Download - vasculitis
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Supervised by Dr. Ragaa abd el-kader
Professor of Rheumatology Alexandria University
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CALSSIFICATION OF VASCILITIS AND ITS PATHOGENESIS
This part is made by: Asmaa Ahmed Mahmoud Salem …….(187)
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Vasculitis is inflammation of blood vessels, often with ischemia, necrosis, and occlusive changes
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classification according to cause:
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classification according to vessle size :
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calssification according to Pathogenesis:
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calssification according to Immuno pathology
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1.Deposition of circulating antigen-antibody complex within the vessel wall. This leads to complement activation and
chemotactic 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 immunopathologic mechanism in vasculitis are:
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are Antibodies directed against certain proteins in the cytoplasmic granules of neutrophils and monocytes
ANCA Associated Diseases•Wegener’s granulomatosis•Microscopic polyangiitis-70% positive•Churg-Strauss syndrome-about 50%•Renal limited (pauci-immune) vasculitis•Drug-induced ANCA-associated vasculitis
ANCA
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Types
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Temporal Arteritis
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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."
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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
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Associated conditions• 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.
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Symptoms: 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:
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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.
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Complications: 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.
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Complications:(cont.)• 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.
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• 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.
Complications:(cont.)
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Diagnosis
Physical exam
Laboratory tests
Biopsy
Imaging studies
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1-Physical exam:• 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.
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2-Laboratory tests:• LFTs, liver function tests, are abnormal particularly
raised ALP- alkaline phosphatase• Erythrocyte sedimentation rate, an inflammatory
marker, >60 mm/hour (normal 10–40 mm/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.
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3-Biopsy: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 1.5–3 cm length is 85-90% sensitive. So, a negative result does not definitely rule out the diagnosis.
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4-Imaging studies:• 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
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Trea
tmen
t
:
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Treatment:Corticosteroids: typically high-dose prednisone (40–60 mg 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 2–4 weeks, and slowly tapered over 9–12 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
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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.
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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.
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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.
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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.
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Large vessel Small vesselMedium vessel
Takayasu`s
arteritisGiant cell arteritis
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First DescriptionThe first case of Takayasu’s arteritis was described in 1908 by Dr. Mikito Takayasu at the Annual Meeting of the Japan Ophthalmology Society.
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definitionTakayasu arteritis is a chronic inflammation of the large blood vessel (aorta).
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Histopathology
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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.
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Types of Involvement in Takayasu’s Arteritis
Classical Takayasu’s
Pulmonary Arteries
DescendingAorta
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Takayasu’s arteritis is not known.Some evidence suggests that an infection of some viral, bacterial, or other occurring in a person with other predisposing factors
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Clinical picture
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Symptoms
Systemic stage40-50% Inflammation
Fever,fatigue, weight loss.
Arthralgia and non-specific pains.
Tndeness.
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SymptomsOcclusive stage50-60%
Ischaemic phenomena
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Occlusive stage
Vascular
claudication of jaw or extremities. back pain (due to involvement of the aorta).syncope (rare).hypertension (the commonest presentation in children).
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Occlusive stage
Neurological
DizzinessHeadachesTIAsvisual disturbanceSeizuresstroke
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Occlusive stage
Cardiac : angina dyspnoea (from congestive cardiac failure - the primary cause of death)
Pulmonary :Haemoptysispleuritis
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Occlusive stageGastrointestinal abdominal pain
renal haematuria
Dermatological rashes including :erythema multiforme induratum
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Signs systolic BP difference >10 mmHg
between arms . Peripheral pulses may not be
palpable. Arterial bruits over any large
artery and bruit of aortic regurgitation.
Hypertension in 50% due to renal artery involvement.
Ophthalmoscopic changes. Anaemia . Muscle wasting. Skin vasculitis .
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Diagnostic criteria From the American College of Rheumatology
3 of 6 of the following should be present:
1.Age at onset ≤40 years. 2.Claudication of the extremities. 3.Reduced pulsation of one or both brachial arteries 4.>10 mmHg BP difference between arms 5.Bruit over one or both subclavians or the abdominal aorta. 6. Arteriographic narrowing/occlusion of the entire aorta, its primary branches or large arteries in upper/lower limbs .
