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Vasculitis

Content:

Vasculitis is a heterogeneous group of diseases, which are based on inflammation of the vascular wall with subsequent damage to some organs and systems.



Classification

Currently there is no single classification of vasculitis. In practice, the most commonly used classification of vasculitis, depending on the size of the affected vessel:

  1. Vasculitis of large vessels:
  • Temporal arteritis is a predominant lesion of the extracranial branches of the carotid artery and some parts of the aorta, caused by rheumatism and is usually observed after 40 years.
  • Takayasu syndrome - inflammation of the aorta, as well as its main branches, is more common at a young age (40 years old).
  1. Vasculitis of vessels of average caliber:
  • Kawasaki disease is an inflammation of the arteries of any caliber, mostly of medium diameter. Causes damage to the coronary arteries, aorta and veins. It is observed in most cases in children.
  • Nodular panarteritis is a necrotizing inflammation of the arteries of small and medium diameter without damaging the vessels of microcirculatory rust and the development of glomerulonephritis.
  1. Vasculitis of small vessels:
  • Leukocytoclastic cutaneous angiitis - only the skin is affected, glomerulonephritis does not develop.
  • Essential cryoglobulinemic vasculitis is a lesion of the capillaries and venules, which leads to the defeat of the glomerular apparatus of the kidneys and small vessels of the skin.
  • Schönlein-Henoch disease - inflammation of all vessels of the microvasculature: venules, arterioles, capillaries. The skin, joints, digestive organs and kidney glomeruli are involved in the pathological process.
  • Microscopic polyangiitis - necrotizing inflammation of small and medium-sized vessels, necrotizing glomerulonephritis, pulmonary capillary.
  • Chardz-Stross syndrome is an inflammation of the airways that causes asthma and eosinophilia in the blood and is accompanied by vasculitis of small and medium diameter vessels.
  • Wegener's granulomatosis - necrotizing glomerulonephritis, inflammation of the respiratory system, vascular lesion of the microvasculature, as well as medium-caliber arteries.

Some of the vasculitis are systemic (several body systems are affected), while for others only the skin is involved in the pathological process.

Causes of vasculitis

The most common causes of vasculitis are:

  1. Bacterial and viral infections. Narpimer, revealed that some types of vasculitis more often occur after suffering hepatitis B.
  2. Allergy to:
  • drugs (more than 150 drugs are known that can trigger vasculitis development: antibiotics, sulfa and iodine-containing drugs, radiopaque substances, analgesics, B vitamins, etc.).
  • own, endogenous antigens with rheumatism, systemic lupus erythematosus, paraneoplastic syndrome, congenital insufficiency of the components of the blood complement.
  1. Heredity.

In some cases, the exact cause of vasculitis cannot be established.

Temporal arteritis

Temporal arteritis is cunning in that it can cause vision loss due to the death of the retina and optic nerve fibers as a result of impaired blood circulation.

Main symptoms:

  • intense headache, especially in the temporal and occipital regions,
  • double vision, temporary blindness,
  • lack of pulse in peripheral arteries,
  • “Intermittent claudication” in the area of ​​the masticatory, temporal muscles and tongue, when, due to the resulting pain, it is necessary to stop the movement of these muscles,
  • fever,
  • soreness and nodularity of the arteries, especially the temporal,
  • recurrent neck pain
  • omission of a century
  • loss of appetite, malaise.

The diagnosis is made on the basis of a biopsy of the temporal artery, the clinical picture of the disease and angiography.

Treatment is carried out with corticosteroid drugs for a long time, with a gradual decrease in dosage, in order to avoid the appearance of withdrawal syndrome. Refusal of treatment may trigger the development of irreversible blindness.

Takayasu syndrome

Takayasu syndrome is a systemic vasculitis, the cause of which is unknown. It is more common in Asian women aged 15 to 30 years and in everyday life is known as "the disease, accompanied by a lack of pulse." In Takayasu syndrome, inflammation affects the aortic arch and large arteries and gradually leads to blockage.

Symptoms

At the beginning of the disease in almost half of the cases occur:

  • fever,
  • malaise,
  • joint pain,
  • sleep disturbance
  • fatigue,
  • weight loss,
  • anemia.

The process is gradually chronized and the disease acquires one of the following clinical forms of the course:

  • Type I - lack of pulse, damage to the arteries of the subclavian and brachiocephalic trunk.
  • Type II - a combination of symptoms of types I and III.
  • Type III - atypical narrowing (stenosis) in the region of the thoracic or abdominal aorta at a certain distance from the arc and main branches (this type is most common).
  • Type IV - expansion and stretching of the site of the aorta and its main branches.

Diagnosis and treatment

The diagnosis is determined on the basis of complete absence / weakening of the pulse, the appearance of noise over the affected areas of the arteries, the appearance of symptoms of regional ischemia, as well as angiography data (conical or even narrowing of the thoracic, abdominal and aortic arch and its branches) and ultrasound (Doppler).

