Satoshi Fujikawa

Systemic sclerosis in children: A national retrospective survey in Japan

A retrospective questionnaire survey of pediatric departments, for childhood collagen disease from 1985 to 1994 was used to clarify the clinical features of scleroderma in Japan. In the primary survey, 0.9% of the children with a rheumatic condition had scleroderma. Answers to this questionnaire were received on 18 (localized 9; systemic 9) patients from 15 institutions. In order to examine systemic sclerosis (SSc), seven cases of SSc in Japanese articles during the same period as the questionnaire were added to these answers and compared to the Japanese epidemic study investigated by Fukuyama in 1974. There were 16 children, seven boys and nine girls, with SSc during the 10‐year period in Japan. The mean age of onset of symptoms was 8.0 ± 2.8 years and the age at diagnosis was 10.1 ± 3.0 years. Eighty percent of children had Raynauds phenomenon at the onset of SSc, and skin and musculoskeletal involvement was highly recognized during the course of the disease. Atrophy of the frenulum linguae and lung fibrosis were commonly seen in SSc. In serological studies, 80% of children have antinuclear antibodies and approximately 50% of patients have anti‐Scl‐70 (topoisomelase I) antibodies at the onset and during the course of childhood SSc. The prognosis is poor, as remission occurred in only one child. The clinical symptoms and examination of serological autoimmune antibodies were supportive of an early diagnosis of SSc. When compared to the previous national survey of children with SSc, the present results showed that the male‐to‐female ratio was reduced, the age at onset was low, the positive incidence of serological autoimmune antibodies elevated, and the usage of vasodilators and nonsteroid anti‐inflammatory drugs (NSAID) increased, with corticosteroids decreased. But, the positive percentage of clinical symptoms were not changed in both studies. For a complete retrospective nationwide epidemic survey carried out on children with scleroderma, especially SSc, it is important to include dermatology departments.

Clinical significance of anti-streptococcal esterase (ASE) determination in rheumatic fever and other streptococcal diseases.

Streptococcal esterase is known as one of the extracellular products of Group A streptococci. In this paper, an antibody of streptococcal esterase titers in patients with rheumatic fever, acute glomerulonephritis and other streptococcal infections were determined. In cases with acute rheumatic fever and acute glomerulonephritis, ASE titers showed almost the same changes as ASO, ASK and ADN-B. The characteristics of the ASE determination, however, is that small changes in the titers could be detected because the final definition of this titer was determined by photometer.

Upper limit of normal anti‐strepto‐polysaccharide (ASP) level and significance of ASP determination

C-polysaccharide, one of the cellular components of streptococcal cell wall, provides group-specific antibodies and has been reported to have cross-immunity with glycoproteins of human cardiac tissue, especially of cardiac valve and aorta. Therefore, the measurement of the ASP (anti-strepto-polysaccharide) titer may be considered to give the direct proof of group A streptococcal infection and be useful in investigating the pathogenesis of rheumatic heart disease. Recently Kimura and his co-workers, Department of Bacterioimmunology of Nippon Medical College, have provided a new passive hemagglutination technique for the determination of ASP level, using formalized sheep red cells. Using this method, we measured ASP titer in the sera from the patients with rheumatic fever, rheumatic valvular heart disease, acute glomerulonephritis, scarlet fever, vascular purpura and MCLS and compared them with other antibody titers against extracellular antigens including ASO, ASK and ADN-B. The upper limit of normal ASP titers in 184 healthy school children in the present study was 1:32 by this method and those of 1:64 or more were considered abnormal or “positive”. In rheumatic fever with active carditis, A S 0 and ASK showed high levels in all 3 cases, but ASP was at a high level in one of 3 cases; this was not coincident with previous reports. But in an inactive phase of rheumatic fever, the high level of ASP titer was observed for a long time in comparison with antibodies against extracellular antigens, and especially among the cases of rheumatic heart disease which passed 2 years or more after the onset, 38% of the patients took the high level of ASP titer despite of the low levels of ASO, ASK and ADN-B titers. In acute glomerulonephritis, the titer of ASP was high in 82% of the patients in the acute phase and 50% of the patients continuously had the high level of ASP titer in the inactive phase. In scarlet fever, 36% of the patients showed high ASP titers. In vascular purpura, many cases took high ASP titers in comparison with antibodies against extracellular antigens and many patients of this disease may be considered to be of streptococcal infection. In MCLSt a high ASP level was observed only in 10% of the cases, and antibodies against extracellular an tigens was in a low level in all cases. The correlation coefficient between ASP and antibodies against extracellular antigens was r=-0.04 to 0.39, and therefore it can be considered that ASP is independent of other antibodies. The correlation coefficient between A S 0 and ASK was r=0.44 in rheumatic fever and r=0.45 in acute glomerulonephritis, and r=0.65 between A S 0 and ADN-B in rheumatic fever.