Pathology of Joints/Muscles

Signs and symptoms of Scleroderma

Signs and symptoms of Scleroderma

• Calcinosis

• Raynaud’s Phenomenon

• Esophageal dysfunction

• Sclerodactyly

• Telangiectasis

Calcinosis

Calcium deposits in the skin

Raynaud’s Phenomenon

Spasms of blood vessels in response to cold or stress; sudden blanching, cyanosis, and erythema of the fingers and toes as the walls of blood vessels supplying the hands and feet become narrowed

Esophageal dysfunctions

Acid reflux and decrease in motility of esophagus

Sclerodactyly

Thickening and tightening of the skin on the fingers and hands

Telangiectasis

Dilations of capillaries causing red marks on surface of skin

Pathogenesis of Scleroderma

• Excessive collagen deposits within blood vessels, pericapillary space and within the skin, causing scarring and fibrosis

• Tied to the skin and vasoconstriction, so it distinguishes from other autoimmune disorders

How is the skin affected by Scleroderma?

Skin becomes tight, smooth, and waxy or shiny and seems bound down to underlying structures

What are the neuromusculoskeletal manifestations of scleroderma?

• Disuse atrophy of muscle because of limited joint motion secondary to skin, joint, or tendon involvement

• Arthralgia affects the wrist, hands, ankles, and knees

How is the viscera affected by scleroderma?

• GI motility dysfunction affects the esophagus and anorectal regions, causing reflex, heartburn, dysphagia, and bloating after meals

• Cardiac involvement can be manifested as myocardial or pericardial disease, conduction system disease, or arrhythmias

• Pulmonary involvement is characterized by pulmonary arterial hypertension and pulmonary vascular disease

Implications for functional performance and rehabilitation professionals’ role in Scleroderma

• Mitigate risks to mobility through exercise and joint protection techniques

• Skin protection

• Stress management

• Monitor vitals signs with activity

Osteoarthritis

Slowly evolving articular disease that appears to originate in the cartilage and affects the underlying bone, soft tissues, and synovial fluid. Leads to loss of mobility, chronic pain, deformity, and loss of function

Pathogenesis for Osteoarthritis

• Loss of hyaline cartilage, hypertrophic changes in neighboring bone and joint capsule, synovial inflammation, and focal calcifications of the cartilage

• Progressive loss of cartilage, inflammation develops, with resulting bony overgrowth, ligament laxity, and progressive muscle weakness and atrophy accompanied by joint pain

• Joint space narrows

Etiological factors for Osteoarthritis

Multifactorial, including biomechanics injury, biochemistry, previous injury, inherited predisposition. Injuries to joint ligaments and meniscus, joint malalignments, and subsequent decreases in muscle length

Clinical manifestations of Osteoarthritis

• Begins in joints on ONE side of the body

• Primarily the hips, knees, spine, hands, feet

• Inflammation, redness, warmth, swelling

• Brief morning stiffness

• No system symptoms

Incidence for Osteoarthritis

40 year old and older; affects most adults over the age of 65

Onset for osteoarthritis

Gradual onset

Implications for functional performance/rehabilitation professionals’ roles for Osteoarthritis

• Poor correlation between the extent of radiographic degenerative changes the presence of symptoms

• Joint protection

  • Pt’s must minimize the mechanical stress on the involved joint(s). can do this by using postural supports, knee braces, and orthoses during an exercise program to vary the stresses

  • Proper posture

  • Evaluating the need for assistive ambulatory device, a shock-absorbent shoe insert, or heel wedge, etc

• Exercise

  • Attempts to alleviate pain through pharmacologic or physical modalities may not improve symptoms unless accompanied by some form of physical conditioning

  • Educate pt to use ice prior to exercise and to incorporate a program of submaximal exercise to warm up before beginning exercise program.

Rheumatoid arthritis is what kind of disease?

Chronic systemic inflammatory disease

What are the etiological factors of Rheumatoid Arthritis?

Unknown, combination of genetic and environmental factors

For Rheumatoid Arthritis, _______ are 3x more likely get this disease than _______.

Women; men

Pathogenesis of Rheumatoid Arthritis

Joint inflammation because of massive infiltration of immune cells, especially T lymphocytes, into the synovial fluid

Clinical manifestations of Rheumatoid Arthritis

• Symmetrical joint distribution

• Can affect any joint but tends to be in upper extremities

• Inflammation

• Prolonged morning stiffness

• System presentation: fatigue, weight loss, fever

Onset for Rheumatoid Arthritis

May be sudden or progress over weeks to months; exacerbation/remission cycle

Rheumatoid Arthritis is more debilitating for who?

