Exam 2 Connective Tissue Disorders

What are the major clinical features common to connective tissue disorders?

• Joint pain/stiffness

• Muscle weakness

• Skin rashes/texture changes

• Organ dysfunction/damage

• Fatigue, SOB, malaise, fever, weight loss

What is the typical disease pattern of connective tissue disorders?

Often relapsing/remitting, with possible acute flares/worsening.

Why is early diagnosis important in connective tissue disease?

Early diagnosis is associated with better outcomes.

Why can connective tissue diseases affect multiple body systems?

Connective tissue is widespread and provides support, protection, binding, transport, and immune functions; therefore disease can affect joints, muscles, skin, blood vessels, and organs.

What common demographic pattern is seen across the 3 CTDs in this lecture?

They are most common in women, generally presenting in early-to-mid adulthood.

What organ systems commonly become involved in these CTDs?

Lungs, heart, blood vessels, muscles, nerves, and skin.

What vascular phenomenon is commonly associated with these CTDs?

Raynaud's phenomenon.

What type of disease is SLE?

Autoimmune connective tissue disorder.

Who is most commonly affected by SLE?

Women (~85%), with onset typically 20–40 years old.

How does SLE incidence vary by race according to the lecture?

Approximately:

• 1:400 Black women

• 1:800 Latina women

• 1:1,100 White women

What are potential triggers for SLE?

UV exposure, smoking/pollution; the lecture notes no strong genetic markers.

What are common clinical manifestations of SLE?

Think systemic disease: fatigue, joint/muscle symptoms, skin involvement, and potential organ dysfunction/damage.

What lab findings are associated with SLE?

• ↑ ANA

• ↓ RBCs

• ↓ WBCs

• ↓ platelets

• ↑ ESR

• ↑ CRP

What additional testing may help diagnose SLE?

Antibody testing and sometimes tissue biopsy of an involved organ.

What medications are commonly used to manage SLE?

• Antimalarials

• Steroids

• NSAIDs

• Immunosuppressives

• Blood thinners

• Monoclonal antibodies

• Acthar Gel

What non-pharmacological management is recommended for SLE?

• Mediterranean diet

• Vitamin B6/C

• Smoking cessation

• Mental health support

• Exercise

Why can exercise programming be challenging in SLE?

Disease flares and organ involvement make consistent activity plans difficult.

What is an important PT education point for patients with SLE?

Educate patients about the benefits of increased physical activity, while adapting activity to disease status.

What environmental considerations are important for SLE?

UV protection and appropriate layers if the patient experiences Raynaud's.

What should PT consider regarding joints during an SLE flare?

Avoid using severely flared joints and assess for osteonecrosis.

What precautions are important if an SLE patient is anticoagulated or thrombocytopenic?

Increased bleeding/bruising risk → modify treatment and monitor appropriately.

What should PT monitor if an SLE patient has cardiopulmonary involvement?

Cardiovascular and pulmonary symptoms, along with exercise tolerance.

What general exercise target is given for SLE?

Progress to:

• 150–300 min/week moderate activity

• 40–75% HRR

• Strength ≥2 days/week

• Stretching

• 3–5 sessions/week

What type of disease are polymyositis and dermatomyositis?

Autoimmune connective tissue disorders characterized by inflammatory myopathy.

What are possible triggers/etiologic factors of polymyositis and dermatomyositis?

Genetic predisposition with possible triggers including:

• UV exposure

• Infection

• Malignancy

• Drugs

What are the typical ages of onset for polymyositis and dermatomyositis?

5–14 years and 45–64 years

What is the sex distribution for polymyositis and dermatomyositis?

Women are affected approximately 2–3× more than men.

What are the hallmark clinical features of polymyositis/dermatomyositis?

Muscle weakness (symmetrical & proximal pattern) + distinctive skin rash.

Which muscle groups are commonly weak with polymyositis/dermatomyositis?

• Quads & glutes

• Deltoids & biceps

• Neck flexors

What percentage of patients develop symmetrical proximal weakness within 1 year?

80%

What important systemic complications can occur with polymyositis/dermatomyositis?

• ILD (~20%)

• Dysphagia

• Myocarditis

• Joint pain

• Vasculitis

• Internal malignancy (10–20%)

Why is dysphagia important in polymyositis/dermatomyositis?

It indicates involvement beyond skeletal muscle and creates additional functional/safety concerns.

Why is ILD important for PT?

It can cause significant respiratory impairment, and respiratory failure may progress rapidly.

What labs/tests may be used to evaluate polymyositis/dermatomyositis?

• ↑ Interferon

• ↑ CK/muscle enzymes

• Troponin → heart involvement

• ANA

• Myositis-specific antibodies

• EMG

• PFTs

• MRI

• Muscle/skin biopsy

Why might troponin be checked in polymyositis/dermatomyositis?

To assess for cardiac involvement/myocarditis.

What should be managed first in severe polymyositis/dermatomyositis?

Malignancy or ILD

What medications may be used for polymyositis/dermatomyositis?

• High-dose prednisone

• IVIG

• Methotrexate

• Mycophenolate mofetil

• Antimalarials

• Topical corticosteroids

Does exercise worsen muscle inflammation in myositis?

No. exercise increases peripheral blood anti-inflammatory cytokines.

What benefits can cardiovascular training provide for polymyositis/dermatomyositis?

Improved VO₂max and skeletal-muscle mitochondrial function.

What pulmonary PT interventions may be used with polymyositis/dermatomyositis?

• O₂ management

• Weaning from ventilation/ECMO when appropriate

• Diaphragmatic strengthening

What type/intensity of strengthening is preferred for polymyositis/dermatomyositis?

Concentric strengthening, approximately 20–30% of 1RM.

Is blood-flow restriction training considered safe in polymyositis/dermatomyositis?

Yes

What type of disease is scleroderma?

Autoimmune connective tissue disorder.

What is the etiology of scleroderma?

Unclear; no specific genetic markers. It is likely driven by vascular changes.

What happens to the fingers/skin in scleroderma?

Swelling + Raynaud's → edema is replaced by fibrotic tissue, causing tight/thickened tissue.

What is the typical epidemiology of scleroderma?

• Incidence: 1–2/100,000

• Onset: 30–50 years

• Female:male ≈ 4:1

What major pulmonary complications occur with scleroderma?

Pulmonary fibrosis/ILD and pulmonary arterial hypertension (~70%).

What cardiovascular complications can occur with scleroderma?

Pericarditis and arrhythmias.