Causes and mechanism of development (Etiopathogenesis)
The pathology is most common in young people. The disease rarely occurs spontaneously and is usually preceded by certain triggers. The main precipitating factors include prior acute infections, episodes of hypothermia, allergic reactions (to food products or medications), as well as alcohol consumption. In addition, there is a clear link with chronic viral diseases, specifically hepatitis B and C viruses.
The pathological process is based on an immunological failure. In response to a trigger, an antigen-antibody cascade reaction is initiated. Circulating immune complexes (CIC) consisting predominantly of immunoglobulins begin to actively form in the bloodstream. These complexes deposit on the inner lining of small blood vessels, causing systemic hyperergic inflammation, microthrombosis, and increased permeability.
Main clinical syndromes
Disease manifestation is usually acute and accompanied by general symptoms: fever and signs of pronounced intoxication. The full clinical picture comprises four leading syndromes:
- Cutaneous hemorrhagic syndrome. The hallmark of the disease. Symmetric rashes appear on the skin as pinprick hemorrhages (petechiae) or larger spots (purpura). The preferred localization of the rash is the extensor surfaces of the lower extremities. The elements often coalesce, and in severe cases, they may become necrotic.
- Articular syndrome. Manifests as joint pain (arthralgia) or the development of full-blown arthritis. An important feature is that the inflammation is benign and resolves without leaving joint deformities.
- Abdominal syndrome. Characterized by sharp abdominal pain. The danger lies in the high risk of gastrointestinal bleeding. It is important to remember that abdominal symptoms may be the initial presentation of the disease, appearing before the skin rash.
- Renal syndrome. Manifests as glomerulonephritis, occurring in hematuric (blood in urine) or nephrotic variants. This is the most serious manifestation of the disease due to the risk of a rapidly progressive course resulting in chronic renal failure (CRF).
Differential diagnosis
When establishing a diagnosis, the clinician must exclude the secondary nature of hemorrhagic purpura, which can mimic an independent disease. Differential diagnosis is carried out with the following pathologies:
- Infective endocarditis.
- Septic conditions (specifically meningococcemia, which is also characterized by a hemorrhagic rash).
- Chronic viral hepatitis.
Management tactics and prognosis
The long-term prognosis for the patient directly depends on the severity of the renal syndrome: the rate of progression of glomerulonephritis and the pace of development of renal failure.
In the acute phase of the disease, strict semi-bed rest is prescribed for the patient. Basic drug therapy includes the administration of desensitizing medications. In severe cases, particularly in the presence of abdominal syndrome, glucocorticosteroids (prednisolone) are indicated. Plasmapheresis is used as an additional treatment method. This procedure helps remove pathogenic circulating immune complexes from the blood.
Briefly: answers to topical questions
Which conditions are used in the differential diagnosis of the cutaneous syndrome in hemorrhagic vasculitis?
Differential diagnosis of the cutaneous syndrome (purpura) in hemorrhagic vasculitis is primarily performed with conditions that cause secondary purpura.
Diseases for differential diagnosis:
- Infective endocarditis — the secondary nature of the rash must be excluded.
- Sepsis — specifically presenting as meningococcemia.
- Chronic hepatitis.
| Disease | Features of differential diagnosis |
|---|---|
| Subacute infective endocarditis | Fever, glomerulonephritis, leukopenia, anemia, and elevated ESR are present. Blood cultures are performed in doubtful cases. |
| Rheumatoid arthritis | Has a chronic course spanning many years, persistent joint deformities, and high titers of rheumatoid factor. |
Which groups of drugs, apart from desensitizing agents and GCS, are used to treat hemorrhagic vasculitis?
In addition to desensitizing agents and glucocorticosteroids, three main groups of drugs are used depending on the severity of clinical manifestations.
- Antiplatelet agents — form the basis of therapy in mild cases of the disease (dipyridamole, pentoxifylline, or ticlopidine are used).
- Anticoagulants — heparin preparations serve as the basis of treatment for moderate and severe forms of vasculitis.
- Cytostatics — are prescribed exclusively for high clinical and laboratory activity of glomerulonephritis (the drug of choice is cyclophosphamide in combination with glucocorticoids).
The clinical picture forms a classic tetrad (CSAP): Skin (purpura), Joints (arthritis), Abdomen (pain), Kidneys (nephritis).
Class and exam questions
Does vasculitis always start with a rash on the legs?
No, the disease may debut with a pronounced abdominal syndrome (sharp abdominal pain) even before the appearance of the characteristic skin rash.
Does joint syndrome in this vasculitis lead to disability?
No, arthritis in hemorrhagic vasculitis is benign and resolves without causing irreversible joint deformities.
What determines the severity and prognosis of the disease?
The prognosis directly depends on the degree of renal involvement—the presence of glomerulonephritis and the risk of its transition to chronic renal failure.
What else to study on this topic
- Mechanisms of formation and pathogenic action of circulating immune complexes (CIC)
- Hematuric and nephrotic variants of glomerulonephritis
- Algorithm of differential diagnosis with meningococcemia
- The role of hepatitis B and C viruses in the development of systemic vasculitis
- Indications and mechanism of action of plasmapheresis in immune complex pathologies
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