Subcutaneous immunoglobulin 16.5 % given by the rapid push method in patients with inborn errors of immunity: a prospective multicenter study of quality of life

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Abstract

BACKGROUND: Replacement therapy with immunoglobulin preparations is the basic therapy for patients with impaired antibody formation in the context of inborn errors of immunity. The introduction of replacement therapy with subcutaneous immunoglobulin (SCIG) into clinical immunological practice has allowed patients with inborn errors of immunity, regardless of age, to carry out therapy independently at home. This circumstance has played a leading role in improving the quality of life of patients.

AIM: To assess the quality of life of patients and adherence to therapy during the transition from intravenous immunoglobulin replacement therapy to SCIG therapy.

MATERIALS AND METHODS: A total of 233 patients of different ages with inborn errors of immunity were enrolled in the study. All patients were switched from intravenous immunoglobulin to SCIG 16.5 % therapy. SCIG was administered every 7–10 days for at least 12 months via the rapid push method. Assessment of quality of life and adherence to SCIG therapy was carried out 6 and 12 months after the start of therapy using a questionnaire.

RESULTS: Based on the results of the assessment of the quality of life of patients using a questionnaire, patients of all age groups under 18 years and their parents demonstrated improvement in the social sphere, academic performance at school, and physical activity against the background of switching to SCIG. According to the survey, 84 % of patients did not experience technical difficulties and 83 % wanted to continue the SCIG infusion.

CONCLUSION: Performing manual infusion of SCIG at home has a positive effect on the quality of life of patients and helps improve adherence to therapy.

About the authors

Asmik Ya. Avedova

Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology

Author for correspondence.
Email: asya.avedova95@mail.ru
ORCID iD: 0000-0002-9399-6534
Russian Federation, Moscow

Elena V. Deripapa

Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology

Email: deripapa@mail.ru
ORCID iD: 0000-0002-9083-4783

Cand. Sci. (Medicine)

Russian Federation, Moscow

Yulia A. Rodina

Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology

Email: rodina.julija@rambler.ru
ORCID iD: 0000-0001-9857-4456

MD, Cand. Sci. (Medicine)

Russian Federation, Moscow

Anna A. Mukhina

Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology

Email: ffmanya@yandex.ru
ORCID iD: 0000-0002-3305-1694
SPIN-code: 3925-3328

MD, Cand. Sci. (Medicine)

Russian Federation, Moscow

Elena A. Latysheva

National Research Center Institute of Immunology

Email: ealat@mail.ru
ORCID iD: 0000-0002-1606-205X
SPIN-code: 2063-7973

MD, Dr. Sci. (Medicine)

Russian Federation, Moscow

Daria V. Yukhacheva

Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology

Email: yukhachevadv@gmail.com
ORCID iD: 0000-0001-9078-8206
SPIN-code: 3052-1661

MD, Cand. Sci. (Medicine)

Russian Federation, Moscow

Vasily I. Burlakov

Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology

Email: sarovas@yandex.ru
ORCID iD: 0000-0003-1267-9957
Russian Federation, Moscow

Nellie Yu. Kan

Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology

Email: nkan123@yandex.ru
ORCID iD: 0000-0003-3564-6496
SPIN-code: 2163-8349

Cand. Sci. (Medicine)

Russian Federation, Moscow

Daria V. Bogdanova

Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology

Email: miss.daryabogdanova@yandex.ru
ORCID iD: 0000-0003-2897-5208
SPIN-code: 4123-9308
Russian Federation, Moscow

Anna A. Moiseeva

Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology

Email: lira_belaqua@mail.ru
ORCID iD: 0000-0002-6134-3811
SPIN-code: 2482-8500
Russian Federation, Moscow

Natalia B. Kuzmenko

Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology

Email: plunge@list.ru
ORCID iD: 0000-0002-1669-8621
SPIN-code: 6281-2729

MD, Cand. Sci. (Medicine)

Russian Federation, Moscow

Zoya A. Nesterenko

Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology

Email: nesterenkozoya7@gmail.com
ORCID iD: 0000-0003-4427-054X
SPIN-code: 5957-1752
Russian Federation, Moscow

Ekaterina A. Deordieva

Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology

Email: deor2005@yandex.ru
ORCID iD: 0000-0002-8208-2075

MD, Cand. Sci. (Medicine)

Russian Federation, Moscow

Anna L. Ogneva

Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology

Email: ania.tulip@mail.ru
ORCID iD: 0000-0002-8697-4206

MD, Cand. Sci. (Medicine)