Men

What condition affects multiple joints and deformities in the fingers are common (like ulnar deviation, swan neck and boutonniere)?

Rheumatoid Arthritis

Implications for functional performance/rehabilitation professionals’ roles for Rheumatoid Arthritis?

• Exercise prescription

  • Moderate-level exercise for 30 minutes per 4 or 5 days to increase physical fitness

  • Lack of exercise → increased joint stiffness, muscle weakness, muscle atrophy, increased risk of fracture and deformity

• Strength and aerobic training

  • Dynamic strengthening (gradually increasing resistance) through the full available ROM improves muscle strength, joint stabilization, and physical function

  • Low load resistive muscle training

  • Aerobic and aquatic exercise

• Modalities

  • Preparatory for exercise/activity participation

  • Cold is more suitable but heat is typically preferred

  • Superficial heat (e.g, paraffin baths, moist hot packs, hydrotherapy, aquatic therapy) is recommended for acute flare-ups

  • Electrotherapeutic modalities and thermotherapy physical agents are used to obtain pain relief, control inflammation, and reduce joint stiffness

Etiological factors of Rhabdomyolysis

Prolonged immobilization in older adults after a fall

Pathogenesis of Rhabdomyolysis

• Rapid breakdown of skeletal muscle tissue because of a mechanical, physical, or chemical traumatic injury

• Large release of the creatine phosphokinase enzymes, myoglobin, and other cell byproducts into the blood system

  • Acute renal failure

Clinical manifestations of Rhabdomyolysis

• Excessive fatigue and inability to recover from a strenuous event

• Muscle pain (myalgia) and weakness ranging from mild to severe

• Change in the color of urine

Implications for functional performance/Rehabilitation professionals’ roles for Rhabdomyolysis

• Pt with a history of exertional rhabdomyolysis: should be cautioned regarding participation in extreme exertional activities and event

• Recovery from statin-induced rhabdomyolysis should prompt the therapist to assess the individual for impaired muscle performance, especially proximal muscle weakness including the inspiratory muscles

• Fall-related rhabdomyolysis:

  • Why did they fall?

  • How long did they stay on the ground?

Polymyalgia Rheumatic

Diffuse pain and stiffness in multiple muscle groups that primarily affect the shoulder and pelvic girdle musculature

Risk factors/incidence for Polymyalgia Rheumatic

• Female, age, and race

  • Women are affected twice as often, the disease is rare before 50 years old; most cases occur after 70 years old

  • White women

Etiological factors of Polymyalgia Rheumatic

Unknown cause, genetic factors, infection, or an autoimmune malfunction may play a role

Pathogenesis of Polymyalgia Rheumatic

• Despite complains of pain and stiffness in muscles, this disease is NOT associated with any histologic abnormalities

  • Arching and stiffness typical of this condition are caused by joint inflammation

What medication do patients with polymyalgia rheumatic respond to rapidly?

Prednisone

The diagnosis of what condition is based on the presence of a constellation of finding and the pt’s response to a trial of prednisone?

Polymyalgia Rheumatic

Clinical manifestations of Polymyalgia Rheumatic

• May begin gradually, taking days or weeks for symptoms to become fully evident, but more often it develops suddenly and the person wakes up one morning feeling stiff and sore for no apparent reason

• Symptoms are often bilateral and symmetrical, affecting the neck, sternoclavicular joints, shoulders, hips, low back, buttocks

• Painful stiffness lasts more than 1 hours in the morning on arising and is a hallmark feature of this disorder

• Muscle weakness is not the problem. Pain and stiffness are the primary issues.

Implications for functional performance/Rehabilitation professionals’ roles for Polymyalgia Rheumatic

• Therapist must be aware of the potential risk of giant cell or temporal arteritis

  • An adult older than 65 with sudden onset of temporal headaches, exquisite tenderness over the temporal artery, scalp sensitivity, or visual complaints should be seen by a physician immediately as this vasculitis is associated with stroke and blindness

• Medical adherence

  • Ensure that patient is taking prednisone as directed

• As the disease progresses, ADLS become difficult. Bed mobility and sit-to-stand transfers are affected

• Inflammatory response involving bursitis and tenosynovitis therefore use ultrasound as a deep heating agent in the presence of inflammation

Connective tissue disease

May present as a single entity or may have features of multiple autoimmune diseases

