rspНаучно-практическая ревматологияRheumatology Science and Practice1995-44841995-4492IMA-PRESS, LLC10.14412/1995-4484-2019-539-544rsp-2788Research ArticleОРИГИНАЛЬНЫЕ ИССЛЕДОВАНИЯORIGINAL RESEARCHКлинико-лабораторная характеристика больных системной склеродермией, позитивных по антителам к рибонуклеопротеинуClinical and laboratory characteristics of patients with systemic sclerosis positive for anti-ribonucleoprotein antibodiesШаяхметоваР. У.ShayakhmetovaR. U.

Шаяхметова Рушана Ульфатовна.

115522, Москва, Каширское шоссе, 34А.

Rushana Shayakhmetova.

34A, Kashirskoe Shosse, Moscow 115522.

rushick@rambler.ruАнаньеваЛ. П.AnanyevaL. P.

115522, Москва, Каширское шоссе, 34А.

34A, Kashirskoe Shosse, Moscow 115522.

КоневаО. А.KonevaO. A.

115522, Москва, Каширское шоссе, 34А.

34A, Kashirskoe Shosse, Moscow 115522.

СтаровойтоваМ. Н.StarovoitovaM. N.

115522, Москва, Каширское шоссе, 34А.

34A, Kashirskoe Shosse, Moscow 115522.

ДесиноваО. В.DesinovaO. V.

115522, Москва, Каширское шоссе, 34А.

34A, Kashirskoe Shosse, Moscow 115522.

ОвсянниковаО. Б.OvsyannikovaO. B.

115522, Москва, Каширское шоссе, 34А.

34A, Kashirskoe Shosse, Moscow 115522.

ГарзановаЛ. А.GarzanovaL. A.

115522, Москва, Каширское шоссе, 34А.

34A, Kashirskoe Shosse, Moscow 115522.

Научно-исследовательский институт ревматологии им. В.А. НасоновойРоссияV.A. Nasonova Research Institute of RheumatologyRussian Federation201916112019575539544Copyright © Шаяхметова Р.У., Ананьева Л.П., Конева О.А., Старовойтова М.Н., Десинова О.В., Овсянникова О.Б., Гарзанова Л.А., 20192019Шаяхметова Р.У., Ананьева Л.П., Конева О.А., Старовойтова М.Н., Десинова О.В., Овсянникова О.Б., Гарзанова Л.А.Shayakhmetova R.U., Ananyeva L.P., Koneva O.A., Starovoitova M.N., Desinova O.V., Ovsyannikova O.B., Garzanova L.A.Данная работа распространяется под лицензией Creative Commons Attribution 4.0.This work is licensed under a Creative Commons Attribution 4.0 License.https://rsp.mediar-press.net/rsp/article/view/2788

Среди пациентов, соответствующих критериям системной склеродермии (ССД),  имеется подгруппа больных, у которых не выявляются специфичные для ССД антиядерные антитела, но имеются антитела к рибонуклеопротеину (анти-U1РНП). Клиническое значение  этого вида антиядерных антител при ССД не ясно. Наличие анти-U1РНП представляет  большой интерес, так как они не только присутствуют при других ревматических заболеваниях, но и рассматриваются как маркер смешанного заболевания соединительной ткани.

Цель исследования – выявить частоту анти-U1РНП у больных ССД и дать клиническую и лабораторную  характеристику  больных, позитивных по этим антителам.

Материал и методы

Материал и методы. Обследовано  330 больных, соответствующих критериям ССД (ACR/EULAR 2013), наблюдавшихся в ФГБНУ  НИИР им. В.А. Насоновой в период с 2012 по 2017 г. Анти-U1РНП определялись иммуноферментным методом (референсные значения 0–25 Ед/мл).

