Autonomic function testing and symptom severity in patients with suspected mast cell activation disorders

Area of Science:

• Mast cell activation disorders
• Autonomic nervous system function
• Hematology

Background:

• Mast cell activation disorders (MCAD), including hereditary alpha tryptasemia (HaT) and systemic mastocytosis, are increasingly recognized.
• Dysautonomia, or autonomic dysfunction, can significantly impact quality of life in these patients.
• Formal classification criteria exist for MCAD and related conditions.

Purpose of the Study:

• To assess autonomic nervous system severity in patients with MCAD, HaT, and systemic mastocytosis.
• To compare objective and subjective measures of autonomic dysfunction in these populations.
• To determine the association between specific MCAD classifications and autonomic disease severity.

Main Methods:

• Evaluation of objective autonomic function tests.
• Assessment of subjective patient-reported autonomic symptom severity.
• Analysis of data from patients with formally classified MCAD, HaT, and systemic mastocytosis.

Main Results:

• Autonomic disease severity was objectively mild across all patient groups.
• Subjective measures indicated moderate to severe autonomic dysfunction in all subjects.
• MCAD presence did not consistently alter subjective or objective severity.
• HaT was associated with lower objective, but not subjective, autonomic severity measures.

Conclusions:

• Formal classification criteria are suitable for assessing MCAD and related disorders.
• Suspected dysautonomia in MCAD patients warrants a comprehensive workup.
• Incorporating both objective and subjective measures is crucial for evaluating autonomic dysfunction in these conditions.