Cerebral vasculitis

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Cerebral vasculitis
Other namesCentral nervous system vasculitis
Specialty Cardiology, neurology, rheumatology   OOjs UI icon edit-ltr-progressive.svg

Cerebral vasculitis (sometimes the word angiitis is used instead of "vasculitis") is vasculitis (inflammation of the blood vessel wall) involving the brain and occasionally the spinal cord. [1] It affects all of the vessels: very small blood vessels (capillaries), medium-size blood vessels (arterioles and venules), or large blood vessels (arteries and veins). If blood flow in a vessel with vasculitis is reduced or stopped, the parts of the body that receive blood from that vessel begins to die. It may produce a wide range of neurological symptoms, such as headache, skin rashes, feeling very tired, joint pains, difficulty moving or coordinating part of the body, changes in sensation, and alterations in perception, thought or behavior, as well as the phenomena of a mass lesion in the brain leading to coma and herniation. Some of its signs and symptoms may resemble multiple sclerosis. [2] 10% have associated bleeding in the brain. [3]

Contents

Causes

"Primary" angiitis/vasculitis of the central nervous system (PACNS) is said to be present if there is no underlying cause. The exact mechanism of the primary disease is unknown, but the fundamental mechanism of all vasculitides is autoimmunity. Other possible secondary causes of cerebral vasculitis are infections, systemic auto-immune diseases such as systemic lupus erythematosus (SLE) and rheumatoid arthritis, medications and drugs (amphetamine, cocaine and heroin), some forms of cancer (lymphomas, leukemia and lung cancer) and other forms of systemic vasculitis such as granulomatosis with polyangiitis, polyarteritis nodosa or Behçet's disease. It may imitate, and is in turn imitated by, a number of other diseases that affect the blood vessels of the brain diffusely such as fibromuscular dysplasia and thrombotic thrombocytopenic purpura. [3]

Diagnosis

Cerebral angiography and magnetic resonance imaging, family medical history, symptoms, a complete physical examination, and ultimately biopsy of the brain, are often required for the diagnosis. Also, many lab tests must be done for the diagnosis; tests may reveal anemia (a shortage of red blood cells), a high white blood cell count, a high platelet count, allergic reactions, immune complexes, antibodies (tools the body uses to fight off threats) and elevation of inflammatory markers. [4] Another crucial part in the diagnosis of cerebral vasculitis is the use of imaging techniques. Techniques such as conventional digital subtraction angiography (DSA) and magnetic resonance imaging (MRI) are used to find and monitor cerebral involvement.[ citation needed ]

Treatment

Treatment is first with many different high-dose steroids, namely glucocorticoids. Then, if symptoms do not improve additional immunosuppression such as cyclophosphamide are added to decrease the immune system's attack on the body's own tissues. [3] Cerebral vasculitis is a very rare condition that is difficult to diagnose, and as a result there are significant variations in the way it is diagnosed and treated. [5]

Specific Diseases

Giant cell arteritis (GCA)

(Also known as temporal arteritis)

Symptoms

  • Fever
  • general uneasiness
  • weight loss
  • inflammation of the muscles causing stiffness in the shoulders; neck; and/or upper arms
  • persisting headache
  • pain in the jaw or ear while eating
  • double vision
  • partial loss of vision or blind spots
  • (on rare occasions) stroke.

Diagnostic criteria

Three or more of the following five criteria must be met:

  • Age 50 years or more
  • New developed headache
  • Tenderness of the superficial temporal artery
  • Elevated sedimentation rate, at least 50 mm/hour (blood test that reveals inflammatory activity)
  • Giant cell arteritis in a biopsy specimen from the temporal artery

Takayasu's arteritis

Symptoms

Starts with nonspecific symptoms such as:

  • Localized joint pain
  • Fever
  • Fatigue
  • Headaches
  • Rashes
  • Weight loss
  • Diagnosis usually does not happen until the blockage causes deficient blood flow to the extremities or to a stroke.

Classification criteria

Three or more of the following six criteria must be met:

  • Age when disease starts is under 50
  • Decreased brachial artery pulse
  • Systolic blood pressure differs by more than 10mmHg between arms
  • Cramping caused by exercise in the extremities
  • Abnormal sounds (through stethoscope) over subclavian arteries or abdominal aorta
  • A narrowing or blockage in the aorta, its primary branches, or large arteries as seen through a radiograph of the arteries.