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Investigations
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(A) MRA patient with active TA at diagnosis. There is complete occlusion of the left SCA at its origin (arrow) with numerous collaterals evident and an ostial stenosis of the left common
carotid artery. (B) MRA image from the same patient in remission. No significant progression of the lesions found on the baseline
MRA is seen.
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3D MRA
MRI
Angio
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ManagementDrugs
prednisolone, usually at a starting dose 1 mg/kg/day.
Steroid sparing agents : Cyclophosphamide.
Hypertension should be aggressively managed .
Anticoagulant used to prevent stroke .
Anti-tumour necrosis factor (anti -TNF) has recently been used with encouraging results .
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SurgerySurgical procedures are sometimes required to increase the flow of blood through an artery and procedures undertaken include:
Angioplasty with stenting Vascular bypass procedures Aortic valve replacement Suction thrombectomy of the
subclavian artery
Management
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Complications Complications occur as a result of narrowing or occlusion of the arteries and may include:
Loss of vision Hypertension Stroke Aortic regurgitation Myocardial infarction
Pregnancy Healthy baby
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Prognosis20% have self-limiting monophasic disease. A picture however is emerging of long-term disability and reliance on steroids to reduce the remission rate. The mortality rate is 2-35% over 5 years
Differential diagnosis Acute lymphoblastic leukemia Behcet syndromePolyartheritis nodosaRheumatic fever Giant cell arteritis
Buerger disease Systemic lupus
erythematosis juvenile rheumatoid
arthritis Migraine Malignancy
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Takayasu’s arteritisa
rare disease :inflammation in the walls of the largest arteries in the body: the aorta and its major branches.
“pulseless disease.”
more common among young women
diagnosed by angiography & MRA
The problems caused by narrowed or blocked arteries
treated with glucocorticoids (prednisone and others)& other medications that suppress the immune system.
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•Polyarteritis nodosa is an autoimmune disease affecting the medium sized arteries
•Most cases of PAN occur in the 4th or 5th decade, although it can occur at any age .
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• symptoms result from ischaemic damage to affected organs, often the skin, heart, kidneys, and nervous system.
• Generalised symptoms include fever, fatigue, weakness, loss of appetite, and weight loss. Muscle and joint aches are common. The skin may show rashes, swelling, ulcers.
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• Peripheral neuropathies are very common (50 to 70%). This includes tingling, numbness and/or pain in the hands, arms, feet, and legs.
• Central nervous system (CNS) lesions may occur 2 to 3 years after the
onset of PAN and may lead to cognitive dysfunction, decreased alertness, seizures and neurologic deficits.
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Skin affection include: • purpura, •livedo reticularis,• ulcers, •nodules •or gangrene. •Skin involvement occurs most often on the legs and is very painful
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• Renal artery vasculitis may lead to impaired kidney function, and renovascular hypertension.
• Testicular infarction cause testicular pain
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• .affection of the mesenteric
artery can cause Abdominal pain,
gastrointestinal bleeding (occasionally is mistaken for inflammatory bowel disease)
Hemorrhage, bowel infarction, and perforation are rare, but
very serious
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1. Routine laboratory tests may provide important clues to PAN, but there is no single blood test that is diagnostic of this disease. Most patients with PAN have elevated ESRs.
2. If there is skin or muscle/nerve involvement, a skin or muscle/nerve biopsy can be extremely helpful in coming to a definite diagnosis of PAN.
3. Nerve conduction studies are a non-invasive way of identifying nerves that are involved by the inflammation.
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4-An angiogram of the abdominal and renal blood vessels may also be very helpful in diagnosing PAN. microaneurysms most often affect the arteries leading to the kidneys, liver or gastrointestinal tract.
