Vasculitis affecting small blood vessels: ANCA-associated and Non-ANCA
Vessels such as arterioles, venules and capillaries, which are smaller than the size of arteries, are classified as small vessels. Vasculitides of these vessels may be broadly divided into anti-neutrophil cytoplasmic antibodies (ANCA)-associated and non-ANCA vasculitis. ANCA-associated small vessel vasculitis is the more common of the two aforementioned categories of small vessel vasculitides and includes Churg-Strauss syndrome, Wegner’s granulomatosis and drug-induced small vessel inflammation. Non-ANCA small vessel vasculitides include those caused by paraneoplastic syndromes, immune complex deposition and inflammatory bowel disease.
ANCAs are mainly IgG specific
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The typical patient presents with glomerulonephritis and pulmonary capillaritis, which are inflammatory pathologies of the small vessels of the kidneys and lungs, respectively. Due to its ability to cause the aforementioned pathologies, MPA acts as a predisposing factor for the development of pulmonary-renal-syndrome. This syndrome includes Goodpasture Syndrome (a rare autoimmune disorder that allows for the destruction of the basement membrane in the lungs and kidneys by antibodies), Systemic Lupus Erythematosus (an autoimmune mediated disorder affecting the kidneys, skin, joints and other healthy tissue/organs) and Wegner’s Granulomatosis, which is discussed in the following …show more content…
Involvement of the latter system manifests as inflammation of the sinuses and middle ear, which may lead to hearing loss.
glomerulonephritis and pulmonary capillaritis
Figure 23 illustrates the differential diagnosis of ANCA and non–ANCA-associated vasculitis. About 10 percent of patients with microscopic polyangiitis (the most common type of ANCA–associated small vessel vasculitis) and Wegener's granulomatosis have negative assays for ANCA; however, this finding does not completely rule out these diseases and ANCA titers do not always correlate with disease activity.3 On the other hand, a positive ANCA assay result is not solely diagnostic of ANCA–associated vasculitis.
ANCA associated vasculitides of small blood vessels
Non-ANCA associated vasculitides of small blood
Gas in 1968 described Acute Posterior Multifocal Placoid Pigment Epitheliopathy (APMPPE) as being a condition that caused acute and rapid loss of central vision due to multiple pale lesions at the level of Retinal pigment epithelium (RPE) in the posterior pole. (1) It is a self-limiting condition which recovers spontaneously over a three weeks period leaving residual pigment epithelial alterations.(2) The typical features in acute phase include cream colored placoid lesions at the level of RPE, early hypofluorescence and late hyperfluorescence of the lesions on Fundus fluorescein angiography (FFA).(2) It is usually followed by a viral flu like prodrome in 1/3 of the patients. Neurological manifestations of APMPPE include Headaches being commonest while others are paraesthesias, vertigo, psychosis and more severe complications, Cerebrospinal fluid pleocytosis stroke and cerebral vasculitis.(3, 4) Association of APMPPE with systemic Vasculitis in the patients with positive perinuclear antineutrophil cytoplasmic antibody has been reported.(5, 6) Other systemic inflammatory diseases suggesting an underlying immune mediated or an inflammatory mechanism include erythema nodosum,(7-9) juvenile rheumatoid arthritis,(10) thyroiditis,(11, 12) nephritis,(5, 6, 13) ulcerative colitis(14) and Adenoviral infections(15) . Many granulomatous diseases have documented associations with APMPPE, including Wegener’s granulomatosis,(16-18) Pulmonary tuberculosis(19) and Sarcoidosis.
that cause dark red blotches on the skin, usually on the face). Laboratory tests are another consideration of diagnosis. Tests consist of anti-nuclear anti-body (ANA) counts and anti-topoisomerase (an enzyme that reduces super-coiling in DNA by breaking and rejoining one or both strands of DNA). High ANA’s and low anti-topoisomerases are found in patients with Raynaud syndrome. (Desai, 2003) “Patients with circulating autoantibodies, antinuclear antibodies, and anti-Scl 70 antibodies are at (an) increased risk of developing a connective tissue disease. Systemic sclerosis is the connective tissue disease most frequently associated with Raynaud’s phenomenon.” (Bowling, 2003) This syndrome is described as primary Raynaud phenomenon (PRP) if is not associated with another disorder and as secondary Raynaud phenomenon (SRP) if it occurs in association with another disorder.
Mcgrogan, A., Franssen, C. F. and De Vries, C. S. 2011. The incidence of primary glomerulonephritis worldwide: a systematic review of the literature. Nephrology Dialysis Transplantation, 26 (2), pp. 414-430.
In 1931 the first case of Wegener’s Disease was discovered by Heinz Klinger, a German medical student (although it was not known as Wegener’s Disease at that time). It was not until several years later in 1936, that three more cases were discovered. A German pathologist, Friedrich Wegener described and found this disorder as a distinct form of Vasculitis, a rare blood vessel inflammation that since the 1950’s has been called Wegener’s Disease Granulomatosis. (http://www.hopkinsvasculitis.org/types-vasculitis/wegeners-granulomatosis/2016)
Rheumatoid arthritis is a chronic inflammatory and an autoimmune disease that occurs when the immune system mistakenly attacks the body’s tissue (Rheumatoid arthritis, 2017). This disease affects the entire body, which is called a systemic (means entire body) disease. Arthritis is derived from the word part arthr-, which means “joint,” and -itis, which means “inflammation,” so altogether it means “inflammation of the joints.” It creates inflammation that causes the tissue that lines the inside of joints (synovium) to thicken. About 1.5 million people in the U.S. are affected. It affects all races, but it affects three times as many women than men (What is Rheumatoid Arthritis, n.d.). Overtime, rheumatoid arthritis causes painful swelling that can potentially result in bone erosion or joint deformity, which leads up to physical disabilities. RA can affect more than just your joints, but can spread to body systems, skin, eyes, lungs, heart, blood vessels, e.t.c (Rheumatoid arthritis, 2017).