Treatment therapy

  • 50% of patients respond to corticosteroid therapy alone in early phases
  • Methotrexate or Azathioprine are an alternative to corticosteroid immunosuppressants
  • There have been studies on Mycophenolate mofetil and anti-TNF therapies
  • In Takayasu’s arteritis it is vital to combine drug treatments often with low-dose aspirin or statin

Polyarteritis nodosa (PAN)

Symptoms

  • Systemic illness with fever
  • General feeling of discomfort or uneasiness with cause difficult to identify
  • Weight loss
  • Arthritis
  • Black discoloration of skin primarily on the extremities
  • Severe inadequate blood supply to the extremities
  • Ischemic stroke, hemorrhages and a progressive encephalopathy with or without seizures may occur

Diagnostic Criteria

Three or more of the following ten criteria are required:

  • More than 4 kg (8.8 lb) weight loss
  • Lace-like purplish discoloration of the skin (livedo reticularis)
  • Testicular pain
  • Pain in a muscle or group of muscles (myalgias)
  • Damage to peripheral nerves
  • Elevation of blood pressure by more than 90 mmHg
  • Creatinine serum levels greater than 1,5 mg/dl
  • Hepatitis B or C virus antibodies
  • An aneurysm or occlusion as shown in a pathologic arteriography
  • Histology findings typical of PAN

Treatment therapy

Granulomatosis with polyangiitis (GPA)

Symptoms

  • Men are affected twice as often as women
  • Compression of structures surrounding the nose and paranasal sinuses
  • Diabetes insipidus
  • Abnormal protrusion of the eyeball(s)
  • Nonseptic meningitis
  • Affection of the lung and kidney due to destruction of the arteries and veins
  • Ischemic stroke, hemorrhages, or encephalopathy with possible seizures

Diagnostic Criteria

Two or more of the following four criteria are required:

  • Necrotizing ulcerating inflammation of nose, sinuses, mouth or pharynx
  • Irregular lung infiltrates
  • Nephritis
  • Granulomatous vascular and perivascular inflammation

Treatment Therapy

Related Research Articles

Giant cell arteritis Medical condition

Giant cell arteritis (GCA), also called temporal arteritis, is an inflammatory autoimmune disease of large blood vessels. Symptoms may include headache, pain over the temples, flu-like symptoms, double vision, and difficulty opening the mouth. Complication can include blockage of the artery to the eye with resulting blindness, aortic dissection, and aortic aneurysm. GCA is frequently associated with polymyalgia rheumatica.

Mycophenolic acid

Mycophenolic acid (MPA) is an immunosuppressant medication used to prevent rejection following organ transplantation and to treat autoimmune conditions such as Crohn's disease and lupus. Specifically it is used following kidney, heart, and liver transplantation. It can be given by mouth or by injection into a vein. It comes as mycophenolate sodium and mycophenolate mofetil.

Vasculitis Medical disorders that destroy blood vessels by inflammation

Vasculitis is a group of disorders that destroy blood vessels by inflammation. Both arteries and veins are affected. Lymphangitis is sometimes considered a type of vasculitis. Vasculitis is primarily caused by leukocyte migration and resultant damage. Although both occur in vasculitis, inflammation of veins (phlebitis) or arteries (arteritis) on their own are separate entities.

Granulomatosis with polyangiitis Medical condition

Granulomatosis with polyangiitis (GPA), previously known as Wegener's granulomatosis (WG), is an extremely rare long-term systemic disorder that involves the formation of granulomas and inflammation of blood vessels (vasculitis). It is a form of vasculitis that affects small- and medium-size vessels in many organs but most commonly affects the upper respiratory tract, lungs and kidneys. The signs and symptoms of GPA are highly varied and reflect which organs are supplied by the affected blood vessels. Typical signs and symptoms include nosebleeds, stuffy nose and crustiness of nasal secretions, and inflammation of the uveal layer of the eye. Damage to the heart, lungs and kidneys can be fatal.

