Brittany Harris FNP-C

NEW CLINICAL PRACTICE GUIDELINES- AGA Practice Update on GI Manifestations and Autonomic or Immune Dysfunction in Hypermobile Ehlers-Danlos Syndrome, READ Below 👇
A new Clinical Practice Update published in Clinical Gastroenterology and Hepatology from the American Gastroenterological Association and a group of experts provides long-overdue guidance for clinicians treating patients with hypermobile Ehlers-Danlos syndrome (hEDS) or hypermobility spectrum disorders (HSDs) who also experience gut-brain interaction disorders (DGBIs). There are 16 best practice advice statements in the publication.

16 Best Practice Advice Statements

CLINICAL ASSOICIATIONS: Clinicians should be aware of the observed associations between hEDS or HSDs and POTS and/or MCAS and their overlapping gastrointestinal (GI) manifestations.

WHO TO TEST? Testing for POTS/MCAS should be targeted to patients presenting with clinical manifestations of POTS/MCAS, but universal testing for POTS/MCAS in all patients with hEDS/HSDs is not supported by the current evidence.

SCREENING FOR HYPERMOBILITY/HEDS: Gastroenterologists seeing patients with DGBI should inquire about joint hypermobility and strongly consider incorporating the Beighton score for assessing joint hypermobility into their practice as a screening tool; if the screen is positive, gastroenterologists may consider applying 2017 diagnostic criteria to diagnose or refer to specialist.

TESTING FOR POTS: Testing for POTS through postural vital signs and referral to specialty practices for autonomic testing should be considered in patients with hEDS/HSDs and refractory GI symptoms who also report orthostatic intolerance after exclusion of medication side effects and appropriate lifestyle or behavioral modifications have been attempted but is not required for all patients with hEDS/HSDs who report GI symptoms alone.
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CELIAC TESTING: Testing for celiac disease may be considered earlier in the diagnostic evaluation of patients with hEDS/HSDs who report a variety of GI symptoms and not only limited to those with diarrhea. There is insufficient research to support routine testing for disaccharidase deficiencies or other diet-mediated mechanisms as causes of GI symptoms in hEDS/HSDs.

FUNCTIONAL DEFECATION DISORDERS: Diagnostic testing for functional defecation disorders with anore**al manometry, balloon expulsion test, or defecography should be considered in patients with hEDS/HSDs and lower GI symptoms such as incomplete evacuation given the high prevalence of pelvic floor dysfunction, especially re**al hyposensitivity, in this population.

GASTRIC EMPTYING: In patients with hEDS/HSDs and comorbid POTS who report chronic upper GI symptoms, timely diagnostic testing of gastric motor functions should be considered after appropriate exclusion of anatomical and structural diseases, as abnormal gastric emptying may be more common than in the general population.

MEDICAL MANAGEMENT: Medical management of GI symptoms in hEDS/HSDs and POTS/MCAS should focus on treating the most prominent GI symptoms and abnormal GI function test results. In addition to general DGBIs and GI motility disorder treatment, management should also include treating any symptoms attributable to POTS and/or MCAS.

TESTING FOR MCAS: In patients presenting to gastroenterology providers, testing for mast cell disorders including MCAS should be considered in patients with hEDS/HSDs and DGBI who also present with episodic symptoms that suggest a more generalized mast cell disorder involving 2 or more physiological systems, but current data do not support the use of these tests for routine evaluation of GI symptoms in all patients with hEDS/HSDs without clinical or laboratory evidence of a primary or secondary mast cell disorder.

TRYPTASE TESTING: If MCAS is suspected, diagnostic testing with serum tryptase levels collected at baseline and 1-4 hours following symptom flares may be considered by the gastroenterologist; increases of 20% above baseline plus 2 ng/mL are necessary to demonstrate evidence of mast cell activation.

ADDITIONAL ALLERGY TESTING: If a diagnosis of MCAS is supported through clinical and/or laboratory features, patients should be referred to an allergy specialist or mast cell disease research center where additional testing may be performed.

DGBI EVALUATION: Diagnostic evaluation of GI symptoms consistent with DGBI in patients with hEDS/HSDs and comorbid POTS and/or MCAS should follow a similar approach to the evaluation of DGBI as in the general population including the use of a positive symptom-based diagnostic strategy and limited noninvasive testing.
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TREATMENT OF POTS: Treatment of POTS may include increasing fluid and salt intake, exercise training, and use of compression garments. Special pharmacological treatments for volume expansion, heart rate control, and vasoconstriction with integrated care from multiple specialties should be considered in patients who do not respond to conservative lifestyle measures.

TREATMENT OF MCAS: When MCAS is suspected, patients can benefit from treatment with histamine receptor antagonists and/or mast cell stabilizers, in addition to avoiding triggers such as certain foods, alcohol, strong smells, temperature changes, mechanical stimuli, emotional distress, or specific medications.

NUTRITION: Besides general nutritional support, special diets including a gastroparesis diet and various elimination diets can be considered for improving GI symptoms. Dietary interventions should be delivered with appropriate nutritional counseling or guidance to avoid the pitfalls of restrictive eating.

MANAGEMENT OF GI SYMPTOMS: Management of chronic GI symptoms in patients with hEDS/HSDs who do not exhibit symptoms consistent with POTS or MCAS should align with existing approaches to management of DGBI and GI motility disorders in the general population, including integrated multidisciplinary care involving multiple specialties, where appropriate.

Source: https://pubmed.ncbi.nlm.nih.gov/40387691/

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https://news.va.gov/press-room/va-launches-mdma-assisted-mental-health-therapy-trial/

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https://ja.ma/4d4ucpW

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PMCID: PMC3411859 NIHMSID: NIHMS371296 PMID: 22549432