The Vasculitis, symptoms and treatment of vasculitis
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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 certain organs and systems.



Classification

At present there is no unified classification of vasculitis. In practice, the classification of vasculitis is most often used depending on the size of the affected vessel:

  1. Vasculitis of large vessels:
  • Temporal arteritis, a predominant lesion of the extracranial branches of the carotid artery and certain aortic sites, arises from rheumatism and is usually observed after 40 years.
  • Takayasu's syndrome - inflammation of the aorta, as well as its major branches, is more common at a young age (dote 40 years).
  1. Vasculitis of medium-sized vessels:
  • Kawasaki disease - inflammation of the arteries of any caliber, mainly of medium diameter. It leads to the defeat of 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 affecting the vessels of the microcirculatory rust and the development of glomerulonephritis.
  1. Vasculitis of small vessels:
  • Cutaneous angiitis leukocytoclastic - affects only the skin, glomerulonephritis does not develop.
  • Essential vasculitis cryoglobulinemic - the defeat of capillaries and venules, which leads to the defeat of the glomerular apparatus of the kidneys and small vessels of the skin.
  • Shenlaine-Henoch disease - inflammation of all vessels of the microcirculatory bed: venules, arterioles, capillaries. The pathological process involves the skin, joints, digestive organs and glomeruli of the kidneys.
  • Microscopic polyangiitis - necrotizing inflammation of small and medium vessels, necrotizing glomerulonephritis, pulmonary capillaritis.
  • The Charge-Strauss syndrome is an inflammation of the respiratory tract causing asthma and eosinophilia in the blood and accompanied by vasculitis of small and medium diameter vessels.
  • Wegener's granulomatosis is necrotizing glomerulonephritis, inflammation of the respiratory system, vascular lesions of the microcirculatory bed, and medium-sized arteries.

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

Causes of vasculitis

The most common causes of vasculitis are:

  1. Bacterial and viral infections. Narimer, it is revealed that some types of vasculitis occur more often after hepatitis B.
  2. Allergies to:
  • medicines (more than 150 medicines known to trigger the development of vasculitis are known: antibiotics, sulfonamide and iodine preparations, radiocontrast agents, analgesics, B vitamins, etc.).
  • own, endogenous antigens for rheumatism, systemic lupus erythematosus, paraneoplastic syndrome, congenital insufficiency of blood complement components.
  1. Heredity.

In some cases, the exact cause of the appearance of vasculitis can not be established.

Temporal arteritis

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

The main symptoms are:

  • intense head pain, especially in the temporal and occipital areas,
  • double vision, seizures of temporary blindness,
  • absence of pulse on peripheral arteries,
  • "Intermittent claudication" in the area of ​​masticatory, temporal muscles and tongue, when due to the arising pains it is necessary to stop the movement made by these muscles,
  • increased body temperature,
  • soreness and nodularity of the arteries, especially the temporal,
  • periodic pain in the neck,
  • omission of the century,
  • decreased appetite, malaise.

Diagnosis is based on a temporal artery biopsy, a clinical picture of the disease and angiography.

Treatment is performed with corticosteroid drugs for a long time, with a gradual decrease in dosage to avoid the occurrence of withdrawal syndrome. Refusal of treatment can provoke the development of irreversible blindness.

Takayasu's syndrome

Takayasu's syndrome is a systemic vasculitis, the cause of which is unknown. It is more common in Asian women between the ages of 15 and 30 and is commonly known as "a disease accompanied by a lack of pulse." With Takayasu's syndrome inflammation affects the arch of the aorta and large arteries and leads gradually to their blockage.

Symptoms

At the onset of the disease, in almost half the cases, there are:

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

Gradually, the process is chronicized and the disease acquires one of the following clinical forms of the course:

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

Diagnosis and treatment

The diagnosis is based on complete absence / loss of pulse, the appearance of noises over the affected areas of arteries, the appearance of symptoms of regional ischemia, and angiography data (conical or uniform narrowing of the thoracic, abdominal and aortic arch and its branches) and ultrasound (dopplerography).

Treatment is based on a long-term intake of corticosteroid drugs (prednisolone), which lasts at least 12 months. In addition, vascular drugs (komplamin, prodektin), anticoagulants, antihypertensives and cardiac glycosides are prescribed. In some cases, surgical intervention is indicated.

Kawasaki disease

Kawasaki disease is a systemic arteritis causing damage affecting large, medium and small arteries, which is often combined with a mucocutaneous lymphatic syndrome. It occurs usually in childhood.

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

The mechanism of development of Kawasaki's disease is the development of antibodies to the inner shell 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 reaction.

Symptoms

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

The main symptoms of Kawasaki are as follows:

  1. The temperature rises for 2 weeks.
  2. Leather:

- Occurrence of urticaria rash in the perineum, trunk and proximal limbs during the first 5 weeks from the onset of the disease.

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

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

  1. Eyes:

- bilateral conjunctivitis ,

- Anterior uveitis.

  1. Oral cavity:

- redness and dryness of the mucosa,

Bleeding and cracks in the lips,

- signs of catarrhal angina,

- bright red language.

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

- myocarditis,

  • heart failure,
  • pericarditis,

- aneurysms of the coronary arteries, the 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. Organs of digestion:

- stomach ache,

- diarrhea,

- vomiting.

  1. Other symptoms that occur rarely:

- Aseptic meningitis,

- Urethritis.

