- Causes of vasculitis
- Temporal arteritis
- Takayasu's syndrome
- Kawasaki disease
- Nasal panarteritis
- Essential vasculitis cryoglobulinemic (ECV)
- Shenlaine-Henoch disease
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:
- Vasculitis of large vessels:
- Temporal arteritis - a predominant lesion of the extracranial branches of the carotid artery and some aortic areas, 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 (dot 40 years).
- 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 panarritis - necrotizing inflammation of small and medium diameter arteries without vascular injury of the microcirculatory rust and development of glomerulonephritis.
- Vasculitis of small vessels:
- Cutaneous angiitis leukocytoclastic - only the skin is affected, 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. In the pathological process, the skin, joints, digestive organs and glomerulus of the kidneys are involved.
- 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 vessels of small and medium diameter.
- Wegener's granulomatosis is necrotizing glomerulonephritis, inflammation of the respiratory system, vascular lesions of the microcirculatory bed, and medium-sized arteries.
Causes of vasculitis
The most common causes of vasculitis are:
- Bacterial and viral infections. Narimer, it is revealed that some types of vasculitis occur more often after hepatitis B.
- Allergies to:
- Medicinal products (more than 150 drugs known to be able to trigger the development of vasculitis: 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.
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 chewing, 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 carried out 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 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 known in everyday life 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.
At the onset of the disease, in almost half the cases, there are:
- joint pain,
- Sleep disorder,
- weight loss,
Gradually, the process is chronicized and the disease acquires one of the following clinical forms of the course:
- I type - lack of pulse, defeat of the arteries of subclavian and brachiocephalic trunk.
- II type - a combination of symptoms of types I and III.
- Type III - 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 determined on the basis of complete absence / loss of pulse, the appearance of noises over the affected areas of arteries, the appearance of symptoms of regional ischemia, as well as angiography (conical or uniform narrowing of the thoracic, abdominal and aortic arch and its branches) and ultrasound (dopplerography).
Treatment is based on long-term use of corticosteroid drugs (prednisolone), which lasts at least 12 months. In addition, vascular drugs (komplamin, prodectin), anticoagulants, antihypertensives and cardiac glycosides are prescribed. In some cases, surgical intervention is indicated.
Kawasaki disease is a systemic arteritis causing damage affecting large, medium and small arteries, which is often combined with dermal-mucous 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-Barr, retroviruses or rickettsia. It is also possible hereditary predisposition.
At the basis of the mechanism of development of Kawasaki disease lies the development of antibodies to the inner shell of the vessels: on the membranes of the endothelial cells of the arteries appears the Kawasaki antigen, and antibodies, interacting with it, cause a corresponding inflammatory reaction.
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:
- The temperature rises for 2 weeks.
- 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 area of the palms and soles.
- Development of erythema, which after 2-3 weeks gives way to ecdysis.
- bilateral conjunctivitis ,
- Anterior uveitis.
- Oral cavity:
- redness and dryness of the mucosa,
Bleeding and cracks in the lips,
- signs of catarrhal angina,
- bright red language.
- Lymphatic system: an increase in cervical lymph nodes.
- The cardiovascular system:
- heart failure,
- 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.
- arthralgia (pain),
- development of polyarthritis of small, as well as knee and ankle joints.
- Organs of digestion:
- stomach ache,
- Other symptoms that occur rarely:
- Aseptic meningitis,
Diagnosis and treatment
The diagnosis of Kawasaki's disease is based on the following basic criteria:
- The duration of the fever is not less than 5 days.
- 2-sided conjunctivitis (reddening of the conjunctiva).
- Reddening of the tonsils, swelling and dry lips, "crimson" tongue.
- Peripheral parts of the limbs: erythema, edema, skin peeling.
- Polymorphic rash.
- Cervical lymphadenopathy.
If there are 5 of 6 criteria, the diagnosis is confirmed.
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 cicatrical 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 ),
The defeat of the vascular wall is accompanied by increased blood coagulability and the development of secondary thrombosis.
Symptoms of a general nature first appear:
- Fever that occurs after taking corticosteroids and does not stop 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 be a few months of loss of body weight 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:
- Renal vessels.
- Organs of the abdominal cavity.
- Peripheral nervous system.
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 insufficiency for several years.
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.
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.
The defeat of the blood vessels in the nodular panarteritis leads to the emergence of small-focal heart attacks, rhythm disturbance, progressive cardiosclerosis and development, ultimately, heart failure.
Almost half of the patients develop polyneuritis, manifested by sharp pains, parasthesias and paresis (especially in the lower extremities).
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:
- Classic - polysyndromicity manifestations, the presence of weight loss, fever, kidney damage, myalgia, polyneuritis.
- Asthmatic - bronchospasm, which makes this disease similar to ordinary bronchial asthma.
- Skin-thrombangiotic - isolated lesion of the skin and subcutaneous fat against a background of weight loss, fever and muscle pain.
- Mono-organic - persistent arterial hypertension due to renal vascular disease, which comes to the fore.
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 is carried out with the help of corticosteroid drugs and immunosuppressants. Also in parallel, funds are allocated to improve blood microcirculation and rheology, and, 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.
The main features of ECB are:
- Hemorrhagic purpura, which is a small-spotted or petechial non-pruritic 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,
- An increase in ESR,
- Presence of rheumatoid factor,
- Detection of cryoglobulins.
In addition, CT of chest, liver ultrasound, biopsy of affected skin and kidneys are prescribed.
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,
- Food allergy.
There are several forms of the disease:
- Simple (cutaneous) - hemorrhagic rash, some parts 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.
- Articular (rheumatoid) - pain in the large joints of the lower limbs, often accompanied by myalgia and skin lesions.
- Abdominal - spastic abdominal pain, periodic nausea and vomiting, gastrointestinal bleeding.
- Renal - proteinuria, macro- or microglobulinuria, development of glomerulonephritis.
- 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.
- 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:
- Palpable purple.
- The onset of the disease is before the age of 20.
- Pain in the abdomen, which increase after eating.
- Detection of granulocytes during arteriolar and venular biopsy.
Treatment is reduced to the appointment of a hypoallergenic diet, the use of antiaggregants and anticoagulants, glucocorticosteroids and immunosuppressants.