University of Missouri
School of Health Professions
Physical Therapy 7890
Rheumatic Diseases
I.
Introduction
A.
Importance
1. more than 100 diseases
2. chronic, multisystem
3. affect people of all ages
4. once called collagen or connective tissue (CT) diseases
5. characterized by inflammation
6. autoimmunity and molecular mimicry
7. genetics or inheritance
8. rehabilitation personnel need to develop a collaborative relationship with patient; over time, different team
members may take lead as advisor
B.
II.
Some Major Classifications
1. inflammatory diseases (e.g. rheumatoid arthritis, systemic sclerosis)
2. juvenile arthritis (three types)
3. seronegative spondyloarthropathies (e.g. ankylosing spondylitis, reactive arthritis)
4. osteoarthritis (including traumatic arthritis)
5. vasculitis (e.g. polymyalgia rheumatica)
6. crystalline deposit diseases (gout, pseudogout)
7. Sjogren's Syndrome and Sicca Syndrome
8. infectous (viral, including HIV; bacterial)
9. fibromyalgia
10. miscellaneous diseases with CT component (e.g. sarcoidosis)
Seronegative Spondyloarthropathies:
A.
Common features
1.
HLA-B27 present
2.
seronegative refers to lack of Rheumatoid Factor (RF) in serum
3.
spine and sacroiliac involvement to varying degrees
4.
inflammation of the entheses (enthesis = singular)
a.
tissue site where tendons, ligaments and joint capsule merge with the periosteum and bone cortex
(Sharpey’s fibers)
b.
inflammation leads to eventual ossification of ligaments
and ankylosis of joints; bone spurs where tendons and
ligaments attach may form
5.
unique systemic involvement in the different diseases (ankylosing spondylitis, Reiter’s syndrome (reactive
arthritis), psoriatic arthritis, enteropathic arthritis (ulcerative colitis or Crohn’s disease), and Whipple’s disease
B.
Ankylosing Spondylitis
1.
Definition: a chronic inflammatory disease primarily of axial skeleton.
2.
Population: 90% male, diagnosed before age 40
a.
average age of diagnosis = 28
b.
females usually have less extensive spinal involvement
c.
high incidence in some native SW American tribes
(Pima); Blacks rare
3.
Early complaints: low back stiffness and pain often starting in the teen years
4.
Joint involvement: symmetric involvement of SI joints, vertebrae, shoulders, hips
5.
X-rays: SI joint pseudowidening (erosions) followed by ankylosis; bridging syndesmophytes between
vertebrae produce “poker spine” or “bamboo spine”
6.
Labs: positive blood test for HLA-B27.
7.
Course: gradual ankylosis of axial skeleton
8.
Prognosis: lifespan essentially normal. Extraarticular disease: iritis may threaten vision. About 75% remain
regularly employed.
9.
Medical management: NSAIDS; sulfasalazine. Regular eye exams.
10.
PT/OT: education: posture training and ROM exercise; adaptive equipment (see handout on areas to address)
C.
Psoriatic Arthritis (PsA)
1.
Definition: inflammatory arthritis associated with psoriasis
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III.
Population: men and women affected almost equally; onset 30-50
Appearance: psoriasis; DIP arthritis; nail lesions, including
pitting; sausage digits (toes); shortening and instability of digits (mutilans deformity; opera glass deformity)
Joint involvement: small joints hands and feet; SI, lumbar spine.
Radiographs: erosive arthritis (“pencil in cup” with bone destruction); asymmetric involvement of SI joints
Labs: no specific diagnostic tests
Course: exacerbation/remission or slowly progressive
Prognosis: variable, but life expectancy relatively unaffected
Medical Management: NSAIDS, DMARDs including methotrexate, BRMs, analgesics
PT/OT: education; ROM similar to RA; posture similar to AS; adaptive equipment; shoes; assistive devices for
gait; pool therapy; UV; pain management with modalities at home similar to RA
D.
Reactive Arthritis or Reiter’s Syndrome
1.
