Vasculitis, Causes of Inflamed Blood Vessels, Symptoms

Vasculitis, or vasculitides, refers to a broad group of diseases that affect blood vessels. The basis is inflammation to necrosis, i.e. death of the vessel wall. 

Inflammation can affect any blood vessel of the human body, from the smallest to the largest - the aorta. The site of action is equally extensive, i.e. anywhere in the body.

How the disease manifests itself is also a matter of multiplicity.

Basically, the course of vasculitis depends on the location, size, extent and also the number of affected blood vessels.

The blood vessel is primarily damaged by inflammation and this can cause several conditions.

An example is the reduction of the space for blood flow. A blood vessel can be narrowed partially, but also completely. 

In the case of partial restriction, some blood flow is maintained, but this may not be sufficient. If there is a total restriction, the part of the tissue or organ behind the vessel closure is not perfused and ischemia, even infarction, occurs.

Partial restriction of flow through a blood vessel is referred to as stenosis.
Stenosis is a morbid, i.e. pathological, narrowing or deterioration of permeability, in this case of the blood vessel. 
Complete restriction = cessation of blood flow and is referred to as obliteration, meaning the disappearance of a cavity, an opening, in case of the vasculitis of a blood vessel.

As a consequence of inflammation, the damaged blood vessel can also widen, forming a so-called aneurysm, i.e. an outward bulging. The blood vessel is weakened in this part and there is a risk of rupture with bleeding.

Neither state is ideal from a prognosis perspective. Therefore, early detection of vasculitis and its treatment is very important.

Want to know more?
Vasculitides are divided into a wide group of subtypes.
And each has its own specifics.
Read on.

Vasculitis is a large group of diseases

If one hears the term vasculitis, one imagines inflammation of a blood vessel. The inflammation affects the vascular wall.

Thrombosis can form on the damaged part, which reduces the space for blood flow. As noted, this can be partial, i.e. stenosis, or complete, i.e. obliteration.

The second mechanism is that the weakened part of the blood vessel bulges: an aneurysm is formed. And the latter is risky in terms of rupture and bleeding.

Neither of these conditions can be underestimated. Since we don't know where in the body the inflammation will occur, there is no typical picture of how it will progress. 

However, one thing is certain.

Restricted blood flow will cause poor circulation. For vital organs such as the brain, heart or kidneys, difficulties occur immediately. But even with damage to the smaller blood vessels of the skin, there is a complication of the medical condition that requires a solution.

So wherever the blood vessel is damaged, early detection of the cause and early treatment is necessary. And what may be the cause of inflammation of a blood vessel is also a broad issue.

In the case of vasculitides, diversity and heterogeneity is the reason that different names for a single disease entity have arisen over time. But, on the other hand, one disease can manifest itself differently in each person. That depends on a number of characteristics of the disease, and for each person with vasculitis an individual approach is important.

So the whole atmosphere around the concept of vasculitides is challenging even for a professional.
And for this reason, the collaboration of doctors from several fields of medicine is necessary.

I set myself the task of putting together knowledge from multiple sources so,to shed some light on a difficult subjectand present the information in a concise, yet factual and understandable way for each person.

Vasculitides are classified by:

• the size of the intervening blood vessel
  • large-vessel vasculitis
  • medium-vessel vasculitis
  • small-vessel vasculitis
• the type of blood vessel
  • aortic inflammation - aortitis
  • inflammation of medium and small arteries - arteritis
  • inflammation of arterioles - arteriolitis
  • inflammation of arterioles, capillaries and venules - vasculitis
  • inflammation of the veins - phlebitis
• localisation
• etiology
  • primary - unclear cause of occurrence
  • secondary - in another disease
    • infectious
    • systemic connective tissue disease, lupus, rheumatoid arthritis, Sjogren's syndrome and others
    • post-medication reactions
    • malignant diseases
    • and others
• pathogenesis
• type of inflammatory reaction
• type of immune disorder
• tissue and organ damage

The division by discoverer is also interesting, which did not quite capture the exact historical conditions of the disease.

Over time, several classifications emerged, but for the sake of unification, the Chapel Hill consensus classification was created and later modified.

