Characterized by redness in the ear and nasal cartilage antihelix which leads to a saddle-nose. And the differential diagnosis must contain gout and rheumatic fever and other immune diseases. He
notes the frequent inflammation of the ear flap, and may affect the
ears or only one, may cause distortion in the ear hearing test is
usually sensorineural and may be progressive, or crispy surprise. Sometimes it may be transferable and be caused by narrowing of external auditory canal. Injury
may be associated Khdot vestibular vertigo, may be responding to
Lachtbar calorie deficient or non-existent, and all patients who show
signs of infection appear to the inner ear infection in the cartilage of
the ear.
Pathogenesis
Pathogenesis is immune where the immune complexes found in patients and antibodies to collagen was noted that the injury is associated with HLA DR4 antigen, as in other immune diseases.
And histological examination does not have distinctive properties, especially pathognomonic of the disease, but notes changes in the cartilage Kvkd basal cells and cells of cartilage basophilia Mahouselh vacuolated chondrocytes and necrosis and inflammation of the vessels and sometimes inflammation of the periosteum of cartilage.
In brief it is an immune disease Besab connective tissue leads to the injury of cartilage, joints, and renal injuries sample
Age - the incidence - of race
The average age of injury 44 years old, but the injury can be seen from the age of 13-84 years
Infected females and males equally
Affects all races and breeds, but more commonly when the eggs
Important clinical signsThe main symptoms:
Bilateral ear injury
Injury audio
Arthralgia
Inflammation of the larynx and trachea
Inflammation of the ribs and Gdharifaa Costochondritis
Cardiac and vascular injury
Emboli and venous and arterial Arterial and venous thromboses
Renal injury
Diagnostic criteria DIAGNOSTIC CRITERIAAnd called Mc Adam's Signs
Relapsing inflammation of the cartilage of the Sioanin (for Lazntin)
Inflammation of the joints of the nostril is Non-erosive, inflammatory polyarthritis
Inflammation of the nasal cartilages
Ocular inflammation inflammatory in-kind
Inflammation of the cartilage respiratory tract Respiratory tract chondritis
Dysfunction audio vestibular Cochlear and / or vestibular dysfunction
The disease is diagnosed the presence of one of the following facts:
The presence of clinical trials Alamattin or more.
Histological proof of infection with a single sign.
Two goals separate cartilage inflammation responded well to steroids and immunosuppression.
Rates of events associated with infection polychondritis
Chondritis of auricles 90%
Arthropathy 80%
Chondritis of Respiratory Tract 55%
% Nasal Chondritis (Saddle Nose Deformitiy) 50
Ocular Inflammation 50%
Cochlear / VestibularDamage 50%
% Anemia 10
Clinical characteristics of the course of the disease
The beginning of the disease is characterized by a sharp and sudden, and that 91% of patients get their ears and the pain can be accompanied by pain in the throat and hoarseness of the voice and in some cases gets respiratory blockage is interpreter. It can be seen as articulated polyarthriti injury or articular pain and polyarthralgia that can be transient or may lead to changes articulated. In some patients initially observed recurrent inflammation of the solid and repeatedly scleritis or episcleritis
The vestibular and auditory changes. Vestibular often be the result of a malfunction with the outcome of a blood vasculiti inflammation and cartilage hurt, but less common than other symptoms often lead to confusion in diagnosis Kachtbah syndrome Cogan Cogan's syndrome, which Mitravq with some show of this disease.
Treatment
In simple cases is given non-steroid anti-inflammatory
Steroids public road
Inhibitors such as immunity Mitatroxat or imuran and cyclosporine
Dapsone and dapsone and pencillamine penicillamine in less severe cases
In severe cases, some prefer to use relapsing Aeltsikulovosvamad cyclophosphamide. The first line when there are demonstrations of the disease did not respond to steroids and Almitatroxat Alazzatiuperen and azathioprine or methotrexate.
A biopsy paratracheal Tracheostomy in respiratory tract obstruction in some cases
Demonstrations in kind Ocular Manifestations
Episcleritis 39%
Scleritis 14%
Iritis 9%
Retinopathy 9%
Muscle Paresis 5%
Optic Neuritis 4%
4% Peripheral Corneal Thinning
WarningThe survival rate with observation for ten years is 75% and some studies showed a 90% either so increase complications conjunction anemia and complications as mentioned that these people Mahpin to Khbathat spinal myelodysplastic malignancies.
Predisposing factors for the warning in a bad Relapsing polychondritis:
At all ages: the presence of anemia
At older ages or equal to 50 years:
The existence of saddle-nose Saddle nose
Tracheal stenosis and throat Tracheolaryngeal strictures
Arthritis Arthritis
Inflammation of the vessels
Microhematuria.
