What is the recommended management approach for hypertension in a patient with hEDS and MCAS?

Management of Hypertension in a Patient with hEDS and MCAS

For a patient with hEDS and MCAS presenting with elevated MAP readings over 100 (including 111), a calcium channel blocker (CCB) such as amlodipine should be the first-line antihypertensive medication, potentially combined with a low-dose ACE inhibitor if needed for adequate blood pressure control.

Understanding the Clinical Context

Patients with hypermobile Ehlers-Danlos Syndrome (hEDS) and Mast Cell Activation Syndrome (MCAS) present unique challenges in hypertension management due to:

• Autonomic dysfunction often present in hEDS
• Potential for medication reactions in MCAS
• Increased cardiovascular risk profile
• Need for careful medication selection to avoid exacerbating existing symptoms

Initial Assessment of Hypertension Severity

Based on the MAP reading of 111 and other readings over 100:

• This represents Stage 1-2 hypertension requiring pharmacological intervention
• Target blood pressure should be <130/80 mmHg according to current guidelines 1
• Immediate treatment is warranted given the sustained elevation and comorbidities

Medication Selection Algorithm

First-line therapy:

1. Calcium Channel Blockers (CCBs)
   • Preferred first-line agent for patients with MCAS and hEDS
   • Less likely to trigger mast cell degranulation than other antihypertensives
   • Amlodipine 5mg daily is an appropriate starting dose 1, 2
   • Can be titrated up to 10mg if needed

Second-line options (if CCB alone is insufficient):

1. Low-dose ACE inhibitor (e.g., lisinopril 5-10mg)

   • Add if blood pressure remains >140/90 mmHg after 2-4 weeks on CCB
   • Monitor carefully for hypotension due to potential autonomic dysfunction in hEDS

2. Consider low-dose thiazide-like diuretic (e.g., chlorthalidone 12.5mg)

   • As third-line agent if dual therapy is insufficient
   • Preferred over hydrochlorothiazide due to longer duration of action 1

Medications to avoid or use with caution:

• Beta-blockers: May worsen fatigue in hEDS patients
• Alpha-blockers: Risk of orthostatic hypotension in patients with autonomic dysfunction
• Clonidine: Should be avoided in patients with heart failure risk 1

Monitoring Protocol

1. Home blood pressure monitoring twice daily for 2 weeks after initiating or changing therapy
2. Follow-up visit within 4 weeks of treatment initiation
3. Target blood pressure of <130/80 mmHg 1
4. Monitor for orthostatic hypotension, especially with position changes

Lifestyle Modifications

While pharmacotherapy is necessary, lifestyle modifications remain important:

• Sodium restriction (<2,300 mg/day)
• Regular physical activity as tolerated (considering joint hypermobility limitations)
• DASH-style eating pattern with increased potassium intake
• Weight management if applicable
• Stress reduction techniques

Special Considerations for hEDS and MCAS

• Patients with MCAS and hEDS have a higher prevalence of autonomic dysfunction 3
• Medication reactions are more common, so start at lower doses and titrate slowly
• Fixed-dose combinations may improve adherence but limit dosing flexibility
• Consider potential drug interactions with any MCAS medications the patient may be taking

Follow-up Plan

• Reassess blood pressure control in 2-4 weeks
• If target not achieved, add or adjust medications following the algorithm above
• Once stable, follow up every 3-6 months
• Annual assessment for target organ damage

By following this approach, hypertension can be effectively managed while minimizing the risk of exacerbating symptoms related to hEDS and MCAS.