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Clinical Characteristics of Juvenile Systemic Sclerosis in Japanese To the Editor: The severity of systemic sclerosis (SSc) is highly variable. Since disease onset before age 16 years is quite rare in patients with SSc1, the clinical characteristics of juvenile SSc have not been fully clarified to date. We determined retrospectively the clinical features of Japanese SSc patients younger than age 16 years in comparison with adult Japanese patients with SSc. All 184 SSc patients referred to our hospital during a period of 11 years (1992–2003) were eligible for the study. All patients fulfilled the criteria for SSc proposed by the American College of Rheumatology2. Patients with localized scleroderma were excluded. The cases were grouped into diffuse cutaneous SSc (dSSc) or limited cutaneous SSc (lSSc) according to the classification system proposed by LeRoy, et al3. Complete histories, physical examinations, and laboratory tests including high resolution computed tomography (HRCT) of the chest were conducted for all patients at the first visit and during followup. We evaluated general health by blood sampling and urinalysis at every third month, and cardiopulmonary condition by chest radiograph semiannually, with more examinations if necessary. Organ system involvement was defined as described4,5. Among 184 Japanese SSc patients, 5 were classified as having juvenile SSc. Clinical profiles of these patients are summarized in Table 1. Concerning the individual profiles of these patients, a male patient (Patient 1) first presented at the age of 6 months, with skin sclerosis on the foot that was noted by his mother. At the first visit, at age 2 years, 11 months, he showed skin sclerosis of the whole body, with 24 points on the Modified Rodnan total skin thickness score (TSS). Assessment was the same as for an adult assessment and the TSS obtained was the average score of 2 physicians. Skin biopsy of the foot revealed an increase in thickened collagen fibers in the dermis. He had no internal involvement other than esophageal hypomotility. His skin sclerosis gradually improved to 15 TSS points, 9 years after starting treatment with prednisolone (PSL). A 7-year-old girl (Patient 2) with disease onset at age 5 years was admitted to our hospital because of skin sclerosis on the extremities, with 27 TSS points. She had no internal involvement. One year after starting treatment with PSL, skin sclerosis improved to 17 TSS points. A 13-year-old boy (Patient 3) who developed skin sclerosis on the hands at age 8 years was admitted to hospital with whole-body skin sclerosis, with 27 TSS points. Although treatment with PSL had been started at age 11 years at another hospital, skin sclerosis had not improved. HRCT showed pulmonary fibrosis that had developed slowly. In a girl (Patient 4) who presented with Raynaud's phenomenon at age 9 years, skin sclerosis had rapidly developed on the upper arms within 6 months, resulting in 19 TSS points. Treatment with PSL was started and skin sclerosis improved to 15 points after 5.5 years' followup. A boy (Patient 5) experienced Raynaud's phenomenon at age 13 years; skin sclerosis developed on the upper arms, but not the trunk, at age 17 years (TSS 11 points). He was treated with PSL, which appeared to be effective for his skin sclerosis (TSS 8 points after 8 years' PSL treatment). The clinical and laboratory features of juvenile SSc were compared with those of adult SSc. The prevalence of dSSc in patients with juvenile SSc was significantly higher than in patients with adult SSc (p < 0.05; Table 2. ). Furthermore, TSS of those with juvenile SSc were significantly higher than those of adult SSc (p < 0.05). There was no difference in the prevalence of organ involvement between juvenile and adult SSc patients. Juvenile SSc exhibited an increased frequency of antitopoisomerase I (topo I) antibody positivity compared to adult SSc patients (100% vs 29%; p < 0.05). When profiles of juvenile SSc patients were