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1. Weight loss of > 4 kg since beginning of illness
2. Livedo reticularis 3. Testicular pain or tenderness 4. Myalgias, weakness, or leg tenderness 5. Mononeuropathy or polyneuropathy 6. Development of hypertension 7. Elevated BUN or creatinine unrelated to
dehydration or obstruction 8. Presence of hepatitis B surface antigen or
antibody in serum 9. Arteriogram demonstrating aneurysms or
occlusions of the visceral arteries 10.Biopsy of small or medium-sized artery
containing granulocytes
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• patients are treated with high doses of corticosteroids. Other immunosuppressive drugs are also added for patients who are especially ill. In most cases of PAN now, if diagnosed early enough the disease can be controlled, and often cured.
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Wagner Granulomatosis
BYAsmaaHamed
191
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Definition: Wegener's granulomatosis (WG) is a chronic
granulomatous necrotizing vasculitis predominantly affecting the upper and lower respiratory tracts and the kidney.
Epidemiology: The onset of Wegener's granulomatosis can
occur at any age, but it most often occurs between the ages of 30 and 50.
Caucasians are most likely to develop Wegener's granulomatosis.
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Etiology:The cause of Wegener’s Granulomatosis is not known.
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Pathophysiology:
• Inflammation with granuloma formation.
• Anti-neutrophil cytoplasmic antibodies (ANCAs) are responsible for the inflammation in Wegener's. This type of ANCA is also known as cANCA, with the c indicating cytoplasmic.
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Clinical Picture:
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SINUS SIGNS & SYMPTOMS
• rhinorrhea.
• Discolored nasal discharge
• Bloody nasal discharge, nose bleeding
• Ulcerations of the mucous membranes (sores or crusting)
• perforation of the nasal septum may develop, and collapse of the nasal bridge (called saddle nose deformity).
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Respiratory SIGNS & SYMPTOMS
Pulmonary nodules
infiltrates
cavitary lesions
pulmonary hemorrhage bronchial stenosis
subglottic stenosis
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KIDNEY SIGNS & SYMPTOMS Rapidly progressive segmental necrotizing
glomerulonephritis leading to chronic renal failure.
MUSCULOSKELETAL SYSTEM SIGNS & SYMPTOMS
Pain, joint swelling affects two-thirds of
patients. It does not lead to permanent joint damage
or deformities
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SKIN SIGNS & SYMPTOMS
Nearly half of people with Wegener's granulomatosis develop skin lesions
Skin sores or rashes that appear as small red or purple patches, small blister-like lesions, ulcers or small nodules
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EYES SIGNS & SYMPTOMSConjunctivitis, scleritis,
episcleritis, Uveitis Weakened vision or double
vision. Double vision or a
decrease in vision .
Oral CavityStrawberry gingivitis. Bone destruction with
loosening of teeth. Non-specific ulcerations
throughout oral mucosa.
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Criteria:In 1990, the American College of Rheumatology
accepted classification criteria for Wegener's. Nasal or oral inflammation:
• painful or painless oral ulcers or • purulent or bloody nasal discharge
Lungs: abnormal chest X-ray with: • nodules, • infiltrates or • cavities
Kidneys: urinary sediment with: • microhematuria or • red cell casts
Biopsy: granulomatous inflammation • within the arterial wall or • in the perivascular area
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According to the Chapel Hill Consensus Conference (CHCC) (1992):
• a granulomatous inflammation involving the respiratory tract, and
• a vasculitis of small to medium-size vessels.
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Diagnosis:Laboratory investigation
CBC Anti-Neutrophil CytoplasmicAutoantibodies (ANCA)
RadiologyLung X-ray show cavities or masses,infiltrates, solid nodules. A sinus X-ray or computed tomography(CT) scan
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TISSUE BIOPSY
The only sure way to confirm a diagnosis of Wegener's granulomatosis
Sites nasal passages, airways,
or lungs Finding leukocytoclastic vasculitis
with necrotic changes and granulomatous inflammation (clumps of typically arranged white blood cells) on microscopy.
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TREATMENT A-DRUG THERAPY:
1-corticosteroids
2-Cyclophosphamide.
