Hemorrhagic vasculitis

Author Ольга Кияница

2018-11-07

Among all primary vasculitis, hemorrhagic vasculitis or Schönlein-Henoch disease is most common. This pathology is also known as rheumatic purpura. Against the background of this disorder, a shift occurs in the hemostasis system with the subsequent development of microthrombovasculitis. It can occur both at lightning speed and chronically, so the treatment is individually.

Hemorrhagic vasculitis (GDV or Schönlein-Genoha disease) is an inflammatory disease of the vasculitis group, in which small blood vessels (capillaries) are affected. Usually is a self-limited pathological condition. Synonyms - allergic purpura, allergic vasculitis, anaphylactoid purpura, hemorrhagic capillary toxicosis, leukocytoclastic vasculitis, nonthrombocytopenic idiopathic purpura, rheumatic purpura, vascular purpura.

This is the most common form of vascular inflammation in children (among vasculitis), which leads to inflammatory changes in small blood vessels.

The basis of the development of hemorrhagic vasculitis is the impairment of the immune system, which causes inflammation of the vascular wall. The disease is most often defined in children under 5 years of age, and in such cases the fulminant form of GDV most often develops. With timely treatment can improve the quality of life of the patient.

Video: Schonlein-Henoch Purpura - Hemorrhagic Vasculitis

Signs of hemorrhagic vasculitis

Symptoms of GDV usually begin suddenly and can be expressed as follows:

  • Headache.
  • Fever.
  • Loss of appetite
  • Spasms.
  • Abdominal pain.
  • Painful menstruation.
  • Urticaria
  • Bloody diarrhea.
  • Joint pain.

Red or purple spots usually appear on the skin and are called petechiae. In the classic case of a rash appear on the buttocks, back and legs .

(A and B) Classic skin lesions with GDV with palpable purpura on the limbs. (C) Arthritis and purpura on the lower limbs.(D) Bullous and necrotic lesions of the lower limbs in a patient with a complex course of purpura.

Inflammatory changes associated with GDV can also develop in the joints, kidneys, digestive system and, in rare cases, the brain and spinal cord (central nervous system).

Features of the manifestations of different forms of hemorrhagic vasculitis:

  1. With Schonlein purpura, the skin and joints are affected, but there is no inflammation of the gastrointestinal tract.
  2. In Genokha purpura, purple spots on the skin and acute processes in the abdominal cavity, such as glomerulonephritis (a type of kidney disease), are determined in patients. With this form of GDV, the joints are not affected.

In addition to the characteristic red spots on the skin, headache, loss of appetite and / or fever can be detected.

  • The skin usually becomes red (diffuse erythema).
  • Abdominal pains become most pronounced at night.
  • Blood may be present in the stool, and abnormal bleeding (hemorrhage) from the gastrointestinal tract is diagnosed as bloody diarrhea.
  • Pain in the joints (arthralgia) can be determined in any joint, but most often it affects the knee and in the area of ​​the ankles.
  • Some people with GDV have vomiting and diarrhea, while others have severe constipation and unusually dark stools (melena).

Spots of purpura are caused by small hemorrhages under the skin and are not associated with abnormally low platelet count (a non-thrombocytopenic disorder), which is characteristic of some other forms of purpura. Other skin lesions associated with GDV include large blisters (hives) or ulcers (tissue necrosis), especially on the buttocks and legs.

Swelling can occur on the face and neck due to abnormal accumulation of fluid in the soft tissues of the affected areas (angioedema). In rare cases, edema develops in the larynx, which can cause breathing difficulties that can lead to life-threatening respiratory complications.

One-quarter to one-half of patients with GDV experience problems with renal function, such as glomerulonephritis , in which renal tissue is damaged. As a result, the process of purifying blood from various metabolites is disturbed. The pathological process is expressed in the determination of blood in the urine (hematuria) and inflammatory changes in the kidneys. Some people may develop severe kidney disease, including nephropathy, chronic kidney inflammation (nephritis) and / or nephrotic syndrome, leading to renal failure.

