What is a vasculitis? Vasculitis is a group of pathologies united by the presence of an inflammatory process affecting any blood vessel (arteries, arterioles, veins, venules or capillaries)

They are divided into two categories:

• primary vasculitis,
• secondary vasculitis.

In the first case it is not possible to establish the presence of a specific cause, while in the second case the pathology can arise following infections, the intake of drugs or toxins, inflammatory diseases and neoplasms.

The symptoms are not unique and vary according to some characteristics such as: size, location, extent of organ involvement, degree and type of inflammation.

The first description of a vasculitis process dates back to 1761, by a doctor from Forlì Giovan Battista Morgagni, who described a pathology belonging to this group in his work “De sedibus et causis morborum per anatomeninvestigatis” (subsequently defined as “Takayasu’s arteritis “).

A further contribution came in 1808 from the English physician, founder of dermatology, Robert Willan, who described one of the most common skin manifestations, purpura, in his treatise “On cutaneous diseases”.

In 1866 it was the turn of two German doctors, Rudolf Robert Maier and Adolf Kussmaul, who reported a detailed analysis of periarthritis nodosa following an autopsy.

In 1968, during the Capri conference, Anthony S. Fauci, G. Marone, M. Condorelli, L.M. Liechtenstein provided the first classification of vasculitis: this was implemented a few years later by the American College of Rheumatology (ACR), which provided sensitivity and specificity criteria for their diagnosis.

Coming to the present day, the latest classification of the pathology comes to us from the Consensus Conferences, held in 1982 and 2012 in Chapel Hill; from these was born the current classification incorporated in the ICD-10.

What is vasculitis and how to recognize it?

A vasculitis is an inflammation of the blood vessels and can arise without a determinable cause, in this case we speak of primary vasculitis.

Alternatively, it can be the result of autoimmune processes, an infectious process or another pathological condition, in which case we speak of secondary vasculitis.

Furthermore, the latter can be triggered by drugs, toxins or other external agents.

Vasculitis can affect any blood vessel and, for this reason, it is very difficult to classify its symptoms and manifestations, which depend on various factors such as: the size of the vessel, its location and the degree of involvement of the organ.

Vasculitis: the causes

Usually, the main cause of the onset of these pathologies is attributable to an overactivity of the immune system, which erroneously recognizes the blood vessel cells as foreign and attacks them as occurs in response to an infection by viruses or potentially pathogenic bacteria.

The causes of this reaction are not clearly defined but can be traced back to different types of infection, to some types of tumor and immune system disorders, or to the use of a specific drug, we will therefore speak of secondary vasculitis.

In the event that there is no known cause that may have led to the onset of this pathology, it is referred to as primary vasculitis.

In the case of secondary vasculitis, there are several diseases that can be associated with its appearance

• Infections: Many cases of vasculitis are the result of hepatitis C virus infection, while hepatitis B can result in the clinical presentation of polyarteritis nodosa;
• autoimmune diseases: vasculitis can occur as a result of certain diseases of the immune system, such as rheumatoid arthritis, systemic lupus erythematosus, juvenile dermatomyositis, and scleroderma;
• allergic reactions: exposure to chemicals (insecticides and petroleum products) and drugs – such as amphetamines, sulfonamides, beta-lactams, oral contraceptives, NSAIDs, quinolones and some vaccines – can cause vasculitis;
• blood cell tumors: A lymphoproliferative or myeloproliferative neoplasm can cause vasculitis.

Vasculitis: the symptoms

As previously mentioned, the symptoms of vasculitis vary based on many factors that can influence both the site and the extent of the manifestation.

The main parameter to be evaluated is the location of the affected blood vessel and the extent of the disease, which can be mild or become extremely disabling.

Among the main symptoms that we can list, it is good to underline the manifestations of systemic inflammation such as fever, night sweats, asthenia, anorexia, weight loss, arthralgias and arthritis.

The most serious symptomatic manifestations are:

• alveolar haemorrhage (characterized by persistent or recurrent pulmonary haemorrhage);
• rapidly progressive glomerulonephritis: disorder of the glomeruli (clusters of microscopic kidney blood vessels), characterized by tissue edema, arterial hypertension and the presence of red blood cells in the urine;
• mesenteric ischemia (disruption of intestinal blood flow);
• vision loss in patients with giant cell arteritis.

In the case of involvement of small and medium blood vessels, the skin lesion is very frequent and can manifest itself as palpable purpura, urticaria, ulcers, livedo reticularis and nodules.

A further classification of symptoms takes into account two main aspects: the size of the affected vessel and the affected organ.

Depending on the size of the blood vessels affected by the disease, we recognize more common symptoms for each category:

Small size:

• palpable purpura (1-3 mm)
• papules (very small)
• bleeding
• urticaria
• vesicles
• livedo reticulate (rarely)

Medium size:

• ulcers
• nodules
• livedo reticulate
• papulo-necrotic lesions
• hypertension
• possible damage to the renal vessels

Big dimensions:

• ischemia
• hypertension
• aneurysms
• dissection, hemorrhage or rupture

As regards the bodies concerned:

• heart: myocardial infarction, hypertension and gangrene
• joints: arthritis
• kidneys: dark urine or hematuria and glomerulonephritis
• skin: nodules, ulcers, bruises or hives, purpura and livedo reticularis
• lungs: shortness of breath and hemoptysis (coughing up blood)
• eyes: redness, itching and burning, photosensitivity, reduced visual acuity and blindness
• gastrointestinal tract: oral aphthae and ulcers, abdominal pain and intestinal perforation
• nose, throat and ears: sinusitis, ulcers, tinnitus and hearing loss
• nerves: numbness, tingling, weakness in various parts of the body, loss of sensation or strength in hands and feet, and pain in arms and legs
• brain: headache, stroke, muscle weakness, and paralysis (inability to move)

The types of vasculitis

There are many forms of vasculitis and each of them is characterized by a specific clinical picture.