Table: Chapel Hill consensus classification

Read also:

• Rheumatoid arthritis
• Rheumatism is harmful to the whole organism and at any age
• Systemic lupus erythematosus 
• Sclerodermia
• Sjögren syndrome
• Polymyositis and dermatomyositis 
• Raynaud syndrome

What causes them? This is not exactly clarified.

Some risk factors for the development of the disease are mentioned, such as genetic predisposition, autoimmunity, infection, complications of other systemic diseases, allergic reaction to certain drugs. Thus, multifactorial interaction - mutual, multiple factors. 

The beginning of the problem may be a certain trigger.

Vasculitis can have different causes. From this point of view, they are divided into primary and secondary.

Classification of vasculitides by cause of occurrence:

1. primary - the exact cause of the disease is unknown
2. secondary - the basis is another disease, for example, infection, oncological disease, systemic connective tissue disease, etc.

Primary forms are mostly autoimmune diseases. Their development may be caused by an undetermined trigger and the interaction of several factors. Examples include the presence of genetic predisposition.

Secondary vasculitides reflect the existence of another disease. Distinguishing the form of vasculitis is important in its treatment. 

The cause of the secondary form is, for example:

• infection - by bacteria, viruses or parasites
• diffuse connective tissue diseases such as rheumatoid arthritis, lupus and others
• oncological malignant disease
• for other systemic diseases such as sarcoidosis
• caused by medicines
• consultative vasculitis
• after transplantation

Symptoms depend on a number of factors, such as the location and extent of damage to the blood vessel. A small, non-serious disease may not manifest itself, or run its course mildly.

The opposite is the involvement of a larger extent, or a large blood vessel. Alternatively, a medium-sized blood vessel that supplies blood to tissues and organs.

The blood vessel may be inflamed in one place. The inflammatory damage is mostly well circumscribed. Another example is if it alternates an area of inflammation with a healthy section. In this case, only one, but also several vessels may be affected at the same time.

Damage to the blood vessel will manifest as thrombosis with flow restriction. Blood clots can subsequently embolize to another part of the body. The aforementioned aneurysm of the vessel is a risk for disruption of the vessel wall and the development of dissection or rupture of the vessel and bleeding.

A serious condition is mainly aortic dissection, when blood through the disrupted endothelium penetrates between the layers of the vascular wall. Depending on the extent, it threatens a person's life.

Rupture - rupture of the aorta with massive bleeding ends in death.

General non-specific symptoms of this group of diseases:

• general weakness
• fatigue, exhaustion, prolonged, unexplained
• increase in body temperature to fever
• sweating
• lack of appetite and weight loss
• abdominal pain
• headache
• dizziness to fainting
• paleness of the skin
• joint and muscle pain
• kidney failure
• persistent sinusitis
• hearing impairment
• conjunctivitis
• new-onset hypertension
• skin manifestations are also common 
  • mostly occur symmetrically and in some cases primarily on the lower limbs 
  • purpura - red to purple skin seeding
  • livedo reticularis - blotchy red to pink skin of the lower limbs
  • erythema - flat skin redness
  • petechiae - pinpoint, round spots that appear on the skin as a result of bleeding
  • Hyperpigmentation
  • subcutaneous nodes
  • skin defects - ulcers
  • to necrosis of the skin - death of part of the skin

As an example, here are some diseases and their symptoms...

Large-vessel vasculitis

The main representative is Takayasu's arteritis and giant cell arteritis. Both diseases have a common marker, that is, inflammation of large and medium vessels, hence the aorta and its branches.

The diseases are so close to each other that they are thought to be one disease that manifests itself in two different forms.

They are united by the presence of symptoms such as fatigue, inflammation of blood vessels, fever, hypertension already present at a young age, a lateral difference when measuring blood pressure in the upper extremities or unmeasurable blood pressure (due to damage to the aorta).

The association of neurological symptoms indicates a problem with blood flow to the brain, when it can occur together with visual impairment.

The search for the exact cause is complex.

Example:

Ischemia of the lower limb is mostly caused by atherosclerosis.

As opposed to...

