA, and that pustulotic SpA also would be classified as a member of SNSA. This led us to suggest the possibility to change the concept of SNSA proposed by Moll et al. The optimal treatment remains to be defined, but sulfasalazine was effectively used with almost all patients in combination with nonsteroidal anti-inflammatory drugs.

Tubulointerstitial Nephritis Associated with Enteritis and Sacroiliitis

“…However, some cases have reported associations between ankylosing spondylitis and abnormal findings on a urinalysis (hematuria, proteinuria, or urinary cast) (21). Cases of HLA-B51-positive ankylosing spondylitis have also been reported in Japan (22). The association between ankylosing spondylitis and sacroiliitis is well known (23).…”

A Case of Human Leukocyte Antigen (HLA) B27-Positive Intestinal Behçet's Disease with Crohn's Disease-Like Anal Fistulas

“…21 HLA-B27 may constitute a significant prognostic marker for disease severity. 22,23 A relationship between HLA-B51 and SpA was reported, 24,25 and some SpA cases without HLA-B27 but with HLA-B51 24 could not fulfill either the international or Japanese criteria for Behçet's disease. 2,4 The present case was HLA-B51 negative but HLA-B27 positive; moreover, the patient's father was a patient with AS.…”