connective tissue diseases in acute care

what is connective tissue

1 or the 4 basic tissue types in the body (nervous, muscle, epithelial, and connective tissue)

what is the purpose of connective tissue

-protects (skeletal)

-supports

-binds (anchor in the body -ex:GI)

-transport (blood -O2 and nutrients, waste)

-immunity (lymphatic - WBC)

what are the 5 types of specialized connective tissue

-bone

-adipose

-cartilage

-blood

-reticular

what are the 3 things that make up connective tissue

cells, fibers and extracellular matrix

what are symptoms of connective tissue disorder

-joint pain and stiffness

-muscle weakness

-skin rashes or texture changes

-organ dysfunction and/or damage

*symptoms are everywhere there is CT

what are the non-specific constitutional symptoms of connective tissue disorder

-fatigue

-SOB

-malaise

-fever

-weight loss

do all patients with connective tissue disorders have the same symptoms

no

what pattern can be seen with connective tissue disorders

relapse/remit

-can have acute worsening

____ diagnosis is crucial for best outcome

early

what are the 2 subcategories of connective tissue diseases

inherited

autoimmune

what are the types of inherited connective tissue disorders

-ehlers-danlos syndrome

-marfan syndrome

-osteogenesis imperfecta

what are the types of autoimmune connective tissue diseases

-systemic lupus erythematosus (SLE)

-polymyositis and dermatomyositis

-scleroderma

-rheumatoid arthritis (RA)

-sjogren’s syndrome

-vasculitis

-ankylosing spondylitis

-mixed connective tissue disease

lupus epidemiology

85% women

most often occurs in black women → latinas→white women

what is the onset of lupus

20-40 y/o

what is the pathology of lupus

autoimmune

likely triggered by UV light, smoking/pollution, no strong genetic markers

lupus leads to ____ lifespan and ____ QOL

shortened; worse

symptoms of lupus

Skin and Hair: photosensitivity, butterfly rash, alopecia, nail integrity, Raynauds

Heart: pleuritic chest pain, murmurs, pericarditis, myocarditis

Kidneys: inflammation, failure **poor prognosis

Joint and muscles: swollen joints, arthritis **often first sign

Mental: cog dysfunction, fever, seizures, psychosis, headaches, dizziness

Lungs: pleuritic chest pain, pneumonia, inflammation

Blood: inflammation, anemia, blood clots

Mouth: ulcers

how is lupus diagnosed

1. labs

   1. tissue biopsy

what labs are taken for lupus

1. antinuclear antibodies (ANAs): high

2. CBC: low RBC, low WBC, low platelets

3. erythrocyte sedimentation rate (ESR): high

4. C-reactive protein (CRP): high

5. antibody labs: done for initial diagnosis

what are the medications for lupus

1. antimalarial meds : decrease antibodies (hydroxachloraquine)

2. steroids (topical or oral): decrease pain/inflamation/WBC activity (prednisone)

3. NSAIDS: decrease inflammation/pain (ibuprofen/naproxen)

4. immunosuppressives: stop immune system from attacking, organ involvement (methotrexate)

5. blood thinners: decrease blood clots (coumadin)

6. monoclonal antibodies (mAbs): protein in lab to find a substance in body

7. Acthar gel: decreased inflammation, help make cortisol

when is pregnancy advised for pt with lupus

when SLE is well controlled and no teratogenic meds are being used

what is the medical treatment for lupus

1. diet: increase vitamin B6, vitamin C. Mediterranean diet

2. smoking cessation

3. mental health support

4. exercise

physical therapy intervention

-variability of disease process (flares), organ involvement can make consistent activity plan challenging

-should be educating on benefits of increased activity

-environment: UV protection, layers if Raynauds

-avoid using joints that are flared - should be checked for osteonecrosis

-caution if pt is anticoagulated/thrombocytopenia

-monitor if having cardio/pulm sxs

-currently no specific outcome measure for this population

PT intervention goal

-work up to 150-300 min of moderate intensity activity (40-75% HR)

-strength 2+ days/week

-stretch

-3-5 sessions per week

polymyositis/dermatomyositis epidemiology

-women 2-3X more than men

-incidence 5-10 per 1 million per year

-prevalence 10-20 per 100,000

polymyositis/dermatomyositis onset

5-14 years and 45-65 years

polymyositis/dermatomyositis pathology

autoimmune

likely genetic predisposition, triggered by UV exposure, infection, malignancy drugs

polymyositis/dermatomyositis clinical picture

-idiopathic inflammatory myopathies

-muscle weakness and distinctive skin rash

-ILD affects 20% pts

-coexisting internal malignancy in 10-20% cases

-dysphagia (neck inflammation)