Cause of death in patients with Relapsing polychondritis:
Respiratory complications such as narrowing of the respiratory tract and pneumonia
And cardiovascular complications Cardiovascular complications such as aneurysm rupture or blood Awasabh Dsamat heart or inflammation of the vessels.
Infectious complications
Accompanied by infection with malignancy
Pathogenesis
Pathogenesis is immune where the immune complexes found in patients and antibodies to collagen was noted that the injury is associated with HLA DR4 antigen, as in other immune diseases.
And histological examination does not have distinctive properties, especially pathognomonic of the disease, but notes changes in the cartilage Kvkd basal cells and cells of cartilage basophilia Mahouselh vacuolated chondrocytes and necrosis and inflammation of the vessels and sometimes inflammation of the periosteum of cartilage.
In brief it is an immune disease Besab connective tissue leads to the injury of cartilage, joints, and renal injuries sample
Age - the incidence - of race
The average age of injury 44 years old, but the injury can be seen from the age of 13-84 years
Infected females and males equally
Affects all races and breeds, but more commonly when the eggs
Important clinical signsThe main symptoms:
Bilateral ear injury
Injury audio
Arthralgia
Inflammation of the larynx and trachea
Inflammation of the ribs and Gdharifaa Costochondritis
Cardiac and vascular injury
Emboli and venous and arterial Arterial and venous thromboses
Renal injury
Diagnostic criteria DIAGNOSTIC CRITERIAAnd called Mc Adam's Signs
Relapsing inflammation of the cartilage of the Sioanin (for Lazntin)
Inflammation of the joints of the nostril is Non-erosive, inflammatory polyarthritis
Inflammation of the nasal cartilages
Ocular inflammation inflammatory in-kind
Inflammation of the cartilage respiratory tract Respiratory tract chondritis
Dysfunction audio vestibular Cochlear and / or vestibular dysfunction
The disease is diagnosed the presence of one of the following facts:
The presence of clinical trials Alamattin or more.
Histological proof of infection with a single sign.
Two goals separate cartilage inflammation responded well to steroids and immunosuppression.
Rates of events associated with infection polychondritis
Chondritis of auricles 90%
Arthropathy 80%
Chondritis of Respiratory Tract 55%
% Nasal Chondritis (Saddle Nose Deformitiy) 50
Ocular Inflammation 50%
Cochlear / VestibularDamage 50%
% Anemia 10
Clinical characteristics of the course of the disease
The beginning of the disease is characterized by a sharp and sudden, and that 91% of patients get their ears and the pain can be accompanied by pain in the throat and hoarseness of the voice and in some cases gets respiratory blockage is interpreter. It can be seen as articulated polyarthriti injury or articular pain and polyarthralgia that can be transient or may lead to changes articulated. In some patients initially observed recurrent inflammation of the solid and repeatedly scleritis or episcleritis
The vestibular and auditory changes. Vestibular often be the result of a malfunction with the outcome of a blood vasculiti inflammation and cartilage hurt, but less common than other symptoms often lead to confusion in diagnosis Kachtbah syndrome Cogan Cogan's syndrome, which Mitravq with some show of this disease.
Treatment
In simple cases is given non-steroid anti-inflammatory
Steroids public road
Inhibitors such as immunity Mitatroxat or imuran and cyclosporine
Dapsone and dapsone and pencillamine penicillamine in less severe cases
In severe cases, some prefer to use relapsing Aeltsikulovosvamad cyclophosphamide. The first line when there are demonstrations of the disease did not respond to steroids and Almitatroxat Alazzatiuperen and azathioprine or methotrexate.
A biopsy paratracheal Tracheostomy in respiratory tract obstruction in some cases
Demonstrations in kind Ocular Manifestations
Episcleritis 39%
Scleritis 14%
Iritis 9%
Retinopathy 9%
Muscle Paresis 5%
Optic Neuritis 4%
4% Peripheral Corneal Thinning
WarningThe survival rate with observation for ten years is 75% and some studies showed a 90% either so increase complications conjunction anemia and complications as mentioned that these people Mahpin to Khbathat spinal myelodysplastic malignancies.
Predisposing factors for the warning in a bad Relapsing polychondritis:
At all ages: the presence of anemia
At older ages or equal to 50 years:
The existence of saddle-nose Saddle nose
Tracheal stenosis and throat Tracheolaryngeal strictures
Arthritis Arthritis
Inflammation of the vessels
Microhematuria.
Cause of death in patients with Relapsing polychondritis:
Respiratory complications such as narrowing of the respiratory tract and pneumonia
And cardiovascular complications Cardiovascular complications such as aneurysm rupture or blood Awasabh Dsamat heart or inflammation of the vessels.
Infectious complications
Accompanied by infection with malignancy
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