Ehlers-Danlos Syndromes (EDS) are a group of hereditary disorders that affect connective tissue throughout the body. Connective tissue provides structure and support to skin, joints, blood vessels, and organs, but in EDS it is compromised due to defects in collagen production or processing. A “flare-up” is defined as a temporary, acute worsening of typical symptoms, often including a significant increase in pain, fatigue, and joint instability. Because EDS affects many body systems, flares can manifest as a widespread escalation of symptom severity. Identifying the specific triggers that precipitate these episodes is necessary for managing the condition and stabilizing daily life.
Mechanical and Physical Strain
The inherent laxity and fragility of connective tissue in EDS make the body highly susceptible to flares triggered by mechanical and physical stress. Normal daily activities can push the compromised joints beyond their limits, initiating a cascade of painful symptoms. Hypermobile joints, which lack the firm structural support of typical ligaments, require surrounding muscles to work harder to maintain stability.
This constant overcompensation by the muscles leads to chronic strain and fatigue, making even minor overexertion a common flare-inducing event. Activities like walking for an extended duration, standing in one place for too long, or engaging in repetitive tasks can cross the threshold of tolerance. The resulting micro-trauma places undue stress on the already weakened tendons and ligaments, leading to increased joint pain and instability, sometimes manifesting as frequent subluxations or partial dislocations.
Improper posture and sudden, uncontrolled movements or injuries also represent significant mechanical triggers. When the body is subjected to a sudden jolt, or if stability is not properly maintained, the joint capsule stretches too far, initiating a protective, painful response. This instability can irritate or entrap nerves, leading to shooting pain and further muscle guarding, which locks the body into a flare cycle.
Systemic and Inflammatory Responses
Flare-ups can also be driven by internal systemic processes, particularly those involving inflammation and hormonal changes. The body’s response to an infection, whether a minor viral illness or a bacterial infection, places systemic stress on the body. This stress can trigger a widespread inflammatory response that exacerbates underlying EDS symptoms, leading to profound fatigue and increased musculoskeletal pain.
Hormonal fluctuations represent a common and often cyclical trigger for many individuals with EDS. Hormones, especially estrogen, influence the elasticity and laxity of connective tissues. Many individuals report a worsening of symptoms during the luteal phase of the menstrual cycle, which occurs after ovulation. During this 10 to 14-day window, increased ligament laxity can translate directly into greater joint instability and subsequent pain.
Major hormonal shifts, such as those occurring during pregnancy, perimenopause, or postpartum, can similarly affect connective tissue integrity and precipitate severe flares. Sleep deprivation acts as another powerful systemic stressor, preventing the body from performing necessary recovery and repair functions. Poor quality sleep correlates strongly with increased pain and fatigue, creating a self-perpetuating cycle that lowers the pain threshold.
Autonomic and Environmental Factors
A significant number of EDS flares are linked to the autonomic nervous system (ANS) and various environmental stressors. The ANS regulates involuntary bodily functions like heart rate, blood pressure, and digestion; dysfunction in this system, known as dysautonomia, is common in EDS. Psychological stress, including chronic anxiety or emotional trauma, directly impacts the ANS by activating the sympathetic “fight-or-flight” response. This sustained activation increases muscle tension and promotes inflammation, which lowers the body’s pain threshold and leads to a physical flare.
Dysautonomia often manifests as Postural Orthostatic Tachycardia Syndrome (POTS), where standing causes an abnormal increase in heart rate, leading to symptoms like dizziness, fatigue, and presyncope. Triggers that worsen POTS symptoms, such as dehydration, physical strain, and especially heat, can quickly initiate a flare. Hot weather causes vasodilation, or the widening of blood vessels, which can exacerbate blood pooling and orthostatic intolerance.
Changes in the external environment, such as shifts in barometric pressure, can also affect joint pain and stiffness. Furthermore, a common co-occurring condition is Mast Cell Activation Syndrome (MCAS), where mast cells inappropriately release inflammatory mediators. In individuals with MCAS, flares can be triggered by a wide range of environmental factors, including strong scents, certain foods, pollen, temperature extremes, and pressure.