Systemic vasculitis refers to serious pathologies that cause disruption to all internal organs and can often lead to death.
The danger of the situation is that many diseases of this group have similar symptoms, which makes it difficult to diagnose them.
These include nodular periarteritis, which is still called Kussmaul-Mayer disease.
Causes and risk factors
Nodular periarteritis is a disease characterized by by the lesion of small and medium arteries , leading to a disruption of blood supply to internal organs and the development of all kinds of pathologies. For the first time it was described in 1866 by two doctors - Kussmaul and Mayer.
The etiology of nodular periarteritis has not been fully clarified, but the results of the study showed that plays a key role in its development by the increased sensitivity of the organism.
Various factors( infectious and viral diseases, intoxications, intake of certain medicines, hypothermia, etc.) lead to the emergence of an allergy with a characteristic reaction of the immune system to allergens.
One of the most likely risk factors for this disease, experts believe exposure to the hepatitis B virus .In addition, there are theories about the impact on the development of nodular periarteritis cytolomegavirus, rubella, the Epstein-Barr virus, hepatitis C .The assumptions about hereditary predisposition to pathology are also not excluded.
Classification of
In the clinical course of nodular periarteritis, several variants are distinguished:
- classical( with renal-polyneuric or renal-visceral symptoms);
- asthmatic;
- monoorganic;
- is a skin-thrombangiotic.
The most mild form of nodular periarteritis is skin , which is not characterized by viroropathies( extensive lesions of internal organs).Its main sign is subcutaneous or cutaneous nodules, which are located on the limbs along the course of the vascular bundle. Patients in one way or another retain working capacity and social skills;In addition, they often have persistent remissions.
This photo shows the manifestations of the cutaneous nodular periarteritis:
Mono-organ pericarteritis nodular differs by changes in the vessels , which is established by biopsy or examination of the removed organ.
The disease course can be benign, slow progressing, recurring, rapidly progressing and acute .In particular, slow progression is characteristic of the thrombangiotic variant, but the disease in this case can be characterized by arterial hypertension, microcirculatory disorders and peripheral neuritis.
Danger and complications of
Sometimes, with acute course and accompanying negative factors, the disease develops with lightning speed, causing the patient's death in a few months. In other cases, in the absence of adequate treatment, the nodular periarteritis can cause the following complications:
- infarctions and sclerosis of different organs;
- perforation of ulcers;
- rupture of aneurysms;
- of uremia;
- strokes;
- of the gangrene of the intestine;
- encephalomyelitis.
Similar complications often lead to complete or partial disability of patients.
Symptoms of
The symptomatology of nodular periarteritis resembles the symptoms of other diseases - in particular, inflammation of the arteries and the Sharg-Strauss syndrome .
In addition, they are very diverse and can affect any organ, which greatly complicates the diagnosis of .The most common signs of the pathology are:
- The long-lasting fever , which is immune to antibiotics.
- Weight loss, lack of appetite , weakness and decreased efficiency.
- Changes in the skin of include pallor( "marbling") of the limbs, as well as skin eruptions of hemorrhagic, erythematous, urticaric type. Approximately 20% of patients palpable painful subcutaneous nodules, which are detected along the course of vascular nerve trunks of the extremities.
- Strong muscular pains ( especially calf muscles are susceptible to pain), atrophy and muscle weakness, tenderness in palpation. Less common are polyarthritis, mainly affecting large joints.
- Cardiovascular disorders of , which can lead to the development of angina pectoris, myocardial infarction, cardiosclerosis, rhythm and blockade disorders. The most common symptom is hypertension, and in 10% of cases, mitral valve insufficiency is noted.
- Kidney damage is noted in an overwhelming number of patients, with 70-97% diagnosed with vascular nephropathy, less often proteinuria, cylindruria, microhematuria. Renal failure can develop rapidly enough and eventually lead to a kidney infarction due to thrombosis of the artery.
- Lesions of the lungs with the development of nodular periarteritis cause chest pain, shortness of breath, cough, hemoptysis.
- Disorders of the digestive tract are characterized by pain in different parts of the abdomen, nausea, vomiting, frequent loose stools with mucus and blood.
- The lesions of the nervous system are expressed by polyneuritis, the symptoms of which are pains in the limbs, numbness, impaired sensation, paresis. In a number of patients, the disease affects the peripheral arteries of the limbs, which can lead to ischemia of the fingers and even gangrene.
- Eye lesions of include malignant retinopathy, thickening of the vessels of the fundus and their aneurysmal enlargement.
- Disorders of the endocrine system - in 80% of male patients epididymitis and orchitis are noted;in addition, there are cases of violations of the adrenal and thyroid gland functioning.
When should I see a doctor?
The first symptoms that should alert a patient are fever of unclear etiology and strong weakness of , especially if it is accompanied by skin lesions.
Diagnosis
The diagnosis of "nodular periarteritis" is based on patient complaints, laboratory tests and studies in cases where another option is completely excluded .The diagnostic methods that are used in this case include:
- Urinalysis .With nodular periarteritis in patients noted proteinuria, microhematuria, and cylindruria.
- General blood analysis .In the blood of patients are found hyperthrombocytosis, leukocytosis, anemia.
- Biochemical blood test .In case of disease in the blood of patients the following changes occur: increase of sialic acids, fractions of y- and a-globulins, fibrin, SRP, seromucoid.
- Doppler ultrasound of kidney vessels .The study should determine the stenosis of vessels, which is also characteristic of nodular periarteritis.
- Radiography of lungs .In the course of the procedure, there is an increase and deformation of the pulmonary pattern.
- Angiography of the .Method of radiographic examination of vessels, designed to identify affected segments.
- Biopsy of .The procedure involves examining a sample of the blood vessel tissue under a microscope for analysis of the diagnosis. Sometimes patients undergo liver or kidney biopsy.
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Treatment
Therapy of nodular periarteritis should be long( at least 2-3 years) and include primarily hormonal drugs. The mild forms of the disease are corrected by high doses of corticosteroids( the most effective is " Prednisolone ").In a break between taking these medications medicines are prescribed pyrazolone series or acetylsalicylic acid.
For complications in the form of arterial hypertension and nephrotic syndrome, cytostatics-immunosuppressors are used. If the nodular periarteritis is accompanied by the syndrome of ICE and hyperthrombocytosis, specialists prescribe trental, heparin, and quarantil, and with muscular atrophy and neuritis, the treatment tactics include hydrotherapy, massage and physical therapy.
Prophylaxis and prognosis
Prognosis of nodular periarteritis depends on the form, stage and course of the disease. The course of complex forms accompanied by vascular lesions is prognostically unfavorable: remission occurs only in 50% of patients.
Preventative measures include strengthening the body and preventing any factors that can trigger the development of nodular periarteritis( uncontrolled intake of medications, infectious and viral diseases, prolonged exposure to ultraviolet, etc.).
An important condition is an increase in the immunity of : a healthy way of life, proper nutrition, regimen and moderate physical activity.
Nodular periarteritis - is quite a serious disease, the outcome of which largely depends on the timely diagnosis of .Any characteristic symptoms, especially if they occur in men aged 30 to 60 years, require immediate consultation of a specialist and a complete examination of the body.