No abstract

Oesophageal motility and lower oesophageal sphincter (LOS) competence were investigated in 13 patients with progressive systemic sclerosis (PSS) and in 16 patients with localized scleroderma (LS) by means of oesophageal manometry and 24-h pH monitoring of the distal oesophagus. Results were compared with those of a control group consisting of asymptomatic volunteers. Marked abnormalities in oesophageal motility and in acid exposure in the distal oesophagus were observed in PSS patients only. The mean resting pressure of the LOS was 10.1 +/- 1.5 mmHg in PSS, 21.4 +/- 1.1 mmHg in LS, and 23.8 +/- 2.0 mmHg in asymptomatic controls. Overall sphincter length was 24.1 +/- 3.4 mm in PSS, 31.1 +/- 1.6 mm in LS, and 39.0 +/- 2.0 mm in the control group. Spincter abdominal length was 12.1 +/- 2 mm, 15.4 +/- 1 mm, and 25.0 +/- 1 mm, respectively. The amplitude and duration of oesophageal waves were markedly reduced at 5, 10, and 15 cm above the LOS in PSS patients, with only the upper part of their gullet being spared. An abnormal acid exposure in the distal oesophagus was observed in 84.6% of PSS patients, whereas only 18.2% (2 of 11) of pH-tested LS patients had an abnormal 24-h pH test. These data show that a marked oesophageal involvement is present only in the systemic form of scleroderma. Oesophageal tests may be useful for a circumstantial diagnosis whenever the diagnosis of PSS is uncertain; however, their use does not seem to be justified as routine in patients with LS.

Clinical, radiological and scintigraphic studies and HLA type assessment were performed in 38 subjects, constituting all the first-degree members of three generations of the families of six patients affected with ankylosing spondylitis (AS). The families included both parents, all siblings and all children of the probands. Definite AS was found in three men and possible AS in another. In another man and in a woman, a diagnosis of asymptomatic bilateral sacroiliitis was made. These six subjects indicate a family prevalence of AS reaching 15.8%. HLA B27 was present in 20 individuals (52.6%), including those with definite and possible AS and the case with asymptomatic sacroiliitis. The woman with asymptomatic sacroiliitis lacked HLA B27 antigen. Our study confirms the familial occurrence of AS, but it shows the occurrence to be lower than that previously reported.

To investigate the value of synovial fluid analysis in predicting the articular evolution of juvenile chronic arthritis, synovial fluid from 29 patients with oligoarticular onset juvenile chronic arthritis were examined prospectively. The patients were subsequently classified after a three year period of observation as having polyarticular (10 patients

Cardiac valvular involvement in patients with rheumatoid arthritis is rare and seldom so severe as to require surgery. The present report is concerned with a case of rheumatoid aortic incompetence successfully treated by prosthetic valve replacement. As suggested by previous similar reports, surgery is the most effective therapy for clinically significant rheumatoid endocarditis.