Lecture 17 - Vasculitides

Question 1

Vasculitis is a diagnosis of ______. Tissue biopsy is the gold standard for diagnosis.

Answer 1

Exclusion

Question 2

____ cell Arteritis is a large vessel vasculitis that typically affects the aorta and its branches (including carotid and external ophthalmic). It’s most common in adults over ____ years old, and it’s the most common systemic vasculitis in adults. There is a risk of _____ loss from this disease.

Patients typically present with ______ for the last couple weeks (should make sense since the disease affects the carotids).

Answer 2

Giant cel Arteritis

50 years old

Vision loss

Headache

Question 3

GCA histology shows _______ (full thickness) inflammation with elastin and collagen disruption. Which T-cells are highly implicated in infiltrate in this disease?

Answer 3

Transmural

CD4+ TH-17 T-cells

Question 4

_______-______ Purpura is the most common vasculitis in kids. It affects small vessels and typically occurs following an upper ______ illness. It is characterized by a tetrad of symptoms including 1. Palpable ______ in ALL patients, 2. Arthritis/Arthralgia, 3. Abdominal pain, and 4. Renal disease. Imunofluorescence will show Ig___ deposits in the small blood vessels.

Answer 4

Henoch-Schonlein Purpura

Respiratory

Purpura

IgA

Question 5

Leukocytoclasia describes ______ degeneration which forms ____ dust. This is indicative of a Leukocytoclastic vasculitis, but it doesn’t tell you the cause.

Answer 5

Neutrophil

Nuclear dust

Question 6

ANCA-Associated Small vessel vasculitis is caused by Anti-____ Cytoplasmic Abs (ANCA). Two important proteins that are targeted are _____ (a protease in neutrophil granules that contributes to formation of antimicrobial peptides) and _____ (enzyme in neutrophil granules that catalyzes H2O2). These proteins are moved to the neutrophil plasma membrane, where they can interact with _____ molecules on the endothelial lining of small vessels. This is considered Priming the neutrophils, after which they can degranulate, releasing ROS that damages the endothelium.

Answer 6

Anti-Neutrophil Cytoplasmic Abs

PR3

MPO

Adhesion molecules

Question 7

Basically, ANCAs role in the pathogenesis of ANCA-associated vasculitis (AAV) includes three main things. 1. Priming neutrophils by upregulating PR3 and MPO. 2. Activating _____ (which molecule?) of the complement system. 3. Increaseing cross-talk between Th-__ and Th-__ T-cells as well as differentiation of B-cells via IL-21.

Answer 7

1. Priming neutrophils via PR3 and MPO
2. Activating C5a
3. Th-1 and Th-17

Question 8

Granulomatosis with Polyangitis (GPA), Microscopic Polyangitis (MPA), and Eosinophilic Granulomatosis with Polyangitis (EGPA) are three conditions that can arise from ANCA-assiociated vasculitis. While they all result in _____ vasculitis, EGPA will show eosinophilic infiltrate and is associated with uncontrolled _____ and eosinophilia.

In GPA, 90% of patients are ANCA+, with the Ab/antigen combo being ___-ANCA/___3.

For MPA, 90% are ANCA+, with the Ab/antigen combo being __-ANCA/____.

For EGPA, only 50% are ANCA+, 70% of whom show the same AB/antigen combo as MPA.

Answer 8

Necrotizing vasculitis

Asthma

c-ANCA/PR3

p-ANCA/MPO

Question 9

Early symptoms of GPA often involves the ______ airway.

Answer 9

Upper

Question 10

There is no _____ involvement in MPA, unlike with GPA, and lung disease is mostly capilarities (hemorrhage) in MPA vs nodules or cavitary lesions in GPA.

Answer 10

Sinus