Practical management strategies for benign hypermobility syndromes

Abstract

Purpose of reviewPatients with symptomatic hypermobility syndrome such as hypermobile Ehlers-Danlos syndromes (hEDS) and hypermobility spectrum disorders (HSD) commonly present to rheumatologists with joint pain and functional disability. Providers often have difficulty with diagnosis due to a lack of knowledge on the range of associated manifestations and the available therapeutic modalities. This review will discuss recent updates on diagnostic measures and treatment options for rheumatologists to help patients navigate hEDS/ HSD. Recent findingsThis article describes newer diagnostic measures and assessment of hEDS/HSD manifestations. Evidence supporting physical therapy and occupational therapy is provided, as well as recent updates on assistive devices, compressive garments, orthoses, and surgical interventions. Given patient heterogeneity specific guidance about the amount and type of therapies required to produce a beneficial effect is lacking. Treatment should be individualized, and many of the studies focus on regional joint complaints rather than a whole-body approach.

Pelvic Organ Prolapse in Ehlers-Danlos Syndrome

“…Rheumatologists are often the first physicians involved with caring for EDS patients. Unfortunately, there is no single drug therapy to treat the pain and other comorbidities in patients with EDS [ 6 ]. Instead, therapy is often guided by the patient's symptomatology.…”

“…Instead, therapy is often guided by the patient's symptomatology. Nonsteroidal anti-inflammatory drugs are the first line for pain, with ibuprofen being most effective and best tolerated [ 6 ]. While neuropathic modulators including tricyclic antidepressants, anticonvulsants, serotonin, and norepinephrine reuptake inhibitors are traditionally useful for centralized pain, their use in this patient population is limited secondary to 47% of patients noting adverse effects including worsening dysautonomia [ 6 ].…”

“…Nonsteroidal anti-inflammatory drugs are the first line for pain, with ibuprofen being most effective and best tolerated [ 6 ]. While neuropathic modulators including tricyclic antidepressants, anticonvulsants, serotonin, and norepinephrine reuptake inhibitors are traditionally useful for centralized pain, their use in this patient population is limited secondary to 47% of patients noting adverse effects including worsening dysautonomia [ 6 ]. For patients with specific pelvic pain not responsive to oral analgesia or conservative management, injection with long-acting corticosteroids can be used [ 7 ].…”

“…A randomized control trial has shown that a six-week generalized program improving strength and fitness resulted in significant improvements in pain scores and improvement in self-esteem [ 8 , 9 ]. Another study showed specifically that patients with pelvic girdle pain respond well to targeted physical therapy [ 6 ]. There is no specific time frame after which therapy is considered effective, as training is often riddled with physical and mental setbacks, but the basic approach includes strength training, proprioceptive exercises, and gentle stretching [ 10 ].…”