Vasculitis

Signs and symptoms

• General symptoms: Fever, unintentional weight loss
• Skin: Palpable purpura, livedo reticularis
• Muscles and joints: Muscle pain or inflammation, joint pain or joint swelling
• Nervous system: Mononeuritis multiplex, headache, stroke, tinnitus, reduced visual acuity, acute visual loss
• Heart and arteries: Heart attack, high blood pressure, gangrene
• Respiratory tract: Nose bleeds, bloody cough, lung infiltrates
• GI tract: Abdominal pain, bloody stool, perforations
• Kidneys: Inflammation of the kidney's filtration units

  Cause

Classification

• Underlying cause. For example, the cause of syphilitic aortitis is infectious However, the causes of many forms of vasculitis are poorly understood. There is usually an immune component, but the trigger is often not identified. In these cases, the antibody found is sometimes used in classification, as in ANCA-associated vasculitides.
• Location of the affected vessels. For example, ICD-10 classifies "vasculitis limited to skin" with skin conditions, and "necrotizing vasculopathies" with musculoskeletal system and connective tissue conditions. Arteritis/phlebitis on their own are classified with circulatory conditions.
• Type or size of the blood vessels that they predominantly affect. Apart from the arteritis/phlebitis distinction mentioned above, vasculitis is often classified by the caliber of the vessel affected. However, there can be some variation in the size of the vessels affected.

• Large vessel: Takayasu's arteritis, Temporal arteritis
• Medium vessel: Buerger's disease, Kawasaki disease, Polyarteritis nodosa
• Small vessel: Behçet's syndrome, Eosinophilic granulomatosis with polyangiitis, Cutaneous vasculitis, granulomatosis with polyangiitis, Henoch–Schönlein purpura, and microscopic polyangiitis. Condition of some disorders have vasculitis as their main feature. The major types are given in the table below:

• Rheumatic diseases, such as rheumatoid arthritis, systemic lupus erythematosus, and dermatomyositis
• Cancer, such as lymphomas
• Infections, such as hepatitis C
• Exposure to chemicals and drugs, such as amphetamines, cocaine, and anthrax vaccines which contain the Anthrax Protective Antigen as the primary ingredient.

• Granulomatosis with polyangiitis
• Eosinophilic granulomatosis with polyangiitis
• Microscopic polyangiitis

  Diagnosis

• Laboratory tests of blood or body fluids are performed for patients with active vasculitis. Their results will generally show signs of inflammation in the body, such as increased erythrocyte sedimentation rate, elevated C-reactive protein, anemia, increased white blood cell count and eosinophilia. Other possible findings are elevated antineutrophil cytoplasmic antibody levels and hematuria.
• Other organ functional tests may be abnormal. Specific abnormalities depend on the degree of various organs involvement. A Brain SPECT can show decreased blood flow to the brain and brain damage.
• The definite diagnosis of vasculitis is established after a biopsy of involved organ or tissue, such as skin, sinuses, lung, nerve, brain, and kidney. The biopsy elucidates the pattern of blood vessel inflammation.
• An alternative to biopsy can be an angiogram. It can demonstrate characteristic patterns of inflammation in affected blood vessels.
• 18F-fluorodeoxyglucose positron emission tomography/computed tomography has become a widely used imaging tool in patients with suspected Large Vessel Vasculitis, due to the enhanced glucose metabolism of inflamed vessel walls. The combined evaluation of the intensity and the extension of FDG vessel uptake at diagnosis can predict the clinical course of the disease, separating patients with favourable or complicated progress.
• Acute onset of vasculitis-like symptoms in small children or babies may instead be the life-threatening purpura fulminans, usually associated with severe infection.

Treatment