Etiology and pathogenesis
The exact cause of the disease cannot always be determined. The pathogenesis is based on immune disorders, according to which three types of the disease are distinguished (depending on the presence of specific antibodies and the immunofluorescence microscopy pattern of the biopsy specimen).
The main etiological factors include:
- Infectious processes: previous streptococcal infection, sepsis, viral diseases, and subacute infective endocarditis.
- Systemic and rheumatic pathology: systemic lupus erythematosus (SLE), Goodpasture syndrome, hemorrhagic vasculitis, and microscopic polyangiitis. When renal involvement is secondary, prominent clinical markers of the underlying systemic disease are observed concurrently.
Clinical presentation
The disease is characterized by an extremely rapid and severe course. The classical presentation manifests as the simultaneous appearance of three key syndromes:
- Pronounced nephrotic syndrome.
- Arterial hypertension.
- Rapidly progressive renal failure.
Of particular concern is arterial hypertension, which very rapidly acquires a malignant course. The eyes are the primary target organ: severe retinopathy and papilledema develop. In extreme cases, this leads to retinal detachment and total vision loss.
The natural history of the disease has an exclusively fatal prognosis. Spontaneous remissions do not occur. If the patient receives no treatment, uremia develops within the next 6–8 months, accompanied by severe anemia, hyperkalemia, and pericarditis, inevitably leading to a fatal outcome.
Principles of intensive care
Given the malignant nature of the pathology, the only correct tactic is immediate and maximally aggressive therapy.
Main areas of treatment:
- Pulse therapy: intravenous administration of ultra-high doses of cytotoxic drugs and glucocorticoids (prednisolone).
- Plasmapheresis: extracorporeal blood purification in large volumes (up to 5 liters of plasma are removed). The method is particularly indicated for patients with high titers of anti-glomerular basement membrane antibodies.
- Four-component regimen: simultaneous use of glucocorticoids, cytostatics, antiplatelet agents, and heparin.
To preserve the patient's life in the acute phase or in case of irreversible loss of renal function, renal replacement therapy — hemodialysis — is used. Subsequently, the option of kidney transplantation is considered.
Briefly: answers to topical questions
What three immunological types of rapidly progressive glomerulonephritis are distinguished based on pathogenesis?
Depending on pathogenesis, three immunopathogenetic types of rapidly progressive glomerulonephritis are distinguished.
- Antibody-mediated type (anti-GBM) — caused by specific autoantibodies against the glomerular basement membrane (target is type IV collagen), yields linear fluorescence.
- Immune complex type — associated with the deposition of preformed immune complexes in the mesangium and capillary walls, characterized by granular fluorescence.
- Pauci-immune type (pauci-immune) — characterized by the absence of immune deposits in the glomeruli, while antineutrophil cytoplasmic antibodies (ANCAs) are detected in the serum.
Which specific drugs are used for pulse therapy in rapidly progressive glomerulonephritis?
Ultra-high doses of glucocorticoids and cytostatics are used for pulse therapy in rapidly progressive glomerulonephritis.
- Glucocorticoids — specifically, prednisolone is used.
- Cytostatics — specific drug names from this group in the context of pulse therapy for this disease are not specified.
Additionally, a four-component regimen may be used, including a combination of cytostatics, glucocorticoids, heparin, and antiplatelet agents.
The classical triad in RPGN occurs simultaneously: Hypertension, Nephrotic syndrome, Renal failure (can be memorized as "HNR — Heinous Necrosis of Renal function").
Class and exam questions
Why is RPGN called malignant glomerulonephritis?
Due to its fatal natural history: spontaneous remissions are impossible, and without aggressive therapy, the patient dies from uremia within a few months.
What is the morphological essence of the disease?
The key marker is the formation of so-called extracapillary crescents in the renal glomeruli, accompanied by severe tissue damage and tubular involvement.
What is the main goal of performing high-volume plasmapheresis in this pathology?
Plasmapheresis physically removes aggressive pathogenic antibodies from the bloodstream, primarily anti-glomerular basement membrane antibodies.
What else to study on this topic
- The mechanism of formation of extracapillary crescents in the glomeruli
- Three immunological types of RPGN: characteristics of antibodies and IF data
- Differential diagnosis with manifestations of polyarteritis nodosa and SLE
- Pathogenesis of retinal damage in malignant arterial hypertension
- Pulse therapy protocols and dosages in the four-component regimen
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