Systemic vasculitides: It comprises of collection of disorders characterized by presence of fibrinoid necrosis and inflammation of blood vessel walls. The blood vessels are comprised of arteries that pass oxygen-rich blood to the tissues of the body and veins that return oxygen-depleted blood from the tissues to the lungs for oxygen. Vasculitis is characterized by inflammation and damage to the walls of various blood vessels.

Signs and Symptoms

The signs and symptoms of vasculitis vary depending on which blood vessels and, as a result, which organ systems are affected

. Small vessel Vasculitides:
Primary small vessel vasculitis : It is an autoimmune disease of unknown aetiology with environmental and genetic factors contributing to it. It is commonly associated with antineutrophil cytoplasm antibody (ANCA).
ANCA interferes with normal processes that resolve inflammation by disrupting apoptotic pathways and preventing apoptotic cell removal that in turn allows progression to secondary necrosis.
There are several vasculitides that affect small vessels.

. Churg-Strauss arteritis. Affects medium and small vessels with vascular and extravascular granulomatosis. It involves arteries of lungs and skin, but may be generalized. Churg-Strauss syndrome, in particular, occurs in patients with a history of asthma or allergy and features inflammation of blood vessels in the lungs, skin, nerves, and abdomen. The blood vessels involved in Churg-Strauss syndrome are small arteries.

Signs and Symptoms: There are three stages of Churg-Strauss syndrome,

Allergic: This is usually is the first phase (prodromal) of Churg-Strauss syndrome.

Allergic rhinitis: This affects the mucous membranes of your nose, causing runny nose, sneezing and itching.

Hypereosinophilia: In this phase of Churg-Strauss syndrome, your body produces abnormally high numbers of eosinophils.
. During this phase, the signs and symptoms include:
. Fever
. Weight loss
. Fatigue
. Night sweats
. Cough
. Pulmonary involvement : Dyspnoea, alveolar haemorrhage or pleurisy and with non specific pulmonary infiltrates on chest radiography. Asthma precede development of vasculitism often associated with non-specific symptoms.
. Cardiac involvement is a late manifestation.
. Gastrointestinal : Pain ,diarrhoea and ascites.
Systemic vasculitis:
With treatment in an earlier stage, you may not progress to this stage. The vasculitis commonly affects blood vessels in your skin, heart, lungs, central nervous system, musculoskeletal system, and gastrointestinal tract. It can also affect your kidneys. Signs and symptoms depend on which organs are affected.

. Microscopic polyarteritis/polyangiitis. Affects capillaries, venules, or arterioles. Thought to be part of a group that includes Wegeners since both are associated with ANCA and similar extrapulmonary manifestations. This form of vasculitis affects small-sized blood vessels in your kidneys, lungs and skin.
. Hypersensitivity vasculitis (allergic vasculitis). Usually due to a hypersensitivity reaction to a known drug. Drugs most commonly implicated are penicillin, sulphonamides and thiazide diuretics. There is presence of skin vaculitis with palpable petechiae or purpura. The primary sign of hypersensitivity vasculitis is red spots on your skin.
. Henoch Scholein purpura is a systemic vasculitis and characterized IgA deposition and is most common in children below 5 years of age.

Cause: The exact cause is not known but there is a upper respiratory tract infection.

Signs and Symptoms
. Rash usually affecting the buttocks and lower limbs
. Arthralgia
. Abdominal pain and bloody diarrhoea.
. Glomerulonephritis with increasing age.

Other organs may also be involved , C.N.S. - seizures may occur.
Pulmonary haemorrhage may occur if lungs are involved.

Diagnosis: Skin biopsy reveals leucocytoclastic vasculitis with IgE deposition in blood vessels or dermoepidermal junction.

Renal histology shows IgA deposition in the mesangium.