Definition: sterile joint inflammation after a sexually transmitted or GI infection (Salmonella, Shigella,
Yersinia, Campylobacter; Chlamydia, Ureaplasma) in a genetically-susceptible individual.
2.
Population: men 6:1 to women; Blacks less affected.
3.
Appearance: conjunctivitis, keratodermia blenorrhagicum; oral
and penile ulcers; sausage digits (toes) caused by tenosynovitis of
toe tendons;
4.
Joint involvement: asymmetric involvement of SI, lumbar spine.
5.
Radiographs: heel spurs; asymmetric involvement of SI joints
6.
Labs: no specific diagnostic tests
7.
Course: exacerbation/remission; can be indolent (not causing pain)
8.
Prognosis: life expectancy relatively unaffected
9.
Medical Management: NSAIDS, DMARDs including methotrexate, analgesics; local steroid injections.
10.
PT/OT: education; posture similar to AS; heel cups or lifts, arch supports, shoes suitable for calcaneal spur;
Achilles stretching
E.
Enteropathic Arthritis
1.
Description: arthritis associated with inflammatory bowel disease,
including Crohn’s disease and ulcerative colitis. Also Whipple’s
disease and intestinal bypass arthritis.
2.
Population: varies with the specific disease; in obese persons after bypass surgery (reversal of surgery
eliminates joint symptoms)
3.
HLA-B27 frequently present in persons with these diseases who also develop spinal, SI, and peripheral arthritis
4.
Management directed at the primary disease (including colectomy in ulcerative colitis). PT/OT similar to AS
if symptoms persist.
Inflammatory Diseases:
A.
Rheumatoid Arthritis
1. Definition: a chronic inflammatory disease with symmetrical polyarticular involvement,
morning stiffness, malaise, and fatigue
2. Population: females 2.5 X male rate; increasing incidence with age up to age 70; all races
3. Early complaints: symmetric polyarthritis, morning stiffness > 1 hr., fatigue, malaise; etiology may be linked to
viral infection (Gould)
4. Joint involvement: commonly 2nd and 3rd MCPs and PIPs; MTPs, wrists, knees, elbows.
5. Other system involvement: rheumatoid nodules; ocular, pulmonary, cardiac, vasculitis; Sjogren’s S.
6. Radiographs show symmetrical joint space narrowing, juxtaarticular erosions, osteopenia.
7. Labs: presence of rheumatoid factor (RF) in 85%; increased erythrocyte sedimentation rate (ESR); C-reactive
protein; hypergammaglobulinemia, anemia, eosinophilia, thrombocytosis, occasionally hypocomplementemia
8. Course: three general types; self-limited; persistent manageable; progressive with premature mortality. Usually
improves with pregnancy, worsens after. Exacerbation/remission typical. Advanced disease will ankylose, which
halts the inflammation, becoming a “burned out” joint.
9. Prognosis: depends on type; better with aggressive early medical/pharmacologic management
10. Medical management: NSAIDs, DMARDs, corticosteroids, BRMs, analgesics
11. PT/OT: education about exercise, rest, adaptive equipment, shoes, stiffness and pain management.
B.
Systemic Lupus Erythematosus (SLE)
1. Definition: a chronic, complex autoimmune inflammatory disease affecting multiple organ systems: skin, renal,
cardiovascular, pulmonary, neuropsychiatric and musculoskeletal
2. Population: women of childbearing age; outnumber male patients 10:1; higher incidence in Blacks and Hispanics.
3. Joint involvement or deformity:
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a. nondeforming laxity in fingers called Jaccoud's deformity
b. reversible, nondeforming ulnar deviation and swan necking can occur: Jacoud’s Arthropathy
c. avascular necrosis (usually hip; rarely, proximal humerus) with long term steroid use.
Labs: positive indirect antinuclear antibody (ANA); positive anti-dsDNA, Sm antigen; several other autoantibodies
possible.
Course: extremely variable. Pregnancy complicates management but is possible. Patients advised to avoid
pregnancy during a "lupus flare".
