Knut Dale

Family Studies in Bechterew's Syndrome (Ankylosing Spondylitis): II. Prevalences of Symptoms and Signs in Relatives of HLA 827 Negative Probands

All available adult first-degree relatives of 9 probands with HLAB27 negative ankylosing spondylitis were examined. Completion rate was 90%. Prevalences of symptoms and signs and distributions of chest and spinal mobility are presented. No secondary case of Bechterews syndrome was found among the relatives, compared with 10% among the relatives of HLAB27 positive probands (p = 0.05). We conclude from this study and that of relatives of HLAB27 positive probands, that a HLAB27 negative relative runs a low risk of contracting Bechterews syndrome, irrespective of the HLAB27 status of the proband.

Radiographic Changes, HLA B27 and Back Pain in Patients with Psoriasis or Acute Anterior Uveitis

Twenty-five female and 23 male consecutive patients hospitalized because of severe psoriasis, and 34 consecutive patients hospitalized because of acute anterior uveitis (AAU) were examined. Twelve of the patients with AAU (35%) and 11 of the patients with psoriasis (23%) had definite sacro-iliitis, which was unilateral in 6 (55%) of the former and in 2 (17%) of the latter. No definite radiographic differences in sacro-iliitis between HLA B27-positive and -negative patients could be detected. The radiographic changes of the dorso-lumbar junction (Th9-L2) related to the diseases, were seen only in HLA B27 positive patients with sacro-iliitis. Scattered lateral syndesmophytes were the commonest paravertebral ossifications in patients with psoriasis, whereas more diffuse and more numerous lateral mixed osteophytes appeared most frequently in AAU patients. Parasyndesmophytes were seen both in patients with psoriasis and in those with AAU. Asymptomatic sacro-iliitis was frequently found in unilateral, psoriatic sacro-iliitis (4 out of 6 patients) but was not restricted to this group. No radiographic indications of aortic valve insufficiency or apical lung fibrosis were found.

Dynamic MR-imaging in the diagnosis of pigmented villonodular synovitis of the knee

The aim of this report was to study the rate of contrast enhancement at MRI in pigmented villonodular synovitis (PVNS). Dynamic MRI was performed in a patient with PVNS just before and 1 week and 3 years following synovectomy of the knee. As opposed to patients with rheumatic disease the patient with PVNS showed a slower enhancement rate before synovectomy than 1 week after. At 3 years follow-up the patient with PVNS showed an enhancement rate just above that of the normal controls. The results indicate that PVNS has features more common to hyperplastic or neoplastic lesions than to inflammatory ones. The results at 3 years follow-up also suggest that dynamic MRI may indicate the efficacy of arthroscopic synovectomy.The aim of this report was to study the rate of contrast enhancement at MRI in pigmented villonodular synovitis (PVNS). Dynamic MRI was performed in a patient with PVNS just before and 1 week and 3 years following synovectomy of the knee. As opposed to patients with rheumatic disease the patient with PVNS showed a slower enhancement rate before synovectomy than 1 week after. At 3 years follow-up the patient with PVNS showed an enhancement rate just above that of the normal controls. The results indicate that PVNS has features more common to hyperplastic or neoplastic lesions than to inflammatory ones. The results at 3 years follow-up also suggest that dynamic MRI may indicate the efficacy of arthroscopic synovectomy.

Bechterew's Syndrome (Ankylosing Spondylitis): A Syndrome with Distinct Subgroups

The results of tests for associations among radiographic findings of the dorsolumbar spine, peripheral joints, tendon insertions and the pubic symphysis are presented. Ankylosis of sacro-iliac joints, ankylosis of apophyseal joints, bridging syndesmophytes, ossified interspinous ligament, block vertebrae, arthritis of the pubic symphysis and new bone formation of the ischium were strongly mutually associated. They probably belong to the same subgroup of disease. Such findings were negatively associated with distal peripheral joint arthritis. Mixed osteophytes, parasyndesmophytes or shining corners (anterior spondylitis) showed associations suggesting that the etiology may be mixed. A late stage of sacro-iliitis, regressive changes, characterized by narrow joint spaces without extensive ankylosis and with minimal sclerosis (grade IV sacro-iliitis) was associated with distal peripheral joints arthritis, but negatively associated with signs of ankylosing processes of the dorsolumbar spine. On the basis of the radiographic findings, distinct subgroups of AS could be identified. These results confirm our previous results of an association pattern between clinical findings. We would also recommend that the grading system of sacro-iliitis put forward by Dale be adopted, since it turned out that this grading system often distinguished between distinct subgroups of this heterogeneous condition(s).

