Vasculitis is an inflammatory disease that affects the blood vessels. The inflammation can cause blood vessel walls to thicken, reducing the breadth of the route through the channel. Organ and tissue damage can occur when blood flow is limited.
Vasculitis comes in a variety of forms, the most of which are uncommon. Vasculitis can damage one or many organs. The condition might endure for a short time or for a long time.
Vasculitis affects people of all ages, however some kinds are more frequent in specific age groups. You may improve without therapy depending on the type you have. The majority of kinds necessitate the use of medicines to reduce inflammation and avoid flare-ups.
Vasculitis is a Disease that Affects People
Vasculitis is a term used to describe a group of rare illnesses defined by the inflammation of blood vessels. Acute (short-term) vasculitis is one kind, whereas chronic (long-term) vasculitis is another (long-term).
There is currently no cure for vasculitis, although early identification and therapy can help to alleviate symptoms and slow the disease’s development.
The following are examples of vasculitis:
- Arteritis of the giant cells
- Granulomatosis with polyangiitis (also known as Wegener’s granulomatosis) is a kind of granulomatosis with polyangiitis.
- Polyangiitis on a microscopic scale
- Polyangiitis with eosinophilic granulomatosis (formerly known as Churg-Strauss syndrome)
- Polyarteritis nodosa is a kind of polyarteritis.
- Behcet’s illness is a condition that affects people of all ages.
- Arteritis Takayasu
Our rheumatologists have all undergone extensive training and experience in the treatment of vasculitic diseases. Depending on the kind of vasculitis, other sub-specialists may be engaged in the patient’s care.
Vasculitis in Children (VC)
Henoch-Schonlein purpura (also known as IgA vasculitis) and Kawasaki illness are the most prevalent forms of vasculitis in children.
Vasculitis can manifest as polyarteritis nodosa, Takayasu arteritis, ANCA-associated vasculitis, Behcet’s disease, or intrinsic vasculitis of the central nervous system. All of these illnesses are extremely rare in children.
Vasculitis Necrotizing (VN)
The term “necrotizing vasculitis” refers to a category of diseases characterised by inflammation of the blood vessel walls. The nomenclature of these diseases and how the problem produces sickness are determined in part by the size of the damaged blood vessels.
Vasculitis may affect every system of the body and cause a variety of symptoms.
Symptoms for the majority of kinds can include:
- Loss of body weight
- Discomfort (tiredness)
- Muscle spasms
- Vasculitis can be anything from a minor ailment to a potentially fatal condition.
- If severe vasculitis is detected and treated early, irreparable harm can be avoided. Blood tests, biopsy of afflicted tissue, and angiography are often used to diagnose vasculitis.
- Vascular disorders are inflammatory illnesses that are frequently treated with glucocorticoids.
- Other immune-suppressing drugs may be administered to patients as well. These can aid individuals with severe illness or allow them to take glucocorticoid dosages that are lower.
Vasculitis has an unknown underlying aetiology. Vasculitis is considered to be caused by immune system malfunctions in the majority of instances.
Certain medicines, such as sulphur drugs, penicillin, propylthiouracil, and other pharmaceuticals, poisons, or other inhaled environmental irritants, may cause allergic responses or hypersensitivity, resulting in vasculitis. Fungal, parasite, or viral infections can cause other types.
Vasculitis has been linked to autoimmune disorders in rare cases. When the body’s natural defences against “foreign” or invading organisms (e.g., antibodies) start attacking healthy tissue for unexplained reasons, autoimmune diseases develop.
Most of the time, the aetiology of these vasculitis illnesses is unknown. Immune system dysfunction and blood vessel inflammation are typical symptoms.
Each kind of vasculitis has its unique set of symptoms, which are mostly determined by the organs that are afflicted.
Vasculitis can manifest itself in a variety of ways, for example:
- Kawasaki disease is a condition that affects people who have had a stroke
- Behçet’s disease is a condition that affects people.
- Polyarteritis nodosa is a kind of polyarteritis that affects the joints.
- Polyangiitis with granulomatosis
- Takayasu’s arteritis is a kind of arthritis that affects the joints.
- Churg-Strauss syndrome is a condition that affects a person’s ability to think clearly.
- Temporal arteritis, and giant cell arteritis
- Henoch-Schönlein purpura is a kind of purpura caused by the Henoch-Schönlein syndrome.
Vasculitis can also occur in conjunction with the following conditions:
- Viruses (such as hepatitis B),
- Chemicals exposure (such as amphetamines and cocaine),
- Cancers (lymphoma and multiple myeloma, for example), and
- Rheumatoid arthritis is a kind of arthritis that affects the joints (such as rheumatoid arthritis and systemic lupus erythematosus).
Which Organ Systems are Likely to be Impacted?
It’s worth noting that not every organ system in every patient will be impacted. The kind of vasculitis, as well as the pattern of organ involvement (and symptoms), are unique to each person (category).
A wide range of rashes, the most well-known of which is “palpable purpura” –purplish–red patches that often appear on the legs. The term “palpable” refers to how easily these areas may be felt by the examiner’s fingertips.
Symptoms range from full–blown arthritis to aches and pains in the joints that aren’t accompanied by visible swelling (arthralgias). Henoch-Schönlein purpura is a kind of cutaneous vasculitis characterised by palpable purpura and arthritis (note the right ankle swelling).
A skin sample confirmed the diagnosis, with immunofluorescence revealing IgA accumulation in blood vessel walls.
Sinus, Nose and Ears
Chronic sinus congestion and “infections” that last longer than they should; hearing loss; nasal septum inflammation, which can lead to a perforation or collapse of the bridge of the nose, as illustrated in the illustration below.
May damage either blood vessels leading to the eyes, causing abrupt vision loss, or tiny blood vessels within the eyes, causing retinal issues, sclera (the white portion of the eyes) thinning, inflammation within the eye’s various chambers, and conjunctivitis (“pinkeye”).
A patient with systemic lupus erythematosus has retinal vasculitis, as seen here (lupus). Vasculitis-caused retinal infarction is shown by the white patches.
How do we Treat Vasculitis?
- Many types of paediatric vasculitis are effectively treated at Boston Children’s Hospital. Our rheumatologists, who have the greatest experience detecting and treating vasculitis, are part of one of the biggest paediatric rheumatology departments in the United States, seeing over 4,000 outpatients and over 1,000 inpatients each year.
- The Samara Jan Turkel Clinical Center for Pediatric Autoimmune Disease brings together paediatric rheumatologists and consulting experts from throughout the hospital to provide children with vasculitis with comprehensive, coordinated care.
- We’ve formed innovative cross-departmental collaborations, such as the Dermatology-Rheumatology Center, which brings together rheumatologists and dermatologists to treat children with skin-related vasculitis. Another example is the Multiple Manifestations of Autoimmune Disease Clinic, where rheumatologists and immunologists collaborate to assist children with a variety of autoimmune disorders, including vasculitis, for whom there is no single diagnosis.