Henoch–Schönlein purpura Medical condition

Henoch–Schönlein purpura (HSP), also known as IgA vasculitis, is a disease of the skin, mucous membranes, and sometimes other organs that most commonly affects children. In the skin, the disease causes palpable purpura, often with joint pain and abdominal pain. With kidney involvement, there may be a loss of small amounts of blood and protein in the urine, but this usually goes unnoticed; in a small proportion of cases, the kidney involvement proceeds to chronic kidney disease. HSP is often preceded by an infection, such as a throat infection.

Eosinophilic granulomatosis with polyangiitis Medical condition

Eosinophilic granulomatosis with polyangiitis (EGPA), formerly known as allergic granulomatosis, is an extremely rare autoimmune condition that causes inflammation of small and medium-sized blood vessels (vasculitis) in persons with a history of airway allergic hypersensitivity (atopy).

Kawasaki disease Disease found in young children

Kawasaki disease is a syndrome of unknown cause that results in a fever and mainly affects children under 5 years of age. It is a form of vasculitis, where blood vessels become inflamed throughout the body. The fever typically lasts for more than five days and is not affected by usual medications. Other common symptoms include large lymph nodes in the neck, a rash in the genital area, lips, palms, or soles of the feet, and red eyes. Within three weeks of the onset, the skin from the hands and feet may peel, after which recovery typically occurs. In some children, coronary artery aneurysms form in the heart.

Thromboangiitis obliterans Medical condition

Thromboangiitis obliterans, also known as Buerger disease, is a recurring progressive inflammation and thrombosis (clotting) of small and medium arteries and veins of the hands and feet. It is strongly associated with use of tobacco products, primarily from smoking, but is also associated with smokeless tobacco.

Polyarteritis nodosa Medical condition

Polyarteritis nodosa (PAN) is a systemic necrotizing inflammation of blood vessels (vasculitis) affecting medium-sized muscular arteries, typically involving the arteries of the kidneys and other internal organs but generally sparing the lungs' circulation. Small aneurysms are strung like the beads of a rosary, therefore making this "rosary sign" an important diagnostic feature of the vasculitis. PAN is sometimes associated with infection by the hepatitis B or hepatitis C virus. The condition may be present in infants.

Takayasus arteritis Medical condition

Takayasu's arteritis (TA), also known as aortic arch syndrome, nonspecific aortoarteritis, and pulseless disease, is a form of large vessel granulomatous vasculitis with massive intimal fibrosis and vascular narrowing, most commonly affecting young or middle-age women of Asian descent, though anyone can be affected. It mainly affects the aorta and its branches, as well as the pulmonary arteries. Females are about 8–9 times more likely to be affected than males.

Arteritis Medical condition

Arteritis is the inflammation of the walls of arteries, usually as a result of infection or autoimmune response. Arteritis, a complex disorder, is still not entirely understood. Arteritis may be distinguished by its different types, based on the organ systems affected by the disease. A complication of arteritis is thrombosis, which can be fatal. Arteritis and phlebitis are forms of vasculitis.

Cryoglobulinemia Medical condition

Cryoglobulinemia is a medical condition in which the blood contains large amounts of pathological cold sensitive antibodies called cryoglobulins – proteins that become insoluble at reduced temperatures. This should be contrasted with cold agglutinins, which cause agglutination of red blood cells.

Polymyalgia rheumatica Medical condition

Polymyalgia rheumatica (PMR) is a syndrome with pain or stiffness, usually in the neck, shoulders, upper arms, and hips, but which may occur all over the body. The pain can be very sudden, or can occur gradually over a period. Most people with PMR wake up in the morning with pain in their muscles; however, cases have occurred in which the person has developed the pain during the evenings or has pain and stiffness all day long.

Relapsing polychondritis Medical condition

Relapsing polychondritis is a multi-systemic condition characterized by repeated episodes of inflammation and deterioration of cartilage. The often painful disease can cause joint deformity and be life-threatening if the respiratory tract, heart valves, or blood vessels are affected. The exact mechanism is poorly understood, but it is thought to be related to an immune-mediated attack on particular proteins that are abundant in cartilage.

