Autoimmune Rheumatic Diseases

Question 1

What is a rheumatologist?

Answer 1

Treats many different organs systems, autoimmune disease and drug to modulate their function

Question 2

What organs can be affected by rheumatic disease?

Answer 2

Skin, blood vessels, nerves, muscles, bones/joints, internal organs and gut

Question 3

What is vasculitis (Skin)

Answer 3

Is an inflammatory skin diseases which causes widening of the blood vessels causing things such as stroke, MI’s, abdominal pain, fevers, headaches, nose bleeds, vision loss, weight loss etc.

Question 4

Do all rheumatoid diseases go into all blood vessels?

Answer 4

No - some will be found in smaller blood vessels, others in larger ones such as the aorta.

Question 5

What is the symptoms and causes of the skin disease = small vessel vasculitis-henoch schonlein purpura (IgA vasculitis)?

Answer 5

Rashes, stomach aches, arthritics, renal disease and is caused by IgA deposits in the blood vessels of the kidney, skin or gut.

There is expansion of B and T cells

Question 6

The symptoms of the skin disease kawasaki and cause?

Answer 6

Coronary artery aneurysms, peeling skin around finger nails, red eyes, cracked lips, swelling ankles

Caused by infection associated pyroptosis and oxidative stress which activates innate and acquired immunity leading to arterial wall damage.

Question 7

What are the symptoms and causes of the nerve disease polyarteritis nodosa?

Answer 7

Medium sized artery disease which causes gangrine in the extremeties and foot drop - where you cannot keep you foot up. It also causes kidney aneurism

Question 8

What are the symptoms and causes of giant cel arteritis?

Answer 8

The aorta and vessels next to the eye become inflamed (you can see this on their skin) if not treated within 4 weeks causes blindness. Within about 3 days there is no more symptoms and the patient will be fine

Question 9

What are the symptoms and causes myositis?

Answer 9

Causes rashes, discolorisation on the back, knuckles and under eyes. Also causes muscle weakness. Caused by inflammation surrounds blood vessels and atrophy of fibers near border of fascicle.

This is caused by cancer because your body is trying to fight the cancer. Also, caused by checkpoint inhibitor treatments.

Question 10

What causes these types of diseases?

Answer 10

You genes, environment, or we dont really know

Question 11

What are the symptoms of RA?

Answer 11

Swelling of joints
Joint tenderness
systemic malaise
loss of energy
severe morning stiffness

Question 12

Why would you go early for treatment?

Answer 12

It progresses from feet and hands to all over the body anywhere there is synovial membranes and can be stopped.

Question 13

What do people with RA develop around their knees?

Answer 13

Pannus which is a swelling full of fibroblasts, plasma cells, endothelium, T lymphocytes, macrophages etc.

Question 14

Is RA a normal response to injury?

Answer 14

No its a big response to really small injuries this could be because of lack of regulation of the immune response or more cells.

Question 15

What does the pannus tissue do to bone?

Answer 15

it can eat away at it

Question 16

Smoking and RA?

Answer 16

Smoking destroys cells in lungs bringing immune system to the lungs. If you are genetically predisposed to RA you are going to have an exagerated immune response. This immune response produces ACPA antibodies which bind harshly to MHC 1 causing good T cell reactions.

Question 17

What are some extra-articular manifestations of Rheumatoid?

Answer 17

Ocular, heart inflammation, vascular, skin, lungs, neurological (stroke)

Question 18

Is rheumatoid vasculitis a late complication of RA?

Answer 18

Yes and this can cause gangrine, eyes damage,

Question 19

How do drugs manage RA?

Answer 19

Systemic DMARDs

Interfere with cytokine function - Anti-TNFalpha and anti-IL-6 (be careful as if someone had TB at some point this may come back).

Inhibit signal (CTLA4-Ig-Abatacept)

Deplete B cells - rituximab

Block Jak stat pathway

Question 20

How does tofacitinib work?

Answer 20

IL-6 and other cytokines use JAK3-STAT signalling to increase inflammation and this blocks this reducing inflammation

Question 21

What is psoriatic arthritis?

Answer 21

Can occur in feet, spine, hands etc. Arthritis can get so bad it can melt bones away

Question 22

Th17 and psoriatic arthritis

Answer 22

Tendons and ligans go into bones and if there is too many Th17 cells this will cause a big immune response to the little hurts these tendons get e.g strain from carrying bags

The IL-17 triggers macrophages, fibroblasts etc which also release inflammatory cytokines

Question 23

What are biological therapies for psoriatic arthritis?

Answer 23

Anti-TNF

IL-17 inhibitor

IL-12/23 inhibitor which blocks p40 of IL12 and IL23

IL-17 receptor antibody for skin psoriasis

Question 24

What organs does lupus affect?

Answer 24

Most of them - mouth ulcers, renauts, kidneys failure, hair loss, face rash.

Question 25

What is the pathophysiology of Lupus?

Answer 25

Triggers cause immune system disregulation or defective apoptosis.

The defective apoptosis causes modified nucleosomal material which is taken up by plasma cells to make Interferons and then either the innate immune response or the adaptive immune response.

The immune system disregulation causes T and B cells and cytokine defects and the adaptive immune response.

Question 26

What does type 1 interferon do?

Answer 26

Decreases Treg cells, increases NETosis and induces B cells proliferation and loss of tolerance.

Question 27

What are the biological therapies of lupus?

Answer 27

Rituximab - didnt actually show any significstn benifit.

Belimumab - unhibits the soluble form of a B cell survival factor (BAFF) which normally promotes the formation and survival of memory B cells and plasmablasts making autoantibodies.

IFN - trials show mixed results to date.

Question 28

What is systemic sclerosis?

Answer 28

Tight fibrosis skin which entirely encases the body it also happens in the gut causing increased bacteria, the lungs (interstitial fibrosis, pulmonary hypertension), cardiac muscles (arythmias, CCF) and renal (accelerated HT, renal crisis)