Post on 11-Oct-2015
Embed Size (px)
Supervised by Dr. Ragaa abd el-kaderProfessor of Rheumatology Alexandria University
CALSSIFICATION OF VASCILITIS AND ITS PATHOGENESIS This part is made by: Asmaa Ahmed Mahmoud Salem .(187)
Vasculitis is inflammation of blood vessels, often with ischemia, necrosis, and occlusive changes
classification according to cause:
classification according to vessle size :
calssification according to Pathogenesis:
calssification according to Immuno pathology
Deposition of circulating antigen-antibody complexwithin the vessel wall. This leads to complement activation andchemotactic attraction of neutrophils by complementcomponents. Subsequent phagocytosis of such complexes with Liberation of neutrophil granular products leads to vasculardamage.
2.Cell-mediated hypersensitivity: Antigenic exposure may attract lymphocytes which liberate cytokines causing tissue damage and further activation of macrophages and lymphocytes.
3. Failure to clear the antigen may lead to persistent inflammation and eventual formation of epithelioid cells and giant cells, giving rise to a granulomatous tissue reaction.
The possible immunopathologicmechanism in vasculitis are:
are Antibodies directed against certain proteins in the cytoplasmic granules of neutrophils and monocytes
ANCA Associated DiseasesWegeners granulomatosisMicroscopic polyangiitis-70% positiveChurg-Strauss syndrome-about 50%Renal limited (pauci-immune) vasculitisDrug-induced ANCA-associated vasculitis
Giant-cell arteritis (GCA or temporal arteritis)
is an inflammatory disease of blood vessels most commonly involving large and medium arteries of the head.The name (giant cell arteritis) reflects the type of inflammatory cell involved as seen on a biopsy.It is also known as "Cranial arteritis" and "Horton's disease."
Temporal Arteritis Risk Factors
Many serious complications may arise from Temporal Arteritis :strokes can be just one of these. Other dangerous health issues include : blindness, paralysis and aortic aneurysm. In worst cases, it may even lead to death.It is because of the potentially serious complications that Temporal Arteritis needs to be treated as soon as it is diagnosed. Early treatment prevents any serious physical damage and helps the patient make a faster recovery. If you have persistent headaches or any other symptoms characteristic of Temporal Arteritis, it is necessary that you begin the treatment immediately
polymyalgia rheumatica (PMR): which is characterized by sudden onset of pain and stiffness in muscles (pelvis, shoulder) of the body and is seen in the elderly. Other diseases related with temporal arteritis are : systemic lupus erythematosus, rheumatoid arthritis severe infections.
It is more common in females than males by a ratio of 3:1. The mean age of onset is about 70 years, and it is rare in those less than 50 years of age.Patients present with:
New type of severe headache fevertenderness and sensitivity on the scalpjaw claudication (pain in jaw when chewing)tongue claudication (pain in tongue when chewing) and necrosisreduced visual acuity (blurred vision)acute visual loss (sudden blindness)diplopia (double vision)acute tinnitus (ringing in the ears) Shoulder PainCough:People with Temporal Arteritis can also suffer from a bad case of dry cough.The inflammation may affect blood supply to the eye and blurred vision or sudden blindness may occur. In 76% of cases involving the eye, the ophthalmic artery is involved causing anterior ischemic optic neuropathy Loss of vision in both eyes may occur very abruptly and this disease is therefore a medical emergency.
Giant cell arteritis can cause the following complications:
Blindness: This is the most serious complication of GCA. The swelling that occurs with giant cell arteritis narrows your blood vessels, reducing the amount of blood and therefore oxygen and vital nutrients that reaches your body's tissues. Diminished blood flow to your eyes can cause sudden, painless vision loss in one or, in rare cases, both eyes. Unfortunately, blindness is usually permanent.
Aortic aneurysm. Having giant cell arteritis increases your risk of aneurysm. An aortic aneurysm is a serious condition because it may burst, causing life-threatening internal bleeding. Because it may occur even years after the initial diagnosis of GCA.