3-Methotrexate
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4-Bactrim
5-Bisphosphonates (Fosamax)
6-Folic acid or folinic acid
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B-SURGERY: Severe subglottic stenosis, tracheotomy is required. kidney transplant in cases of renal failure
Prognosis:Without treatment, people with this disease can die within a few months.
With treatment, most people who receive corticosteroids and cyclophosphamide get much better.
However, the disease may return in about half of all patients. In these cases, the disease usually comes back within 2 years of stopping treatment.
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• Presented by: asmaa shaltot
Microscopic polyangiitis
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Also known as "Microscopic polyarteritis," "Microscopic polyarteritis nodosa" It is an ill-defined autoimmune disease characterized by necrotizing, small-vessel vasculitis
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The process is begun with an autoimmune process of unknown etiology that triggers production of p-ANCA( is usually directed against myeloperoxidase (MPO).
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In the form of glomerulonephritis which causes hematuria, proteinuria and RBC casts are present.
Renal
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About 1/3 of patients have a purpuric rash .
Nail bed infarcts and splinter hemorrhages may occur.
Skin
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Mild symptoms of rhinitis, epistaxis, and sinusitis Alveolar hemorrhage pulmonary fibrosis chest x-ray bilateral patchy infiltrates
Respiratory
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Abdominal pain, nausea, vomiting, diarrhea, and bloody stools.
Vasculitis peripheral neuritis and mononeuritis multiplex.
Neurologic symptoms include numbness or tingling in the arm, hand, leg, or foot.
GIT
Neurologic
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Over time, muscle wasting that is secondary to the nerve damage may result.
later, cerebral vasculitis cerebral hemorrhage, infarction, seizures, or headache.
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If the eyes are affected, episcleritis, conjunctivitis and uveitis usually result.
The FIVE most common clinical manifestations of MPA are:
Kidney inflammation (~ 80% of patients). Weight loss (> 70%). Skin lesions (> 60%). Nerve damage (60%). Fevers (55%).
Ocular
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ANCA positive.
ESR and C-reactive protein levels are elevated.
laboratory tests
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CBC:◘WBC and platelet counts are elevated
◘ Anemia of chronic disease is common.
◘ An acute drop in Hct suggests alveolar hemorrhage or hemorrhage in the GI tract.
Serum creatinine should be measured
periodically to check for renal involvement.
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Urinalysis: (to check for hematuria, proteinuria, and cellular casts) should be done.
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Tissue biopsy may be needed to make the diagnosis of MPA.
Biobsy
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A steroid (usually prednisone) in combination with a cytotoxic agent [cyclophosphamide (CYC)] is typically the first combination of medications to be prescribed.
Treatment
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After control of the disease CYC is then typically switched to azathioprine or
methotrexate .
Prednisone is usually discontinued after approximately 6 months.
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Churg-Strauss Syndrome
Presented by: asmaa hassan 193
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Churg-Strauss Syndrome Defination• Churg-Strauss syndrome (CSS), or allergic
granulomatous angiitis, is a rare syndrome that affects small- to medium- sized arteries and veins. This disease was first described in 1951 by Dr. Jacob Churg and Dr. Lotte Strauss as a syndrome consisting of :
1. 1-Asthma 2. 2-eosinophiliaeosinophilia3. 3-fever4. 4-vasculitis of various organ systems
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Causes
Environmental Genetics
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Clinical Picture:
1-Type of the patient-is a middle aged individual with a history of new-onset or newly-worsened asthma.-male=female
2-Classic symptoms and signs of Churg-Strauss Syndrome
- is a highly variable illness
mild symptoms
severe or life-threatening complications
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Stages of Churg-Strauss syndrome
There are three stages, or phases, of Churg-Strauss syndrome but not everyone develops all three phases or in the same order
A)-Allergic stage This is usually the first stage of Churg-Strauss
syndrome. It's marked by a number of allergic reactions, including:
a-Asthma:Asthma symptoms may begin long before the onset of vasculitis
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b-Hay fever (allergic rhinitis):This affects the mucous membranes of the nose causing runny nose sneezing itching
c-Sinus pain and inflammation (sinusitis): there is facial pain and develop nasal polyps
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(B)-Eosinophilic stage An eosinophil is one subtypes of white blood cell.