Diagnosis and treatment

The diagnosis of Kawasaki's disease is based on the following basic criteria:

  1. The duration of the fever is not less than 5 days.
  2. 2-sided conjunctivitis (reddening of the conjunctiva).
  3. Reddening of the tonsils, swelling and dry lips, "crimson" tongue.
  4. Peripheral parts of the limbs: erythema, edema, skin peeling.
  5. Polymorphic rash.
  6. Cervical lymphadenopathy.

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

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

Node panarteritis

Panarteritis nodular is a necrotizing vasculitis that affects small and medium arteries and leads to secondary damage to tissues and organs. Consequences of severe panarteritis are cicatricial changes, hemorrhages and heart attacks.

The causes of nodular panarteritis are diverse. These include:

  • allergy to medicines, vaccines, serums,
  • food and cold allergy,
  • viral infection (hepatitis B, herpes, cytomegalovirus ),
  • Pollinosis.

The defeat of the vascular wall is accompanied by increased blood coagulability and the development of secondary thrombosis.

Symptoms

Symptoms of a general nature first appear:

  • fever, passing after taking corticosteroids and not stopping with antibiotics,
  • musculo-articular pain, especially in calf muscles and large joints,
  • progressive weight loss, it is more pronounced than with oncological diseases and can account for several months of weight loss of 30-40 kg.

Classical nodular panarteritis is characterized by five major syndromes that determine the entire picture of the disease and include a lesion:

  1. Renal vessels.
  2. Organs of the abdominal cavity.
  3. The lungs.
  4. Hearts.
  5. Peripheral nervous system.

Kidneys

The kidney damage is observed in 9 out of 10 patients and manifests itself:

  • persistent increase in blood pressure,
  • severe retinopathy,
  • micro- and macrohematuria,
  • development of the perineal hematoma,
  • the progression of signs of renal failure for several years.

Abdomen

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

  • diffuse, persistent, intensifying pain,
  • Diarrhea (up to 10 times a day),
  • decrease or lack of appetite,
  • nausea, vomiting.

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

Lungs

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

A heart

The defeat of the heart vessels with nodular panarteritis leads to the appearance of small-focal infarctions, rhythm disturbance, progressive cardiosclerosis and development, and ultimately, heart failure.

Nervous system

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

Other

The defeat of the vessels of one or another area can lead to necrosis or gangrene of the organ.

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

  1. Classic - polysyndromicity manifestations, the presence of weight loss, fever, kidney damage, myalgia, polyneuritis.
  2. Asthmatic - bronchospasm, which makes this disease similar to ordinary bronchial asthma.
  3. Skin-thrombangiotic - isolated lesion of the skin and subcutaneous fat against the background of weight loss, fever and muscle pain.
  4. Mono-organic - persistent arterial hypertension due to renal vascular disease, which comes to the fore.

Diagnostics

Nodular panarritis is diagnosed on the basis of 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 corticosteroids and immunosuppressants. Also in parallel, funds are allocated to improve blood microcirculation and rheology, and also, if necessary, antihypertensive, analgesic, antiasthmatics.

Essential vasculitis cryoglobulinemic (ECV)

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

The causes of ECV development have not been fully established, however, there is a connection with the presence of chronic viral hepatitis C and B. Cryoglobulins, which are whey proteins that can fold at temperatures below 37 degrees, practically clog the small vessels and lead to the formation of hemorrhagic purpura.

Symptoms

The main features of ECB are:

  • hemorrhagic purpura, which is a small-spotted or petechial non-judicious rash in the region of the shins, abdomen, buttocks;
  • migratory polyartralgia of a symmetrical nature involving proximal interphalangeal, metacarpophalangeal, knee, as well as ankle and elbow joints;
  • Raynaud's syndrome ;
  • polyneuropathy (parasthesia, hyperesthesia, numbness in the lower extremities),
  • dry Sjogren's syndrome,
  • enlargement of the liver, kidneys,
  • development of glomerulonephritis and renal failure,
  • pain in the muscles,
  • abdominal pain, lung infarction, etc.

Diagnosis and treatment

The most characteristic changes for ECV are:

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

In addition, CT of chest, liver ultrasound, biopsy of affected skin and kidneys are prescribed.

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

Shenlaine-Henoch disease

Shenlaine-Henoch disease is a 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,
  • supercooling,
  • food allergy.

Symptoms

There are several forms of the disease:

  1. Simple (cutaneous) - hemorrhagic rash, some of which can be necrotic. 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. Articular (rheumatoid) - pain in the large joints of the lower limbs, often accompanied by myalgia and skin lesions.
  3. Abdominal - spastic abdominal pain, periodic nausea and vomiting, gastrointestinal bleeding.
  4. Renal - proteinuria, macro- or microglobulinuria, development of glomerulonephritis.
  5. Lightning fast - extensive hemorrhage, gangrene of the feet, brushes, other necrotic lesions, which often occur in children of the first 2 years of life after a child's infection.
  6. Mixed - is characterized by the presence of symptoms of several forms of vasculitis.

Diagnosis and treatment

The diagnosis of rheumatic purpura is based on 4 main criteria:

  1. Palpable purple.
  2. The onset of the disease is before the age of 20.
  3. Pain in the abdomen, which increase after eating.
  4. Identification of granulocytes in biopsy arterioles and venules.

Treatment is reduced to the appointment of a hypoallergenic diet, the use of antiaggregants and anticoagulants, glucocorticosteroids and immunosuppressants.


| 24 November 2014 | | 2 064 | Uncategorized
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