Vasculitis can be classified based on various factors such as

• the triggering cause
• the location of the affected blood vessels:
• Cerebral
• Cutaneous
• Systemic

The type or caliber of blood vessels affected:

Vasculitis of the large blood vessels

Illnesses:

• Behçet’s disease, chronic relapsing multisystem vasculitis, causing inflammation of the mucous membranes
• Giant cell arteritis, a disease affecting the thoracic aorta, the large arteries emerging from the aorta in the neck, and the extracranial branches of the carotid arteries
• Takayasu’s arteritis, inflammatory disease affecting the aorta, its branches and pulmonary arteries

Symptoms:

Limb claudication

• Differences in blood pressure measurement or pulse absent or of different intensity in the limbs
• Ischemic symptoms of the central nervous system (e.g. stroke)

Vasculitis of the middle blood vessels:

Illnesses:

• Cutaneous vasculitis of the medium vessels
• Polyarteritis nodosa, systemic necrotizing vasculitis typically affecting medium-sized muscular arteries

Symptoms:

Symptoms of tissue infarction in affected organs, such as:

• Muscles: myalgias
• Nerves: mononeuropathy multiplex
• Gastrointestinal tract: mesenteric ischemia
• Kidney: New onset of hypertension (due to renal artery involvement)
• Skin: ulcers, nodules and livedo reticularis.

Vasculitis of the Small Blood Vessels:

Illnesses:

• Eosinophilic granulomatosis with polyangiitis, systemic vasculitis of small to medium vessels, characterized by asthma, transient pulmonary infiltrates and hypereosinophilia
• Cryoglobulinemic vasculitis, a rare multisystem disease characterized by the presence of circulating cryoprecipitable immune complexes in the serum
• Granulomatosis with polyangiitis
• Immunoglobulin A storage vasculitis (formerly known as Henoch-Schönlein purpura)
• Microscopic polyangiitis
• Cutaneous small vessel vasculitis

Symptoms:

• Symptoms of tissue infarction in affected organs similar to those of vasculitis involving medium-sized vessels, except skin lesions which tend to be purpuric
• At the renal level: glomerulonephritis

How is vasculitis diagnosed

First of all, it is necessary to consult a doctor who will evaluate the patient’s state of health and prescribe the necessary tests.

The first level of investigation involves performing blood tests or analyzing other body fluids in order to identify the possible presence of an active inflammatory disease that could indicate the presence of vasculitis.

The main values that must be carefully monitored and the result of which must be interpreted in the light of the clinical picture are:

• increased erythrocyte sedimentation rate (ESR)
• increased C-reactive protein (CRP)
• anemia
• increased white blood cell count and eosinophilia
• high levels of anti-neutrophil cytoplasmic antibodies (rarely)
• hematuria (rarely)

An angiogram (a contrast-enhanced x-ray of blood vessels) may show characteristic signs of inflammation of the affected vessels.

However, for the definitive diagnosis of vasculitis it is necessary to perform a biopsy of the involved vessel, i.e. the removal of a portion of the affected blood vessel.

Vasculitis: the most effective treatments

The treatment of vasculitis varies according to the etiology, type and extent and/or severity of the pathology.

In the case of secondary vasculitis, for example, the first approach involves removing the triggering cause (in the case of drugs, infections, tumors, etc.).

In the case of primary vasculitis, on the other hand, the treatment aims to induce remission using cytotoxic immunosuppressants or high-dose corticosteroids, usually for at least 3/6 months or in any case until sufficient remission of the inflammatory symptoms.

All patients treated with immunosuppressants should be regularly monitored and tested for tuberculosis and hepatitis B, as these diseases may reactivate following the administration of these therapies.

Based on the severity of the vasculitis, the treatments differ in:

• induction of remission for life-threatening vasculitis: administration of corticosteroids, often in combination with cyclophosphamide or rituximab;
• induction of remission for less severe vasculitis: administration of corticosteroids associated with milder immunosuppressants or rituximab;
• maintenance of the remissive state: methotrexate, azathioprine or rituximab is used, in combination with lower doses of corticosteroids.

Vasculitis: effects on daily life

An individual suffering from vasculitis must necessarily undergo periodic checks to verify the state of the disease and to be able to act promptly on any worsening of the clinical picture.

In cases where remission is obtained thanks to immunosuppressive therapies, the patient must take care to undergo periodic checks to verify not only the state of the disease, but also to evaluate any side effects of the treatment in question.

Even if the vasculitis is in remission, it is good practice to continue with periodic checks as it is possible that the disease will recur at any time.

Long-term drug treatment is often able to control symptoms, ensuring the patient a good quality of life.

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