Normally, eosinophils comprise 5% or less of the total white blood cell count. In CSS, the percentage of eosinophils may reach as high as 60%. In the picture below, the eosinophils are shown by the dark pink stain.
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Signs and symptoms of eosinophilia may include:
* Fever * Weight loss * Asthma * Fatigue * Night sweats * Cough * Abdominal pain * Gastrointestinal bleeding
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(C)-Vasculitic stage-the hallmark of this stage of Churg-Strauss
syndrome is severe blood vessel inflammation (vasculitis).
-By narrowing blood vessels, inflammation reduces blood flow to vital organs and tissues throughout the body, including the skin, heart, peripheral nervous system, muscles, bones and digestive tract.
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Nose• * Sinusitis, • * Nasal polyps
Lung * Pulmonary infiltrates * Bleeding into the lungs * Diffuse interstitial lung disease
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Skin• Rashes• Palpable purpura• Nodules • often at sites of pressure, • such as the elbow
Kidney• Glomerulonephritis• Hypertension
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Gastrointestinal
Heart
Granuloma sometimes found in spleen
congestive heart failure or a heart attack
Nerve
pain
numbness in extremities
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Diagnosis:1-abnormal blood tests (eosinophilia, in particular)
.2-Elevated (ESR) and (CRP) levels3-ANCA is present in approximately 40% of
patients4-Elevated serum IgE levels 5-In addition to a detailed history and physical
examination, blood tests and imaging studies6-nerve conduction tests, and tissue biopsies
(e.g., of lung, skin, or nerve)
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The ACR selected 6 disease features (criteria)
patient should have at least 4 of the 6 ACR criteria.These criteria include: 1. asthma 2. eosinophilia [>10% on differential WBC count] 3. mononeuropathy 4. transient pulmonary infiltrates on chest X-rays 5. paranasal sinus abnormalities 6. biopsy containing a blood vessel with
extravascular eosinophils
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Treatment
oral
intravenous )usually methylprednisolone(
prednisone
2-immunosuppressive drugs. ,such as azathioprine
methotrexate,or cyclophosphamide may be used in addition to prednisone.
11--steroidssteroids
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Immune Complex-Mediated Small Vessel Vasculitis
There are four principal subtypes: • hypersensitivity vasculitis;• Cryoglobulinemic vasculitis;• Henoch–Schonlein purpura (HSP); • Hypocomplementemic urticarial vasculitis.
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Hypersensitivity cutaneous Vasculitis:
Presented by :Asmaa khairy beltagy 194
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Hypersensitivity cutaneous Vasculitis:
Definition:• immune complex small-vessel vasculitis
that is restricted to the skin • not associated with any other form of
primary or secondary vasculitis.• not associated other organ involvement
(eg, the glomeruli or pulmonary capillaries)
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cutaneous leukocytoclastic angiitis
hypersensitivity vasculitis
leukocytoclastic vasculitis
cutaneous small-vessel vasculitis
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Epidemiology:• incidence 10-30 cases per million per
year.
• Race
• Sex
• Age
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Causes of hypersensitivity vasculitis:
• half of patients no inciting agent can be identified
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Other causes:• Medications:
• Infections:
• Some malignancies:
• Foods or food additives:
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Pathogenesis:• Arthus reaction :
• Immunegenecity:
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Clinical findings:Skin• palpable purpura , non palpable purpura,• papules,• vesicles, pustules, • urticaria, ulcers,• non palpable laisions (macules and patches) • splinter heamorrhage. • occur in symmetric fashion over dependent regions, ie,
the lower extremities or buttocks. • occur in cohorts or "crops" that are the same age. • may be asymptomatic or accompanied by a burning or
tingling sensation
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• Joints Hypersensitivity vasculitis is sometimes
accompanied by arthralgias and even frank arthritis, with a predominance for large joints.
