ARTERIAL INFLAMATORY DISEASES

 VASCULITIS

Vasculitis is inflammation of your blood vessels. It causes changes in the blood vessel walls, including thickening, weakening, narrowing or scarring. These changes can restrict blood flow, resulting in organ and tissue damage.

Vasculitis might affect just one organ, or several. The condition can be short term (acute) or long lasting (chronic).

Vasculitis is also known as angiitis and arteritis.

TYPES OF VASCULITIS

There are many types of vasculitis, and most of them are rare.

• Behcet’s disease
• Buerger’s disease
• Chrug Strauss syndrome
• Crygobulinemia
• Giant cell arteritis
• Granulomatosis with polyangitis
• Henoch scholein purpura
• Kawasaki disease
• Takayasu’s arteritis

SYMPTOMS

The signs and symptoms of vasculitis vary greatly. They're often related to decreased blood flow throughout the body

GENERAL SIGNS AND SYMPTOMS

General signs and symptoms of vasculitis include:

• Fever
• Headache
• Fatigue
• Weight loss
• General aches and pains
• Night sweats
• Rash
• Nerve problems, such as numbness or weakness

SIGNS AND SYMPTOMS FOR SPECIFIC TYPE OF VASCULITIS

Other signs and symptoms are related only to certain types of vasculitis. The symptoms can develop early and rapidly or in later stages of the disease

• Behcet's disease. This condition causes inflammation of your arteries and veins. Signs and symptoms include mouth and genital ulcers, eye inflammation, and acne-like skin lesions.
• Buerger's disease (thromboangiitis obliterans) . This condition causes inflammation and clots in the blood vessels of your hands and feet, resulting in pain and ulcers in these areas. Rarely, Buerger's disease can affect blood vessels in the abdomen, brain and heart. It is also called thromboangiitis obliterans.
• Churg-Strauss syndrome (Eosinophilic granulomatosis with polyangiitis). This condition is very rare. It mainly affects the lungs, skin, kidneys, heart and nerves in your limbs. Signs and symptoms vary greatly and include asthma, skin changes, nerve pain and nasal allergies.

• Cryoglobulinemia. This condition results from abnormal proteins in the blood. Signs and symptoms include rash, joint pain, weakness, and numbness or tingling.
• Giant cell arteritis. This condition is an inflammation of the arteries in your head, especially at the temples. Giant cell arteritis can cause headaches, scalp tenderness, jaw pain, blurred or double vision, and even blindness. It is also called temporal arteritis.
• Granulomatosis with polyangiitis. This condition causes inflammation of the blood vessels in your nose, sinuses, throat, lungs and kidneys. Signs and symptoms include nasal stuffiness, sinus infections, nosebleeds and possibly coughing up blood. But most people don't have noticeable symptoms until the damage is more advanced.
• Henoch-Schonlein purpura (IgA vasculitis). This condition is more common in children than in adults, and causes inflammation of the smallest blood vessels (capillaries) of your skin, joints, bowel and kidneys. Signs and symptoms include abdominal pain, blood in the urine, joint pain, and a rash on your buttocks or lower legs.
• Hypersensitivity vasculitis. Sometimes called allergic vasculitis, the primary sign of this condition is red spots on your skin, usually on your lower legs. It can be triggered by an infection or an adverse reaction to medicine.
• Kawasaki disease. This condition most often affects children younger than age 5. Signs and symptoms include fever, rash and redness of the eyes. It is also called mucocutaneous lymph node syndrome.

• Microscopic polyangiitis. This form of vasculitis affects small blood vessels, usually those in the kidneys, lungs or nerves. You may develop abdominal pain and a rash, fever, muscle pain and weight loss. If the lungs are affected, you may cough up blood.
• Polyarteritis nodosa. This form of vasculitis usually affects the kidneys, the digestive tract, the nerves and the skin. Signs and symptoms include a rash, general malaise, weight loss, muscle and joint pain, abdominal pain after eating, high blood pressure, muscle pain and weakness, and kidney problems.
• Takayasu's arteritis. This form of vasculitis affects the larger arteries in the body, including the aorta. Signs and symptoms include joint pain, loss of pulse, high blood pressure, night sweats, fever, general malaise, appetite loss, headaches and visual changes.
• Wegener granulomatosisThis is a necrotizing vasculitis associated with the triad:

 • Necrotizing or granulomatous vasculitis of small to medium vessels mostly in the lung and upper airway

 • Necrotizing granulomas of the upper and lower respiratory tract • Glomerulonephritis A T-cell hypersensitivity to an inhaled agent or microbe is likely causal, although PR3-ANCAs are present in 95% of cases

NONINFECTIOUS VASCULITIS

IMMUNE COMPLEX ASSOCIATED VASCULITIS

Immune complex–associated vasculitis is caused by vascular deposition of circulating antigen-antibody complexes (e.g., DNA/ anti-DNA complexes in systemic lupus erythematosus).

MECHANISM OF ACTION

Vascular injury arises from complement activation or the recruitment of Fc-receptor-bearing cells . Although the nature of the initiating antigen is not always known, immune complex deposition typically underlies the vasculitis associated with drug hypersensitivities (antibodies against the agent itself or directed against modified self-proteins); in vasculitis secondary to viral infections, antibodies are directed against viral proteins (e.g., hepatitis B surface antigen in 30% of patients with polyarthritis nodosa).

ANTINEUTROPHIL CYTOPLASMIC ANTIBODIES

Antineutrophil cytoplasmic antibodies (ANCAs) are a heterogeneous group of autoantibodies directed against the constituents of neutrophil primary granules, monocyte lysosomes, or EC:

 • Anti-myeloperoxidase (MPO-ANCA) is directed against the lysosomal constituent involved in generating oxygen free radicals; it is also called perinuclear-ANCA (p-ANCA). This antibody is characteristically seen in microscopic polyangiitis and Churg-Strauss syndrome.

 • Anti-proteinase 3 (PR3-ANCA) is directed against a neutrophil azurophiic granule constituent, also called cytoplasmic ANCA (c-ANCA). This is characteristically associated with Wegener granulomatosis.

ANTI ENDOTHELIAL CELL BODIES

Anti-endothelial cell antibodies may underlie certain vasculitides such as Kawasaki disease. Anti-endothelial cell antibodies (AECAs) are those that can bind to endothelial cells (ECs) via variable region-specific interactions. The identification and quantification of AECAs varies depending on the technique used. The best approach would be to combine at least two different methods. Thus, AECA measurement cannot be considered a diagnostic tool, but the detection and titers of AECAs are associated with disease activity in various systemic vasculitis diseases. AECAs have been described in almost all primary systemic vasculitis diseases but also in many secondary vasculitis diseases, with the identification of various antigens