Vasculitis Health Dictionary

A group of diseases affecting CONNECTIVE TISSUE. The term is really outdated since there is no evidence that collagen is primarily involved. Fibrinoid NECROSIS and VASCULITIS are two ‘characteristics’, and autoimmunity reaction may occur in the connective tissue. The latter affects blood vessels and causes secondary damage in the connective tissue. Such conditions are sometimes described as collagen vascular diseases, examples being RHEUMATOID ARTHRITIS, SYSTEMIC LUPUS ERYTHEMATOSUS (SLE), and SCLERODERMA.... collagen diseases

‘Rheumatism’ is the colloquial term for nonspeci?c musculoskeletal symptoms arising in the joints, ligaments, tendons and muscles. ‘Arthritis’ describes a pathological musculoskeletal disorder. Most common are sprains of ligaments, strains of tendons and muscles,

BURSITIS, TENDINITIS and non-speci?c back pain (see BACKACHE).

Osteoarthritis (OA) rarely starts before 40, but by the age of 80 affects 80 per cent of the population. There are structural and functional changes in the articular cartilage, as well as changes in the collagenous matrix of tendons and ligaments. OA is not purely ‘wear and tear’; various sub-groups have a genetic component. Early OA may be precipitated by localised alteration in anatomy, such as a fracture or infection of a joint. Reactive new bone growth typically occurs, causing sclerosis (hardening) beneath the joint, and osteophytes – outgrowths of bone – are characteristic at the margins of the joint. The most common sites are the ?rst metatarsal (great toe), spinal facet joints, the knee, the base of the thumb and the terminal ?nger joints (Heberden’s nodes).

OA has a slow but variable course, with periods of pain and low-grade in?ammation. Acute in?ammation, common in the knee, may result from release of pyrophosphate crystals, causing pseudo-gout.

Urate gout results from crystallisation of URIC ACID in joints, against a background of hyperuricaemia. This high concentration of uric acid in the blood may result from genetic and environmental factors, such as excess dietary purines, alcohol or diuretic drugs.

In?ammatory arthritis is less common than OA, but potentially much more serious. Several types exist, including: SPONDYLARTHRITIS This affects younger men, chie?y involving spinal and leg joints. This may lead to in?ammation and eventual ossi?cation of the enthesis – that is, where the ligaments and tendons are inserted into the bone around joints. This may be associated with disorders in other parts of the body: skin in?ammation (PSORIASIS), bowel and genito-urinary in?ammation, sometimes resulting in infection of the organs (such as dysentery). The syndromes most clearly delineated are ankylosing spondylitis (see SPINE AND SPINAL CORD, DISEASES AND INJURIES OF), psoriatic or colitic spondylitis, and REITER’S SYNDROME. The diagnosis is made clinically and radiologically; no association has been found with autoantibodies (see AUTOANTIBODY). A particularly clear gene locus, HLA B27, has been identi?ed in ankylosing spondylitis. Psoriasis can be associated with a characteristic peripheral arthritis.

Systemic autoimmune rheumatic diseases (see AUTOIMMUNE DISORDERS). RHEUMATOID ARTHRITIS (RA) – see also main entry. The most common of these diseases. Acute in?ammation causes lymphoid synovitis, leading to erosion of the cartilage, associated joints and soft tissues. Fibrosis follows, causing deformity. Autoantibodies are common, particularly Rheumatoid Factor. A common complication of RA is Sjögren’s syndrome, when in?ammation of the mucosal glands may result in a dry mouth and eyes. SYSTEMIC LUPUS ERYTHEMATOSUS (SLE) and various overlap syndromes occur, such as systemic sclerosis and dermatomyositis. Autoantibodies against nuclear proteins such as DNA lead to deposits of immune complexes and VASCULITIS in various tissues, such as kidney, brain, skin and lungs. This may lead to various symptoms, and sometimes even to organ failure.

Infective arthritis includes: SEPTIC ARTHRITIS An uncommon but potentially fatal disease if not diagnosed and treated early with approriate antibiotics. Common causes are TUBERCLE bacilli and staphylococci (see STAPHYLOCOCCUS). Particularly at risk are the elderly and the immunologically vulnerable, such as those under treatment for cancer, or on CORTICOSTEROIDS or IMMUNOSUPPRESSANT drugs. RHEUMATIC FEVER Now rare in western countries. Resulting from an immunological reaction to a streptococcal infection, it is characterised by migratory arthritis, rash and cardiac involvement.

Other infections which may be associated with arthritis include rubella (German measles), parvovirus and LYME DISEASE.

