Hives in autonomic disorders: a cutaneous marker of a distinct symptom phenotype.
Hives are reported by a significant portion of patients with POTS and NMH, and their frequency is associated with greater symptom burden.
Where it sits
this study against the rest of the desmopressin corpusSummary and findings
This study measured the frequency of hives in patients with Postural Orthostatic Tachycardia Syndrome (POTS) and Neurally-Mediated Hypotension (NMH). Among 188 respondents, 42.6% reported hives sometimes, and 17.6% reported hives often or always. The study found associations between hives frequency and increased symptom burden across various autonomic symptom domains.
Abstract
Background Postural Orthostatic Tachycardia Syndrome (POTS) and Neurally-Mediated Hypotension (NMH) are heterogeneous syndromes characterized by dysautonomia and multisystem symptoms. Mast cell activation, often manifesting as hives, has been proposed as a contributing mechanism, but its prevalence and clinical relevance in POTS and NMH are poorly defined. Method Patients from the Johns Hopkins POTS Clinic completed surveys assessing hives frequency and symptom burden using the Malmö POTS, the Composite Autonomic Symptom Score (COMPASS)-31, and a pain questionnaire. Associations between hives and clinical features were evaluated among patients with confirmed POTS, NMH, or clinically diagnosed orthostatic intolerance. Result Among 188 respondents, 80 (42.6%) reported hives sometimes and 33 (17.6%) reported hives often or always. Increasing hives frequency was associated with higher Malmö POTS scores and greater autonomic symptom burden across multiple COMPASS-31 subdomains, including gastrointestinal, bladder, and vasomotor symptoms (all p < 0.05). Hives was also associated with pain (OR 3.47, 95% CI 1.54-7.77, p = 0.002) and tingling (OR 5.73, CI 2.15-15.26, p < 0.001), but not orthostatic symptoms. These associations persisted after multivariable adjustment. Conclusion Hives are common in orthostatic intolerance syndromes and are associated with increased symptom burden. Future studies are needed to clarify the role of mast cell activation and evaluate mast cell-targeted therapies.
Background
This paper addresses the prevalence and clinical relevance of hives in patients with POTS and NMH, conditions characterized by dysautonomia and various symptoms. Prior research has suggested a potential link between mast cell activation and hives, but the extent of this association in these syndromes is not well defined. Understanding this relationship could inform future research and therapeutic approaches.
Methods
The study involved patients from the Johns Hopkins POTS Clinic who completed surveys assessing hives frequency and symptom burden. The primary outcome measures included the Malmö POTS, the Composite Autonomic Symptom Score (COMPASS)-31, and a pain questionnaire. The population consisted of patients with confirmed POTS, NMH, or clinically diagnosed orthostatic intolerance, with a total sample size of 188.
Results
Among the 188 respondents, 42.6% reported hives sometimes, and 17.6% reported hives often or always. Increasing frequency of hives was significantly associated with higher Malmö POTS scores and greater symptom burden across multiple COMPASS-31 subdomains (all p<0.05). Additionally, hives were associated with pain (OR 3.47, 95% CI 1.54-7.77, p=0.002) and tingling (OR 5.73, CI 2.15-15.26, p<0.001), but not with orthostatic symptoms.
Interpretation
The findings suggest that hives are a common symptom in patients with orthostatic intolerance syndromes and are linked to increased symptom burden. While the associations are statistically significant, the clinical significance of these findings requires further investigation. Limitations include reliance on self-reported data and a relatively small sample size, which may affect the robustness of the conclusions.
Key findings
- 42.6% reported hives sometimes, n=188.
- 17.6% reported hives often or always, n=188.
- Hives frequency was associated with higher Malmö POTS scores (p<0.05).
- Hives were associated with pain (OR 3.47, 95% CI 1.54-7.77, p=0.002).
- Hives were associated with tingling (OR 5.73, CI 2.15-15.26, p<0.001).
Limitations
- self-reported data may introduce bias
- sample size of 188 may limit generalizability
- no control group for comparison
- cross-sectional design limits causal inference