Hypermobility, EDS, JIA, and Chronic Pain

Generalized joint hypermobility (GJH)

5 or more joints affected by hypermobility

Classified as medical condition if pt has other sx such as chronic pain or fatigue

Ehlers-Danlos Syndromes (EDS)

Group of inheritable connective tissue disorders including GJH and skin hyperextensibility

Can only be diagnosed in adults, not children

Hypermobility spectrum disorder

Hypermobile disorders that do not meet criteria of EDS

Systemic sx of HSD and EDS

Chronic pain, fatigue, dysautonomia, proprioceptive, and coordination deficits

Often co-occurs with with developmental coordination disorder (DCD), ASD, and ADHD

Vascular EDS (vEDS)

Form of EDS characterized by translucent skin, prominent veins and eyes

Pts often bruise easily and are at risk for aortic rupture

Signs to watch in gait (HSD)

Decreased knee flexion, knee hyperextension, excessive pronation in stance

Juvenile idiopathic arthritis (JIA)

Group of conditions that present with arthritis of unknown etiology. Diagnosis of exclusion

Sx last at least 6 weeks with onset before 16 years old

Sx include swelling, end range pain, stiffness, loss of ROM, protective muscle spasms, chronic systemic inflammation

Joint changes in systemic inflammation

Increased production of synovial fluid

Stretched and weakened joint capsule

Ligamentous laxity and joint instability

Overgrowth of synovium (pannus)

Demineralization of bone, osteophyte formation

Thinning and loss of articular cartilage

Oligoarthritis

Most common form of JIA. Low grade inflammation in 4 or less joints

Commonly affects knees, ankles, and elbows while hips and small joints spared

Systemic effects uncommon, but may have iridocyclitis

Onset in early childhood, often 2-4y/o

Polyarticular arthritis

JIA that affects 5 or more joints within the first 6 months of sx appearing; often appear in late childhood or early adolescence. Characterized by swollen and warm joints

Sx are bilateral and affect both large and small jts. May have mild systemic sx such as low grade fever.

May or may not have rheumatoid factor (RF)

Systemic arthritis

JIA that can appear at any point in childhood. Characterized by systemic fever for at least 2 weeks and presence of rash on trunk and limbs

Sx symmetrical and polyarticular. Global inflammation that presents with joint pain

Systemic sx include rash, pleuritis, swollen lymph nodes, spleen enlargement, and serositis

Enthesitis

Form of JIA where inflammation present at tendon-to-bone insertions. Commonly affects heels and knees

May co-occur with juvenile spondyloarthritis, juvenile ankylosing spondylitis

Sx caused by T calls creating inflammation in tendons and subchondral bone marrow

Goals of pharm management

Induce remissions, control arthritis, prevent jt erosion, manage sx outside of jts

Methotrexate (MTX)

Commonly used med for polyJIA and sJIA

Signs of compensatory motor behavior (JIA)

Unusual WBing in crawling, scooting instead of walking, flexed knee posture in gait

Nociceptive pain

Pain that signals a threat to the body’s tissues in response to actual or perceived noxious stimuli

Neuropathic pain

Pain caused by lesions or disease in the somatosensory nervous system

Nociplastic pain

Pain from altered nociception, not due to tissue or nervous system damage

Pain threshold

The minimum intensity of stimuli needed to elicit a pain response

Sensitization

Increased pain response of nociceptors to normal or subthreshold levels of input

Central sensitization

Increased responsiveness of nociceptive neurons in CNS to normal or subthreshold input