Primary Systemic Vasculitis

Vasculitis (angiitis) is an inflammation of blood vessels (aortitis, arteritis, arterioles, capillaritis, phlebitis, lymphangitis). Vasculitis can be destructive (necrotic), productive and granulomatous. Due to the depth of inflammatory injury endovasculites, endomes vasculitis and pan vasculitis are differentiated.vasculitis

Finally, inflammation of blood vessels leads to sclerosis of the vascular wall with its calcification. Vasculitis can be regional and systemic, primary and secondary.

The secondary vasculitis develops in different generalized infectious and noninfectious processes such as sepsis, rheumatic fever, syphilis, tuberculosis, SLE, malignant tumors, in patients with serum sickness and other allergic conditions.

Primary vasculitis act as original diseases. These are not widely spread diseases; these are represented by nonspecific aortoarteritis (Takayasu’s disease), temporal (cranial) arteritis (Horton’s disease), polyarteritis no dosa, Wegener’s granulomatosis, Buerger’s disease (thrombi angiitis obliterans) and some other diseases. Takayasu’s disease and Horton’s disease are based predominantly on reactions of hypersensitivity of the delayed type with the formation of granulomatous inflammation. In all other vasculitis circulating immune complexes play the decisive role. As inflammation in primary vasculitis is of immunogenic origin antibiotics are not effective in contrast to immunosuppressive drugs.

Takayasu’s disease occurs predominantly in young women and is characterized by immunogenic granulomatous inflammation of aorta and medium-sized and small arteries. The process is characterized by necrosis, sclerosis, and thrombosis of the arteries. Finally, inflammation leads to prominent sclerotic narrowing of arteries with numerous ischemic complications of the brain, heart, limbs and even eyes.

Temporal (cranial) giant-cell arteritis (Horton’s disease) affects mainly temporal arteries, but in some patients, it may combine with other types of arterites. The disease develops mostly in old people of both sexes. In the end-stage of the disease fibrotic thickening of the intima and scarring of the media develop with thrombosis of arteries. Clinically the disease manifests itself by facial pains and headache and ischemic complications in the brain, eyes.

Polyarteritis Nodosa – the disease develops mainly in young and middle-aged patients (mostly in men). It is characterized by immunogenic inflammation of medium-sized arteries of different organs, but predominantly of kidneys, heart, digestive tract and the brain. Microscopically necrosis, inflammation with thrombosis and sclerosis of all layers of the vascular wall is found. Clinically severe thoracic and abdominal ischemic pains are typical. Myocardial infarction, infarctions of the kidneys, of the brain and gangrenous appendicitis are typical for the disease.

Wegener’s granulomatosis – in this disease there is vasculitis and necrotizing granulomatous inflammation involves the nose and respiratory tract, the lungs, and kidneys in various combinations. Clinically nasal bleedings, pulmonary bleedings, and hematuria are highly typical.

Patients die because of necrotic pulmonary injuries or renal failure due to severe glomerulonephritis.

Buerger’s disease (thrombi angiitis obliterans) – is characterized by the simultaneous affection of arteries and veins of the low limbs. Smoking is a very significant risk factor for the disease. Morphologically inflammation, sclerosis, and thrombosis of arteries and veins are found; in the end-stage of the process gangrene of the legs may develop.

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