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Complications• residual hyperpigmentation
• scars (in the case of ulcerated lesions)
• recurrent disease
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Differential Diagnosis• Other vasculitides• Henoch-Schönlein purpura• Mixed cryoglobulinemia• Microscopic polyangiitis• Churg-Strauss syndrome• Wegener granulomatosis• Polyarteritis nodosa• Systemic autoimmune conditions• Systemic lupus erythematosus• Rheumatoid arthritis• Miscellaneous• Other types of drug eruptions• Thrombotic thrombocytopenic purpura.• Infective endocarditis.• Pregnancy associated purpura.
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Diagnosis1. ACR Criteria for Classification of
Hypersensitivity Vasculitis
2. Laboratory Findings and imaging
3. Biopsy
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1. American College of Rheumatology 1990 Criteria for the Classification of Hypersensitivity Vasculitis
1. Age at disease onset >16 years.2. Offending medication at disease onset.3. Palpable purpura.4. Maculopapular rash.5. Biopsy including arteriole and venule, showing neutrophils
perivascular
three of these five criteria has Sensitivity = 71%; specificity = 83.9%.
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2.Laboratory Findings and imagingTestTypical Result
Complete blood cell count, with differentialNormal
ElectrolytesNormalLiver function testsNormalUrinalysis with microscopyNormalErythrocyte sedimentation rate/C-reactive protein
Mild to moderate elevations in <50% of patients
ANANegativeRheumatoid factorNegativeC3, C4NormalANCANegativeAntihepatitis B and C assaysNegativeCryoglobulinsNegativeChest radiography, CT and MRINormal
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3.Biopsy:• Light microscopy:• 24 – 48 hours appearance of a lesion• from non ulcerated leision• If there are ulcers , it should be taken
from edges of the ulcer.• Cellular infiltrate of neutrophils and
lymphocytes (lymphocytes rich infiltrates may be seen in new
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• Direct immuneflourescent microscopy (DIF)
this patient had a history of ventricular septal defect that was complicated by streptococcal septicemia and was associated with IgA and IgM vascular immunoglobulin deposition
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Diagnostic algorithm :Pesentation consistent with small vessel
vasculitis
HistoryMdication
Infection e.g HCVConnecive tissue
disease
Review of systems and
physical ex. to exclude extra
cutaneous manifestation
Work-up
Skin biopsy
histoogy DIF
Blood work
radiologyCBC ANA
ANCARF
CryoglobulinsC3, C4
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Treatment:1. Elevation of the legs2. or compression stockings3. removal of the offending agent4. Mild cases: NSAID5. For persistent disease: colchicine,
hydroxychloroquine or dapsone 6. refractory or more severe cases:
immunosuppressive agents e.g glucocorticoids or Azathioprine
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ByAsmaa Samy Farag El Naggar 195
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DefinitionCryoglobulinimic vasculitis (CV): It is a systemic vasculitis secondary to circulating immune complex deposition in small blood vessels.
The name literally means “cold antibody in the blood”
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Classification of CryoglobulinemiaA. Brouet classification: based on cryoglobulin
composition
Classification of CryoglobulinsComposition of cryoprecipitates
Type I cryoglobulinemia monoclonal Ig, usually IgM or, less frequently, IgG or IgA
Type II mixed cryoglobulinemiapolyclonal IgGs + monoclonal IgM
Type III mixed cryoglobulinemia
polyclonal IgGs + polyclonal IgMs
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B. Classification based on the association of the syndrome with an underlying disease:
1 .Essential, or idiopathic
2 .Secondary
Distinct disorder; it can be classified among systemic vasculitides.
lymphoproliferative disorder,
autoimmune disease, infectious disease
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Epidemiology The prevalence varies from country to country(related to the
endemicity of HCV ).
No racial predilection.
female: male = 3:1.
Mean age = 42-52 ys.
