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What is the diagnostic criteria for Takayasu Arteritis?
A diagnosis of Takayasu arteritis is established in patients who meet three or more of these criteria.
- Age at disease onset ≤40 yearsClaudication of the extremities
- Decreased pulsation of one or both brachial arteries
- Difference of at least 10 mm Hg in systolic blood pressure between the arms
- Bruit over one or both subclavian arteries or the abdominal aorta
- Arteriographic studies showing narrowing or occlusion of the entire aorta, its primary branches, or large arteries in the proximal upper or lower extremities that is not related to arteriosclerosis, fibromuscular dysplasia, or other causesa
Clinical manifestations of Takayasu Arteritis?
Takayasu arteritis has an initial inflammatory phase followed by a pulseless phase. The initial phase is characterized by fever, arthralgia, myalgia, malaise, and weight loss that can last weeks to months. ESR and C-reactive protein are elevated. The pulseless phase is characterized by symptoms of vascular insufficiency, such as arm or leg claudication, or hypertension that is usually caused by renal artery stenosis. Many patients with Takayasu arteritis do not seek medical attention until they enter this disease phase.
What is the treatment for Takayasu Arteritis?
High-dose corticosteroid therapy is indicated in the inflammatory phase. Elevated inflammatory markers, constitutional symptoms, or evidence of progressive arterial narrowing indicate the need for treatment. Uncontrolled trials suggest that methotrexate and tumor necrosis factor α inhibitors are effective steroid-sparing agents. In addition, cyclophosphamide is used in patients with severe refractory Takayasu arteritis.After the inflammatory phase of Takayasu arteritis has been controlled, affected patients may still have symptoms of vascular insufficiency due to fixed arterial narrowing. Aspirin may be used in this setting, but some patients require vascular intervention with revascularization. The outcome of coronary artery bypass graft surgery, angioplasty, and stenting is not optimal in these patients, and revascularization is not indicated until inflammation has been controlled.
What is Polyarteritis nodosa?
a necrotizing vasculitis of the medium-sized arteries.Polyarteritis nodosa has a peak age of onset between 40 and 60 years. Approximately 50% of cases of polyarteritis nodosa are associated with hepatitis B virus infection, usually of recent acquisition. The disorders also occur, but less frequently, in patients with hepatitis C virus infection.
Clinical manifestations and diagnosis of Polyarteritis nodosa?
Patients with polyarteritis nodosa typically present with fever, abdominal pain, arthralgia, and weight loss that develop over days to months. Two thirds of these patients have mononeuritis multiplex, and one third have hypertension, testicular pain, and cutaneous involvement including nodules, ulcers, purpura, and livedo reticularis.The kidneys, peripheral nerves, and heart also are commonly affected. Aneurysm formation is common, especially in the mesenteric vessels. Ischemia, not glomerulonephritis, causes renal disease in patients with this condition.Patients with polyarteritis nodosa usually have anemia, leukocytosis, and an elevated ESR. ANCA assays are almost always negative, particularly in patients with concomitant hepatitis B virus infection. In patients with a compatible clinical presentation, biopsy is generally taken from the skin or a sural nerve. Radiographic imaging of the mesenteric or renal arteries can also be used to establish a definitive diagnosis of polyarteritis nodosa. Characteristic findings of this condition include aneurysms and stenoses of the medium-sized vessels.
Treatment of Polyarteritis nodosa?
- High-dose corticosteroid therapyPatients with a poor prognosis or with disease that is not controlled by corticosteroids should receive cyclophosphamide in addition to corticosteroid therapy.
- Short-term high-dose corticosteroid therapy accompanied by an antiviral agent such as lamivudine is indicated in patients with concomitant hepatitis B virus infection.
What are the large-vessel vasculitis?
- Polymyalgia rheumatica
- Takayasu arteritis
What is the clinical manifestation of Polymyalgia Rheumatica?
What is the treatment?
- characterized by aching in the shoulders, neck, and hip girdle region; fatigue; and malaise that develop over weeks to months. ESR and C-reactive protein levels are elevated.
- Affected patients respond rapidly and dramatically to low-dose prednisone (10 to 20 mg/d).
What are the small-vessel vasculitis?
- Wegener Granulomatosis
- Microscopic polyangiitis
- Churg-Strauss syndrome
- Henoch-Schonlein purpura
- Cryoglobulinemic vasculitis
- Cutaneous leukocytoclastic vasculitis
Clinical manifestations and diagnosis of Wegener's?
- affects the respiratory tract and the kidneys. More than 70% of affected patients present with upper-airway symptoms, particularly sinusitis. Nasal, inner ear, or laryngotracheal involvement can also occur. Cartilage and tissue destruction can result in nasal septal perforation or a saddle nose deformity.
- Up to 90% of patients have pulmonary manifestations that can include cough, hemoptysis, or pleurisy. Radiographs in these patients show infiltrates or nodules that are often cavitary, as well as pulmonary hemorrhage
- Renal involvement occurs in 80% of patients. Renal manifestations usually are preceded by pulmonary disease and can be rapidly progressive. Other commonly affected organs include the eyes (scleritis, keratitis, uveitis, and an inflammatory soft-tissue mass in the orbital fossa known as retro-orbital pseudotumor), skin (purpura, nodules, and ulcers), and nerves (mononeuritis multiplex). Patients with Wegener granulomatosis also have an increased risk for venous thromboembolism.
Treatment of Wegener's
- High-dose corticosteroid therapy accompanied by a 3- to 6-month course of oral cyclophosphamide
- After remission has been achieved, cyclophosphamide can be switched to azathioprine or methotrexate.
- Treatment should be continued for at least 18 months.
