Vascultiits Flashcards
Vasculitis
Damage and inflammation of blood vessels, the WBC in blood flowing releases small molecules which makes the blood vessels look like foreign to the immune system, autoimmune disorders.
Three categories : large vessel vasculitis - flow decreases, pressure decreases, vessels bulge inwards -> blocking blood flow, a bruit (Auscultated), a thrill (palpated), medium vessel vasculitis - scaring results in complete blow keg, stagnant blood further leads to blood clot formation, bulging out occurs, aneurysm are common, small vessel vasculitis - easy breakage occurs, bleeding.
Vasculitis pathophysiology
When the protein attached to the endothelium of blood vessels are mistaken to be foreign, the antibodies attach to them and alert WBCs, activating an immune response, the WBCs start releasing immune peptides and further recruit more WBCs to the site and cause inflammation. The. Molecules released further travel downstream causing damage at various sites of blood vessels. The WBC further infiltrate into the blood vessel walla -> upon blood vessel wall damage certain tissue factors get released into blood -> the clotting factors get mixed with the tissue factors -> clotting cascade gets activated -> activates a clotting factors get mixed called Fibrinogen -> Fibrinogen becomes fibrin and forms a clot at the damaged site, this whole process is called a fibrinogen necrosis -> leads to scarring -> narrowing of vessels occurs.
Temporal arteritis
A type of large vessel vasculitis, affects temporal artery - also called Giant Cell arteritis
Symptoms : fever, chills, night sweats, fatigue, myalgia. The inflammation also leads to formation of immune complexes which get stuck in joints causing pain. Very common for women of age around 60 years old
Symptoms : fatigue, night sweats, chills, arthritis
Affects both external carotid artery and superficial temporal artery
More specific symptoms : headache lateral, mandibular pain
Also affects ophthalmic artery, symptoms : blindness, blurred vision, double vision
Migraines are seen to be a common misdiagnoses due to common symptoms
Difference between migraines and temporal arteritis is the age , ESR ( Normal - RBCS repel each other due to being negatively charges, inflammation : RBCs become positively charges and clump / stack over each others, leading to higher sedimentation rate than normal )
Diagnoses : biopsy of temporal artery, microscopic view will shows Granuloma
Treatment : steroids
Takayasu arterities
Aorta branches off into the branches : Brachiocephalic, left common carotid and left subclavian. Takayasu arterities affects the three branches of aorta.
Braciocephalic ( branches into right subclavian and right common carotid, due to fibrosis in brachiocephalic from repeated inflammation, causes narrowing -> Force decreases, BP decreases, Rate decreases and flow decreases.
Left common carotid and left subclavian has the same effect a Brachiocephalic, due to low BP, no pulse is found on pulse points such as Radial, therefore the diseases is called Pulseless Disease (the right side has a better chance for pulse detection than left, blood pressure will be low on both sides but left would b much lower compared to the right, decreased blood flow to heart occurs leading to vision and neuro problems, dizziness, drowsy, blurry vision, double vision, bruit can be heard at carotid arteries and thrill can be felt as well
Diagnoses : Increased ESR,, Biopsy (show Granulomas)
Treatment : steroids
Polyarterities Nodosa
Many arteries are inflamed and are modular. Hepatitis B is found in 20-30 % patients and may be the proteins in this virus can cause molecular mimicry (self proteins that look similar to foreign proteins get antibodies made against them by the immune system) further leading to immune response and vasculitis.
Diagnoses : biopsy (Fibrinoid neurosis : fibrinogen clot in blood vessels walls present as well as lots of debris / dead cells -> Fibrosis (scaring occurs ) -> healing -> Fibrinoid necrosis, continuation of this cycle leads to weakening of blood vessels walls present as-> bulging occurs -> Aneurysm ), rteriogram ( visualizes inside of blood vessels )
Pulmonary arteries are not damaged by vasculitis
Treatment : steroids, Cyclophosphamide (prevents cellular division ).
Kawasaki disease
Usually occurs in children,
Symptoms : fever lasting for more than 5 days, rash in mouth, Pam’s, feet’s and red eyes
Causes: unknown, also known as Mucocutaneous Lymph node syndrome, swelling of lymph node is also found
This is a type of medium vessel vasculitis . Symptoms can affect heart if not treated -> it can cause blood clots in heart blood vessels and prevent it from contracting. It can also eventually cause MI, pericardial effusion.
Some other symptoms : bright red tongue, desquamation ( skin peeling off palms and soles of feet)
Differential diagnoses : scarlet fever (can present symptoms like Kawasaki, occurs as a progression of strep throat ), atypical / incomplete Kawasaki : when there is three or less symptoms present, next step in this case would be to get a ESR / CRP, further to confirm supplemental labs are ordered : ALT increases, platelets increases, WBC increases, albumin decreases (if more than three supplemental labs come out true -> Kawasaki can be diagnosed, if less than three then a Echocardiogram is used (show presence of Aneurysm, Throbosis, Regurgitation, Rupture of blood vessels, Paericardial effusion)
Treatment : Aspirin, IVIG (immunoglobulins are delivered through IV to fight off Kawasaki antibodies )
Burger disease
Blood vessels narrow in fingers cause muscle pain -> Claudication, if the black age is very severe and completely stopping blood flow -> Autoamputation occurs (finger fall off).
Two six: Autoamputation and Claudication
Also known as Thromboangitis obliterans
Also affects feet’s causing for foot pain.
Diagnoses : ultrasound, Angiography (shows narrowing of arterities, cork screwing pattern (arteries try to grow and regrow new arteries ).
Rick factors : smoking,
Treatment : stop smoking
Churg - Strauss Syndrome
Vasculitis of small blood vessels. Misdiagnosed as allergic reaction or asthma due to similar Sox like runny nose, but eosinophils are found very low in blood with asthma and allergic reaction whereas with Churg Strauss Eosinophils will be high in blood. Another blood test performed to confirm is PANCA (anti Neutrophilic Cytoplasmic antibodies) those are seen in autoimmune disease normally. Biopsy is also used -> shows Granulomas.
Henoch - Schnolein purpura
Small vessel vasculitis, ANCA (Anti Neutrophilic Cytoplasmic Antibodies ) are not found, instead of IgA are found. It is associated with URI -> initiate molecular mimicry like IgA and body starts vast inflammation
Sx: joint pain, bloody urine, bloody stool, abdominal pain, skin lesions on butts / legs -> palpable / bumpy.
Diagnoses : elevated IgA
Treatment : steroids
Most common in kids.