In rare cases, part of the victim’s intestine may fold into itself ( invagination ). This leads to significant pain and, if conservative measures do not solve the problem, surgical intervention may be required.

When the central nervous system is involved, people with this disorder may experience severe headaches, perceptual changes, visual difficulties (atrophy of the optic nerve) and / or convulsions.

Causes of hemorrhagic vasculitis

The exact cause of GDV is not fully elucidated, although research shows that it is associated with an abnormal response of the immune system. In some cases, the causative factor is an extreme allergic reaction that develops to some offensive substances (for example, food or drugs).

Studies show that hemorrhagic vasculitis can be caused by dysfunction of the immune system (i.e. an increase in the number of IgA immune complexes). Autoimmune disorders occur when the body’s natural defense against “alien” or invading agents (such as antibodies) begins to attack healthy tissue for unknown reasons.

It has been suggested that this disorder may be the result of:

  • An extreme allergic reaction to certain foods, such as chocolate, milk, eggs or beans.
  • Various drugs (for example, nifedipine, diltiazem, cefuroxime, diclofenac, etc.), bacteria (for example, Streptococcus) and insect bites were also indicated as possible causes of GDV.
  • Rubella precedes the first symptoms of GDV in approximately 30% of cases.
  • About 66% of cases of respiratory infection of the upper respiratory tract precede the onset of symptoms by about 1–3 weeks.

The connection of GDV with viral infections has not been proven.

Video: Causes of Vasculitis

Prevalence

GDV is a rare disorder that affects more often men than women. The disease can occur in all age groups, but a high prevalence is observed among children.

In children, the initial symptoms usually begin after 2 years and last for about 4 weeks. The disease in such cases is characterized by a mild course. About 50% of sick children suffer one or more relapses within two to three months. The frequency of relapses appears to be higher in those children who had a more severe onset of the disease.

The age of affected children generally ranges from 2 to 11 years. In the US, for every 100,000 school-age children, there are about 14 cases. As a rule, a benign (non-threatening) disorder appears in most cases for self-treatment (self-restraint).

Related violations

Ordinary purpura is the most common type of disease, most common in women over 50 years of age. In the absence of damage, skin lesions occur more often than subcutaneous hemorrhage. However, after surgery or even minor injuries, the fragility of the blood vessels leads to excessive bleeding.

Bleeding can be reduced as a result of short-term corticosteroid therapy or the introduction of estrogen in postmenopausal women.

Tsinga , a type of purpura, is the result of a deficiency in vitamin C in the diet. Symptoms may include:

  • General weakness.
  • Anemia
  • Loosening of the gums.
  • The tendency to hemorrhages under the skin and mucous membranes that line the mouth and gastrointestinal tract.

Scurvy is rare in modern civilizations, except in regions remote from modern living conditions. Improved diet and the availability of foods containing vitamin C contributes to the prevention of the disease.

Gardner-Diamond syndrome, or autoerythrocyte syndrome , a rare disorder that is sometimes called painful bruising syndrome. It is characterized by spotted purpuras, mainly in young women. The occurrence of pathology is associated with an autoimmune disorder.

Vasculitis, or angiitis , is a vascular inflammatory disease that can occur alone or in combination with other allergic or rheumatic diseases. Inflammation of the vascular walls contributes to spasm of the blood vessels and can cause insufficient blood supply to certain areas of the body (ischemia), tissue death (necrosis) and / or the formation of blood clots (thrombosis). Vessels of any size or any part of the vascular system may be affected. Symptoms relate to the system involved and may include:

  • Fever.
  • Headache.
  • Deep loss of appetite.
  • Weight loss
  • Weakness.
  • Abdominal pain.
  • Diarrhea
  • Pain in the muscles and joints.

Since there are many forms of vasculitis, there are plenty of reasons for its development. Some types may be caused by allergic reactions or hypersensitivity to certain drugs, such as penicillin, propylthiouracil, other drugs, toxins, and various inhaled stimuli. Some forms can occur due to a fungal infection, parasites or viral infections, while in some cases there is no obvious reason.