Etiological factors and pathogenesis of Connective tissue disease

• Unknown but possibly self-antigens and infectious agents in the pathogenesis have been suggested

• Most found in women younger than 50 years old

Implications for functional performance/Rehabilitation professionals’ roles for Connective tissue disease

• The younger onset of age means people with it will likely be working, might be parents

  • Work modification/energy conservation

  • Psychological/emotional support

• Pts should be encouraged to develop regular exercise habits and participate in an active lifestyle

Clinical manifestations of Connective tissue disease

• Combines features of

  • Lupus (rash, Raynaud phenomenon, arthritis, arthralgia)

  • Scleroderma (swollen hands, esophageal hypomotility, pulmonary interstitial disease)

  • Polymyositis (inflammatory myositis)

  • Polyarthralgia

• Pulmonary, cardiac, and renal involvement

• Trigeminal sensory neuropathy appears to occur much more frequently than in other rheumatic diseases

Gout

Elevated level of serum uric acid and the deposition of urate crystals in the joint, soft tissues, and kidneys

Etiological factors of Gout

• Unexplained impairment in uric acid excretion by the kidneys

• Family history and age increases risk

• Heavy alcohol consumption, obesity, renal insufficiency

Pathogenesis of Gout

• In healthy people, uric acid dissolves in the blood, passes through the kidneys and then is excreted through the urine

• If the body produces more uric acid than the kidneys can process or if the kidneys are unable to handle normal levels of uric acid, the acid level in the blood rises

• High levels of uric acid in the blood may precipitate out and accumulate in body tissues, forming supersaturated body fluids, including the joints and kidneys

• Crystal aggregates triggers an inflammatory response, resulting in local tissue necrosis and a proliferation of fibrous tissue

Uric Acid

Substance that normally forms when the body breaks down cellular waste produced called purines

Asymptomatic Hyperuricemia

Serum urate of more than 7 mg/dL

Acute Gouty Arthritis

• Acute, monoarticular, inflammatory arthritis manifested by exquisite joint pain occurring suddenly at night

• Erythema, warmth, extreme tenderness, and hypersensitivity are typically present

• First metatarsophalangeal joint (i.e., big toe) is a common site of pain, ankle, instep, knee, wrist, elbow, and finger all can be the site of the initial attack

What is the common site of pain for acute gouty arthritis?

Big toe

Intercritical Gout

Asymptomatic phase, can last months or years

Chronic Gout

Can enter the chronic phase up to a decade after the initial attack, characterized by joint damage, functional loss, and disability

Implications for functional performance/rehabilitation professionals’ roles for Gout

• Education on the causes/risk factors

• Reduce obesity

• Onset of severe joint pain with a swollen, hot joint must always be a concern to therapist

  • Could be gout or septic joint

  • Septic Joint: orthopedic emergency, so when a red, hot painful joint is observed without prior medical diagnoses, immediate medical evaluation is necessary

Myopathy

Nonspecific muscle weakness secondary to an identifiable disease or condition

Critical Illness Myopathy

Associated with prolonged stays in intensive care units

Etiological factors and pathogenesis of Myopathy

• Idiopathic inflammatory myopathies are thought to be immune-mediated processes that are triggered by environmental factors

• Expression of proinflammatory cytokines and expression of MHC class I antigens on muscle fibers are associated with muscle weakness in individuals with active and chronic disease

• Diabetes is associated with myopathy of three origins: vascular, neurogenic, and metabolic

• Acquired myopathy can occur as post of a paraneoplastic syndrome

  • Tumors may produce muscle weakness with/without inflammation

• Medications

  • Cholesterol-lowering statins are associated with tendinitis and muscle abnormalities

Clinical Manifestations of Myopathy

• Progressive proximal muscle weakness with varying degrees of pain and tenderness

• Muscles may be acutely inflamed and painful to move and touch

• Muscle weakness and easy fatigability eventually compromise aerobic capacity and affect the person’s endurance and ability to work, social, and complete ADLs

Implications for functional performance/Rehabilitation professionals’ roles for Myopathy

• Acute care

  • The effects of ICU-acquired myopathy are even more pronounced in persons who are both on bed rest and critically ill

  • Psychologic and emotional support of the patient and the family

• Exercise

  • Early rehabilitation with careful application of rest and exercise

  • Begins in the acute phase with stretching and PROM

    • Upper extremity splinting and lower extremity bracing may be necessary to prevent contractures, prolong mobility, and enhance functional skills

  • Client education with energy conservation and joint protection.