Prognosis: early detection and aggressive, appropriate management before major organ damage occurs has
dramatically improved life expectancy
Medical management: prednisone; NSAIDS (not Ibuprofen); sunscreen
PT/OT: need to understand what organ system is involved with each referral. Patient education. Energy
conservation measures; aerobic conditioning, post-op hip surgery; post-CVA; psychiatric OT.
C.
Systemic Sclerosis (PSS, SSc; Scleroderma)
1. Definition: a generalized disorder of the small blood vessels and diffuse connective tissues (ischemia) with excess
deposition of fibrous tissue involving skin and internal organs.
a. two types of systemic involvement: diffuse cutaneous systemic sclerosis (dcSSc) and limited cutaneous
systemic sclerosis (lcSSc or CREST syndrome).
b. CREST: chondrocalcinosis, Reynaud's phenomenon, esophageal dysmotility, sclerodactyly, telangiectasia-lcSSc has a better prognosis than dcSSc.
c. other forms of scleroderma (morphea, linear) can be disfiguring, but are not life-threatening to the children
who may develop them.
2. Population: age 30-50; all races, slightly more in Blacks; women 3-4:1
3. Early complaints: Reynaud's phenomenon; puffy fingers, nonpitting edema, and taut skin of hands; disease usually
slow in onset; rarely fast
4. Appearance: joints involved after skin changes render movement difficult. Puffy skin changes start in acral areas:
fingers, toes, nose, ears; disease progresses to involve entire body in dcSSc. Skin becomes leathery; tendons may
squeak upon movement; facial expression disappears.
5. Labs: serum antinuclear and anticentromere antibodies
6. Course: extraordinarily variable
7. Prognosis: when limited to skin, good. Renal SSc was cause of death for many until ACE inhibitors brought blood
pressure down. Now lung SSc often cause of death.
8. Medical Management: Symptomatic only. ACE inhibitors for renal disease; for Reynaud's, antispasmodics for
vascular spasm (reserpine)and vasodilators (diltiazem). Finger ulcers treated with antibiotics, nitroglycerine paste.
9. PT/OT: holistic team approach; education including finer points of ROM, including face, TMJ, thorax; pain
management; careful thermal Rx available at home; massage; selected joint mobilization ; work simplification and
energy conservation; adaptive equipment.
D.
Polymyositis and Dermatomyositis
1. Definition: uncommon disorders involving inflammation and degeneration of skeletal muscle resulting in weakness
and atrophy
2. Populations:
a. bimodal peaks at ages 5-14 and 45-65
b. more females than males
3. Complaints: weakness, difficulty standing up and climbing stairs, arthralgias, fever, muscle tenderness
4. Clinical appearance: fever, muscle weakness
a. dermatomyositis has skin features: heliotrope discoloration of periorbital area, especially eyelids; scaly rash
(Gottron's papules) over dorsum of joints, especially knees, MCP and PIP joints; sun exposure may exacerbate
a rash over face, neck, and back similar to SLE.
b. polymyositis does not have skin features, but may be associated with a cancer.
5. Labs: elevated serum enzymes CK and aldolase. Muscle biopsy. Electromyography
6. Course: extremely variable
7. Prognosis: 80% average survival is 5 years. Those with skin manifestations have a better prognosis. About half of
adult polymyositis patients will have an occult or preexisting malignancy. Successful Rx of the cancer may cause
myositis to abate.
8. Medical treatment; corticosteroids; occasionally azathioprine or methotrexate.
9. PT/OT: usually not needed if medicine brings disease under control; when needed, ROM, passive or active;
contracture management, graded strengthening; adaptive equipment.
E.