Treatment is based on long-term use of corticosteroid drugs (prednisone), which lasts at least 12 months. Additionally, vascular drugs (komplamin, prodectin), anticoagulants, antihypertensive drugs and cardiac glycosides are prescribed. In some cases, surgery is indicated.

Kawasaki disease

Kawasaki disease is a systemic arteritis that causes damage to large, medium and small arteries, which is often combined with mucosal lymphatic syndrome. It usually occurs in childhood.

It is believed that the main cause of the development of this disease is infection with herpes viruses, Epstein-Barr, retroviruses or rickettsiae. Hereditary predisposition is also possible.

The basis of the mechanism for the development of Kawasaki disease is the production of antibodies to the inner lining of the vessels: the Kawasaki antigen appears on the membranes of the endothelial cells of the arteries, and the antibodies, interacting with it, cause a corresponding inflammatory response.

Symptoms

Usually. There are 3 main periods of the disease: acute (up to 1.5 weeks), subacute (up to 1 month) and recovery period (up to several years).

The main symptoms of Kawasaki are as follows:

  1. Temperature increase within 2 weeks.
  2. Leather:

- The appearance of an urticarial rash in the crotch area, torso and proximal extremities during the first 5 weeks of the onset of the disease.

- The occurrence of painful seals in the palms and soles.

- The development of erythema, which after 2-3 weeks gives way to scaling.

  1. Eyes:

- bilateral conjunctivitis ,

- anterior uveitis.

  1. Oral cavity:

- redness and dryness of the mucous,

- bleeding and cracked lips,

- signs of catarrhal angina,

- bright red tongue.

  1. Lymphatic system: an increase in cervical lymph nodes.
  2. The cardiovascular system:

- myocarditis,

  • heart failure,
  • pericarditis,

- coronary artery aneurysm, development of myocardial infarction within 1.5 months from the onset of the disease,

- the formation of aneurysms in the ulnar, iliac, femoral, subclavian arteries,

- development of mitral or aortic insufficiency.

  1. Joints:

- arthralgia (pain),

- development of polyarthritis of small, as well as knee and ankle joints.

  1. Digestive organs:

- stomach ache,

- diarrhea,

- vomiting.

  1. Other symptoms that rarely occur:

- aseptic meningitis,

- urethritis.

Diagnosis and treatment

The diagnosis of Kawasaki disease is made on the basis of the following basic criteria:

  1. Fever duration of at least 5 days.
  2. 2-sided conjunctivitis (conjunctival redness).
  3. Redness of the tonsils, swelling and dry lips, "crimson" tongue.
  4. Peripheral limbs: erythema, swelling, peeling of the skin.
  5. Polymorphic rash.
  6. Cervical lymphadenopathy.

If 5 of 6 criteria are present, the diagnosis is considered confirmed.

Treatment of Kawasaki disease is carried out by prescribing immunoglobulin and aspirin.

Nodular panarteritis

Panarteritis nodosa is a necrotizing vasculitis that affects the small and medium arteries and leads to a secondary lesion of tissues and organs. The consequences of severe panarteritis are cicatricial changes, hemorrhages and heart attacks.

The causes of nodular panarteritis are varied. These include:

  • allergy to drugs, vaccines, serums,
  • food and cold allergies,
  • viral infection (hepatitis B, herpes, cytomegalovirus ),
  • pollinosis.

Damage to the vascular wall is accompanied by increased blood clotting and the development of secondary thrombosis.

Symptoms

First, symptoms of a general nature appear:

  • fever that passes after taking corticosteroids and does not stop with antibiotics,
  • muscle and joint pain, especially in the calf muscles and large joints,
  • progressive weight loss, it is more pronounced than with cancer and can be a weight loss of 30-40 kg in a few months.

Classic nodular panarteritis is characterized by five main syndromes that define the whole picture of the disease and include lesions:

  1. Vascular kidney.
  2. Abdominal organs.
  3. Lung.
  4. Hearts.
  5. Peripheral nervous system.

Kidney

Kidney damage occurs in 9 out of 10 patients and manifests itself:

  • persistent increase in blood pressure
  • severe retinopathy,
  • micro and gross hematuria,
  • development of kidney hematoma,
  • progression of signs of renal failure within a few years.

Abdomen

The organs and vessels of the abdominal cavity are affected at the very beginning of the development of the disease. Appear:

  • spilled, persistent, increasing in intensity of pain,
  • diarrhea (up to 10 times a day),
  • decrease or lack of appetite
  • nausea, vomiting.

In some cases, peritonitis or gastrointestinal bleeding occurs due to intestinal gangrene or perforation of the ulcer.

Lungs

Lung damage in a third of patients is manifested by bronchospasm, as well as the occurrence of eosinophilic pulmonary infiltrates. Also characterized by the development of vascular pneumonia, characterized by unproductive cough, hemoptysis, and increasing signs of respiratory failure.