Treatment Septic arthritis is the only type that can be cured using antibiotics, while the principles of treatment for the others are similar: to reduce risk factors (such as hyperuricaemia); to suppress in?ammation; to improve function with physiotherapy; and, in the event of joint failure, to perform surgical arthroplasty. NON-STEROIDAL ANTI-INFLAMMATORY DRUGS (NSAIDS) include aspirin, paracetamol and many recently developed ones, such as the proprionic acid derivatives IBUPROFEN and naproxen, along with other drugs that have similar properties such as PIROXICAM. They all carry a risk of toxicity, such as renal dysfunction, or gastrointestinal irritation with haemorrhage. Stronger suppression of in?ammation requires corticosteroids and CYTOTOXIC drugs such as azathioprine or cyclophosphamide. Recent research promises more speci?c and less toxic anti-in?ammatory drugs, such as the monoclonal antibodies like in?iximab. An important treatment for some osteoarthritic joints is surgical replacement of the joints.... joints, diseases of

A metabolite of PENICILLIN which is one of the CHELATING AGENTS. It is sometimes used in RHEUMATOID ARTHRITIS that has not responded to the ?rst-line remedies and it is particularly useful when the disease is complicated by VASCULITIS. Penicillamine is also used as an antidote to poisoning by heavy metals, particularly copper and lead, as it is able to bind these metals and so remove their toxic effects. Because of its ability to bind copper it is also used in WILSON’S DISEASE where there is a de?ciency in the copper-binding protein so that copper is able to become deposited in the brain and liver, damaging these tissues.... penicillamine

This is a disorder in which large ulcerating lesions appear suddenly and dramatically in the skin. It is the result of underlying VASCULITIS. It is usually the result of in?ammatory bowel disease such as ULCERATIVE COLITIS or CROHN’S DISEASE but can be associated with RHEUMATOID ARTHRITIS.... pyoderma gangrenosum

A net-like, purple or blue mottling of the skin, usually on the lower legs, caused by the enlargement of blood vessels beneath the skin.

It is more common in people with vasculitis and those who suffer from excessive sensitivity to cold.

The condition is harmless, and tends to be worse in cold weather.... livedo reticularis

Veins are the blood vessels that convey blood back from the tissues towards the heart. Two common conditions that affect them are THROMBOSIS and varicosities (see below).

Varicose veins are dilated tortuous veins occurring in about 15 per cent of adults – women more than men. They most commonly occur in the legs but may also occur in the anal canal (HAEMORRHOIDS) and in the oesophagus (due to liver disease).

Normally blood ?ows from the subcutaneous tissues to the super?cial veins which drain via perforating veins into the deep veins of the leg. This ?ow, back towards the heart, is aided by valves within the veins. When these valves fail, increased pressure is exerted on the blood vessels leading to dilatations known as varicose veins.

Treatment is needed to prevent complications such as ulceration and bleeding, or for

cosmetic purposes. Treatment alternatives include injection with sclerosing agents to obliterate the lumen of the veins (sclerotherapy), or surgery; in the elderly or un?t, an elastic stocking may su?ce. One operation is the Trendelenburg operation in which the saphenous vein is disconnected from the femoral vein and individual varicose veins are avulsed. (See also VASCULITIS.)

Thrombosis Thrombosis occurs when blood, which is normally a liquid, clots within the vein to form a semisolid thrombus (clot). This occurs through a combination of reduced blood ?ow and hypercoagulability (a reduced threshold for clotting). The most common site for this to occur is in the deep veins of the leg, where it is known as a deep-vein thrombosis (DVT).

Predisposing factors include immobility (leading to reduced blood ?ow), such as during long journeys (e.g. plane ?ights) where there is little opportunity to stretch one’s legs; surgery (leading to temporary post-operative immobility and hypercoagulability of blood); oestrogen administration (low-dose oestrogen oral contraceptives carry a very low relative risk); and several medical illnesses such as heart failure, stroke and malignancy.

Deep-vein thrombosis presents as a tender, warm, red swelling of the calf. Diagnosis may be con?rmed by venogram (an X-ray taken following injection of contrast medium into the foot veins) or by ultrasound scanning looking for ?ow within the veins.

Prevention is important. This is why patients are mobilised and/or given leg exercises very soon after an operation, even major surgery. People should avoid sitting for long periods, particularly if the edge of the seat is hard, thus impeding venous return from the legs. Car drivers should stop regularly on a long journey and walk around; airline travellers should, where possible, walk round the aisle(s) and also exercise and massage their leg muscles, as well as drinking ample non-alcoholic ?uids.

Diagnosis and treatment are important because there is a risk that the clotted blood within the vein becomes dislodged and travels up the venous system to become lodged in the pulmonary arteries. This is known as PULMONARY EMBOLISM.

Treatment is directed at thinning the blood with ANTICOAGULANTS, initially with heparin and subsequently with WARFARIN for a period of time while the clot resolves.

Blocked super?cial veins are described as super?cial thrombophlebitis, which produces in?ammation over the vein. It responds to antiin?ammatory analgesics. Occasionally heparin and ANTIBIOTICS are required to treat associated thrombosis and infection.... veins, diseases of

A skin condition, also known as nettle rash or hives, that is characterized by the development of itchy weals, usually on the limbs and trunk. Large weals may merge to form irregular, raised patches.

