In giant cell arteritis, the large arteries of the upper region of the body are involved. The patient usually complains of severe, unilateral (rarely bilateral) temporal or occipital headache [4]. The sclap may be tender and there may be jaw claudication and sore throat. Involvement of the ophthalmic arteries may lead to loss of vision.

Takayasu arteritis is a chronic granulomatous inflammation of elastic arteries (such as the aorta and its branches). Early inflammatory symptoms of the disease include fever, arthralgia, myalgia and pain over the involved area. In the late stage of the disease, there is occlusion of the arteries and symptoms such as syncope, dizziness, amaurosis fugax, stroke, angina, pulmonary hypertension and claudication are seen. Peripheral pulses are diminished and blood pressure asymmetry is also observed.

Polyarteritis nodosa affects medium-sized arteries of almost all regions of the bpdy. The patient has fever, malaise, weight loss and myalgia. There are many other symptoms related to the organs involved. These include neurological (mononeuritis multiplex), abdominal (abdominal pain and gastrointestinal hemorrhage), respiratory (chest pain, pulmonary infiltrates and consolidation), renal (hematuria, proteinuria, hypertension and acute or chronic renal failure), cardiac (coronary arteritis and pericarditis) and dermatological (palpable purpurae, subcutaneous hemorrhages and gangrene).

Kawasaki disease also involves medium-sized vessels and is a systemic disease. The various clinical features of this disease include fever lasting over 5 days, bilateral congestion of the conjunctiva, erythema of the lips, buccal mucosa and the tongue, cervical lymphadenopathy, polymorphous exanthema, coronary dilation and erythema of the palms and soles [5].

Wegner’s granulomatosis usually presents with involvement of the upper and lower respiratory tract. Upper respiratory tract lesions include recurrent rhinitis, sinusitis, epistaxis and serous otitis media. Lower respiratory tract lesions manifest with cough, hemoptysis, chest pain, dyspnea and caveating lung disease [6].

Churg-Strauss granulomatosis also involves the respiratory tract (with manifestations such as allergic rhinitis, nasal polyposis and asthma) along with cutaneous lesions (nodular and popular rashes), nervous involvement (with mononeuritis multiplex) and cardiac disorders (such as myocardial infarction, cardiomyopathy and pericarditis) [7].

Henoch-Schönlein purpurae manifests with the formation of purpurae on the buttocks and lower legs. Angioedema, intussesception, rectal bleeding and nephritis are also seen in as many as 40-50% of the cases.

The following investigations are helpful in establishing the diagnosis of vasculitis.

• Erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) level: These parameters are raised in most types of vasculitis.
• Temporal artery biopsy: It is a definitive diagnostic test for giant cell arteritis.
• Aortography: Aortography is used to demonstrate stenosis in the aortic branches in the diagnosis of Kawasaki disease.
• Angiography: Angiography of systemic vessels can help in diagnosing polyarteritis nodosa [8].
• ANCA (Antineutrophilic cytoplasmic antibodies): These antibodies are present in Wegner’s granulomatosis, Churg-Strauss granulomatosis, microscopic polyangitis and sometimes in Kawasaki disease [9] [10].
• Biopsy of the affected organ: This is helpful in diagnosing polyarteritis nodosa if enough clinical evidence is not available.

In most types of vasculitis, corticosteroids are the first line drugs used to control the symptoms and limit the progress of the disease. In some types of vasculitis (such as giant cell arteritis and Churg-Strauss granulomatosis) corticosteroids alone are sufficient and cause a dramatic reduction in the symptoms.

In other types of vasculitis (including Takayasu arteritis, polyarteritis nodosa and small vessel vasculitis), corticosteroids may not be sufficient alone and are therefore combined with immunosuppressive agents such as azathioprine and cyclophosphamide.

If hepatitis B is present in association with polyarteritis nodosa, hepatitis B antiviral therapy should be given.

Corticosteroids are not given in Kawasaki disease since they may worsen the dilation of the coronary arteries. In this disease, aspirin and high dose intravenous immunoglobulins are given instead.

In Takayasu arteritis, the occluded or aneurysmal sections of the coronary vessels may make surgical interventions necessary.

The prognosis of the patients with vasculitis is not very good as there is no definite cure. Damage of the systemic organs may also increase the morbidity. Lifelong steroid therapy may be required and this predisposes the individual to many other systemic side effects.