Russian Federation, Moscow

Viktoria O. Bludova

Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology

Email: voevodina_1996@list.ru
ORCID iD: 0000-0003-0960-3089
Russian Federation, Moscow

Anna A. Roppelt

Clinical City Hospital No. 52

Email: roppelt_anna@mail.ru
ORCID iD: 0000-0001-5132-1267
SPIN-code: 7249-4423

Moscow Center of Allergy and Immunology, MD, Cand. Sci. (Medicine)

Russian Federation, Moscow

Daria S. Fomina

Clinical City Hospital No. 52

Email: daria_fomina@mail.ru
ORCID iD: 0000-0002-5083-6637
SPIN-code: 3023-4538

Moscow Center of Allergy and Immunology, MD, Cand. Sci. (Medicine), Assistant Professor

Russian Federation, Moscow

Natalia V. Zinovieva

Children’s City Clinical Hospital No. 9 named after G.N. Speransky

Email: nvzinov@gmail.com
ORCID iD: 0000-0002-6926-2055

MD, Cand. Sci. (Medicine)

Russian Federation, Moscow

Yulia N. Sevostyanova

Children’s City Clinical Hospital No. 9 named after G.N. Speransky

Email: mayuliya@yandex.ru
ORCID iD: 0009-0004-4328-7803
Russian Federation, Moscow

Linara R. Kalmeteva

Republican Children’s Clinical Hospital

Email: l_kalmetieva@mail.ru
ORCID iD: 0000-0003-1866-0640
Russian Federation, Ufa

Dilara D. Prolygina

Republican Children’s Clinical Hospital

Email: drkb.gem.immun@mail.ru
ORCID iD: 0000-0002-5797-4370
Russian Federation, Ufa

Liudmila Yu. Barycheva

Stavropol State Medical University

Email: for_ludmila@inbox.ru
ORCID iD: 0000-0002-4069-0566
SPIN-code: 8946-3393

MD, Dr. Sci. (Medicine)

Russian Federation, Stavropol

Olga S. Selezneva

Regional Children’s Clinical Hospital

Email: selezneva-doc@mail.ru
ORCID iD: 0000-0003-1907-4168
Russian Federation, Rostov-on-Don

Natalia V. Shakhova

Altai State Medical University

Email: Natalia.shakhova@mail.ru
ORCID iD: 0000-0002-7143-8259
SPIN-code: 3290-8520

MD, Dr. Sci. (Medicine)

Russian Federation, Barnaul

Olga M. Laba

Regional Children’s Clinical Hospital

Email: labaom@mail.ru
ORCID iD: 0000-0001-5784-1263
Russian Federation, Yaroslavl

Elena V. Vlasova

Regional Children’s Clinical Hospital

Email: evvlasova@mail.ru
ORCID iD: 0000-0002-6678-5360
Russian Federation, Yekaterinburg

Alla V. Gorenkova

Arkhangelsk Regional Children’s Clinical Hospital named after P.G. Vyzhletsova

Email: domgav63@rambler.ru
ORCID iD: 0009-0008-2514-1594
Russian Federation, Arkhangelsk

Elena V. Timofeeva

Nizhny Novgorod Regional Children’s Clinical Hospital

Email: elenatim-nn@mail.ru
ORCID iD: 0000-0003-3908-1947
Russian Federation, Nizhny Novgorod

Olga V. Trusova

First Saint Petersburg State Medical University named after I.P. Pavlov

Email: o-tru@mail.ru
ORCID iD: 0000-0002-0854-1536
SPIN-code: 3938-4377

MD, Dr. Sci. (Medicine)

Russian Federation, Saint Petersburg

Marina N. Guseva

First Saint Petersburg State Medical University named after I.P. Pavlov, Saint Petersburg

Email: gusevamarina@mail.ru
ORCID iD: 0000-0002-3997-3390
SPIN-code: 3535-9896
Russian Federation, Saint Petersburg

Natalia B. Yudina

Voronezh Regional Children’s Clinical Hospital No. 1

Email: n.yudina@list.ru
ORCID iD: 0009-0001-7498-0836
SPIN-code: 3908-7166
Russian Federation, Voronezh

Evgenia A. Grevtseva

Voronezh Regional Children’s Clinical Hospital No. 1

Email: grievtsieva85@mail.ru
ORCID iD: 0009-0004-9437-4851
SPIN-code: 3717-6562
Russian Federation, Voronezh