Juvenile Ankylosing Spondylitis and HLA B27 Homozygosity

Ten consecutive patients with juvenile ankylosing spondylitis were investigated in a prospective study. At least one of the parents of each patient had a positive history of probable or definite ankylosing spondylitis, Reiters syndrome, or acute anterior uveitis. The patients and their parents were examined clinically and were HLA-typed. Nine of the 10 patients exhibited the genetic marker HLA B27, but only one patient was homozygous for HLA B27. This prevalence of homozygosity among HLA B27 positive persons could be expected to occur by chance alone. Based on the limited material studied, we cautiously conclude that HLA B27 homozygosity is probably not responsible for disease onset in childhood.

Radiographic evaluation of patients with Bechterew's syndrome (ankylosing spondylitis) and their first-degree relatives. Findings in the spine and sacro-iliac joints and relations to non-radiographic findings.

One hundred and twenty-two hospitalized patients, 35 years or more of age with confirmed bilateral sacro-iliitis and 239 first-degree relatives, 25 years or more of age, were examined. All but 3 of the patients had confirmed ankylosing spondylitis (AS) in accordance with established criteria. The sacro-iliac joints were scored by a recently developed grading system. The prevalence of grade V sacro-iliits--extensive ankylosis--was greater in HLA B27-positive than in HLA B27-negative patients, and ankylosis of apophyseal joints, ossification of the interspinous ligament, or block vertebrae were not seen in HLA B27-negative patients. Shining corners and/or squared vertebrae were the most frequent findings of the dorsolumbar spine in patients with a disease course of less than 10 years. Mixed osteophytes were rare in patients below 40 years of age. All radiographic inflammatory changes scored in the spine-except for shining corners--were most often seen in patients with a disease history exceeding 20 years. Syndesmophytes and/or ankylosed apophyseal joints were less extensive in female than in male patients. No differences in the severity of radiographic changes in sacro-iliac joints or in frequency, severity or localization of different radiographic changes of the dorsolumbar spine were observed between HLA B27-positive patients with and those without psoriasis or acute anterior uveitis. Definite sacro-iliitis was restricted to HLA B27-positive relatives of HLA B27-positive probands and demonstrated in one-fifth of them. Shining corners and/or squared vertebrae were frequent findings in relatives with sacro-iliitis (45%), whereas apophyseal joint ankylosis, ossification of the interspinous ligament, block vertebrae and bridging syndesmophytes were not seen. Our results support the view that AS is not a defined disease entity and should be regarded as a syndrome. AS is an improper name for all patients fulfilling the Rome and/or New York criteria for definite AS, since many of them will probably never develop ankylosis of the spine or sacro-iliac joints.

Modified Larsen score--a comment.

Sir, In a newly published article on simple function tests in RA (1) the radiologic damage assessment is described to be performed by a `̀ modi® ed Larsen score’ ’ . Since the primary source for this score is not given in the article I would like to give the following information: In 1977 Arvi Larsen and I presented for the ® rst time a radiographic method for therapeutic studies involving both hands and feet from patients with RA (2). This method is still recommended by EULAR (3, 4). For each joint and each stage standard reference ® lms are available (5). The maximal score per joint is 5. In calculating the total, all individual joint scores are summed after multiplying the wrist scores by 5. The maximal total score (Larsen ± Dale) index or `̀ Larsen score’ ’ is 200 per patient. This method has successfully been used in a large number of therapeutic studies. Larsen himself has for the last few years chosen to use a modi® ed method by dividing the total (overall) sum by 40, which is the total number of joints, from the patient. The intention is that the patient shall get a ’ ’mean score’ ’ (1) which in this way is given to each of the patient s 40 joints.