Eosinophilic granulomatosis with polyangiitis and catastrophic multiorgan involvement

Frolova N.F., Volgina G.V., Sysoeva I.L., Usatyuk S.S., Stolyarevich E.S., Iskhakov R.T., Mutovina Z.Yu., Fomina D.S.

1) State Budgetary Institution of Healthcare “Moscow Clinical Research Center, Hospital No.52”, Moscow, Russia; 2) Federal State Budgetary Educational Institution of Higher Education Russian University of Medicine, Moscow, Russia; 3) Federal State Budgetary Institution of Continuing Professional Education Central State Medical Academy of the Administrative Department of the President of the Russian Federation, Moscow, Russia; 4) Federal State Autonomous Educational Institution of Higher Education I.M. Sechenov First Moscow State Medical University, Moscow, Russia

This article presents a unique clinical case of eosinophilic granulomatosis with polyangiitis (EGPA). This case is characterized by three key features: extremely late diagnosis (10 years after symptom onset), catastrophic multiorgan damage (kidney impairment, leading to acute kidney injury requiring dialysis, heart damage similar to Löffler’s endomyocarditis, lung and peripheral nervous system damage), and a significant positive response to personalized therapy. An intensive combination was used to induce remission, including pulse glucocorticoid therapy, therapeutic plasmapheresis, and a combination of rituximab and benralizumab. This approach resulted in rapid and significant clinical and laboratory improvement: normalization of eosinophil levels, regression of neurological deficits, and heart failure. The most significant outcome was the restoration of kidney function, allowing discontinue renal replacement therapy. This observation demonstrates the effectiveness of aggressive, targeted immunosuppression even in critically late diagnosis of severe life-threatening EGPA and highlights the potential of a combination of drugs with different mechanisms of action (B-cell depletion+interleukin-5 blockade) in achieving remission in case of refractory multiorgan involvement.

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About the Authors

Nadiya F. Frolova – Cand.Sci. (Med.), Deputy Chief Physician for Nephrology Care, Head of the Interdistrict Nephrology Center, Moscow Clinical Research Center, Hospital No. 52, Moscow Healthcare Department. Address: 3 Pekhotnaya St., Moscow 123182; Associate Professor, Department of Nephrology, Russian University of Medicine, Russian Federation. Address: Bldg. 4, 1A Kuskovskaya St., Moscow, 111398; e-mail: nadiya.frolova@yandex.ru. ORCID: 0000-0002-6086-5220
Galina V.Volgina – Dr.Sci. (Med.), Professor, Department of Nephrology, Russian University of Medicine. Address: Bldg. 4, 1A Kuskovskaya St., Moscow, 111398;
e-mail: volginagv@mail.ru. ORCID: 0000-0002-7771-4074
Irina L. Sysoeva – Nephrologist, Nephrology Department No. 2, Moscow Clinical Research Center, Hospital No. 52, Moscow Healthcare Department. Address: 3 Pekhotnaya St., Moscow 123182; e-mail: i.l. sysoeva@mail.ru. ORCID: 0000-0002-1904-9846
Sergey S. Usatyuk – Head of Nephrology Department No. 2, Moscow Clinical Research Center, Hospital No. 52, Moscow Healthcare Department. Address: 3 Pekhotnaya St., Moscow 123182; e-mail: usatuk-doc@mail.ru. ORCID: 0000-0002-8742-3860
Ekaterina S. Stolyarevich – Dr.Sci. (Med.), Physician in the Pathology Department, Moscow Clinical Research Center, Hospital No. 52, Moscow Healthcare Department. Address: 3 Pekhotnaya St., Moscow 123182; Professor, Nephrology Department, Russian University of Medicine. Address: Bldg. 4, 1A Kuskovskaya St., Moscow, 111398; E-mail: Stolyarevich@yandex.ru
Rustam T. Iskhakov – Head of the Nephrology Intensive Care Unit, Moscow Clinical Research Center, Hospital No. 52, Moscow Healthcare Department. Address: 3 Pekhotnaya St., Moscow 123182; e-mail: stamius@yandex.ru. ORCID: 0000-0003-2850-4465
Zinaida Yu. Mutovina – Cand.Sci. (Med.), Deputy Chief Physician for Medical Affairs, Moscow Clinical Research Center, Hospital No. 52, Moscow Healthcare Department. Address: 3 Pekhotnaya St., Moscow 123182; Associate Professor, Department of Therapy, Cardiology, and Functional Diagnostics, Central State Medical Academy of the Administrative Directorate of the President of the Russian Federation, with a course in Nephrology. Address: Bldg. 1A, 19 Marshal Timoshenko St., Moscow, 121359;
e-mail: zmutovina@mail.ru. ORCID: 0000-0001-5809-6015
Daria S. Fomina – Dr.Sci. (Med.), Professor, Deputy Director for Research and Clinical Work, Moscow Clinical Research Center, Hospital No. 52, Moscow Healthcare Department. Address: 3 Pekhotnaya St., Moscow 123182, Chief Allergist-Immunologist, Moscow Healthcare Department, Professor, Department of Clinical Immunology and Allergology, Sechenov First Moscow State Medical University. Address: Bldg. 2, 8 Trubetskaya St., Moscow, 119048; e-mail: daria_fomina@mail.ru. ORCID: 0000-0002-5083-6637

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