Difficulties in diagnosing microscopic polyangiitis

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Abstract

Background. This clinical case demonstrates the difficulty of diagnosing systemic vasculitis and the effectiveness of immunosuppressive therapy.

Description of the clinical case. The article presents a clinical case of microscopic polyangiitis in a 61-year-old man. The disease proceeded with predominant kidney damage associated with antineutrophil cytoplasmic antibodies targeting proteinase-3, febrile fever, myalgia, arthralgia, weight loss, and polyneuropathy. The material obtained during the primary kidney biopsy was of little information, since it contained less than 10 glomeruli without crescents. The patient abstained from the proposed repeated nephrobiopsy. Taking into account clinical and laboratory data, ANCA-glomerulonephritis was diagnosed, presumably within the framework of microscopic polyangiitis. Immunosuppressive therapy with glucocorticosteroids and cyclophosphamide made it possible to obtain a rapid remission of the disease. Anti-relapse therapy was not carried out. After 2 years, a relapse of the disease with a decrease in kidney function developed. The patient underwent repeated nephrobopsy, signs of ANCA-associated glomerulonephritis with the presence of crescents in 63% of the glomeruli were found. The prescribed immunosuppressive therapy with glucocorticosteroids and cyclophosphamide led to remission of the disease with restoration of kidney function.

Conclusion. The clinical case illustrates the difficulty of early verification of the diagnosis of microscopic polyangiitis, the high efficiency of immunosuppressive therapy, and the need for long-term anti-relapse therapy.

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About the authors

Olga V. Simonova

Department of Hospital Therapy, Kirov State Medical University

Author for correspondence.
Email: simonova043@mail.ru
ORCID iD: 0000-0002-6021-0486

Dr. Sci. (Med.), Associate Professor at the Department of Hospital Therapy, Kirov State Medical University

Russian Federation, Kirov

Galina A. Postnikova

Department of Hospital Therapy, Kirov State Medical University

Email: postnikovakirov@yandex.ru
ORCID iD: 0000-0002-3289-3419

Cand.Sci. (Med), Associate Professor at the Department of Hospital Therapy, Kirov State Medical University

Russian Federation, Kirov

Ekaterina S. Stolyarevich

Department of Nephrology, FAPE , A.I.Yevdokimov Moscow State University of Medicine and Dentistry

Email: stolyarevich@yandex.ru
ORCID iD: 0000-0002-0402-8348

Dr. Sci. (Med.), Professor at the Department of Nephrology, FAPE, A.I. Yevdokimov Moscow State University of Medicine and Dentistry, Moscow

Russian Federation, Moscow

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Supplementary files

Supplementary Files
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1. JATS XML
2. Fig. 1A. Kidney biopsy from 2019, represented mainly by the medulla. Masson's trichome stain *100

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3. Fig. 1B. Kidney biopsy from 2019. A section of the cortical layer containing two unchanged glomeruli. Focal infiltration of the interstitium with mononuclear cells. PAS*200

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4. Fig. 2A. Glomeruli with global and segmental glomerulosclerosis. Interstitial fibrosis and tubular atrophy. Masson's Trichome stain *100.

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5. Fig. 2B. Two glomeruli with segmental fibrous crescents, one glomerulus looks little changed. PAS*200

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