Granulomatous meningoencephalitis (GME) is an inflammatory disease of the central nervous system (CNS) of dogs and, rarely, cats. It is a form of meningoencephalitis. GME is likely second only to encephalitis caused by canine distemper virus as the most common cause of inflammatory disease of the canine CNS. The disease is more common in female dogs of young and middle age. It has a rapid onset. The lesions of GME exist mainly in the white matter of the cerebrum, brainstem, cerebellum, and spinal cord. The cause is only known to be noninfectious and is considered at this time to be idiopathic. Because lesions resemble those seen in allergic meningoencephalitis, GME is thought to have an immune-mediated cause, but it is also thought that the disease may be based on an abnormal response to an infectious agent. One study searched for viral DNA from canine herpesvirus, canine adenovirus, and canine parvovirus in brain tissue from dogs with GME, necrotizing meningoencephalitis, and necrotizing leukoencephalitis, but failed to find any.

Susacs syndrome Medical condition

Susac's syndrome is a very rare form of microangiopathy characterized by encephalopathy, branch retinal artery occlusions and hearing loss. The cause is unknown but the current thinking is that antibodies are produced against endothelial cells in tiny arteries which leads to damage and the symptoms related to the illness. Despite this being an extremely rare disease, there are 4 registries collecting data on the illness; two are the United States, one in Germany, and one in Portugal.

Cholesterol embolism Medical condition

Cholesterol embolism occurs when cholesterol is released, usually from an atherosclerotic plaque, and travels as an embolus in the bloodstream to lodge causing an obstruction in blood vessels further away. Most commonly this causes skin symptoms, gangrene of the extremities and sometimes kidney failure; problems with other organs may arise, depending on the site at which the cholesterol crystals enter the bloodstream. When the kidneys are involved, the disease is referred to as atheroembolic renal disease. The diagnosis usually involves biopsy from an affected organ. Cholesterol embolism is treated by removing the cause and giving supportive therapy; statin drugs have been found to improve the prognosis.

Necrotizing vasculitis, also called systemic necrotizing vasculitus, is a category of vasculitis, comprising vasculitides that present with necrosis.

Cryofibrinogenemia refers to a condition classified as a fibrinogen disorder in which the chilling of an individual's blood plasma from the normal body temperature of 37 °C to the near-freezing temperature of 4 °C causes the reversible precipitation of a complex containing fibrinogen, fibrin, fibronectin, and, occasionally, small amounts of fibrin split products, albumin, immunoglobulins and other plasma proteins. Returning this plasma to 37 °C resolubilizes the precipitate.

Cutaneous small-vessel vasculitis Medical condition

Cutaneous small-vessel vasculitis (CSVV), also known as hypersensitivity vasculitis, cutaneous leukocytoclastic vasculitis, hypersensitivity angiitis, cutaneous leukocytoclastic angiitis, cutaneous necrotizing vasculitis and cutaneous necrotizing venulitis, is inflammation of small blood vessels, characterized by palpable purpura. It is the most common vasculitis seen in clinical practice.

References

  1. "Cerebral Vasculitis". Prime Health Channel. 19 December 2012. Retrieved 1 May 2015.
  2. Scolding NJ, Jayne DR, Zajicek JP, Meyer PA, Wraight EP, Lockwood CM (January 1997). "Cerebral vasculitis--recognition, diagnosis and management". QJM. 90 (1): 61–73. doi: 10.1093/qjmed/90.1.61 . PMID   9093590.
  3. 1 2 3 Rehman HU (November 2000). "Primary angiitis of the central nervous system". J R Soc Med. 93 (11): 586–8. doi:10.1177/014107680009301108. PMC   1298150 . PMID   11198690. Archived from the original (PDF) on 2009-11-05. Retrieved 2008-07-23.
  4. "Central Nervous System Vasculitis (CNS Vasculitis)". Cleveland Clinic. Retrieved 1 May 2015.
  5. Scolding NJ, Wilson H, Hohlfeld R, Polman C, Leite I, Gilhus N (July 2002). "The recognition, diagnosis and management of cerebral vasculitis: a European survey". Eur. J. Neurol. 9 (4): 343–7. doi: 10.1046/j.1468-1331.2002.00422.x . PMID   12099915. S2CID   18859529.
  6. Berlit, Peter (1 October 2010). "Diagnosis and treatment of cerebral vasculitis". Therapeutic Advances in Neurological Disorders. 3 (1): 29–42. doi:10.1177/1756285609347123. ISSN   1756-2864. PMC   3002614 . PMID   21180634.
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