Systemic vasculitis is a heterogeneous group of clinical syndromes characterized by inflammation and damage to blood vessels. As a rule, they have a severe course, but timely diagnosis and timely therapy significantly improve the prognosis for the patient.
The skin lesions characteristic of systemic vasculitis often lead these patients to dermatologists in the first place. Therefore, despite the fact that systemic vasculitis belongs to the field of rheumatology, every doctor should have knowledge of the typical clinical picture of the most common diseases.
In this article on estet-portal.com, we will look at such variants of systemic vasculitis as Henoch-Schonlein purpura, cryoglobulinemic vasculitis and Churg-Strauss syndrome.
Symptoms of systemic vasculitis: how to recognize the disease in time
The physician should suspect a patient of systemic vasculitis in cases where the disease is characterized by generalized ischemic organ damage and the development of characteristic rashes.
The basis of systemic vascular disease may be narrowing of their lumen, inflammation, thrombosis or aneurysms.
The size and location of the affected blood vessel determine the clinical presentation of systemic vasculitis.
The following general symptoms of systemic vasculitis should be highlighted:
1. Fever and weight loss;
2. Palpable purpura, livedo reticularis, necrotizing skin lesion;
3. Myalgia, myositis, arthralgia, arthritis;
4. Epistaxis, sinusitis, hemoptysis, pneumonitis, asthma;
5. Intermittent claudication, poor peripheral pulse filling;
6. Abdominal pain, melena;
7. Glomerulonephritis;
8. Increased levels of SOE, CRP (C-reactive protein), ANCA (antinuclear antibodies), anemia, leukocytosis, thrombocytosis, proteinuria, hematuria.
For more information on the clinical presentation of systemic vasculitis such as Henoch-Schonlein purpura, cryoglobulinemic vasculitis, and Churg-Strauss syndrome, read further in the article.
Hemorrhagic vasculitis is triggered by cold weather and interseasonal viruses
Shenlein-Genoch Purpura – most common variant of systemic vasculitis
Schönlein-Genoch Purpura — systemic vasculitis, characterized by the development of aseptic inflammation of small vessels and capillaries, microthrombosis, hemorrhagic rash (purpura).
Schonlein-Henoch purpura occurs predominantly in children, but can also occur in adults. The disease is known the same as "capillary toxicosis", characterized by damage to the skin, joints, abdominal cavity and kidneys.
BLOCKCODE The pathogenesis of Henoch-Schonlein purpura is based on increased secretion of immunoglobulin A and subsequent deposition of IgA-containing deposits in the walls of small vessels.
In 90% of cases, the development of the clinical picture of systemic vasculitis is preceded by an infection of the upper respiratory tract.
Schonlein-Henoch purpura is accompanied by symptoms such as arthralgias, abdominal pain, melena and hematuria.
Read also: Allergic vasculitis can take many forms
The disease is characterized by the development of the so-called palpable purpura, which is symmetrically located on the limbs, buttocks and trunk.
Vasculitis usually resolves spontaneously within a week, but relapses may occur. If the disease is accompanied by the development of nephritis or a fulminant course, the prognosis for the patient deteriorates rapidly.
Chronic glomerulonephritis is rare.
Schonlein-Henoch purpura is treated with heparin and glucocorticoids.
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Cryoglobulinemic vasculitis – a formidable complication of infectious diseases
Cryoglobulinemic vasculitis mostly develops against the background of previous infectious diseases, especially hepatitis C. Cases of cryoglobulinemic vasculitis in patients with systemic connective tissue diseases, lymphoproliferative conditions, hepatitis B are described.
cryoglobulinemic vasculitis is characterized by the deposition of cryoglobulinemic complexes (immunoglobulins or immune complexes that precipitate at low temperatures) in the walls of small vessels. Subsequently, this leads to the activation of the complement system, damage to the vascular endothelium and the development of corresponding ischemic lesions.
Typical clinical symptoms of cryoglobulinemic vasculitis: purpura, Raynaud's phenomenon, necrotic skin lesions, ulceration of the lower extremities.
Other clinical manifestations of cryoglobulinemic vasculitis include arthralgia, proteinuria, hematuria, renal failure, hypertension, hepatomegaly, mono- or polyneuropathy, abdominal pain.
Laboratory blood test reveals an increase in the CFU, a high titer of rheumatoid factor and a low complement concentration (especially C4).
Treatment for cryoglobulinemic vasculitis treatment consists of aminoquinoline drugs (plaquenil), anticoagulants, antiplatelet agents and glucocorticoids.
Vasculitis often affects young girls
Systemic vasculitis: clinical picture of Churg-Strauss syndrome
Churg-Strauss Syndrome – a rare disease that occurs in patients with asthma and a aggravated allergic history.
This pathology is also known as "eosinophilic granulomatosis with polyangiitis".
Churg-Strauss syndrome is characterized by damage to small and medium-sized vessels, involvement of the respiratory tract, heart, kidneys and joints, accompanied by fever and weight loss.
Churg-Strauss syndrome is characterized by the formation of necrotizing eosinophilic extravascular granulomas located near small arteries and veins.
In the blood plasma, an increase in the level of eosinophils, CRP (C-reactive protein) and ANCA (antinuclear antibodies) is determined.
Lung x-ray shows patchy shadows and opacities in the lungs caused by granulomatous inflammation of small vessels.
Churg-Strauss syndrome is treated with high doses of systemic glucocorticoids. Damage to the heart (myocarditis), kidneys (glomerulonephritis) and the respiratory system is the cause of frequent deaths.
Thank you for staying with estet-portal.com. Read other interesting articles in the "Dermatology" section. You may also be interested in How to properly treat skin vasculitis depending on the form and stage
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