Результаты и обсуждение

Результаты и обсуждение. Анти-U1РНП обнаружены  у 65 (19,7%) больных ССД (85% были высокопозитивными, 15% – низкопозитивными). Группа включала 8 мужчин и 57 женщин, средний возраст – 46±14 лет. Давность болезни составила 11±7,9 года. Поражение кожи было минимальным, 59 (91%) больных имели лимитированную форму болезни с наличием  отека кистей (склередема) в 40% и склеродактилии – в 60% случаев. Феномен Рейно присутствовал у всех больных. В половине  случаев наблюдались  периферические ишемические нарушения:  дигитальные  рубчики и/или язвочки  (43%), а также некрозы  и язвы других локализаций, которые встречались  относительно редко (8%). Интерстициальное поражение  легких (ИПЛ)  было обнаружено  в 63% случаев. Повышение систолического давления в легочной артерии (СДЛА) ≥40 мм рт. ст. по данным эхокардиографии  (ЭхоКГ) выявлено  в 26% случаев и преимущественно ассоциировалось с наличием  ИПЛ,  у трех больных была подтверждена легочная артериальная гипертензия. Поражение пищевода было обнаружено у 61% пациентов. У трети больных имелись признаки склеродермической кардиопатии. Особенностью группы являлось частое поражение  опорно-двигательного аппарата: суставов (артралгии  и/или синовиты  в 65% случаев) и мышц (миопатия слабой или умеренной  степени выраженности у 43% больных). Нередко повышались СОЭ и уровень С-реактивного белка. Частым было сочетание  с синдромом  Шегрена  (у трети больных). Не было зафиксировано ни одного случая склеродермической почки, средние показатели  функции почек были в пределах нормальных величин, однако у 17% больных скорость  клубочковой фильтрации была ниже 80 мл/мин/м2. Все больные были позитивны по антинуклеарному фактору (Hep-2), кроме того, выявлялись  ревматоидный  фактор (22%), антитела к Ro/SS-A  (41%), La/SS-B (18%), двуспиральной ДНК (42%), Scl70 (7%), антицентромерным антителам (9%); 39 из 55 (71%) высокопозитивных по анти-U1РНП больных соответствовали критериям смешанного заболевания соединительной ткани, предложенным R. Kasukawa и соавт. (1987). Заключение. Проведенное исследование позволяет  обсуждать наличие  особого фенотипа ССД, отличающегося своеобразными клиническими проявлениями на фоне гиперпродукции анти-U1РНП.

Among the patients fulfilling the criteria for systemic sclerosis (SS), there is a subgroup without SS-specific antinuclear antibodies, but positive for anti-ribonucleoprotein (anti-U1 RNP)  antibodies. The clinical significance of this type of antinuclear  antibodies in SS is not clear. The presence of anti-U1  RNP antibodies is of great interest, since they are not only present in other rheumatic diseases, but are also considered as a marker for mixed connective tissue disease. Objective: to reveal the frequency of anti-U1RNP antibodies in patients with SS and to provide the clinical and laboratory characteristics of patients positive for these antibodies.

Subjects and methods

Subjects and methods. 330 patients who fulfilled the 2013 ACR/EULAR criteria for SS and had been followed at the V.A. Nasonova Research Institute of Rheumatology from 2012 to 2017 were included. Anti-U1 RNP were determined by enzyme immunoassay (reference values: 0–25 U/ml).

Results and discussion

Results and discussion. Anti-U1RNP were detected in 65 (19.7%) patients with SS (85% of patients were highly positive; 15% were low-positive). The group included 8 men and 57 women; their mean age was 46±14 years. The disease duration was 11±7.9 years. Skin lesions were minimal; 59 (91%) patients had a limited form of the disease with swelling in the hands (scleredema)  in 40% of cases and sclerodactyly in 60%. Raynaud's phenomenon was present in all the patients. One-half of the cases were observed to have peripheral ischemic disorders: digital scars and/or sores (43%), as well as necroses and ulcers of other sites, which were relatively rare (8%). Interstitial lung disease (ILD)  was identified in 63% of cases. Elevated pulmonary artery systolic pressure (PASP) ≥40 mm Hg, as shown by echocardiography, was detected in 26% of cases and was associated mainly with the presence of ILD; pulmonary arterial hypertension  was  confirmed in three patients. Esophageal lesions were found in 61% of patients. One-third of patients had signs of scleroderma cardiopathy.  The feature of the group was the common  involvement of the locomotor system: joints with arthralgia and/or synovitis in 65% and muscles with mild and moderate myopathy in 43%. Erythrocyte sedimentation rate (ESR) and C-reactive protein levels were frequently elevated. The concurrence with Sjö gren's syndrome was common  (in one-third of patients). None case of scleroderma renal disease was recorded; the mean values of kidney function were within the normal range; however, the glomerular filtration rate was lower than 80 ml/min/m2 in 17% of patients. All the patients were positive for antinuclear  factor (HEp-2); in addition, there was rheumatoid  factor (22%), antibodies against Ro/SS-A  (41%), La/SS-B (18%), double-stranded DNA (42%), Scl70 (7%), and anticentromere antibodies (9%); 39 out of the 55 (71%) patients who were highly anti-U1  RNP-positive fulfilled the mixed connective tissue disease criteria proposed by R. Kasukawa et al. (1987).

Conclusion

Conclusion. The investigation allows one to discuss of the presence of a special phenotype  of SS, which is characterized  by peculiar clinical manifestations in the presence of anti-U1  RNP overproduction.

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The authors declare that there are no conflicts of interest present.