systemic disorders

systemic disorders of joint destruction pathology

inflammatory and involves synovial membranes, cartilage, joint capsule, and surrounding ligaments and tendons

• components of the joint space are being attacked by the immune system

generally manifestations involve what?

multiple joints and other connective tissue structures and organs (also symmetric)

common etiology for systemic joint disorders

autoimmune or genetic disorder

diagnostics for systemic joint disorders

several non-specific lab values indicate the presence of a systemic inflammatory condition

• we use labs to identify inflammatory agents to diagnose for systemic arthritis

what is rheumatoid arthritis?

chronic, systemic autoimmune inflammatory disease (most common)

what does the inflammatory response lead to in rheumatoid arthritis?

accumulation of immune cells and production of abnormal antibody Rheumatoid Factor (RF) and infiltration of the synovium

• congestion and edema of synovial membrane and joint capsule

• fibrous ankylosis, bone atrophy and misalignment → restricts ROM

• fibrous tissue calcifies → bony ankylosis and total immobility

what forms as a result of rheumatoid arthritis?

pannus forms: thick granulation tissue (new cells that don’t belong there)

covers and invades cartilage, destroys joint capsule and bone (destroying joint from inside out)

diagnostic studies for rheumatoid arthritis

labs: RF is measurable in blood (80%), ESR elevated, low C3 or C4 level, synovial fluid has high WBCs

XR reveal bone demineralization and deformities

clinical s/s of rheumatoid arthritis

bilateral symmetric polyarthritis involving smaller joints

diffuse musculoskeletal pain improves with activity

• moving makes them feel better (opposite of osteoarthritis)

warm erythema at joints

malaise

fatigue

sjogren syndrome (lacrimal and salivary gland dysfunction)

• dry eyes and mouth

tx options for rheumatoid arthritis

NSAIDs

corticosteroids

DMARDs (methotrexate) → immunosuppressant

TNF blockers (Humira)

surgical release of tendon

what is systemic lupus erythematosus (SLE)?

chronic, multi-system, inflammatory autoimmune disorder of connective tissue (can happen anywhere in the body)

• may have exacerbations and remissions

what happens in systemic lupus erythematosus (SLE)?

overactive lymphocytes cause antigen-antibody complexes form in basement membrane → leads to further inflammatory destruction

• immune cells are causing changes in the connective tissue

• ligaments, tendons, and capsule deformities

diagnostics for systemic lupus erythematosus (SLE)

elevated ANA, elevated ESR, low C3 and C4 levels, low RBCs, low WBCs, ad lot Plts

clinical s/s of systemic lupus erythematosus (SLE)

may be acute or insidious

polyarthralgia in 90% (arthritis multiple joints)

jaccoud arthropathy

butterfly rash

photophobia

fatigue

pulmonary and cardiac conditions

renal insufficiency

blood dyscrasias

sjogren syndrome

tx options

NSAIDs

glucorticoids

immunosppressants

antimalarials

methotrexate

what is scleroderma?

multisystem, inflammatory connective tissue disease

idiopathic

what does scleroderma affect?

adversely affects skin, blood vessels, synvoium, and microvasculature of internal organs

smooth muscle proliferation → thickened vessel walls constricting blood flow → ischemia → overactive fibroblasts depositing excess collagen leads to fibrosis

diagonstic testing for scleroderma

+ANA, thermography, microvacular imaging

early clinical s/s of scleroderma

Raynaud’s syndrome → common secondary issue because smooth muscle is constricting the blood vessels

polyarthritis in small joints

bilateral edema of fingers/hands/feet

what do the scleroderma s/s progress to?

sclerodactyly (skin tightens and thickens)

flexion contractures

limited ROM

what are the advanced s/s of scleroderma?

muscle atrophy

pulmonary fibrosis and pulmonary hypertension (lungs have lots of blood vessels and smooth muscle)

CHF

pericarditis (heart has lots of blood vessels and smooth muscle)

renal failure

CREST syndrome

tx options for scleroderma

trying optimize circulation → relaxing the smooth muscle and blood vessels

vascular issues: vasodilators

GI issues

• antacids

• PPIs

• promotility Rx

renal issues: ACE inhibitors

pulmonary issues: prostaglandins

what is ankylosing spondylitis (AS)?

fusion of inflamed vertebra (spontaneously), progressive systemic disorder that may affect organs

• M > F (2-1)

• late teens to 20+ years (shows up early in life)

how does ankylosing spondylitis begin?

abnormal inflammatory process in sacroiliac joints and vertebral ligaments that eventually leads to abnormal bone remodeling and deposition causing fusion of vertebra

AS pathogenesis

insidious onset of pain, stiffness, immobility usually begins with the lower back or buttocks

may progress into the upper spine, chest, and neck

over time, the vertebrae may grow together (fuse), causing the spine to become rigid and inflexible → “bamboo spine”

other joints, such as the hips, shoulders, and knees may also be affected

as the spine shrinks, what can it affect?

ribs get tight → can’t take a nice deep breath

clinical s/s of AS

low back pain relieved with activity (not relieved with rest)

severe s/s upon walking

limited ROM in low back

asymmetric peripheral arthritis due to overwork

other tissues/organs AS affects

eyes (uveitis)

aorta and aortic valve

nerve compression

tx options for AS

stretching

ROM exercise

NSAIDs

corticosteroids

methotrexate

joint replacement

what is psoriatic arthritis (PsA)

inflammatory autoimmune disorder

genetic:

• M = F

• 30 - 55y

what is symmetric PsA?

appears in some joints bilaterally

what is asymmetric PsA?

does not appear in same joints bilaterally

what is distal PsA?

local appearance near ends of fingers and toes, nail beds

what is spondylitis PsA?

specific appearance in spine and neck

what is arthritis mutilans PsA?

severe deformities of small joints in fingers and toes

clinical s/s of PsA

patches of thick, red skin that are often covered by silver or white scales

stiffness in the joints, low back, or neck is usually worse in the morning and lasts >30 min (improves with movement)

• may affect joints on one or both sides of the body

• typically affects>1 joint