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Etiology of Mixed Cryoglobulinemia1. There is frequent association between MC and HCV
2. However, the MC is also the result of concomitant genetic
and/or environmental factors, which remain largely
unknown.
A role for HBV < 5% . HCV in MC patients 70% - 100% .
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Etiopathogenesis of MC in HCV
HCV
hepato- and lymphotropic virus
lymphoproliferation & antibody production
Due to the shared expression of CD81 receptors
HCV-dependent gene translocation protects cells against apoptosis Lowering of the cell-activation threshold
HCV syndrome
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Clinical descriptionMeltzer triad: purpura, weakness, arthralgias
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Specific clinical manifestations:
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Specific clinical manifestations:
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Types of presentations: A. Isolated serum mixed cryoglobulins B. Complete cryoglobulinemic syndrome C. Incomplete mixed cryoglobulinemia D.Typical cryoglobulinemic syndrome, but without serum
cryoglobulins
MC can represent a crossroads between some autoimmune disorders and malignancies (B-cell lymphomas, HCC). In only a minority of MC patients a malignancy may develop, generally after a long lasting.
N.B
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Diagnostic methodsThe main diagnostic parameter of MC is the presence of serum
mixed (IgG-IgM) cryoglobulins.
Laboratory Studies:1. Evaluation for serum cryoglobulins2. RF: RF is positive in types II and III.3. Complement evaluation: hypocomplementemia (esp. C4 ).1. Liver function& hepatitis serology. 2. CBC: Leukocytosis &/or Anemia may be present.3. Other
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Imaging Studies:1. Angiography 2. CT imaging may be
considered upon high suspicion of underlying malignancy.
3. Others according to clinical manifestation
Tissue biopsy: It may be required for diagnosis
when patients with vasculitis, renal disease, or both are evaluated.
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Diagnosic criteriacriteria serologicalpathologicalclinicalmajor mixed
cryoglobulins low C4
leukocytoclastic vasculitis
purpura
minorRheumatoid factor + HCV+ HBV +
clonal B-cell infiltrates (liver and/or bone marrow)
chronic hepatitis MPGN peripheral neuropathy skin ulcers
Definite mixed cryoglobulinemia syndrome:a. serum mixed cryoglobulins (± low C4) + purpura + leukocytoclastic vasculitis b. serum mixed cryoglobulins (± low C4)+ 2 minor clinical symptoms + 2 minor serological/pathological findings
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TreatmentThe goal of therapy is to:
1. Treat underlying conditions.2. Limit the precipitant cryoglobulin and the resultant
inflammatory effects.
Asymptomatic cryoglobulinemia no treatment. Secondary cryoglobulinemia treatment of
underlying or associated disease.
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Treatment modalities1. Nonsteroidal anti-inflammatory drugs.
2. Immunosuppressive medications (corticosteroid therapy
cyclophosphamide or azathioprine)
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3. Plasmapheresis is indicated for severe complications
related to cryoprecipitation or serum hyperviscosity.
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4. Pegylated interferon alfa combined with ribavirin.
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5. Rituximab therapy
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Treatment of HCV-associated Mixed Cryoglobulinaemia
State of patientProposed treatmentsAsymptomaticnone
Mild manifestations:purpura, weakness ,arthralgias, arthritis, peripheral sensory neuropathy
LAC-diet, low dosage of steroids other symptomatics
Severe manifestations: nephropathy, skin ulcers, sensory-motor neuropathy, widespread vasculitis, active hepatitis
steroids, plasma exchange, cyclophosphamide, alpha-interferon + ribavirine
Cancer : B-cell NHL, HCCchemotherapy, surgery
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Asmaa toto196
Henoch–Schönlein purpura
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Henoch–Schönlein purpuraDefinision also known as anaphylactoid purpura, rheumatica , purpurais a form of blood vessel inflammation or vasculitis. HSP affects the small vessels called capillaries in the skin and frequently the kidneys
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Epidemiology
• HSP occurs more often in children than in adults, and usually follows an upper respiratory tract infection.. It occurs about twice as often in boys as in girls
The incidence of HSP in children is about 20 per 100,000 children per year;
this makes it the most common vasculitis in childhood
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Etiology • The exact cause of HSP is unknown• HSP can develop after infections with
streptococci (β-haemolytic, Lancefield group A), hepatitis B, herpes simplex virus, parvovirus B19 Drugs linked to HSP, usually as an idiosyncratic reaction,
include the antibiotics vancomycin and ACE inhibitors enalapril and captopril, anti-
inflammatory agent Diclofenac and streptokinase
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Pathophysiology
• Henoch-Schönlein purpura is a small-vessel vasculitis in which complexes of immunoglobulin A (IgA) and complement component 3 (C3) are deposited on arterioles, capillaries, and venules. As with IgA nephropathy, serum levels of IgA are high in HSP and there are identical findings on renal biopsy; however, IgA nephropathy has a prediliction for young adults while HSP is more predominant among children.