Urticaria is generally harmless and usually lasts only a few hours. Sometimes a persistent or recurrent form develops. Dermographism is a less common form

of urticaria in which weals form after the skin is stroked. Urticaria sometimes occurs with angioedema.

The cause of urticaria is often unknown. The most common known cause is an allergic reaction (see allergy), often to a particular food, food additive, or drug. Urticaria may also be caused by exposure to heat, cold, or sunlight. Less commonly, it may be associated with another disorder, such as vasculitis, systemic lupus erythematosus, or cancer.

Itching can be relieved by applying calamine lotion or by taking antihistamine drugs. More severe cases may require corticosteroid drugs. Identifying and avoiding known trigger factors can help prevent future reactions. A tendency to urticaria often disappears in time without treatment.... urticaria

A rare disorder in which granulomas (nodular collections of abnormal cells), associated with areas of chronic tissue inflammation due to vasculitis, develop in the nasal passages, lungs, and kidneys. It is thought that the condition is an autoimmune disorder (in which the body’s natural defences attack its own tissues). Principal symptoms include a bloody nasal discharge, coughing (which sometimes produces bloodstained sputum), breathing difficulty, chest pain, and blood in the urine. There may also be loss of appetite, weight loss, weakness, fatigue, and joint pains.

Treatment is with immunosuppressant drugs, such as cyclophosphamide or azathioprine, combined with corticosteroids to alleviate symptoms and attempt to bring about a remission.

With prompt treatment, most people recover completely within about a year, although kidney failure occasionally develops.

Without treatment, complications may occur, including perforation of the nasal septum, causing deformity of the nose; inflammation of the eyes; a rash, nodules, or ulcers on the skin; and damage to the heart muscle, which may be fatal.... wegener’s granulomatosis

n. see vasculitis.... angiitis

(eosinophilic granulomatosis with polyangiitis) a systemic autoimmune *vasculitis comprising severe asthma, allergic rhinitis, and sinusitis associated with an increased *eosinophil count in the peripheral blood and eosinophilic deposits in the small vessels of the lungs. It usually responds to oral corticosteroids. [J. Churg (1910–2005) and L. Strauss (1913–85), US pathologists]... churg–strauss syndrome

adj. not involving a joint. The term is commonly used to specify a fracture pattern or the position of a bone tumour. It is also used to describe nonarticular or systemic manifestations of severe rheumatoid arthritis; for example, inflammation of the eyes, lungs, and heart, skin nodules and vasculitis, and nerve damage (neuropathy). See also intra-articular; periarticular.... extra-articular

(glomerular nephritis, GN) n. inflammation of the glomeruli, although in practice the term is used for a number of glomerular conditions lacking microscopical signs of inflammation. For instance, *membranous nephropathy is often referred to as membranous glomerulonephritis. GN may be a primary disease, restricted in its clinical manifestations to the kidney, or part of a multisystem disorder, such as systemic *lupus erythematosus or *vasculitis. Its presentation may be acute, with a *nephritic or *nephrotic syndrome; subacute, with rapidly declining renal function over a period of days or weeks; or chronic, with signs of the disease picked up on routine medical examination. Abnormalities of urine analysis are to be expected, with blood, protein, and casts present in variable amounts. Arterial hypertension is a common associated finding. GN is classified according to the different patterns of histological injury seen on renal biopsy specimens; these are examined by light and electron microscopy and by immunofluorescent studies.... glomerulonephritis

any of various rare illnesses characterized by primary lung haemorrhage and rapidly progressive glomerulonephritis. They include *Goodpasture’s disease and, more commonly, the systemic vasculitides (see vasculitis).... goodpasture’s syndrome

generalized inflammation of the glomeruli of the kidneys resulting in a reduction in *glomerular filtration rate, with mild oedema and hypertension resulting from renal salt and water retention. Urine analysis shows the presence of proteinuria and microscopic haematuria with red cell casts. Common and usually self-limiting causes are *Berger’s nephropathy and poststreptococcal glomerulonephritis. Less common but more serious causes of the nephritic syndrome are the vasculitides (see vasculitis) and *Goodpasture’s disease, which, untreated, usually prove fatal.... nephritic syndrome

n. a *monoclonal antibody that acts against the protein CD20, located mainly on the surface of B lymphocytes; it is used in the treatment of many lymphomas and leukaemias, including non-Hodgkin’s lymphoma and chronic lymphocytic leukaemia. It is also licensed for the treatment of severe rheumatoid arthritis and *vasculitis. Side-effects include hypersensitivity reactions and immunosuppression.... rituximab

a reaction that sometimes occurs 7–12 days after injection of a quantity of foreign antigen and is characterized by the deposition of large immune complexes. Since the complexes are deposited in the arteries, kidneys, and joints, the symptoms are those of vasculitis, nephritis, and arthritis.... serum sickness

n. inflammation of the smallest blood vessels (see vasculitis).... telangiitis