compared with adult SSc patients positive for anti-topo I antibodies, pulmonary fibrosis was present in only 20% of juvenile SSc patients, significantly lower than the frequency (88%) in adult patients (p < 0.05; Table 2. ). Studies in other countries have reported that juvenile SSc patients had more severe skin sclerosis (Table 2. )6,7. The frequency of dSSc (80–100%), Raynaud's phenomenon (60–100%), and contracture of phalanges was high. These observations were consistent with the result of our study. Previous studies reported low prevalence of scleroderma renal crisis8: scleroderma renal crisis was not detected in any patient in our study. Other studies have shown that anti-topo I antibodies are frequently detected (27–50%) in juvenile SSc, whereas anticentromere antibodies are rarely detected (Table 2. ). Although a previous study reported relatively higher mortality in patients with juvenile SSc9, a recent study of 135 juvenile SSc patients showed a favorable outcome11. In our study, all juvenile SSc patients survived for the followup period of 9 years. Although racial differences may affect the mortality rate of juvenile SSc, larger longitudinal studies will be needed to confirm mortality rates. Our study suggests that Japanese patients with juvenile SSc have more severe skin sclerosis than adults with SSc, although the frequency of internal organ involvement and the mortality rate is lower than with adult SSc. MAKI MURATA, MD, Department of Dermatology, Kanazawa University Graduate School of Medical Science; SHINICHI SATO, MD, PhD, Department of Dermatology, Kanazawa University Graduate School of Medical Science and Department of Dermatology, Nagasaki University Graduate School of Biomedical Sciences; KAZUHIRO KOMURA, MD, PhD; FUMIAKI SHIRASAKI, MD, PhD; MINORU HASEGAWA, MD, PhD; KAZUHIKO TAKEHARA, MD, PhD, Department of Dermatology, Kanazawa University Graduate School of Medical Science, Nagasaki, Japan. Address reprint requests to Dr. S. Sato, Department of Dermatology, Nagasaki University Graduate School of Biomedical Sciences, 1-7-1 Sakamoto, Nagasaki 852-8501, Japan. E-mail: s-sato@net.nagasaki-u.ac.jp REFERENCES 2. Preliminary criteria for the classification of systemic sclerosis (scleroderma). Subcommittee for Scleroderma Criteria of the American Rheumatism Association Diagnostic and Therapeutic Criteria Committee. Arthritis Rheum 1980;23:581-90. 3. LeRoy EC, Black C, Fleischmajer R, et al. Scleroderma (systemic sclerosis): classification, subsets and pathogenesis. J Rheumatol 1988;15:202-5. 4. Steen VD, Powell DL, Medsger TA Jr. Clinical correlations and prognosis based on serum autoantibodies in patients with systemic sclerosis. Arthritis Rheum 1988;31:196-203. 5. Komura K, Sato S, Hasegawa M, Fujimoto M, Takehara K. Elevated circulating CD40L concentrations in patients with systemic sclerosis. J Rheumatol 2004;31:514-9. 6. Kass H, Hanson V, Patrick J. Scleroderma in childhood. J Pediatr 1966;68:243-56. 7. Suarez-Almazor ME, Catoggio LJ, Maldonado-Cocco JA, Cuttica R, Garcia-Morteo O. Juvenile progressive systemic sclerosis: clinical and serologic findings. Arthritis Rheum 1985;28:699-702. 8. Martinez-Cordero E, Fonseca MC, Aquilar Leon DE, Padilla A. Juvenile systemic sclerosis. J Rheumatol 1993;20:405-7. 9. Kornreich HK, King KK, Bernstein BH, Singsen BH, Hanson V. Scleroderma in childhood. Arthritis Rheum 1977;20:343-50. 10. Vesely R, Vargova V, Ravelli A, et al. Serum level of KL-6 as a marker of interstitial lung disease in patients with juvenile systemic sclerosis. J Rheumatol 2004;31:795-800. 11. Foeldvari I, Zhavania M, Birdi N, et al. Favourable outcome in 135 children with juvenile systemic sclerosis: results of a multi-national survey. Rheumatology Oxford 2000;39:556-9. 12. Lababidi HM, Nasr FW, Khatib Z. Juvenile progressive systemic sclerosis: report of five cases. J Rheumatol 1991;18:885-8. 13. Cassidy JT, Sullivan DB, Dabich L, Petty RE. Scleroderma in children. Arthritis Rheum 1977;20:351-4.
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