Lupus is a chronic inflammatory disease that occurs when your body’s immune system attacks healthy tissue and organs. This inflammation can damage many different bodily systems. Lupus commonly affects the joints, skin, kidneys, lungs, brain and heart. Many people with lupus have found relief for some of their chronic pain through practicing a few different styles of yoga.
When someone has lupus they have a different outlook on life. For patients with this disease, even the easiest task can be painful to the body or joints. Lupus is not contagious. “Lupus is a disease of the immune system. The immune system protects the body from inflection”(http://lupus.webmd.com). With lupus, the immune system attacks the tissue in many parts of the body. Lupus affects women more than men(www.womenshealth.gov). There are many different types of lupus, and each symptom affect different parts of the body. With treatment you can obtain and fulfill a healthy lifestyle.
Polio is a viral disease. It cripples thousands of people and infects even more every year. Even though millions are inoculated, and the polio disease has been successfully purged from hundreds of countries still thousands of people and developing countries are infected and still people are dying. According to the World Health Organization (WHO) polio affects the Central Nervous System, or CNS; by infesting the intestines and transmitting it into the nerves thought the blood vessels. There the virus spreads through the nerve cells to the brain stem or other motor units, while forever damaging the nerves.
Polyarteritis Nodosa is a vasculitis disease that affect medium muscular arteries. Vasculitis is a disease that attacks the blood vessels that make arteries become inflamed. Polyarteritis Nodosa is a rare disease that affect adults, mainly middle ages males. The cause of the disease is unknown but has been related to hepatitis B or C. Organs that are mainly affect is the heart, kidneys and skin. Signs and symptoms are general such as fever, fatigue, loss of appetite and weight loss (Gota). Diagnosing polyarthritis nodosa is difficult, therefore knowing your family and your own medical history will help with diagnosing. Doctors will be able to test with blood work, CT, MRI scans and biopsy. (“Vasculitis Foundation-Polyarteritis Nodosa.”) Once diagnosed the treatment has to happen
Rheumatoid Arthritis is when the joints are chronically inflamed, which happens because it is an autoimmune disease which means that the immune system attacks the body tissues. Although Rheumatoid Arthritis mainly affects the joints, it can also affect other organs.
This leads to the inflammation and potential damaging of the kidneys and eventual kidney disease unless treated. HSP causes an extreme and severe form of IgAN. There are two forms of IgA, IgA1 and IgA2. IgA1 is the only form involved with HSP. “IgA with diminished hinge-region glycosylation are prone to aggregate into macromolecular complexes. These complexes activate the alternative pathway of complement, and then deposit into the renal mesangium” (Saulsbury). The bodies immune response gets triggered by an upper respiratory illness, and produces IgA antibodies. If these IgA antibodies have hinge regions that are not fully developed correctly then they go haywire; producing more and more and clumping together to form complexes that deposit themselves into the kidneys. These malfunctioning IgA1 antibodies also deposit themselves into blood vessel walls, causing them to rupture and blood to pool at the surface of the skin, the cause of the characteristic purple purpura rash (Saulsbury). Individuals who suffer from HSP do not always produce these malfunctioning IgA antibodies, and many researchers are puzzled by and still researching the “come and go” effect of IgAN affliction in correspondence to
This would be a long-term effect, although falling into the category of disability could depend on the seriousness of the deafness, and also whether it worsens in seriousness.
One of the most common mysteries in the world is the development of autoimmune diseases. An autoimmune disease is when the immune system, which usually keeps your body healthy thinks that your healthy cells are antigens and attacks them. This is irony right? It is against properties of evolution for an immune system to attack itself causing sickness and possibly death if untreated. There are about 80 different types of autoimmune diseases, which usually have periods of little to no symptoms and worsening symptoms. What particularly creates confusion in the world is the autoimmune disease, inflammatory bowel disease, which affects almost about five million people worldwide.
Kawasaki disease was discovered by a Japanese pediatrician named Tomisaku Kawasaki. This disease is a “multisystem vasculitis that results in localized coronary artery elastin breakdown and aneurysm formation,” (Lau). This simply means it is a condition that causes inflammation of blood vessel walls in the body. Kawasaki disease is also referred to as “mucocutaneous lymph node syndrome,” by Black’s Medical Dictionary, (Marcovitch). It is most prevalent in infants and toddlers, “mainly in children under five,” (Marcovitch).
The External or Outer Ear - comprises of the auricle or pinna which is the fleshy part of the outer ear. It is cup-shaped and collects and amplifies sound waves which then passes along the ear canal to the ear drum or tympanic membrane. The rim of the auricle is called the helix and the inferior portion is called the lobule. The external auditory canal is a carved tube and contains a few hair and ceruminous glands which are specialized sebaceous or oil glands. These secrete ear wax or cerumen. Both the hairs and the cerumen help prevent dust and foreign objects from entering the ear. A number of people produce large amounts of cerumen, and this sometimes cause the build up to be impacted and can bri...