Cutaneous necrotizing vasculitis is a relatively common inflammatory disease of the blood vessels, affecting veins, arteries and smaller blood vessels (capillaries). This disorder usually affects the skin and can occur alone or in combination with allergic, infectious or rheumatic diseases. Symptoms are:

  • Nodes on the skin.
  • Small hemorrhages under the skin and / or damage to it, which may be red and flat (macula).

They can form in many parts of the body, especially on the back, arms, buttocks and / or legs. In some cases, severely itchy urticaria or ring-shaped ulcers are present. There may also be fever, generalized discomfort (malaise) and / or pain in muscles and joints. The exact cause of cutaneous necrotizing vasculitis is unknown. Some lesions may be caused by an allergic reaction or hypersensitivity to some drugs, such as sulfate or penicillin, toxins and inhaled irritants.

Kawasaki disease is an inflammatory disease in children, characterized by fever, skin rash, swelling of lymph nodes (lymphadenopathy), inflammation of the arteries (polyarteritis) and inflammation of the blood vessels (vasculitis).Inflammatory changes cause destructive lesions in the blood vessels, which can lead to complications associated with the liver, gallbladder and heart. Symptoms may include:

  • Abnormally high body temperature, which begins suddenly and lasts for about two weeks.
  • Redness of the inner part of the eyelids of both eyes (bilateral conjunctivitis).
  • Irritability.
  • Fatigue.
  • Redness (inflammation) of the mouth and tongue (stomatitis).
  • Lip cracking
  • Swelling of the lymph nodes in the neck (cervical adenopathy).
  • Skin rash.

The exact cause of Kawasaki disease is not fully known. This may be due to two previously unknown strains of the bacteria Staphylococcus and Streptococcus and / or a possible immunological abnormality.

Immune thrombocytopenia is a rare platelet disorder characterized by an inexplicably low platelet count in the circulating blood. Symptoms may include:

  • Nosebleeds.
  • Small red or purple spots on the skin, which are hemorrhages under the skin (petechiae) and / or bleeding from the rectum and / or urinary tract.
  • Anemia can contribute to weakness and fatigue.
  • In other people with this disorder, episodes of increased fever and an abnormal enlargement of the spleen are determined.

No specific reason for the development of immune thrombocytopenia is known. Existing data confirm the immunological basis, since most patients have anti-platelet antibodies that can be identified.

Idiopathic thrombocytopenic purpura (ITP) is a rare blood disorder characterized by abnormally low levels of circulating platelets in the blood, abnormal destruction of red blood cells, renal dysfunction, and disorders of the nervous system. Symptoms of this disorder, which start suddenly, may include:

  • Fever
  • Headache.
  • Spots of purpura on the skin and mucous membranes.
  • Joint pain (arthralgia).
  • Partial loss of sensation in the arms or legs (paresis).
  • Change in mental state.
  • Cramps.

The exact cause of the CTP is unknown. It may be associated with an infectious agent or with an autoimmune reaction.

Diagnostics

The diagnosis of GDV can be difficult, especially in adults. The disease is often confused with other forms of vascular inflammation, which are presented above in the “Related disorders” section.

Routine laboratory tests do not allow for the most accurate diagnosis. Platelet count is usually normal, although leukocyte levels and sedimentation can be increased.

According to the classification criteria of the American Society of Rheumatology (ACR), a diagnosis can be made with a sensitivity of 87% and a specificity of 88% if the patient has two of the following four signs-criteria:

  1. Palpable purple.
  2. The patient is more than 20 years old.
  3. Abdominal angina (Compliance with this criterion is determined by the presence of diffuse abdominal pain and blood in the stool).
  4. Histological detection of granulocytes in the vascular wall of arterioles or venules. (Skin biopsy is rarely required).

If these criteria are not clearly expressed, it is necessary that, in addition to palpable purpura, at least one more criterion should be defined in the form of a normal number of platelets. Although abdominal symptoms may precede cutaneous manifestations, no diagnosis is usually made on the skin.

In laboratory tests for GDV most often noted:

  • Moderate increase in erythrocyte sedimentation rate.
  • Anti-nuclear antibody (ANA) is negative.
  • Autoantibodies to neutrophilic granulocyte antigens (ANCA) of the IgG class are not detected.