Poor blood circulation of the upper limb - in this case, atherosclerosis is present only exceptionally, and the true reason may be embolism, thrombosis, but also vasculitis. 

Read the article Peripheral artery disease. 

Takayasu's arteritis

Takayasu's arteritis is a chronic inflammatory disease whose cause is unknown. It is more common in women and in the under 40 age group.

It occurs mostly in Asian populations, where its incidence ranges ranges from 0.3 to 150 new cases per 1 000 000 inhabitants. Takayasu arteritis is estimated to affect 2.6 persons per million annually. The prevalence is 2.6-6.4 persons per million population.

It affects the aorta and its main branches. According to the site of damage, it is divided into 4 subtypes, namely:

• Type I - vessel involvement: aortic arch (Arcus aortae) and its branches, often presenting with aortic valve insufficiency
• Type II - ascending aorta, aortic arch and its branches
• Type III is a combination of type 1 and type 2
• Type IV - damage to pulmonary blood vessels

Symptoms can be difficulties such as:

• fatigue
• weakness
• night sweats
• muscle pain
• joint pain
• weight loss
• upper limb pain, especially exertional
• weakening of the pulse in one upper limb, or complete disappearance
• a lateral difference in blood pressure measurement in the upper extremities of more than 10 mmHg
• large-vessels murmur
• dizziness
• feeling to pass out to passing out
• visual impairment to blindness
• lung damage and pulmonary hypertension
• or high blood pressure due to anemia of the kidneys

The disease has several names, such as:
Aortic arch syndrome,
pulseless disease,
Martorell syndrome,
Takayasu-Onishi syndrome
and other. 

Giant cell arteritis

It is reported to be among the most common vasculitis of the adult population. It mostly affects people over 50 years of age, more precisely between 70 and 80 years of age.

It mostly affects people in North America and Europe, where the incidence is estimated to be 200 to 250 affected per 1 000 000 inhabitants per year. 

It affects large vessels such as the aorta, but mainly the carotid arteries and vertebral (spinal) arteries. But also the smaller blood vessels of the face, such as the temporal artery, the artery of the orbit and the arteries of the brain.

It is also abbreviated as GCA - Giant Cell Arteritis.

CGA presents with the following symptoms:

• slower development

• increased body temperature

• fatigue
• lack of appetite and weight loss
• headache
• visual disturbances, double vision to blindness in one eye
• pain in chewing muscles - exertional
• upper limb pain 
• upper limb anaemia, Raynaud's syndrome to finger necrosis
• encephalopathy - impaired brain function, memory impairment, altered intellect, dementia and others
• and other difficulties associated with the immaturity of the organ concerned

In a significant proportion of cases it runs together with polymyalgia rheumatica.
It is characterized by manifestations such as:
Morning stiffness of the neck,
pain in the muscles of the shoulder and pelvis.

The disease bore several names, such as Horton's disease or Temporal arteritis.

Medium-vessel vasculitis

Already by the name it is obvious that they affect medium-sized blood vessels. Thus, these are mainly blood vessels that lead to the organs.

When the vessel wall is damaged, either stenosis due to thrombosis occurs, but also an aneurysm. 

One of the main representatives is polyarteritis nodosa.

Polyarteritis nodosa

It is also referred to as PAN. In short and loosely translated, it is the involvement of the vascular wall of multiple blood vessels by inflammation with the formation of nodules. It is also called necrotizing vasculitis, multiple nodular inflammation of the arteries.

Nodules arise as a result of necrotizing inflammation of the vessel wall and weakening of the vessel wall when a vascular aneurysm, or bulging, forms.

It is associated with streptococcal infections, but also with hepatitis B or C.

It most commonly affects the blood vessels that carry blood to the kidneys and organs of the gastrointestinal tract (GIT), but also the blood vessels of the heart, muscles and bones. 

