This type of vasculitis is also called non-infectious necrotizing granulomatosis necrotizing granulomatosis or upper respiratory tract. For the first time the disease was described in 1931 as a special type of periarteritis nodosa, in 1936 after a more detailed description of F. Wegener it was named.
Why and how necrotizing vasculitis begins
Wegener's granulomatosis - a systemic vasculitis
Vasculitis - when blood vessels refuse to work
with the development of necrotizing granulomas in the tissues. This form of vasculitis affects small vessels of the upper airways, lungs and kidneys.
The main cause of the disease - an autoimmune process that is allergic to the patient's own tissue, which apparently started after a viral or bacterial infection. In this case, the body produces antibodies against one type of leukocyte - neutrophils, due to which is now called Wegener granulomatosis disease associated with anti-neutrophil cytoplasmic antibody (ANCA). In the walls of arteries developing inflammation with formation of granulomas.
A granuloma is a cluster of cells capable of phagocytosis (dissolution inside pathogens). Externally granuloma nodules appear as different sizes, inside which there is necrosis, after which they are replaced with connective tissue (creating sclerosis), with a gradual decline of organ function.
The disease usually has a subacute or chronic course. In its course, there are three periods:
- initial manifestations of the changes of the upper respiratory tract, the middle ear or eye;
- period generalization visceral (mainly lung and kidney);
- terminal period - with impaired kidney and lungs.
Disease begins gradually with repeated rhinitis, sinusitis or sinusitis. Sometimes the first symptoms of lung disease and if the patient has a strong cough, fever, and sometimes - the blood in the sputum. Purulent inflammation of the middle ear can also be the beginning of vasculitis. Less commonly, the disease begins with damage to eyes, skin and joints. Usually, at the very beginning of the disease the patient has malaise, fever, lack of appetite and weight loss.
The main symptoms of the disease
For necrotizing vasculitis characterized by the classic triad - the defeat of the upper respiratory tract, lung and kidney, along with general signs of disease and less frequent symptoms.
Common signs include fever, which appears both in local and in generalized lesions. More than half of patients have joint damage in the form of joint pain, which usually appear at the peak of disease recurrence. Pains arise in large and small joints. Sometimes it can develop inflammation (arthritis) in large joints. Arthritis often develop in the knee and ankle joints (from two sides).
Changes in the skin and muscles are also developing quite often. A rash on the skin can be varied: hemorrhagic (bleeding a), papular (small hill above the skin), bullous (blisters). The most typical of hemorrhagic rashes, ulcers and necrotic nodules. The degree of skin lesions may be different - from single components to the destruction of vast areas. Elements of the rash often located in large joints, on the buttocks and thighs.
The defeat of the respiratory system
Symptoms of upper respiratory tract are observed in all patients. The disease begins with rhinitis
Rhinitis: a runny nose - is quite serious
Sinusitis - when a puncture is required
. There are pus mixed with blood from the nose, stuffy nose, dry crusts, epistaxis, olfactory disorders, headaches, swelling in the back of the nose, or one half of the face.
Long-term course of the disease leads to the characteristic lesions of the mucous membranes of the pharynx, larynx, trachea, accompanied by a sore throat, hoarseness, and respiratory disorders. Perhaps the destruction of cartilage and bone of the nasal septum, maxillary sinus and the organ of vision.
Sometimes the symptoms of angina
Symptoms of angina - obvious and recognizable
. Purulent processes develop when joining a bacterial infection.
If it affects the lungs appears hacking cough, chest pain, coughing up blood, shortness of breath. The lungs are formed rounded infiltrates, necrosis and decay cavities which are formed, which are then creating sclerosis with formation of pulmonary disease.
The defeat of hearing and vision
Otitis media can be the first sign of the disease, sometimes complicated by paresis of the facial nerve. Perhaps granulomatous destruction of the temporal bone with the development of hearing loss.
The eyes are affected in about half of patients. Granulomatosis orbit of the eye can manifest as swelling in the form of a protrusion of the eyeball (exophthalmos), limiting its mobility, swelling of the eye tissues, including swelling of the optic nerve, which can lead to blindness.
Signs of kidney damage usually appear within a few months after the onset of the disease. In the urine there is a large amount of protein and blood, kidney function has been steadily declining. About a year later the patient may appear high blood pressure. Chronic renal failure is formed in two or three years.
Necrotizing vasculitis - is a serious chronic disease. However, current treatments allow the process to stop when the disease was detected in a timely manner.