Vasculitis
Churg Strauss Syndrome
Churg Strauss Syndrome
Cryoglobulinemia
Cryoglobulinemia
Relapsing Polychondritis 1
Relapsing Polychondritis
Rheumatoid Vasculitis 1
Rheumatoid Vasculitis

Vasculitis is swelling (inflammation) of walls of blood vessels. Blood vessels are of 3 types - arteries (that carry blood from heart to the periphery), veins (that carry blood from various organs back to the heart) and capillaries (small intervening vessels where exchange of gases and nutrients takes place). Blood vessels are spread all over our body and supply blood to every cell. Blood provides oxygen and various nutrients to these cells. It is obvious that blood vessels are equally important as their diseases lead to anomalous blood supply endangering the cells supplied by them.

An inflamed (thickened) blood vessel can narrow and close off or balloon out (aneurysm) and possibly rupture. Vasculitides (plural of vasculitis) are a group of 15 rare diseases which are autoimmune. Blood vessels can be large, medium or small sized. Any blood vessel or organ can be affected by vasculitis. Vasculitis can be localised or generalised (systemic). A particular type of vasculitis is usually restricted to vessels of a particular size. Most vasculitides affect skin and do not cause dysfunction of internal organs. Some forms are short-lasting and innocuous whereas most others are chronic and can be life-threatening. Relapses are common in many types and hence lifetime surveillance is advisable. Vasculitis can affect anyone though some forms are more common in certain groups of patients. Early diagnosis and expert management are essential for successful outcome in these cases.

Genetic Causes:
The exact cause of vasculitides is multifactorial (genes, gender and environment) and is as yet unknown. Secondary vasculitis is due to a known cause and appears in the course of other defined diseases. Treatment consists of control of main disease.

Some causes of secondary vasculitis are listed below:

  1. Infection - Viruses (Hepatitis B and C, HIV, Varicella zoster, etc), bacteria (TB, gonorrhea, streptococci, staphylococci, etc), fungi and others(e.g. syphilis)
  2. Cancer - Most malignancies (solid organ tumors, lymphoma and others) are associated with subsequent development of malignancy.
  3. Drugs - Vaccines and desensitization agents, drugs used for nasal allergies (monteleukast and others from this group), propyl thiouracil, hydralazine, heroin, cocaine, amphetamine, etc. These appear after prolonged exposure to the drug, are usually limited to skin and disappear on early withdrawal of offending agent.
  4. Inflammatory connective tissue diseases – Rheumatoid arthritis, Systemic lupus erythematosus, Sjögren’s syndrome, etc
Some diseases can mimic vasculitis (pseudovasculitis) and need expert advice for a proper diagnosis. Infections (e.g. infective endocarditis), cancer (metastasis or paraneoplastic), scurvy (severe Vitamin C deficiency), embolism (myxoma in heart, infection and cholesterol), thrombosis (Antiphospholipid antibody syndrome, calciphylaxis) and some other diseases may present as multiorgan illness, wrongly diagnosed as vasculitis.

Features:
Fever, fatigue, malaise (feeling unwell), loss of appetite and weight loss are general symptoms seen in most forms of vasculitis. Joint pains, non-deforming arthritis, muscle pains, numbness and weakness also occur in some of these conditions. Symptoms usually last for a long time and may disappear and reappear.

Specific features of some common vasculitides in adults are tabulated below:

Type Sex
Age
Feature
Takayasu’s Arteritis Females (9:1)
15-25 years
Cramping pain in arms or legs on use (claudication),low pulse volume, headache, visual disturbances
Giant Cell Arteritis Females (3:1)
More than 50 years
Headache, jaw claudication while chewing, blurred or double vision(may cause blindness) , scalp tenderness, weakness and pain of shoulder girdle and hips (associated polymyalgia rheumatica)
Polyarteritis Nodosa
(Can be associated with Hepatitis B)
Males (2:1)
40-60 years
Weight loss, high blood pressure, clustered nodules on skin, net-like(reticular) purplish discoloration of skin (livedo), weakness due to involvement of nerves (neuropathy), pain in testes(orchitis), gastrointestinal bleeding
Wegener’s Granulomatosis Males = Females
30-50 years
Sinusitis, crusting rhinitis (choked nose), ‘strawberry’ gums, chronic middle ear disease, blood in sputum, red cells and proteins in urine
Churg Strauss Syndrome Females (2:1)
40-60 years
Long standing asthma, history of allergy, weakness due to involvement of nerves, eosinophils on blood examination
Microscopic Polyangitis Males = Females
30-50 years
Red cells and proteins in urine, blood in sputum, purpura and other skin lesions, neuropathy
Henoch Schonlein Purpura Males = Females
5-20 years
Purpura (small purplish patches under the skin due to bleeding)especially on lower limbs, pain in abdomen, bloody diarrhea, blood in urine
Behcet’s Disease Males = Females
20-35 years
Ulcers in mouth and on genitals, skin nodules (acne-like) and ulcers, painful red eye (uveitis)may lead to photophobia and blindness, pathergy (hyper-reactivity to minor trauma such as pin prick)
Lecocytoclastic Vasculitis Males = Females
30-50 years
Purpura, skin rashes

Vasculitis requires proper assessment for appropriate planning of therapy. Various methods have been developed to assess activity and extent of vasculitis as well as damage caused by the disease. The Birmingham Vasculitis Activity Score (BVAS) is one such most commonly used measure. BVAS assesses disease activity with 48 questions on listed clinical features (ear, nose, throat, skin, lungs, heart and blood vessels, abdomen kidneys, brain and nerves), measurement of body temperature, weight loss and blood pressure as well as examination of urine (proteins and blood) and blood (creatinine). Presence of proteins in urine, kidney failure (creatinine > 1.6 mg/dl), involvement of heart, gastrointestinal tract or nervous system are factors of poor prognosis and require aggressive treatment with steroids and immunosuppressants.

Investigations:
Various tests are required for diagnosis of disease, assessment of organ involvement and monitoring drug therapy. Some of these are expensive but they must be undertaken for proper disease management.

  1. Blood Tests - Haemogram, kidney and liver function tests, sugar and lipids (cholesterol and others). Erythrocyte sedimentation rate and C-reactive protein indicate presence of inflammation. Anti-neutrophil cytoplasmic antibodies (ANCA) are specially tested, in suspected cases of Wegener’s Granulomatosis, Churg Strauss Syndrome and Microscopic Polyangitis.
  2. rine Tests - Routine examination (dip stick for home monitoring) can detect presence of red cells and protein. This indicates involvement of kidneys. A 24-hour urine examination may be ordered in such cases for further assessment.
  3. Imaging - X-ray, ultrasound, computed tomography (CT) and magnetic resonance imaging (MRI) are non-invasive techniques for assessment of blood vessels and various organs. Blood vessels can be further studied by angiography.
  4. Biopsy - Removing a piece of affected tissue is necessary in many cases so that the tissue can be examined under microscope. This surgical procedure is advisable in selected cases for confirmation of diagnosis. Biopsy can also be obtained at bronchoscopy i.e. visualization of airways from inside.

Treatment:

Wegeners GranulomatosisTreatment of vasculitis depends upon the particular type of vasculitis, its severity and associated risk factors. Some types are self-limiting and do not require treatment whereas others require medications under expert supervision. Glucocorticoids (steroids) effectively suppress inflammation and must be used in high doses to start with. The dose can be tapered after a few weeks. Intravenous pulse cyclophosphamide, an immunosuppressant, is a preferred drug in all serious organ-threatening vasculitides. This drug should be continued for 6-12 months and followed by other less toxic drugs such as azathioprin or methotrexate. Mycophenolate mofetil and rituximab have shown promising results in clinical studies. All these drugs require careful monitoring for adverse effects and are best managed by rheumatologists. Help from other super specialists such as cardiologists, nephrologists, pulmonologists and a general surgeon is required in some cases for better management. Regular follow-up is essential even after control of disease as relapses are frequent and must be identified and treated promptly.

Secondary vasculitis needs treatment of original disease along with standard therapy. This applies especially to virus-induced vasculitis (hepatitis B and C, HIV) wherein antiviral therapy is more important. Plasma exchange may be required in these cases. It is also necessary for patients to understand their condition well and learn to cope up with it. Healthy diet, regular exercise, weight control, avoidance of smoking and protection from exposure to cold are important in managing these diseases.


 

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