Abstract

Objective: to estimate frequency of autoantibodies in a cohort of Russian patients with systemic sclerosis (SS) and to investigate clinical associations of these autoantibodies. Subjects and methods. In 2012 to 2015, the investigation enrolled 300 patients (58 men and 242 women) who fulfilled the American College of Rheumatology/European League Against Rheumatism (ACR/EULAR) SS criteria. All patients underwent immunological examination including determination of antinuclear antibodies, anti-topoisomerase I (anti-Scl-70) antibodies, anti-centromere antibodies (ACA), anti-U1-ribonucleoprotein antibodies (anti-U1 RNP), and anti-RNP III antibodies. Results and discussion. The vast majority of patients were middle-aged females with moderate disease duration. There was a preponderance of patients with limited cutaneous form (55.3%); 37.4% patients had diffuse SS; 5.9% had overlap syndrome; and <1% had visceral and juvenile SS. The vast majority (83.8%) of patients were found to have antinuclear factor. Among the SS-associated antibodies, anti-Scl-70 were most common and were revealed in approximately one-half of the patients. ACA was present in only 44 (14.6%) patients. There was a combination of positivity for ACA and anti-Scl-70 antibodies in 3 patients with limited cutaneous SS, including one with an early form of the disease. 26 patients had anti-U1 RNP antibodies. Among them, there was a preponderance of patients with limited cutaneous SS and overlap syndrome. Anti-RNP III antibodies were found in 5.5% of cases, mainly in those with limited cutaneous SS; these were observed in one patient with diffuse SS and interstitial lung disease. No kidney injury was seen in this patient group. Conclusion. The characteristics of the Russian cohort are the preponderance of the limited cutaneous SS and the frequent detection of anti-Scl-70 antibodies in both diffuse and limited cutaneous SS; no correlation of anti-Scl-70 antibodies with rapid progression of the pathological process, with kidney disease.

Highlights

  • Цель – определение частоты выявления аутоантител в когорте российских пациентов с системной склеродермией (ССД), изучение клинических ассоциаций выявленных антител

  • Анти-РНКП III обнаруживались в 5,5% случаев, преимущественно в группе пациентов с лимитированной формой ССД; в одном случае они выявлены у больного с диффузным поражением кожи и выраженным интерстициальным поражением легких

  • There was a preponderance of patients with limited cutaneous form (55.3%); 37.4% patients had diffuse SS; 5.9% had overlap syndrome; and

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Summary

Профиль аутоантител при системной склеродермии

Старовойтова М.Н., Десинова О.В., Конева О.А., Овсянникова О.Б., Алекперов Р.Т., Черкасова М.В., Александрова Е.Н., Ананьева Л.П. Наиболее часто из ССД-ассоциированных антител (приблизительно у половины пациентов) были выявлены антиScl-70. Сочетание позитивности по АЦА и анти-Scl-70 было отмечено у трех пациентов с лимитированной ССД, в том числе у одной – с ранней формой заболевания. Анти-РНКП III обнаруживались в 5,5% случаев, преимущественно в группе пациентов с лимитированной формой ССД; в одном случае они выявлены у больного с диффузным поражением кожи и выраженным интерстициальным поражением легких. Почти у 1/3 (26,7%) пациентов с достоверным диагнозом ССД при позитивности по АНФ ни АЦА, ни анти-Scl-70 не определялись. Сочетание позитивности по АЦА и анти-Scl-70 было отмечено у трех пациентов с лимитированной ССД (одна из них – с ранней формой заболевания).

Спектр и частота обнаружения аутоантител у пациентов с ССД
Нет данных p
Findings
AЦА диффузная лимитированная
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