Patient with H syndrome, cardiogenic shock, multiorgan infiltration, and digital ischemia.
Laura Ventura-EspejoInés Gracia-DarderSilvia Escribá-BoriEva Regina Amador-GonzálezAna Martín-SantiagoJan RamakersPublished in: Pediatric rheumatology online journal (2021)
We report the most severe disease course produced by HS described so far in the literature. Our patient's manifestations included uncommon, new complications such as acute heart failure with severe systolic dysfunction, multi-organ cell infiltrate, and digital ischemia. Most of the clinical symptoms of our patient could have been explained by SARS-CoV-2, demonstrating the importance of a detailed differential diagnosis to ensure optimal treatment. Although the mechanism of autoinflammation of HS remains uncertain, the good response of our patient to Tocilizumab makes a case for the important role of IL-6 in this syndrome and for considering Tocilizumab as a first-line treatment, at least in severely affected patients.
Keyphrases
- case report
- sars cov
- rheumatoid arthritis
- acute heart failure
- end stage renal disease
- heart failure
- systematic review
- blood pressure
- early onset
- chronic kidney disease
- left ventricular
- ejection fraction
- single cell
- newly diagnosed
- peritoneal dialysis
- oxidative stress
- systemic lupus erythematosus
- depressive symptoms
- juvenile idiopathic arthritis
- atrial fibrillation
- patient reported
- combination therapy