Taber's Medical Dictionary
vasculitis
(vas″kyŭ-līt′ĭs )
(vas″kyŭ-lit′ĭ-dēz″ )
pl. vasculitides [L. vasculum, small vessel + -itis ]
Inflammation of blood vessels.
It is usually caused by deposition of antigen-antibody immune complexes or other immune-mediated events. Vasculitis due to immune complexes is seen in patients with systemic lupus erythematosus, rheumatoid arthritis, hepatitis B and C, serum sickness, and drug reactions. Vasculitis found in patients with inflammatory bowel disease, Wegener granulomatosis, graft rejection, polyarthritis nodosa, and temporal arteritis involves other immune-mediated processes. Vasculitis often affects the renal glomeruli, joints, cerebral vessels, testes, or respiratory system.
Vasculitis can affect large, medium-sized, and small blood vessels. When it is found in small blood vessels in the skin, characteristic rashes may be seen. Vasculitis is loosely classified by the size of the vessel involved. Takayasu and giant cell arteritis involve large arteries, including the aorta and carotids. Polyarteritis nodosa and Kawasaki disease involve medium-sized vessels; Wegener granulomatosis, Henoch-Schönlein purpura, and microscopic polyangiitis involve small vessels, particularly in the kidney and respiratory tract.
SYN: SEE: angiitis
VASCULITIS ; SEE: autoimmune disease; SEE: immune complex
SYMPTOMS
Although fever, pain, and malaise are common, the inflammatory changes of the blood vessels are seen primarily through the signs and symptoms associated with the organ or tissues involved. Vasculitis in superficial vessels may present as painful nodules. Inflammation of the glomerular capillaries of the kidney in small vessel vasculitis may produce glomerulonephritis and decreased renal function. When blood vessels of the respiratory tract are involved, pneumonitis, sinusitis, and ulceration of the nasopharynx may result. Involvement of vessels in the heart leads to coronary artery disease and aneurysms.
TREATMENT
Immunosuppressive therapy (with drugs such as cyclophosphamide and prednisone, or monoclonal antibodies such as rituximab) is used to treat most forms of autoimmune-mediated vasculitis.
VASCULITIS
ANCA-associated vasculitis
A rare, systemic autoimmune illness characterized by inflammation and necrosis of small and medium-sized blood vessels in the presence of antineutrophilic cytoplasmic antibodies. The antibodies target myeloperoxidase and proteinase 3, two intracellular proteins. The disease causes damage primarily to the kidneys and lungs.
cryoglobulinemic vasculitis
SEE: cryoglobulinemi
hypersensitivity vasculitis
SEE: leukocytoclastic vasculitis
IgA vasculitis
immunoglobulin A vasculitis A form of small-vessel vasculitis of unknown cause.
SYN: Henoch-Schönlein purpura
HENOCH-SCHÖNLEIN PURPURA
INCIDENCE
It affects children (esp. between the ages of 3 and 5 years) more often than adults, but in any age group it is a rare disease. It typically occurs in the spring.
CAUSES
The cause of HSP is unknown but often occurs after a recent upper respiratory infection. No cause-and-effect association has not been proved.
SYMPTOMS AND SIGNS
It is characterized by the appearance of a purpuric rash on the buttocks and legs and in some patients abdominal pain or gastrointestinal bleeding, polyarticular joint disease, and renal involvement, e.g., glomerulonephritis. The illness usually lasts about 2 to 8 weeks before spontaneously resolving. In some instances (more often in adults than in children) renal failure can complicate the illness.
DIAGNOSIS
The disease is usually clinically obvious because the distribution of the purpuric rash of HSP is unique. When the diagnosis is unclear, biopsies of affected organs, e.g., skin, kidneys, or the intestinal tract may reveal deposits of immunoglobulin A.
TREATMENT
Joint pains respond to rest and administration of nonsteroidal anti-inflammatory drugs. Corticosteroids such as prednisone are used to treat patients with severe gastrointestinal or renal involvement. Hemodialysis is used to support patients who develop renal failure.
PATIENT CARE
Rest is recommended to avoid traumatizing the skin or other organs. Patients should take prescribed medications and see their health care providers for necessary testing and follow up. Maintaining hydration, using ambulatory assistive devices (when joint pains and stiffness limit the ability to walk), and reporting any complications are the hallmarks of patient care. Educating patients about the natural history of the disease (esp. its usual resolution in several weeks) provides reassurance to those patients who have concerns about chronic illness. Patient education also helps HSP patients to know when to report symptoms that suggest worsening or complications of the illness.
HENOCH-SCHÖNLEIN PURPURA
leukocytoclastic vasculitis
A group of systemic diseases characterized by inflammation and necrosis of small blood vessels caused by the destruction of neutrophils and the release of their nuclear material into the blood stream. It causes a classical physical finding in the skin, called palpable purpura, in which small blue or purple papules arise, often on the ankles and legs. Affected patients also usually have fever, joint and muscle pain, swollen glands, and malaise. Causes include some infections, drug reactions, and connective tissue diseases.
SYN: angiitis, hypersensitivity; vasculitis, hypersensitivity
livedoid vasculitis
Vasculitis with bloodclotting that affects small blood vessels in the skin, esp. near the feet and ankles. The cause in most cases is unknown, but it may be associated with diseases such as antiphospholipid antibody syndrome, systemic lupus erythematosus or scleroderma.
SYN: SEE: atrophie blanche
VASCULITIS
rejection vasculitis
Vasculitis that occurs when antigen-antibody complexes are deposited on the walls of small blood vessels in transplanted organs. Although the transplant rejection process is dominated by T-cell-mediated activities, antibodies also may form against the histocompatibility antigens on the transplanted organ and compromise its viability.
SEE: major histocompatibility complex.
renal-limited antineutrophil cytoplasmic antibody associated vasculitis
Vasculitis in which ANCA (antineutrophil cytoplasmic antibody) is found solely in the kidneys, and not in other organs such as the brain, lungs, or testes.
rheumatoid vasculitis
A relatively rare complication of severe rheumatoid arthritis, characterized by blood vessel inflammation, esp. in the skin, eyes (the sclera), and nerves. It can produce deep dermal ulcers, localized skin infarction, ischemia or necrosis of the fingertips, scleritis, and mononeuritis multiplex.