-inflammation of heart (myocarditis)

-inflammation of blood vessels of other tissues/organs such as GI, eyes, and kidneys

-symmetrical proximal weakness w/in 1 year of 80% of cases

-joint pain

dermatomyositis skin sxs

1. heliotrope rash: redness around eyes

2. gottrons papules: reddened areas on fingers

3. Shawl sign: plaques and patches on upper back

polymyositis/dermatomyositis weakness pattern

**hallmark sign

1. LEs: quads and glutes

2. deltoids and biceps

3. neck flexors

how is polymyositis/dermatomyositis diagnosed

1. labs

   1. interferon (IFN): high

   2. serum muscle enzymes (CK, lactate)

   3. troponin for heart involvement (if CK elevated)

   4. ANA as a rule out to lupus

   5. myositis specific autoantibodies

   6. screen for cancer

2. EMG -fibrillations

3. PFTs

4. MRI - muscle anatomy/edema

   1. muscle biopsy

medical diagnosis of ILD

-assessed by CO of chest- “ground glass”, may have pneumonia

-respiratory failure can progress quickly (vent or ECMO)

may get severe enough for transplant

medical treatment for polymyositis/dermatomyositis

1. manage any malignancy or ILD first

2. meds

   1. high dose oral prednisone: 1st line therapy, not effective for skin

   2. IVIG: 1st line for skin issues, effective for myositis too

   3. methotrexate: decrease skin issues

   4. mycophenolate mofetil: skin and muscle issues, 1st line w/ ILD

   5. antimalarials

   6. topical corticosteroids

PT interventions

-exercise does NOT make pts w/ myositis worse →increases peripheral blood anti-inflammatory cytokines

1. cardiovascular training

   1. increased VO2max (walking, biking)

   2. improved mitochondrial function at the skeletal muscle

2. pulmonary training

   1. wean off vent/ECMO →O2 management

   2. diaphragmatic strengthening

3. strength training

   1. concentric strength preferred, 20-30% 1RM

   2. low load high rep

   3. blood flow restriction is safe

scleroderma etiology

-unclear, no genetic markers

-likely driven by vascular changes

-swollen fingers and raynaud’s = edema replaced by fibrotic tissue

scleroderma epidemiology

-incidence: 1-2 per 100,000 in US

-4:1 female to male ratio

scleroderma onset

30-50 yo

scleroderma pathology

autoimmune

scleroderma clinical picture

1. pulmonary fibrosis/ILD, pulmonary arterial hypertension (70%)

   1. 5x increased risk of lung cancer

2. pericarditis, arrhythmias

   1. 7-20% have 5 year mortality rate

3. GI involvement (90%)

4. neuropathies and CNS involvement

5. joint pain, immobility, contractures

scleroderma “CREST” sxs

Calcinosis: calcium deposits on skin

Raynaud’s: spasm of blood vessels in response to cold/stress

Esophageal dysfunction: acid reflux and decreased motility

Sclerodactyly: thickening and tightening of skin on fingers/hands

Telangiectasias: dilation of capillaries causing red marks on skin

scleroderma medical diagnosis

1. skin assessment- tightness

2. labs

   1. positive antinuclear antibody (ANA)

   2. specific antibody testing

3. CXR and PFTs screening for pulmonary involvement

4. punch biopsy

scleroderma medical treatment

1. UVA light therapy

2. meds

   1. immune modulation: high dose oral prednisone and low dose oral methotrexate

   2. inflammation: histamine 1 and histamine 2 blockers

   3. neuropathic pain: doxepin (oral)

3. education: avoid skin trauma (fingers), cold exposure, and smoking

scleroderma PT intervention

1. contracture management (hands, shoulders, face)

2. pain management

3. endurance training

4. balance training (adaptive equipment)

connective tissue disorders are most commen in

women in early-mid adulthood

commonalities of connective tissue diseases

1. skin involvement (each version different)

2. systemic involvement: lungs, heart, blood vessels, muscles, nerves

3. Raynauds

all connective tissue disorders use what type of meds

1. steroids and immunosuppression

increased risk of infection and increased risk of steroid induced muscle wasting