31 Questions
Which autoimmune disease is characterized by skin thickening and can be classified into localized, limited, and diffuse types?
Systemic Sclerosis
What is the leading cause of death in patients with Systemic Sclerosis?
Pulmonary hypertension
Which of the following diseases involves collagen synthesis disorder and can affect heart valves?
Ehler’s-Danlos Syndrome
What is a potential concern for patients with Marfan Syndrome during anesthesia due to their condition?
AI Aortic dissection
Why is the presence of pulmonary hypertension considered an ominous sign in Systemic Sclerosis patients?
It suggests advanced disease and increases perioperative risk
What is a common symptom of rheumatoid arthritis with TMJ and cricoarytenoid involvement?
Limited mouth opening
In Rheumatoid Arthritis, which joints are typically more affected?
Distal joints more than proximal
Which autoimmune disease is known for 'butterfly rash' across the cheeks?
Lupus (SLE)
What can result from chronic pulmonary emboli and vasculopathy in Lupus?
Pulmonary hypertension
Which autoimmune disease has a high risk of thromboembolic events despite a prolonged PTT?
Lupus (SLE)
Systemic Sclerosis is another term for Scleroderma.
True
Raynaud Syndrome is primarily an anesthetic issue during surgery.
False
Ehlers-Danlos Syndrome is characterized by joint hypermobility and vascular fragility.
True
Marfan Syndrome commonly involves short stature and joint stiffness.
False
Pulmonary hypertension is a lesser concern in patients with Systemic Sclerosis.
False
Rheumatoid Arthritis can lead to limited mouth opening and hoarseness due to cricoarytnoid cartilage involvement.
True
In patients with Rheumatoid Arthritis, atlantoaxial subluxation occurs in up to 5% of cases.
False
Lupus nephritis is an early-stage disease and does not progress to end-stage renal failure.
False
Systemic Lupus Erythematosus patients with a long PTT have a low risk of thromboembolic events.
False
______ is the leading cause of death in patients with Systemic Sclerosis.
Pulmonary hypertension
______ Syndrome is characterized by exaggerated vascular response to cold/stress.
Raynaud
______ is a disorder of collagen synthesis that can involve heart valves.
Ehlers-Danlos Syndrome
There is a high correlation between CAD and RA.
True
Rheumatoid arthritis pressure-volume loops can mimic obstructive lung disease patterns.
False
Which of the following is a progressive, inflammatory disease that primarily impacts spine and sacroiliac joints?
Ankylosing spondylitis
Ankylosing spondylitis can cause restrictive lung disease, and affects females much more than males.
False
Which disorder/disease is a systemic autoimmune disease caused by vasculitis and can result in an end-stage renal disease?
Systemic lupus erythematosus
Which sign specifically in an SLE patient requires further workup?
cough, dyspnea, atypical chest pain, orthopnea
Which disorder/disease is a disorder of collagen synthesis and exhibits joint hypermobility? 1
Ehler-Danlos syndrome
What specific workup do patients with Marfan Syndrome need?
cardiac evaluation!!
Which type of Ehler-Danlos Syndrome is more serious because of vascular fragility?
IV
Study Notes
Musculoskeletal and Autoimmune Evaluation
- Two key components: Deformities and Chronic inflammation
- Chronic inflammation can lead to vasculitis and multiorgan dysfunction, affecting CV, pulmonary, renal, hepatic, integumentary, GI, central nervous, and peripheral nervous systems
Rheumatoid Arthritis (RA)
- Chronic inflammation in joints and multiorgan involvement
- Women are affected twice as much as men
- Distal joints are more affected than proximal joints
- TMJ and cricoarytenoid cartilage involvement
- Limited mouth opening, hoarseness, and limited range of motion
- Atlantoaxial subluxation can occur in up to 46% of patients, leading to atlantoaxial instability and cervical myelopathy
- Fever, chronic fatigue, migratory arthritis, and dermatologic manifestations (alopecia, photosensitivity, "butterfly rash")
Systemic Lupus Erythematosus (SLE)
- Chronic inflammation leading to multiorgan involvement
- Interstitial lung disease, pleural effusions, infections, and pulmonary hypertension are common
- Pulmonary hypertension results from chronic pulmonary emboli and vasculopathy
- Premature CAD, pericarditis, myocarditis, and cardiomyopathy
- HTN is difficult to control, and lupus nephritis is end-stage disease
- Anemia and leukopenia are common
- Exam focuses on evaluation of all major organ systems
- Cough, dyspnea, atypical chest pain, and orthopnea require further workup
- Despite long PTT, high risk of thromboembolic events
- Neuro exam including central and peripheral neuropathy/deficits
- EKG, Lytes, CBC, and PTT are indicated
- Frequently have antiphospholipid antibodies, resulting in frequent thromboembolic events
Systemic Sclerosis (SSc)
- Autoimmune disease causing excessive fibrous tissue deposits
- Skin thickening and Raynaud syndrome are common
- Three classes: Localized (just skin), Limited (skin of face and UE, GI involvement), and Diffuse (generalized skin, multiple end-organs)
- Heart failure (R>L), Coronary A fibrosis, and pericarditis
- Pulmonary hypertension is the leading cause of death
- Presence of pulmonary hypertension is an ominous sign and increases perioperative risk
- Airway involvement due to fibrosis
Raynaud Syndrome
- Exaggerated vascular response to cold/stress
- Associated with connective tissue diseases (95% of patients with systemic sclerosis and 30% of patients with Sjogren disease/SLE)
- Cutaneous vasospasm
- Raynaud itself is not an anesthetic issue, but comorbid diseases are a concern
- Very different from peripheral vascular disease
Ehler's-Danlos Syndrome
- Disorder of collagen synthesis
- Joint hypermobility
- Type IV disease is more serious due to vascular fragility
- Type VI involves muscle weakness, scoliosis, and osteopenia
- Can involve heart valves
- Focus on positioning issues
Marfan Syndrome
- Tall, long digits, scoliosis, and valvular heart disease
- AI, aortic dissection, MVP, and MR
- These patients need a complete and thorough cardiac evaluation
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