Symptoms develop in PAN according to the organ affected:

• overall manifestations
  • fatigue
  • weakness
  • increase in body temperature to fever
  • nausea and lack of appetite
  • weight loss
  • muscle and joint pain
• kidneys - ischaemia to heart attack with malfunction and kidney failure, protein and blood in the urine + including renovascular hypertension - severe form of high blood pressure
• digestive tract - abdominal pain, bleeding in the gastrointestinal tract damage to the pancreas gallbladder and other
• nervous system - polyneuropathy and pain, paresthesias, i.e. tingling, sensory disturbances or hemiplegia - stroke, convulsions and also other neurological discomforts
• heart - ischaemic heart disease or heart failure
• muscles and joints - pain and weakness of muscles and joints, atrophy
• skin - skin seeding, erythema (redness of the skin), petechiae to skin nodules, ulceration and necrosis, i.e. skin cells die
  • purple discoloration of the skin is common - purpura, i.e. purple-colored spots and patches that occur on the skin 
• in boys also undescended testicles and their pain

Kawasaki disease

It is preferably a childhood disease, for the age period up to 5 years (average 2 to 3 years). Its cause is unknown.

The highest prevalence is in Asia and in Japanese children.

The disease is mainly risky due to damage to the heart vessels. You can read more about the disease in the article Kawasaki disease on Health.

The course is characterized by symptoms such as:

• sudden onset of the acute phase
• fevers above 40 °C
• bilateral conjunctivitis
• dry mucous membranes, cracked mouth, erythema - engorgement and redness of the tongue - raspberry, also called strawberry tongue
• enlargement of the cervical nodes
• redness of the skin and swelling of the hands
• skin seeding in the groin and chest
• abdominal pain
• inflammation of the joints
• headache
• abdominal pain
• nausea
• weakness
• fatigue

Diagnosis includes history taking and laboratory blood tests (CRP, blood count, sedimentation, biochemistry, antibodies and others) and urine (presence of blood and protein). 

Also, doctors from multiple disciplines collaborate on the diagnosis.

Imaging methods include:

• X-ray
• CT
• MRI
• sonography
• angiography using a contrast agent injected into the blood vessels and X-ray - diagnostic and therapeutic method for diseases of large and medium-sized blood vessels

Alternatively, a biopsy (of the skin, blood vessels, kidneys, lungs and other tissues) is also performed, i.e. taking a small sample and assessing the vasculitis.

The course of the disease depends on the site and extent of the affection. Only one blood vessel in one place may be affected, or one blood vessel in several places, or even several blood vessels.

It can last for a short time, but it can also last a lifetime.

That's not all...

Of course, it also depends on what tissue and organ are unhealed.

In cardiac ischemia, this will be a manifestation of coronary artery disease, heart attack or heart failure. Insufficient blood supply to the brain will be manifested by neurological problems, and in ischemia or infarction of the kidney, renal failure will be present. 

Large-vessel vasculitis is characterized by a characteristic two-stage course when, in the first wave, general symptoms of inflammatory disease such as fatigue, fever, inappetence and weight loss set in.

The second wave of problems comes sometimes years later.

Manifestations of vascular changes may occur together in the acute form. However, in some cases, these complications may appear weeks, months or years apart.

The destroyed vascular endothelium picks up blood clots, and these can gradually clog the blood vessel completely. Alternatively, the blood clot breaks off from the blood vessel and travels through the blood as an embolus. 

An embolus floats in the bloodstream and can cause an embolism, i.e. a blockage of a blood vessel in a completely different place in the human body.

An associated threat of a weakened vessel wall is the aforementioned aneurysm. The latter threatens health and life in terms of dissection and rupture of the vessel with subsequent bleeding.

The course may be mild or severe.

A mild course may be characterized by general discomforts such as fatigue, a tiny skin rash is associated, but it does not pose a more serious threat to a person's health.

Moderate severity will have general symptoms present as well, such as fatigue, increase in body temperature or lack of appetite. Skin manifestations will be present to a greater extent. Joint and muscle pain and late complications may also be present.

In the case of a severe acute course, vital organs are at risk. The risk is their failure due to ischemia and infarction. Plus, bleeding and sudden death are also serious.

Aká je prognóza?

The prognosis depends on the overall course of the disease. It is also influenced by early diagnosis of the disease and early treatment. 

Early treatment is intended to mitigate the progression and, most importantly, to limit the consequences of the disease and permanent organ damage.

If left untreated, the disease increases the risk of permanent organ damage and death.