Vasculitis

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Vasculitis

Cardiovascular system

Vascular disorders

Arterial disease

Angina pectoris

Stable angina

Unstable angina

Myocardial infarction

Prinzmetal angina

Coronary steal syndrome

Peripheral artery disease

Subclavian steal syndrome

Aneurysms

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Vasculitis

Behcet's disease

Kawasaki disease

Hypertension

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Renal artery stenosis

Coarctation of the aorta

Cushing syndrome

Conn syndrome

Pheochromocytoma

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Hypotension

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Abetalipoproteinemia

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Thrombophlebitis

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Lymphedema

Lymphangioma

Shock

Vascular tumors

Human herpesvirus 8 (Kaposi sarcoma)

Angiosarcomas

Congenital heart defects

Truncus arteriosus

Transposition of the great vessels

Total anomalous pulmonary venous return

Tetralogy of Fallot

Hypoplastic left heart syndrome

Patent ductus arteriosus

Ventricular septal defect

Coarctation of the aorta

Atrial septal defect

Cardiac arrhythmias

Atrial flutter

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Premature atrial contraction

Atrioventricular nodal reentrant tachycardia (AVNRT)

Wolff-Parkinson-White syndrome

Ventricular tachycardia

Brugada syndrome

Premature ventricular contraction

Long QT syndrome and Torsade de pointes

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Bundle branch block

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Valvular disorders

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Heart failure

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Pericardial disorders

Pericarditis and pericardial effusion

Cardiac tamponade

Dressler syndrome

Cardiac tumors

Cardiac tumors

Cardiovascular system pathology review

Acyanotic congenital heart defects: Pathology review

Cyanotic congenital heart defects: Pathology review

Atherosclerosis and arteriosclerosis: Pathology review

Coronary artery disease: Pathology review

Peripheral artery disease: Pathology review

Valvular heart disease: Pathology review

Cardiomyopathies: Pathology review

Heart failure: Pathology review

Supraventricular arrhythmias: Pathology review

Ventricular arrhythmias: Pathology review

Heart blocks: Pathology review

Aortic dissections and aneurysms: Pathology review

Pericardial disease: Pathology review

Endocarditis: Pathology review

Hypertension: Pathology review

Shock: Pathology review

Vasculitis: Pathology review

Cardiac and vascular tumors: Pathology review

Dyslipidemias: Pathology review

Assessments

Vasculitis

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USMLE® Step 1 questions

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Vasculitis

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Questions

USMLE® Step 1 style questions USMLE

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A 52-year-old man comes to the clinic due to fatigue, joint pain, and recent appearance of skin lesions on his legs. His medical history is noncontributory except for a car accident as a child which required hospitalization and multiple blood transfusions. He has not seen a physician in more than 10 years. The patient does not use tobacco, alcohol or illicit drugs. He is monogamous with one sexual partner. Temperature is 37.0°C (98.6°F), pulse is 75/min, and blood pressure is 130/85 mmHg. Physical exam shows purpuric papules covering the lower extremities that do not blanch with pressure. The rest of the physical examination is unremarkable. Laboratory tests are obtained and shown below:  

 
 Laboratory value  Result 
 Hemoglobin   15 g/dL 
 Leukocyte count   7,000/mm3 
 Platelets    250,000/mm3 
 ALT  110 U/L 
 AST  75 U/L 
 Rheumatoid factor   Positive   
 Antinuclear antibodies  Negative  
 
 Urinalysis is unremarkable. Serum protein electrophoresis and immunofixation reveals polyclonal IgG and monoclonal IgM bands. Testing for cryoglobulins shows no precipitation after 24 hours. Which of the following tests is most likely to reveal this patient's underlying condition?  

External References

First Aid

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Churg-Strauss syndrome p. 322

autoantibody p. 113

as granulomatous disease p. NaN

labs/findings p. 721

Transcript

Content Reviewers

Rishi Desai, MD, MPH

Contributors

Tanner Marshall, MS

Vincent Waldman, PhD

With vasculitis, you have “inflammation”, of the “blood vessels”, and even though this can happen in arteries or veins, we’re going to focus on vasculitis in arteries because it’s way more common.

Vasculitides—plural for vasculitis—are categorized by the size of the blood vessels they affect, so we have small-vessel, medium-vessel, and large-vessel vasculitis.

Typically vasculitis is due to an autoimmune disease, where the immune system confuses a part of normal body as a foreign invader, and there are a couple of ways this might happen.

Sometimes the body confuses the innermost layer of the blood vessel, which is the endothelial layer, with a foreign pathogen and directly attacks it.

To be a little bit more specific, the white blood cells of the immune system mix up the normal antigens on the endothelial cells with the antigens of foreign invaders like bacteria simply because they look similar—and this is called molecular mimicry.

This autoimmune confusion is thought to be the cause several types of medium-vessel and large-vessel vasculitides.

Other times the immune system attacks healthy cells that are near the vascular endothelium, and the endothelial cells are only getting indirectly damaged.

This is the situation in many small-vessel vasculitides, where the immune system attacks white blood cell enzymes or other non-endothelial cell targets.

Once the endothelium is damaged either directly or indirectly, almost all vasculitis diseases progress in a similar way.

The damaged endothelium exposes the underlying collagen and tissue factor, and these exposed materials increase the chance of blood coagulation.

The blood vessel walls themselves get weaker as they’re more damaged, making aneurysms more likely.

And finally as the vessel wall heals, it becomes harder and stiffer because fibrin is deposited into the vessel walls as part of the healing process.

And actually, that’s vasculitis in a nutshell.

Summary

Vasculitis is a condition in which there is inflammation of the blood vessels, typically caused by immune-mediated damage to the endothelial cells. The inflammation can affect blood vessels of different sizes, ranging from small capillaries to larger arteries and veins. There are many different types of vasculitis, which can affect various parts of the body, including the skin, joints, and internal organs.

Symptoms may include fever, fatigue, weight loss, joint pain, skin rashes, and organ dysfunction, depending on the type and location of the vasculitis. Treatment for vasculitis may involve medications to suppress the immune system and reduce inflammation, such as corticosteroids.

Elsevier

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