CR11: Diseases of Blood Vessels Part 2

Which diseases are characteriezed by medium vessel vasculitis?

• Polyartreitis nodosa

• Kawasaki’s disease

What age group is usually affected by medium vessel vasculitis?

Seen in small children 3-5 years old.

What diseases are characterized by large vessel vasculitis?

• Takayasu’s disease

• Giant cell arthritis

What age group is usually affected by large vessel vasculitis?

Elderly

What are the two divisions of small vessel vasculitis?

ANCA positive and ANCA negative.

Which small-vessel vasculitis diseases are ANCA positive?

• Wegener’s granulomatosis

• Microscopic polyangiitis

• Churg-Strauss Syndrome

Which small-vessel vasculitis diseases are ANCA negative?

• Henoch-Sconlein purpura

• Cryoglobulinemic vasculitis

Who is most susceptible to Buerger’s disease/Thromboangiitis obliterans (TAO)?

SMOKERS and young people <45.

Where does peripheral vasculitis occur in Buerger’s disease/Thromboangiitis obliterans (TAO)?

In small and medium sized vessels.

What are other characteristics of Buerger’s disease/thromboangiitis obliterans?

Reynaud’s phenomenon → decrease in blood flow to fingertips and toes. Leads to cold and painful fingers the more they are used, and potentially loss of the ends of fingers (gangrene).

What is polyarteritis nodosa (PAN)?

An autoimmune disease causing necrotizing inflammation of small and medium sized vessels.

What cardiovascular issues are caused by polyarteritis nodosa?

Microaneurysms, ischemia, and thrombosis.

What organs are damaged by polyarteritis nodosa?

Skin, joints, peripheral nerves, gut, and kidneys. Organ function is decreased by inflammation and necrosis.

Which organ is notably NOT affected by polyarteritis nodosa (PAN)?

LUNGS

What visible symptom can be seen with polyarteritis nodosa?

Palpable nodules with livedo reticularis (mottled skin with clear borders)

What is the new name for Churg-Strauss syndrome?

Eosinophilic granulomatosis with polyangiitis (EGPA)

What is the antineutrophil cytoplasmic antibody (ANCA) test?

Tests for antibodies that attack healthy white neutrophils, causing autoimmune vasculitis. If antibodies are there, the person is ANCA positive.

What are the two kinds of ANCA?

p-ANCA (targets perinuclear area) and c-ANCA (targets granules in cytoplasm)

What is Wegener’s Granulomatosis characterized by?

Hemoptysis (coughing blood), vasculitis, hematuria, strawberry gingivitis, and collapsed bridge of nose.

What is hemoptysis in Wegener’s granulomatosis?

Necrotizing granulomas of the upper and/or lower respiratory tract (coughing up blood).

What is vasculitis in Wegener’s granulomatosis?

Necrotizing granulomatous vasculitis of small arteries and veins, especially the lungs.

What is hematuria in Wegener’s granulomatosis?

Necrotizing glomerulonephritis, causing blood in the urine.

What age group is commonly affected by Takayasu’s disease?

15-45 years of age

What vessels are primarily affected by Takayasu’s disease?

Aortic arch branches

Why is Takayasu’s disease known as the pulseless disease?

There is no detectable pulse in the wrist.

What other symptoms are associated with Takayasu’s disease?

Dizziness, visual changes, and pulmonary hypertension.

What vessel wall changes occur in Takayasyu’s disease?

Intimal thickening, media degeneration, healing with fibrosis

What is Kawasaki disease?

Muco-cutaneous lymph node syndrome

Who is commonly affected by Kawasaki disease?

Infants and children

What are the external symptoms of Kawasaki disease?

Maculopapular rash, red tongue, chipping of skin, inflammation of lips and mouth

How does Kawasaki disease affect blood vessels?

Causes inflammation of coronary arteries and the oral mucosa.

Why is Kawasaki disease considered a self-limiting disease?

It usually goes away on its own.

What is temporal arteritis/giant cell arteritis characterized by?

Focal chronic granulomatous inflammation.

What arteries are associated with temporal arteritis/giant cell arteritis?

Temporal, facial, and opthalmic arteries

What are the symptoms of temporal arteritis/giant cell arteritis?

Headache, fever, weight loss, pain in cheeks and tongue, achiness in shoulder and hip muscles (Polymyalgia Rheumatica), throbbing pain, vision changes, and swollen, tender, red artery sticking out of the face. Can lead to blindness.

How is temporal arteritis/giant cell arteritis diagnosed?

With a temporal artery biopsy; presence of multinucleated giant cells.

What are varicose veins?

Veins that have misdirected valve flow and pooling due to long-term standing.

What are esophageal varicose veins?

Dilated submucosal distal esophageal veins connecting the portal and systemic circulations.

What is the cause of esophageal varicose veins?

Portal hypertension/cirrhosis.

What is a symptom of esophageal varicose veins?

Vomiting out blood.

What are hemorrhoids (rectal varicose veins)?

Inflamed veins at the anorectal junction.

What are hemorrhoids caused by?

Prolonged pelvic congestion due to:

• repeated pregnancies

• chronic constipation

• medications

What are lingual varicies?

Varicose veins on the tongue, lips, and oral cavity

How do lingual varices appear?

Multiple irregular, bluish purple, papular blebs

What is the most common site of varicose veins due to abnormal vascular dilatation?

Legs

What are examples of benign tumors of vascular tissue?

• Hemangioma

• Lymphangioma

• Glomus tumor (glomangioma)

• Pyogenic granuloma (lobular capillary hemangioma)

What are examples of intermediate tumors of vascular tissue?

Hemangioendothelioma, hemangiopericytoma

What are examples of malignant tumors of vascular tissue?

Angiosarcoma (most common), kaposi sarcoma