Stroke. In some cases, a blood clot may form in an affected artery, obstructing blood flow completely, depriving part of your brain of necessary oxygen and nutrients, and causing stroke. This serious condition is an uncommon complication of GCA.
DiagnosisPhysical examLaboratory testsBiopsyImaging studies
Palpation of the head reveals prominent temporal arteries with or without pulsation.The temporal area may be tender.Decreased pulses may be found throughout the body.Evidence of ischemia may be noted on fundal exam.
LFTs, liver function tests, are abnormal particularly raised ALP- alkaline phosphataseErythrocyte sedimentation rate, an inflammatory marker, >60mm/hour (normal 1040mm/hour), but may be normal in approximately 20% of cases.C-reactive protein, another inflammatory marker, is also commonly elevated.Platelets may also be elevated.
The gold standard for diagnosing temporal arteritis . It involves removing a small part of the vessel and examining it microscopically for giant cells infiltrating the tissue. Since the blood vessels are involved in a patchy pattern, there may be unaffected areas on the vessel and the biopsy might have been taken from these parts. Unilateral biopsy of a 1.53cm length is 85-90% sensitive. So, a negative result does not definitely rule out the diagnosis.
U.S: Radiological examination of the temporal artery with ultrasound yields a halo sign. Contrast enhanced brain MRI and CT :is generally negative in this disorder. Recent studies have shown that 3T MRI: using super high resolution imaging and contrast injection can non-invasively diagnose this disorder with high specificity and sensitivity
Corticosteroids: typically high-dose prednisone (4060mg bd), must be started as soon as the diagnosis is suspected (even before the diagnosis is confirmed by biopsy) to prevent irreversible blindness secondary to ophthalmic artery occlusion. Steroids do not prevent the diagnosis from later being confirmed by biopsy. The dose of prednisone is lowered after 24 weeks, and slowly tapered over 912 months. Oral steroids are at least as effective as intravenous steroids,except in the treatment of acute visual loss where intravenous steroids appear to offer significant benefit over oral steroids
Temporal Arteritis Natural Treatment:
Physicians also advise patients to use some natural remedies along with medicines for a faster recovery. Vitamin D and Calcium supplements are very useful in curing the condition. They are also effective in counteracting the long-term effects of Corticosteroid medicines.
Lifestyle and home remedies:Eat a healthy diet. Eating well can help prevent potential problems, such as ;thinning bones, high blood pressure and diabetes.Emphasize fresh fruits and vegetables, whole grains, and lean meats and fish.limiting salt, sugar and alcohol. Be sure to get adequate amounts of calcium and vitamin D. Experts recommend between 1,000 and 1,500 milligrams of calcium and 800 international units (IU) of vitamin D a day.
Lifestyle and home remedies:(cont.)Exercise regularly. Regular aerobic exercise, such as walking, can help prevent bone loss, high blood pressure and diabetes. It also benefits your heart and lungs. many people find that exercise improves their mood and overall sense of well-being.
Temporal Arteritis Prognosis:
For patients with Temporal Arteritis recovery is usually complete. People generally recover fully, though treatment needs to be carried out for 1-2 years or a longer period of time. This prevents any chance of Temporal Arteritis recurrence. When properly treated, Giant Cell Arteritis rarely makes a comeback.
The first case of Takayasus arteritis was described in 1908 by Dr. Mikito Takayasu at the Annual Meeting of the Japan Ophthalmology Society.
definitionTakayasu arteritis is a chronic inflammation of the large blood vessel (aorta).
Epidemiology Incidence worldwide: rare disease 2-3 cases per year per million head of population.
affects women more frequently than men with a 9:1 female:male ratio.
affects young adults up to the age of 40, but is most common in the age range 15-20.
reported all over the world, but is most common in Asia, particularly Japan.
Types of Involvement in Takayasus Arteritis
Classical TakayasusPulmonary ArteriesDescendingAorta
Takayasus arteritis is not known.Some evidence suggests that an infection of some viral, bacterial, or other occurring in a person with other predisposing factors
Inflammation Fever,fatigue, weight loss.
Arthralgia and non-specific pains.
claudication of jaw or extremities. back pain (