All the types of vasculitis are usually of idiopathic origin. No specific causal factors have been identified. Polyarteritis nodosa has been linked to infection with hepatitis B virus.

There is a great variation in the epidemiology of different types of vasculitis. Giant cell arteritis usually occurs in the patients greater than 50 years of age. Takayasu arteritis is more common people less than 40 years of age. Furthermore, it is much more common in females, the male to female ratio being 1:8.

Polyarteritis nodosa affects all age groups; however, the peak incidence is in the fourth and fifth decade of life. Its incidence is twice as high in males as compared to females. Kawasaki disease is most common in Japan and mainly affects children under the age of 5 years. Henoch-Schnonlein purpura is also more common in children and involves boys more frequently as compared to girls [3].

Vasculitis is only the chronic inflammation of the arterial blood vessels. The type of inflammation may differ in various types of vasculitis. The signs and symptoms of these diseases also depend upon the location and size of the arteries involved.

Most of the types of vasculitis are idiopathic in origin and can not be prevented. The risk for the development of polyarteritis nodosa can be reduced by adopting proper preventive measures against hepatitis B infection.

Vasculitis is the inflammation of the wall of the arterial blood vessels. It is not a single disease but a group of diseases involving vessels of different sizes and regions of the body.

Vasculitis is classified into large vessel vasculitis, medium vessel vasculitis and small vessel vasculitis [1]. Large vessel vasculitis has two subtypes; namely giant cell arteritis and Takayasu arteritis. Medium vessel arteritis also consists of two main types: polyarteritis nodosa and Kawasaki disease. Small vessel vasculitis has numerous types including microscopic polyangitis, Wegner’s granulomatosis, Henoch-Schönlein purpura and Churg-Strauss syndrome [2].

Vasculitis means inflammation of the vessels of the body. There are several types of vasculitis which differ in the region and size of the vessel involved. There is no definite cure for the disease and steroids are usually used to control the symptoms of the disease.

1. Jennette JC, Falk RJ, Andrassy K, et al. Nomenclature of systemic vasculitides. Proposal of an international consensus conference. Arthritis and rheumatism. Feb 1994;37(2):187-192.
2. Jennette JC, Falk RJ. Small-vessel vasculitis. The New England journal of medicine. Nov 20 1997;337(21):1512-1523.
   Gardner-Medwin JM, Dolezalova P, Cummins C, Southwood TR.
3. Incidence of Henoch-Schonlein purpura, Kawasaki disease, and rare vasculitides in children of different ethnic origins. Lancet. Oct 19 2002;360(9341):1197-1202.
4. Wasowska-Sznajderman T, Januszewicz W, Chodakowska J, et al. [Giant cell arteritis--presentation of 3 cases]. Polskie Archiwum Medycyny Wewnetrznej. Apr 1994;91(4):298-301.
5. Nebreda Perez V, Sierra Perez E, Gonzalez Alvarez MI, Abella Jimeno A. [Kawasaki disease. Presentation of 8 cases]. Anales espanoles de pediatria. Jun 1987;26(6):444-448.
6. Cabral DA, Uribe AG, Benseler S, et al. Classification, presentation, and initial treatment of Wegener's granulomatosis in childhood. Arthritis and rheumatism. Nov 2009;60(11):3413-3424.
7. Morita H, Kitano Y. Allergic granulomatosis and angiitis of Churg-Strauss syndrome. International journal of dermatology. Oct 1996;35(10):726-728.
8. Schluter A, Hirsch W, Jassoy A, et al. [MR angiography in diagnosis of vasculitis and benign angiopathies of the central nervous system]. RoFo : Fortschritte auf dem Gebiete der Rontgenstrahlen und der Nuklearmedizin. Jun 2001;173(6):522-527.
9. de Groot K, Schnabel A, Gross WL. [ANCA-associated vasculitis (Wegener's granulomatosis, Churg-Strauss syndrome, microscopic polyangiitis). 2. Diagnostic procedure]. Zeitschrift fur Rheumatologie. Sep-Oct 1995;54(5):291-302.
10. Falk RJ, Jennette JC. ANCA small-vessel vasculitis. Journal of the American Society of Nephrology : JASN. Feb 1997;8(2):314-322.