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Signs and symptoms
Purpura, arthritis and abdominal pain are known as the "classic triad" of Henoch–
Schönlein purpura.
Purpura occurs in all cases, joint pains and arthritis in 80%and abdominal pain in 62%.
Some include gastrointestinal hemorrhage as a fourth criterionthis occurs in 33% of cases may lead to intussussception
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Purpura
The purpura typically appear on the legs and
buttocks, but may also be seen on the
arms, face and trunk.
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The abdominal pain • is colicky in character, and may be accompanied
by nausea, vomiting, constipation or diarrhea. There may be blood or mucus in the stools.
The joints involved tend to be the ankles, knees, and elbows ,
but arthritis in the hands and feet is possible ;
the arthritis is nonerosive and hence causes no permanent deformity.
Forty percent have evidence of kidney involvement,
mainly in the form of hematuria (blood in the urine)
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Problems in other organs • such as the central nervous system
(brain and spinal cord) and lungs may occur, but is much less common than in the skin, bowel and kidneys.
Hypertension (high blood pressure) may occurProtein loss and high blood pressure,
Symptoms usually last approximately a month.
Recurrences are not frequent but do occur.
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Diagnosis• The diagnosis is based on the
combination of the symptoms, as very few other diseases cause the same symptoms together
• Blood tests may show elevated creatinine and urea levels (in kidney involvement) raised IgA levels
• The platelet count may be raised, and distinguishes it from diseases where low platelets are the cause of the purpura, such as idiopathic thrombocytopenic purpura
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skin biopsy
• The appearances are of• a hypersensitivity vasculitis• and immunofluorescence• demonstrates IgA and C3 • in the blood vessel wall. • However, overall serum • complement levels are normal
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kidney biopsy• Main findings on kidney biopsy are
increased cells and Ig deposition in the mesangium
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Classification
• the 2006 European League Against Rheumatism (EULAR) and Pediatric Rheumatology Society (PReS)
classification, include palpable purpura as a mandatory criterion together with at least one of the following findings :
diffuse abdominal pain, predominant IgA deposition (confirmed on skin biopsy) ,
acute arthritis in any joint, and renal involvement
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Treatment• Pain killers may be needed for the
abdominal and joint pains
Most patients do not receive therapy because of the high spontaneous recovery rate. Steroids are generally avoided.
However, if they are given early in the disease episode ,the duration of symptoms may be shortened ,and abdominal pain can improve significantly .
Moreover, the chance of severe kidney problems is reduced
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Evidence of worsening kidney damage
• Treatment may be indicated on the basis of the appearance of the biopsy sample; various treatments may be used, ranging from oral steroids to a combination of intravenous methylprednisolone (steroid), cyclophosphamide and dipyridamole followed by prednisone
Intravenous immunoglobulin (IVIG) is occasionally used
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Prognosis• Recovery and recurrence• Overall prognosis is good in most patients,
with one study showing recovery occurring in 94% and 89% of children and adults, respectively (some having needed treatment).In children under ten, the condition recurs in about a third of all cases and usually within the first four months after the initial attack. Recurrence is more common in older children and adults
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