2 articles
Scleroderma Renal Crisis (SRC) is a life-threatening complication of systemic sclerosis (SSc), traditionally associated with anti-RNA polymerase III antibodies, corticosteroid use, and diffuse skin involvement. However, the role of COVID-19 as a potential trigger for SRC remains poorly understood. This study explores the occurrence of COVID-19-associated SRC, focusing on its clinical presentation, underlying risk factors, and outcomes.
We present a case series of two unvaccinated patients with systemic sclerosis who developed SRC following COVID-19 infection, despite the absence of traditional risk factors. Clinical features, laboratory findings, renal histopathology, and disease progression were analyzed to assess potential mechanisms linking SARS-CoV-2 infection to SRC onset. Both patients developed abrupt-onset malignant hypertension and acute kidney injury after supporting the COVID-19. Neither patient had a history of corticosteroid use or known anti-RNA polymerase III positivity, suggesting an alternative mechanism of SRC activation. Notably, both cases had pre-existing renal anomalies (renal developmental abnormality and prior nephrectomy), which may have contributed to increased susceptibility. Despite aggressive management, both patients developed dialysis-dependent renal failure and succumbed to SRC-related complications.
Our findings highlight COVID-19 as a potential trigger for SRC, possibly through endothelial dysfunction, inflammatory cytokine storms, and renal microangiopathy. The presence of pre-existing kidney conditions may further predispose SSc patients to SRC following SARS-CoV-2 infection. Additionally, the lack of vaccination in these cases raises the question of whether COVID-19 immunization could reduce SRC risk. Further research is needed to elucidate the pathophysiology, risk stratification, and long-term outcomes of COVID-19-associated SRC, as well as the role of vaccination in prevention.
Due to the heterogeneous nature of systemic sclerosis, it is difficult to predict disease progression and complications. Despite the discovery of novel autoantibodies associated with systemic sclerosis (SSc), there is an unmet need for biomarkers for diagnosis, disease progression, and response to treatment.
An analytical, qualitative study was performed with a narrative review of literature in the form of a synthesis article. Relevant primary sources published in 2020-2022 were identified and selected, using data extraction and analysis.
Anti-citrullinated protein/peptide antibody could be useful in identifying patients with a more prominent joint disease. Of most interest, the anti-carbamylated protein antibodies (anti-CarP) could be a relevant biomarker related to fibrotic skin and lung disease. Positive anti-RNA (Ribonucleic acid) polymerase III antibody and antinuclear antibodies (ANA) negativity were significantly associated with GAVE (gastral antral vascular ectasia). Autoantibodies against telomeres may help identify systemic sclerosis with lung disease. Osteopontin links myeloid activation and disease progression in systemic sclerosis. CTRP (C1q tumor necrosis factor-related proteins) 9 protein levels may be biomarker of lung disease severity. CD (cluster differentiation) 21-low B cells are linked to vascular damage. L-tyrosine, L-tryptophan, and 1-methyl-adenosine distinguished healthy controls from SSc patients. L-leucine, L-isoleucine, xanthosine, and adenosine monophosphate differentiated between progressing and stable SSc-ILD. CECs (circulating endothelial cells) are a direct indicator of systemic vascular damage. Levels of the protein, galectin-3, are associated with heart involvement in people with systemic sclerosis. Low levels of the galectin-10 protein (Gal-10) in scleroderma associate with inflammation and vascular changes in the lungs, leading to pulmonary arterial hypertension (PAH). High levels of the CD146 protein may be a potential biomarker in identifying people with systemic sclerosis. Blood levels of the protein endocan increased in scleroderma patients who are at risk for pulmonary arterial hypertension . FLCs (free light chain) could be employed as useful potential biomarker of early diagnosis and to follow disease activity.
Novel discovered biomarkers could predict disease development, activity, and severity of diverse organ involvement, predict risk of complications of systemic sclerosis.