- Etanercept is not effective in Wegener granulomatosis
What is Microscopic polyangiitis?
- Microscopic polyangiitis is a necrotizing vasculitis that typically involves the kidneys and lungs. It can affect patients of any age but has a peak age of onset between 30 and 50 years of age. Affected patients frequently present with glomerulonephritis that is often rapidly progressive and commonly results in renal failure; 50% of patients have pulmonary involvement that usually manifests as pulmonary hemorrhage. Fever, arthralgia, purpura, and mononeuritis multiplex can also occur.
- Antimyeloperoxidase antibodies are present in 60% to 85%
- Renal biopsy specimens reveal a pauci-immune necrotizing glomerulonephritis that is indistinguishable from that found in patients with Wegener granulomatosis. Lung biopsy specimens reveal a characteristic pulmonary capillaritis and may reveal inflammatory infiltrates.
Treatment for Microscopic polyangiitis?
corticosteroids and cyclophosphamide to induce remission, followed by maintenance therapy using azathioprine or methotrexate.
Clinical manifestations of Churg-Strauss Syndrome?
Virtually all patients with CSS have asthma; up to 80% of affected patients have a history of rhinitis or sinusitis. Migratory pulmonary infiltrates, mononeuritis multiplex, and purpura are each present in 50% of patients; cardiac and gastrointestinal involvement in 33%; and glomerulonephritis in 20%. Fever, arthralgia, and myalgia may also occur.
Tissue inflammation in patients with CSS is manifested as eosinophilic tissue infiltration or a necrotizing small-vessel vasculitis.
Treatment of Churg-Strauss Syndrome
High-dose corticosteroids are indicated to treat CSS and should be accompanied by cyclophosphamide in patients with renal, gastrointestinal, central nervous system, or cardiac involvement.
Describe the clinical manifestations and diagnosis of Henoch-Shonlein purpura
This condition is characterized by purpura, arthritis, abdominal pain, and renal disease. HSP usually resolves in days to weeks, but a subset of patients has persistent, progressive renal disease.
Skin biopsy specimens demonstrate leukocytoclastic vasculitis and IgA deposition. Renal biopsy specimens reveal a glomerulonephritis with IgA deposition that is indistinguishable from that in patients with IgA nephropathy.
Treatment of Henoch-Schonlein purpura
high-dose corticosteroids are considered for pts with severe glomerulonephritis, but there are no effective treatments for adults with HSP-associated renal disease
What is Cryoglobulinemic Vasculitis?
Cryoglobulins are immunoglobulins that precipitate in the cold and have rheumatoid factor activity. Type I cryoglobulins are monoclonal rheumatoid factors that can be associated with hyperviscosity and can produce ischemic ulcerations in acral areas exposed to the cold, such as the ears, nose, and finger tips. Type I cryoglobulins are present in patients with multiple myeloma and Waldenström macroglobulinemia.
Type II cryoglobulins are monoclonal rheumatoid factors that can be associated with small-vessel vasculitis. Type III cryoglobulins, which are not typically associated with vasculitis, are polyclonal rheumatoid factors that are present in patients with such chronic inflammatory diseases as hepatitis B and C virus infection, endocarditis, systemic lupus erythematosus (SLE), and rheumatoid arthritis.
Clinical manifestations and diagnosis of cryoglobulinemic vasculitis?
Type II cryoglobulinemic vasculitis manifests as palpable purpura, mononeuritis multiplex, hepatosplenomegaly, hypocomplementemia, and glomerulonephritis. Approximately 80% to 95% of patients with type II cryoglobulinemic vasculitis have hepatitis C virus infection, although only 5% of patients with hepatitis C virus infection and cryoglobulins develop vasculitis. A diagnosis of cryoglobulinemic vasculitis is established by the presence of cryoglobulins in the serum.
Treatment of Cryoglobulinemic Vasculitis
Interferon alfa and ribavirin are effective in 75% of patients with mild to moderate cryoglobulinemic vasculitis and hepatitis C virus infection. However, disease may recur if antiviral therapy is discontinued and hepatitis C virus has not been eradicated.
In patients with severe disease, a short course of corticosteroids occasionally accompanied by cyclophosphamide is indicated but should be discontinued once the acute phase of the condition has subsided.
What is Cutaneous Leukocytoclastic Vasculitis?
Cutaneous leukocytoclastic vasculitis can develop in patients with various localized and systemic disorders: 40% of cases are idiopathic; 20% are associated with medications; 22% are related to a recent infection; and 12% are a result of connective tissue diseases, primarily SLE and rheumatoid arthritis. The remaining cases occur in the context of a systemic vasculitis
Clinical manifestations and Diagnosis of Cutaneous leukocytoclastic vasculitis?
Cutaneous leukocytoclastic vasculitis can manifest as palpable purpura, skin nodules, ulcerations, livedo reticularis, or urticaria. The vasculitis typically occurs on the lower extremities, particularly below the knees, or where tightly fitting clothing is worn. Involvement of the upper extremities, trunk, head, or neck should raise suspicion for more severe disease or an associated illness. Symptoms of medication-induced cutaneous vasculitis typically manifest 7 to 21 days after initial exposure to the triggering agent.
Treatment of Cutaneous Leukocytoclastic Vasculitis?
NSAIDs, antihistamines, colchicine, or dapsone is used to manage symptomatic and persistent cutaneous leukocytoclastic vasculitis. Low-dose corticosteroid therapy is indicated if these agents are not effective, and use of a steroid-sparing agent such as methotrexate or azathioprine may be warranted in this setting. Treatment of medication-induced cutaneous leukocytoclastic vasculitis consists of discontinuing the triggering agent.
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