CSS, or Churg-Strauss Syndrome, is a rare and serious disorder involving inflammation of blood vessels. It's also known as eosinophilic granulomatosis with polyangiitis (EGPA). This inflammation can restrict blood flow to organs and tissues, leading to various complications. CSS is characterized by asthma, eosinophilia (an elevated number of eosinophils, a type of white blood cell), and extravascular eosinophilic granulomas (clusters of eosinophils outside of blood vessels). The condition affects small- to medium-sized blood vessels and is considered a necrotizing systemic vasculitis.
However, CSS can also refer to Coffin-Siris syndrome, a rare genetic disorder present at birth (congenital). This condition affects multiple body systems and is characterized by developmental delays, intellectual disability, and distinctive physical features like underdeveloped pinky toenails.
Therefore, the meaning of "CSS disease" depends on the context. It's crucial to clarify which condition is being discussed to avoid confusion.
Churg-Strauss Syndrome (CSS) / Eosinophilic Granulomatosis with Polyangiitis (EGPA):
- Key Characteristics: Blood vessel inflammation, asthma, eosinophilia, extravascular eosinophilic granulomas.
- Impact: Restricted blood flow to organs and tissues, potentially leading to organ damage.
- Nature: A necrotizing systemic vasculitis (inflammation of blood vessels that can cause tissue death).
- References: Multiple sources, including Mayo Clinic, confirm this definition and its characteristics.
Coffin-Siris Syndrome (CSS):
- Key Characteristics: Developmental delays, intellectual disability, underdeveloped pinky toenails, and other physical abnormalities.
- Nature: A rare genetic disorder affecting multiple body systems.
- References: The National Organization for Rare Disorders (NORD) and the Genetic and Rare Diseases Information Center (GARD) provide information on this condition.
To summarize, "CSS disease" is ambiguous without further context. It can refer to either Churg-Strauss Syndrome, a vascular disorder, or Coffin-Siris syndrome, a genetic disorder. The correct interpretation relies heavily on the specific context in which the acronym is used.