Medium vessel vasculitis
. Polyarteritis nodosa. Systemic necrotizing vasculitis and aneurysm formation affecting both medium and small arteries. If only small vessels are affected, it is called microscopic polyangiitis, This form of vasculitis affects small- to medium-sized blood vessels in many parts of the body, including your skin, heart, kidneys, peripheral nerves, muscles and intestines. Signs and symptoms are purpura, skin ulcers, muscle and joint pain, abdominal pain, and kidney problems.
. Wegener's granulomatosis. Systemic vasculitis of medium and small arteries, including venules and arterioles. Most common cause of saddle nose deformity in USA. (nose flattened due to destruction of nasal septum by granulomatous inflammation). This condition causes inflammation of the blood vessels in your nose, sinuses ,throat, lungs, and kidneys. Signs and symptoms can include shortness of breath, nasal stuffiness, chronic sinusitis, nosebleeds and frequent ear infections. nasal or oral inflammation, microscopic hematuria or RBC casts. Peak incidence: ages 40-60 males > females.
. Kawasaki disease :Kawasaki disease is also called mucocutaneous lymph node syndrome because it also affects lymph nodes, skin and the mucous membranes inside the mouth, nose and throat.

Cause: Usually children between 6 months to 8 years are affected. Staphyloccocal and streptococcal toxins are implicated in the pathogenesis. The exact cause is not known.

Signs and Symptoms: The signs and symptoms of Kawasaki disease appear in phases

First phase:
. Fever 4-5 days duration.
. Conjunctival congestion.
. Dry, red fissured lips, strawberry tongue.
. Reddening of oral and pharyngeal mucosa, sore throat.
. Redness of palms and soles
. Macular polymorphous rash on trunk.
. Swollen , cervical nodes, often unilateral

Second phase:
. Peeling of skin on the hands and feet especially tip of fingers and toes.
. Pyuria, urethritis,
. Arthralgia, arthritis i.e. Joint pain
. Diarrhoea, abdominal pain, obstructive jaundice or cholecystitis.

Third phase:
In the third phase of the disease, signs and symptoms slowly go away unless complications develop.

Large vessel vasculitis
. Takayasu arteritis: Primarily affects the aorta and its main branches.This form of vasculitis includes the largest arteries in the body and occurs in young women. Signs and symptoms include back pain, arm weakness or pain with use.(claudication of extremities ), decreased or absent pulses, lightheadedness, headaches, and visual disturbances. Ocular manifestation such as visual loss or field defects, retinal hemorrhages.
. Giant cell (temporal) arteritis: Chronic vasculitis of both large and medium vessels, primarily affecting cranial branches of the arteries arising from the aortic arch.This condition, which usually occurs in people older than 50, is an inflammation of the arteries in your neck, upper body, arms, and most often the head, especially your temples. It can cause headaches, scalp tenderness, jaw pain, blurred or double vision, and even blindness. Giant cell arteritis is often associated with polymyalgia rheumatica.

Diagnosis: Signs and symptoms of vasculitis are similar to many other medical conditions. So it is necessary to rule out other diseases.
. Blood tests:Blood test is carried out to know the E.S.R.( erythrocyte sedimentation rate). R.B.C count,W.B.C. count, platelet count. Some types of vasculitis result in an unusually high or low number of these cells.
. C-reactive protein test :You also may have a test that measures C-reactive protein (CRP), a substance produced by your liver in response to inflammation.
. Blood test to detect anti-neutrophil cytoplasmic antibodies (ANCAs) and other antibodies, such as rheumatoid factor (RF) and antinuclear antibody (ANA). ANCA can indicate a diagnosis of Wegener's granulomatosis or microscopic polyangiitis. RF and ANA elevations can be indications of an associated rheumatoid arthritis or connective tissue disease.
. Imaging studies are carried out to determine whether larger arteries, such as the aorta and its branches, are involved These include ultrasound, computerized tomography (CT) and magnetic resonance imaging (MRI).
. Biopsy :To confirm a diagnosis of vasculitis a small sample (biopsy) of the affected blood vessel is taken.
. The sample is examined for signs of inflammation under a microscope in a laboratory. If there is evidence of scarring, it implies that the condition has been chronic, or long-standing.

. Urine test. This test may detect abnormalities, such as red blood cells and increased amounts of protein, in your urine that often indicate a medical problem.

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