Asya Kh. Ibisheva

Republican Children’s Clinical Hospital named after E.P. Glinka

Email: ibisheva18@mail.ru
ORCID iD: 0000-0003-4732-4515
Russian Federation, Grozny

Zema V. Bambaeva

Regional Children’s Clinical Hospital

Email: z.bambaeva@yandex.ru
ORCID iD: 0000-0002-7541-2793
Russian Federation, Ulan-Ude

Dina V. Mashkovskaya

Republican Children’s Clinical Hospital

Email: mashkovskaya@child-hosp.ru
ORCID iD: 0009-0000-4995-0427
Russian Federation, Simferopol

Svetlana N. Isakova

Research Institute of Fundamental and Clinical Immunology

Email: hell_paradise@mail.ru
ORCID iD: 0009-0007-8368-5250
Russian Federation, Novosibirsk

Almazia R. Shakirova

Children’s Republican Clinical Hospital

Email: almaziaysh@mail.ru
ORCID iD: 0000-0001-9975-3632
SPIN-code: 2666-7175
Russian Federation, Kazan

Ekaterina Yu. Selina

Children’s Republican Clinical Hospital

Email: selina-katya1998@yandex.ru
ORCID iD: 0009-0003-6545-1435
Russian Federation, Kazan

Tatiana V. Shilova

Chelyabinsk Regional Children’s Clinical Hospital

Email: tanya920477@mail.ru
ORCID iD: 0000-0001-9826-9654
SPIN-code: 2028-9392

MD, Cand. Sci. (Medicine)

Russian Federation, Chelyabinsk

Elena S. Zubova

Children’s Regional Hospital

Email: zubovaes@mail.ru
ORCID iD: 0000-0002-9755-8929
SPIN-code: 4140-9615
Russian Federation, Perm

Imani U. Khabaeva

Regional Children’s Clinical Hospital named after N.N. Silishcheva

Email: el25kv@mail.ru
ORCID iD: 0009-0008-7161-4540
Russian Federation, Astrakhan

Ekaterina V. Kitova

Republican Children’s Clinical Hospital

Email: Ek.Burova@yundex.ru
ORCID iD: 0009-0001-6004-2558
Russian Federation, Izhevsk

Anastasia G. Mandzhieva

Regional Clinical Hospital No. 1

Email: mandzhieva81@bk.ru
ORCID iD: 0009-0002-7068-2601
Russian Federation, Tyumen

Svetlana Yu. Starikova

City Children’s Clinical Hospital No. 2 named after V.P. Bisyarina

Email: Starikowa17@mail.ru
ORCID iD: 0000-0002-4321-7301
Russian Federation, Omsk

Tatiana B. Pavlova

Irkutsk State Regional Children’s Clinical Hospital

Email: tabopav@rambler.ru
ORCID iD: 0000-0003-3403-4447
SPIN-code: 4893-7250

MD, Cand. Sci. (Medicine)

Russian Federation, Irkutsk

Elvira N. Tyulyakova

Kaluga Regional Children’s Clinical Hospital

Email: tabopav@rambler.ru
ORCID iD: 0009-0005-1642-4398
Russian Federation, Kaluga

Nikolai S. Grachev

Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology

Email: nick-grachev@yandex.ru
ORCID iD: 0000-0002-4451-3233
SPIN-code: 2836-2349

MD, Dr. Sci. (Medicine), Professor

Russian Federation, Moscow

Anna Yu. Shcherbina

Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology

Email: shcher26@hotmail.com
ORCID iD: 0000-0002-3113-4939
SPIN-code: 6759-0031

MD, Dr. Sci. (Medicine), Professor

Russian Federation, Moscow

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Supplementary files

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1. JATS XML
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2. Fig. 1. Results of a survey of patients under 18 years of age using the PedsQL 4.0 questionnaires. Note. ВВИГ – intravenous immunoglobulin; ПКИГ – subcutaneous immunoglobulin.

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3. Fig. 2. Results of a survey of parents of patients under 18 years of age using the PedsQL 4.0 questionnaires. Note. ВВИГ – intravenous immunoglobulin; ПКИГ – subcutaneous immunoglobulin.

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4. Fig. 3. Results of a survey of patients over 18 years of age using SF-36 questionnaires. p >0.05 for all compared parameters. Note. ВВИГ – intravenous immunoglobulin; ПКИГ – subcutaneous immunoglobulin.

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5. Fig. 4. Results of the questionnaire: a – assessment of patients’ adherence to subcutaneous immunoglobulin therapy; b – assessment of pain syndrome

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