Further diagnosis is associated with symptoms and is used to identify complications. Researches can repeat at change of symptoms and is carried out in uncomplicated cases in out-patient conditions by the pediatrician. In severe cases, performed in the clinic, possibly with additional advice from relevant professionals.

Instrumental diagnostic methods:

  • Ultrasound examination of the abdominal cavity.
  • X-ray examination of the abdominal cavity.
  • Blood test in the stool.
  • Urinalysis to assess kidney damage (there may be significant proteinuria over 100 mg).
  • Biopsy of the kidneys.

The exclusion of brain involvement is performed using magnetic resonance imaging. Exclusion of involvement of the testes is performed by means of Doppler column imaging.

Standard therapy

The main directions of treatment:

  • If people with GDV have allergic reactions, they should strictly avoid irritating substances (for example, certain foods or medications).
  • If there are signs of streptococcal infection, antibiotic therapy is prescribed.
  • Uncomplicated cases of the disease in children often improve spontaneously with age.
  • Most patients do not receive specific treatment, while the prospects for recovery are favorable.
  • Inflammatory reactions are removed nonsteroidal or steroidal anti-inflammatory drugs.

If nonsteroidal anti-inflammatory drugs cannot relieve the symptoms, some patients are given glucocorticoid (steroid) drugs, such as prednisone. These drugs may be helpful in dealing with acute abdominal and joint pain. However, in some cases, soft tissue swelling (angioedema) may be caused by steroid drugs.

Dapsone may be prescribed when prednisone is contraindicated or does not help relieve symptoms. Some studies show that steroids do not shorten the duration of the disease or reduce the frequency or recurrence of symptoms. Other findings indicate that steroid treatment may help reduce the risk of kidney damage.

GDV patients who have developed kidney disease and renal failure, most often need mechanical cleaning of the blood from toxic substances - hemodialysis. Aggressive and supportive therapy may be required during an acute renal crisis.Some patients with severe kidney disease have a kidney transplant. However, the disease can recur in the presence of a transplanted kidney. Other treatments include symptomatic and supportive drugs.

Research therapy

Experimental treatment with combination drug therapy in the form of anticoagulants (that is, heparin and acenocoumarol), corticosteroids, and immunosuppressants was tested in adults with severe cases of GDV. Further research is needed to determine the long-term safety and efficacy of this form of therapy for treating the disorder in question.

Plasmapheresis as a means of removing unwanted substances (toxins, metabolic substances and parts of the plasma) from the blood has also been tested experimentally. The blood in a certain amount is taken from the patient, after which the blood cells are separated from the plasma. Then the plasma of the patient is then replaced with the plasma of another person and the blood is transferred back to the patient. This therapy is still being studied for analysis of side effects and effectiveness. More research is needed before plasmapheresis can be recommended for use in all cases except the most severely flowing GDV.

Intravenous immunoglobulin (IH) was used on an experimental basis to treat some children with severe abdominal pain associated with GDV. Further research is needed to determine the long-term safety and efficacy of immunoglobulins for treating this disorder.

Key points

  • Hemorrhagic vasculitis, or Schönlein-Genoch purpura, is characterized by the occurrence of inflammation of capillaries and other small-caliber vessels.
  • The disease is more often defined in children under 5, but can also occur in other age categories.
  • In hemorrhagic vasculitis, a reduced number of platelets is not detected, which is one of the criteria for diagnosing the disease.
  • The development of hemorrhagic vasculitis is mainly associated with rashes on the skin, especially in the area of ​​the legs, buttocks. The gastrointestinal tract or joints may also be affected.
  • Treatment of hemorrhagic vasculitis is mainly symptomatic, often including non-steroidal anti-inflammatory drugs, and in severe cases - glucocorticosteroids.
  • The prognosis is mostly favorable, and for the purposes of prophylaxis, it is necessary to carefully treat infectious diseases, and in the presence of allergic reactions, follow the doctor’s recommendations for maintaining immune responses within acceptable limits.

Video: Live Great! Hemorrhagic vasculitis


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