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Coronary steal syndrome
Peripheral artery disease
Subclavian steal syndrome
Renal artery stenosis
Coarctation of the aorta
Polycystic kidney disease
Chronic venous insufficiency
Deep vein thrombosis
Human herpesvirus 8 (Kaposi sarcoma)
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
Premature atrial contraction
Atrioventricular nodal reentrant tachycardia (AVNRT)
Premature ventricular contraction
Long QT syndrome and Torsade de pointes
Bundle branch block
Pulseless electrical activity
Tricuspid valve disease
Pulmonary valve disease
Mitral valve disease
Aortic valve disease
Rheumatic heart disease
Pericarditis and pericardial effusion
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
Vasculitis: Pathology review
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Filip Vasiljević, MD
Tanner Marshall, MS
Kaia Chessen, MScBMC
At the clinic, two very different people with very different symptoms showed up. Novakova is a 60 year old woman from Czech Republic who’s been having a headache by the right temple, progressive jaw pain during chewing, and shoulder and hip stiffness, particularly in the morning. Her ESR levels are elevated, and she is beginning to develop vision loss in the right eye. The other person is Hikaru, a cute 3-year-old Japanese boy brought by his mother. He has a red, swollen tongue, unilateral neck swelling, a desquamating rash, and a fever for the past 6 days. An ECG reveals elevation of the ST segment, and an echocardiogram shows evidence of a coronary artery aneurysm.
At first glance, you’d think Novakova and Hikaru symptoms have nothing in common. But the fact is, they both have different forms of vasculitis. The majority of vasculitides are caused by some form of immune-mediated damage. Some are type III hypersensitivities caused by immune complexes that deposit in vessels and cause damage. They could also be triggered by autoantibodies, like anti-neutrophil cytoplasmic antibodies, or ANCA. Lastly, they might be caused by cell-mediated immunity due to monocytes, macrophages or lymphocytes.
Whatever the cause, immune cells cause inflammation, which damages the endothelial wall, exposing the underlying collagen. This results in thrombosis and could lead to ischemia of the organs supplied by the affected vessels. Also, inflammation weakens the structural integrity of the blood vessel wall, leading to aneurysms. In addition, inflammation can spread to the supplied organ itself, causing tissue damage.
Since vasculitides are systemic inflammatory disorders, non-specific symptoms like fever, arthritis, myalgia, weight loss, and night sweats might be present. The symptoms specific to each disease depends on which blood vessels are affected, and we can classify vasculitides into large, medium, and small-vessel vasculitis.
For large vessel vasculitis, let’s start big, with temporal, or giant cell arteritis. Age is the greatest risk factor, and it typically affects people older than 50 years. It's also more common in females of Northern European descent. Giant cell arteritis affects the branches of the carotid artery like the temporal artery. For your exams, remember the person with this disorder often complains of having an unilateral headache located in the temporal area. They might also have fatigue or pain during chewing, or jaw claudication, which is caused by ischemia of the masseter muscle.
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.
Common types of vasculitis include giant cell arteritis, takayasu arteritis, polyarteritis nodosa, kawasaki disease, wegener's granulomatosis, churg-Strauss syndrome, henoch-Schonlein purpura, etc. Symptoms vary depending on the type and location of the vasculitis, but may include fever, fatigue, weight loss, joint pain, skin rashes, and organ dysfunction. Treatment for vasculitis may involve medications to suppress the immune system and reduce inflammation, such as corticosteroids.
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