Polymyalgia Rheumatica / Giant Cell Arteritis / Temporal Arteritis
1. Definition: a systemic inflammatory disorder
2. Population: over 50s; 60% female
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3. Complaints: fatigue and aching of proximal joints; muscle pain, face pain (not headache)
4. Clinical appearance: muscle pain may cause appearance of weakness; with pain control, no
weakness
5. Labs: elevated ESR and anemia
6. Course: untreated may progress to Giant Cell or Temporal Arteritis which can cause blindness
7. Prognosis: in 1-2 years, symptoms usually subside
8. Medical treatment: prednisone; prompt resolution of symptoms (48-72 hr) helps confirm dx.
9. PT: recognize possibility of this disorder in a patient referred for neck/shoulder pain, as early
recognition is crucial to the prevention of vision loss
Juvenile Rheumatoid Arthritis
1. Classification:
a. Pauciarticular: <5 joints after 6 months of disease; 50% of cases; uveitis prevalent
b. Polyarticular: >5 joints after 6 months of disease: 30-40% of cases; joint erosion
c. Systemic-Onset JRA (Still’s Disease): fever, rash, arthralgia; can occur with pleuritis, pericarditis, anemia
2. Possible manifestations as adults:
a. Pauciarticular: osteoporotic; early arthroplasty of large joints (when epiphyses closed); joint ROM
limitations with resulting gait abnormalities; hyperemic overgrowth at joints can result in leg length, or
phalange discrepancies; vision impairments
b. Polyarticular: appearance more like adult RA; cervical fusion common; joint deformities
c. Systemic: growth retardation; multiple systems impaired
IV.
V.
Crystalline Deposit Diseases:
A.
Gout
1. Definition: deposition of monosodium urate monohydrate (MSUM) crystals in joints, kidneys, skin (tophi)
2. Population: 9:1 male to female; onset after age 40
3. Complaints: an exquisitely painful joint--MTP, ankle, knee
4. Clinical appearance: red, hot, tender. A tophus occurs only if untreated for many years
5. Labs: identification of MSUM crystals in synovial fluid or tophi.
6. Course: repeated attacks if medical management is inconsistent
7. Prognosis: satisfactory with adherence to regimen; joint and soft tissue destruction if not
8. Medical management: colchicine, antiinflammatory drugs, occasional intraarticular steroid
9. PT/OT: usually none needed; disease is incidental to another need for rehab
B.
Pseudogout
1. Definition: deposition of calcium pyrophosphate dihydrate (CPDD) in a joint1
2. Population: more women than men; age usually over 50. Most common cause of monoarticular synovitis in an
elderly person.
3. Complaints: exquisite joint pain, but not quite as as severe as gout. Knee most common, followed by wrist,
shoulder, ankle, elbow, 2nd and 3rd MCP joints.
4. Clinical appearance: joint is swollen, red, and warm (often mistaken for OA or gout).
5. Labs: synovial fluid analysis
6. Course: self limiting in 1-3 weeks
7. Prognosis: May recur; attacks provoked by many stressful incidents including illness, surgery, transfusion, etc.
8. Medical management: aspiration; intraarticular corticosteroid;
9. PT/OT: splinting; management similar to OA.
Osteoarthritis:
A. Definition: noninflammatory disorder of joints characterized by deterioration of articular cartilage and secondary new bone
formation. Secondary inflammation is common. Osteoarthrosis is the preferred term if there is no inflammation.
B. Populations: men have higher incidence before age 45; women have higher incidence after age 45. All races affected.
C. Types: primary (hereditary or genetic component) and secondary (acquired, traumatic). There is a lengthy classification list
that goes into detail.
D. Complaints: joint pain and stiffness; functional loss; change in appearance (hands, feet, knees)
E. Clinical appearance: joint enlargement, angulation, decreased ROM, crepitus, gait disturbance
F. Radiographs show asymmetric joint space narrowing, osteophytes, subchondral sclerosis, bone cysts.
** Note that severe radiographic signs correlate only ~50% with clinical reporting of painful symptoms.
G. Course: extremely variable; may even reverse as some cartilage regeneration may occur
H. Prognosis: compatible with full life span, but can interfere with desired activities
I. Medical and surgical treatment: analgesics; antiinflammatory drugs; surgical joint revision and replacement
J. PT/OT: education; exercise; pain management with rest, thermal agents, postoperative care . . .
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