Goodpasture syndrome. Clinical case

I.M. Patrikeeva¹, Yu.A. Lushpaeva², A.Yu. Evenko¹, T.Yu. Malysheva¹, A.G. Bichenova^{3 1}Regional Clinical Hospital No. 1, Tyumen

²Tumen State Medical University, Tyumen

³Multiprofile Clinical Medical Center "Meditsinskii Gorod", Tyumen

Goodpasture syndrome is a disease associated with the formation of anti-glomerular basement membrane antibodies. It belongs to the rare immune complex organ-specific systemic vasculitis. The article presents the clinical case of Goodpasture syndrome in a 23-year-old female patient. The disease manifested with a clinical picture of fulminant course of glomerulonephritis and rapidly progressive azotemia, followed by the pathological process in the lungs in the form of hemorrhagic alveolitis and pulmonary hemorrhage. Laboratory confirmation of the disease was high titers of specific anti-glomerular basement membrane antibodies (anti-GBM antibodies). The point of the described clinical case is the fulminant course of Goodpasture syndrome, despite the conducted immunosuppressive therapy in combination with plasmapheresis, the development of hemorrhagic stroke, coma and the fatal outc ome of the patient. Morphological analysis of the autopsy material showed proliferative nephritis with a very high percentage of crescentic glomeruli and pulmonary vasculitis. The authors focus on the urgent need for revision and adaptation to routine clinical practice of interdisciplinary algorithms for the patient management with Goodpasture syndrome, for the elimination of terminological differences and the determination of the intervention threshold of a primary care physician and a specialist (nephrologist, pulmonologist, rheumatologist).

Keywords: Goodpasture syndrome, anti-glomerular basement membrane antibodies disease, anti-GMB disease, anti-GBM antibodies, crescentic glomerulonephritis, hemorrhagic alveolitis, plasmapheresis.

For citation: Patrikeeva I.M., Lushpaeva Yu.A., Evenko A.Yu. et al. Goodpasture syndrome. Clinical case. RMJ. 2022;6:75–79.