Clinical case of successful treatment of granulomatosis with polyangiitis

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Abstract

Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis is a systemic autoimmune disease characterized by the development of necrotizing inflammation in small blood vessels with damage to various organs and systems. Glucocorticosteroids (GCS) in combination with rituximab or cyclophosphamide are recommended as a pathogenetic therapy for ANCA vasculitis and help restore kidney function and reduce mortality. However, the use of these drugs is associated with certain side effects, primarily this concerns caution with respect to GCS. In this regard, there is a constant search for drugs that could ensure both the achievement of clinical remission of ANCA vasculitis (as the main goal of therapy) and a reduction in the dose, and, ideally, the cancellation of GCS. Based on the data obtained on the role of the alternative complement pathway in the pathogenesis of ANCA vasculitis, avacopan, C5a receptor inhibitor, was proposed for its treatment. In a clinical trial involving patients with ANCA-associated vasculitis, avacopan was non-inferior in achieving remission at week 26 and superior to the GCS therapy group in achieving sustained remission at week 52. All patients in the study also received cyclophosphamide or rituximab. Avacopan is recommended by KDIGO 2024 for inclusion in the induction phase of therapy to reduce the dose of GCS in patients with ANCA glomerulonephritis.

Description of the clinical case. The article presents a case of a patient with granulomatosis with polyangiitis (GPA), who was diagnosed at an advanced stage of the disease, with damage to vital organs and an extremely high risk of death. The severity of the patient's condition required mechanical ventilation with subsequent initiation of venovenous extracorporeal membrane oxygenation (ECMO), as well as renal replacement therapy. Therapy was initiated with a series of plasma exchange sessions, pulse therapy, followed by a transition to oral GCS. Taking into account the severity of glomerulonephritis and infectious complications, the decision of the council was to initiate therapy with avacopan, a drug unregistered in the Russian Federation (in combination with cyclophosphamide and rituximab).

Results. After 8 months from the start of pathogenetic therapy, the patient, who was constantly taking a minimum dose of GCS and avacopan, clinically demonstrated remission of GPA in relation to extrarenal manifestations and partial remission of glomerulonephritis.

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About the authors

Nadiya F. Frolova

City Clinical Hospital No. 52, Moscow Health Department; Russian University of Medicine

Email: nadiya.frolova@yandex.ru
ORCID iD: 0000-0002-6086-5220

Cand.Sci. (Med.), Deputy Chief Physician for Nephrology, Head of the Interdistrict Nephrology Center, City Clinical Hospital No. 52 of the Moscow Healthcare Department; Associate Professor at the Nephrology Department, Faculty of Continuous Professional Education, Russian University of Medicine (RosUniMed)

Russian Federation, Moscow; Moscow

Zinaida Y. Mutovina

City Clinical Hospital No. 52, Moscow Health Department; Central State Medical Academy

Email: zmutovina@mail.ru
ORCID iD: 0000-0001-5809-6015

Cand.Sci. (Med.), Head of the Rheumatology Department No. 1, City Clinical Hospital No. 52 of the Moscow Healthcare Department; Associate Professor of the Department of Therapy, Cardiology and Functional Diagnostics with a course in Nephrology, Central State Medical Academy of the Administrative Dicrectorate of the President of the Russian Federation

Russian Federation, Moscow; Moscow

Margarita V. Kobzar

City Clinical Hospital No. 52, Moscow Health Department

Email: m.v.kobzar@yandex.ru
ORCID iD: 0009-0004-0236-653X

Analyst of the Project Office

Russian Federation, Moscow

Georgy N. Arbolishvili

City Clinical Hospital No. 52, Moscow Health Department; Lomonosov Moscow State University

Email: geodoc@yandex.ru
ORCID iD: 0000-0002-2252-3975

Deputy Chief Physician for Anesthesiology and Critical Care, City Clinical Hospital No. 52 of the Moscow Healthcare Department; Associate Professor at the Department of Anesthesiology and Critical Care, Faculty of Fundamental Medicine, Lomonosov Moscow State University

Russian Federation, Moscow; Moscow

Denis P. Pavlov

City Clinical Hospital No. 52, Moscow Health Department

Email: dr.pavlov@mail.ru
ORCID iD: 0009-0000-0572-8031

Head of the ICU No. 7

Russian Federation, Moscow

Daria Y. Shmeleva

City Clinical Hospital No. 52, Moscow Health Department

Email: dasha068@icloud.com
ORCID iD: 0009-0001-8310-2795

Neurologist of the Rheumatology Department

Russian Federation, Moscow

Farid F. Manerov

City Clinical Hospital No. 52, Moscow Health Department

Email: faridmanerov036@gmail.com
ORCID iD: 0009-0004-6478-6652

Resuscitation Physician

Russian Federation, Moscow

Maryana A. Lysenko

City Clinical Hospital No. 52, Moscow Health Department; Pirogov Russian National Research Medical University named after N.I. Russian Ministry of Health

Author for correspondence.
Email: gkb52@zdrav.mos.ru
ORCID iD: 0000-0001-6010-7975

Dr.Sci. (Med.), Professor, Chief Physician of the City Clinical Hospital No. 52 of the Moscow Healthcare Department; Professor at the Department of General Therapy, Pirogov Russian National Research Medical University

Russian Federation, Moscow; Moscow

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2. Fig. 1. CT scan of the chest organs with contrast from 10/01/2023. CT picture of subtonal consolidation of the right lung, polysegmental infiltration in the left lung. Obstruction of the right upper lobe bronchus. Contents in the bronchial tree on both sides. Small bilateral pleural effusion.

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3. Fig. 2. CT scan of the chest organs from November 2, 2023. Regression of bilateral pulmonary infiltration with replacement by fibrosis is noted.

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4. Fig. 3. CT scan of the chest organs, 12/11/2023

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5. Fig. 4. Scheme of the conducted therapy of ANCA vasculitis

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