A heart

The defeat of the heart vessels in nodular panarteritis leads to the emergence of small focal infarction, arrhythmia, progressive cardiosclerosis and the development, ultimately, of heart failure.

Nervous system

Almost half of the patients develop polyneuritis, manifested by sharp pains, parasthesia and paresis (especially in the lower extremities).

Other

Damage to the vessels of a particular area can lead to necrosis or gangrene of the organ.

According to the clinical course, the following variants of the course of the disease are distinguished:

  1. Classic - polysyndromic manifestations, the presence of weight loss, fever, kidney damage, myalgia, polyneuritis.
  2. Asthmatic - bronchospasm, which makes the disease similar to normal bronchial asthma.
  3. Thromboangiitis skin-an isolated lesion of the skin and subcutaneous fat on the background of weight loss, fever and muscle pain.
  4. Monoorganic - persistent arterial hypertension due to vascular lesions of the kidneys, which comes to the fore.

Diagnostics

Nodular panarteritis is diagnosed based on the history of the disease, a typical clinical picture and laboratory data (neutrophilic leukocytosis, increased ESR, the presence of C-reactive protein, etc.).

Treatment

Treatment is carried out with the help of corticosteroid drugs and immunosuppressants. Also, drugs for improving microcirculation and blood rheology, as well as, if necessary, antihypertensives, painkillers, anti-asthma drugs are also prescribed in parallel.

Essential cryoglobulinemic vasculitis (ECV)

ECV is a systemic vasculitis characterized by the deposition of cryglobulinemic immune complexes in arterioles, capillaries, and venules, resulting in damage to internal organs and systems. It develops mainly in women after 50 years, especially after hypothermia.

The reasons for the development of ECV are not fully established, but there is a connection with the presence of chronic viral hepatitis C and B. Cryoglobulins, which are whey proteins that can coagulate at temperatures below 37 degrees, almost clog small vessels and lead to the formation of hemorrhagic purpura.

Symptoms

The main signs of ECV are:

  • hemorrhagic purpura, which is a small-blooded or petechial non-irritating rash in the legs, abdomen, buttocks;
  • symmetric migratory polyarthralgias with involvement of proximal interphalangeal, metacarpophalangeal, knee, and ankle and elbow joints;
  • Raynaud's syndrome ;
  • polyneuropathy (parastezii, hyperesthesia, numbness in the lower extremities),
  • dry sjogren's syndrome
  • enlarged liver, kidney,
  • development of glomerulonephritis and renal failure,
  • muscle pain
  • abdominal pain, pulmonary infarction, etc.

Diagnosis and treatment

The most characteristic changes for ECV are:

  • increase gamma globulins
  • increased ESR,
  • the presence of rheumatoid factor
  • detection of cryoglobulins.

Additionally, CT scan of the chest, ultrasound of the liver, biopsy of the affected skin and kidneys are prescribed.

Treatment includes plasmapheresis, interferon, glucocorticoids, cyclophosphamide, antiviral therapy, monoclonal antibodies (rituximab).

Schönlein-Henoch disease

Schönlein-Henoch disease is rheumatic purpura or allergic systemic vasculitis, which is based on aseptic inflammation of small vessels, multiple microthrombogenesis, damage to the skin, kidneys, intestines and other internal organs.

In most cases, this vasculitis begins to develop after infectious diseases of the upper respiratory tract. Among other reasons, experts note:

  • taking certain medications, for example, ampicillin, lisinopril, aminazine, enalapril, quinidine, erythromycin, penicillin,
  • insect bites,
  • hypothermia
  • food allergies.

Symptoms

There are several forms of the disease:

  1. Simple (skin) - hemorrhagic rash, some parts of which can necrotize. At the beginning of the disease, the rash is mainly concentrated in the distal parts of the lower limbs, and then spreads to the buttocks and thighs.
  2. Joint (rheumatoid) - pain in large joints of the lower extremities, often accompanied by myalgia and skin lesions.
  3. Abdominal - spastic abdominal pain, recurrent nausea and vomiting, gastrointestinal bleeding.
  4. Renal - proteinuria, macro- or microglobulinuria, development of glomerulonephritis.
  5. Fulminant - extensive hemorrhages, gangrene of the feet, hands, other necrotic lesions that occur more often in children of the first 2 years of life after suffering a childhood infection.
  6. Mixed - characterized by the presence of symptoms of several forms of vasculitis.

Diagnosis and treatment

The diagnosis of rheumatic purpura is made on the basis of 4 main criteria:

  1. Palpable purple.
  2. The onset of the disease before the age of 20.
  3. Abdominal pains that get worse after eating.
  4. Detection of granulocytes in arteriole and venules biopsy.

The treatment comes down to the prescription of a hypoallergenic diet, the administration of antiplatelet agents and anticoagulants, glucocorticosteroids and immunosuppressants.


| November 24, 2014 | | 2,064 | Uncategorized
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