Vasculitis refers to inflammation of vessels including
arteries and veins that contain blood and blood contains the inflammatory cells
including white blood cells e.g. Neutrophil granulocytes, eosinophil,
basophils and mast cells. White blood cells contain granules (sac) contain
toxic substances and digestive enzymes in order to eliminate harmful stimuli.
Now question is what is inflammation?
Inflammation is the
complex biological response of the vascular tissues to harmful stimuli, such as
pathogen, damaged cells, and irritant or a protective immunovascular response
in order to eliminate the initial cause of cell injury and to initiate the
tissue repair. It is considered as innate immunity, compared to adaptive
immunity that is specific for each pathogen to eliminate. All Tissues are
supplied by blood vessels and nerves. Tissue takes nutrient, oxygen and other
substance from blood vessels to survive. Now the question is from where do
blood vessels get the nutrient and oxygen? Get oxygen from respiratory system
and nutrient from foods through GIT. One new question you need to know,
otherwise the question will be existing in your mind as confused and unable to
satisfy, who produces the white blood cells? The Bone marrow is responsible for
production of WBCs. Now you need to go into depth about mechanism of
inflammation. Inflammation caused by infections, trauma, or unknown like
autoimmune. Vasculitis caused by unknown like autoimmune is the main topic
here. White blood cells are considered as immune cells. Once tissue has been
attacked by unknown etiology that is similar to human tissue,
our immune system
produces antibody against that unknown etiology in order to destroy etiology
but it is similar to tissue that is why it destroy the tissue and vessels, this
condition is called autoimmunity. Here our immune cells are unable to recognize
own tissue that is affected. Antibody, Neutrophil and other cells if needed go
to target tissue through blood circulation then cross the endothelium of blood
vessels through vasodilatation caused by histamine and deposited over the
target tissue then release their toxic substance and digestive enzyme in order
to eliminate etiology without harming their own tissue but etiology is similar
to tissue and affect the blood vessels from outside as a result narrowing lumen
of vessels lead to ischemia; blood flow obstruction.
Pictures show mechanism of inflammation of the blood
vessels.
There are three sizes of arteries are small sized, medium
sized and large sized all over the body including lungs, kidneys, heart, head,
eyes, GIT and legs.
Main pathogenesis: Immune complexes, antibody, cytokines and
granulomatosis.
Classification of vasculitis, based on size of involved
vessels:
Large vessel
|
Medium vessel
|
Medium to small vessel
|
Small vessel
|
1. Giant cell arteritis
|
1. Polyarteritis nodosa
|
1. Wegner’s granulomatosis
|
a. Henoch-schonlein purpura
|
2. Takayasu’s arteritis
|
2. Kawasaki disease and
|
2.Churg-strauss syndrome
|
b.Cryoglobulinemic vasculitis
|
3. Hepatitis B virus related
|
3. Microscopic polyangitis
|
C.Cutaneous leukocytoclastic vasculitis.
|
|
Most important is to diagnose the vasculitis through
systemic sign and symptoms and through biopsy of the infected blood vessels,
and also through presence of antibody in the blood whatever the name of the
diseases because the management depends on underlying cause is autoimmunity.
Symptoms of vasculitis:
No specific symptoms are fever, fatigue,
malaise, anorexia, and weight loss.
Mucocutaneous symptoms: mouth ulcer, Reynaud’s phenomenon, vasculitis
purpura, and skin rash
Mononeuritis multiplex, ischemic renal failure related to
arthritis, or glomerulonephritis, arthralgia, arthritis, or myalgia, hematuria,
and asthma.
How to reach to a diagnosis of vasculitis? You need to
exclude other conditions that stimulate vasculitis and conditions which can
cause secondary vasculitis.
A.
Conditions that stimulate
vasculitis are 1. Atherosclerosis, 2. Fibro muscular dysplasia, 3. Vascular spasm,
4. Embolic conditions and 5. Thrombophilia.
B.
Conditions which can cause
secondary vasculitis are 1. Connective tissue disease, 2. Malignancy, 3. Drugs,
4. Hepatitis B and C, and 5. Cryoglobulinemia.
After excluding above, case need some test to identify and
confirm the class of vasculitis.
Investigation:
Biopsies.
Laboratory:
Anti-Neutrophil Cytoplasmic Antibodies (ANCA), rheumatoid factor, serum
cryoglobulins,
CBC, ESR, CRP, urine analysis, renal function test, hepatic
enzymes, viral serology and blood cultures.
Vascular imaging:
Duplex:
describes the flow, velocities and the presence of narrowing or aneurysms.
Angiography: is
the most diagnostic and also CT, MRI angiography.
Other imaging according to organ involvement: like X-ray CT
scan for the chest and paranasal sinuses, echocardiography, abdominal
ultrasonography, and EMG and nerve conductions studies.
Now is the treatment:
Treatment is planned according
to the type and severity of organ involvement, the aggressive nature of the
disease, and the specific type of vasculitis.
Corticosteroids are the mainstay of treatment for the vasculitis
due to it kills all the pathology related to vasculitis. The dose is chosen
according to disease severity and also combination with cyclophosphamide.
How to diagnose the type of vasculitis from symptoms and
test: abdominal pain during eating most
probably mesenteric vessels involvement, stroke or blurring vision suggest
carotid or temporal arteries involvement, associated with asthma most probably
upper respiratory vessels involvement, angina mostly coronary artery
involvement, claudicating during chewing most probably external carotid
arteries involvement . According to size and site of vessels involvement give
the name of diseases. In case of vasculitis, most important is to diagnose the
vasculitis.
Post a Comment