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Methods for Quantitative Detection of Antibody-induced Complement Activation on Red Blood Cells
Published on: January 29, 2014
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Endothelial lesion and complement activation in patients with Scleroderma Renal Crisis
Ney Arencibia Pérez1, María Luisa Agüera Morales1, Rafael Sánchez Sánchez2
1Reina Sofia University Hospital, Nephrology, Av. Menendez Pidal, s/n Córdoba, Spain.
Jornal Brasileiro De Nefrologia
|February 27, 2019
Summary
Scleroderma renal crisis (SRC) may involve thrombotic microangiopathy (TMA) and complement activation, challenging current understanding. This case highlights endothelial damage and TMA in SRC, suggesting a need to redefine the condition.
Area of Science:
- Nephrology
- Rheumatology
- Pathology
Background:
- Scleroderma renal crisis (SRC) is typically defined by vascular endothelial injuries and C4d deposits in kidney biopsies.
- Thrombotic microangiopathy (TMA) has been observed in systemic sclerosis (SSc) but not directly linked to acute kidney injury in SRC.
Observation:
- A patient with scleroderma-dermatomyositis overlap syndrome presented with SRC, exhibiting clinical and histopathological features of TMA.
- Fundus examination revealed severe acute hypertensive retinopathy.
Findings:
- Kidney biopsy demonstrated severe endothelial damage, mucoid intimal widening, arteriolar concentric proliferation, and C3, C4d, and IgM deposition.
- Genetic analysis revealed only membrane cofactor protein (MCP) risk haplotypes, with no other complement gene defects.
Implications:
- This case suggests that TMA and complement activation are key features of kidney damage in SSc patients with SRC.
- Further research correlating clinical and pathological data may necessitate redefining SRC to emphasize acute endothelial injury and complement pathways.
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