Takayasu’s arteritis (TA) is a chronic inflammatory disease affecting large arteries, primarily the aorta and its main branches [4]. It most commonly occurs in young women in the Middle and East Asia.

Aetiology of the disease is unknown. Several aetiologic factors have been proposed, including streptococcal infection and hypersensitivity to tuberculin test. A potential role of circulating immune complexes and circulating antibodies to aortic wall components has also been mentioned. Immunogenetic studies have revealed associations with BW52 and DR4 haplotypes.

The clinical features typically include symptoms of vascular insufficiency of upper limbs, described as arm claudication or arm numbness; loss of pulse or low blood pressure in an arm; and bruits heard most often over the carotids and abdominal aorta, less frequently over the subclavian and femoral arteries. There is often a difference in blood pressure between the arms of more than 30mmHg. Postural nausea is a sign of occlusion of the carotid and vertebrobasilar arterial systems, which may cause a typical partial flexion of the cervical spine. Visual disorders include blurred vision, diplopia or transient unilateral amaurosis. A regular finding is hypertensive retinopathy. Microaneurysms, dilatation of veins and bleeding resemble diabetic retinopathy. Late sequelae include optic nerve atrophy, retinal detachment and vitreous haemorrhage. In the early stages arthralgia, sometimes lower limb synovitis or symmetrical polyarthritis can be observed. Myalgia is frequent and may be misleading. The inflammatory phase of the disease may last for months. Patients are treated for a “fever of unknown origin” for several years before symptoms of vascular insufficiency develop. Skin lesions have often the form of erythema nodosum and lower leg ulcers. Cardiac involvement consists in changes of coronary artery orifices or of their proximal sections. Heart failure is most frequently caused by this disorder or systemic hypertension; myocardial biopsy has proved also myocarditis. Involvement of large- and medium-sized pulmonary arteries is common and leads to pulmonary hypertension, often without a marked clinical manifestation.

Most patients exhibit high ESR and CRP, mild anaemia and leucocytosis, with a finding of elevated levels of all immunoglobulin classes, but no antibodies.

The key role is played by angiographic examination focused on all branches of the aorta. Additional arteriography depends on the respective clinical features; the currently most frequently used method is digital subtraction angiography. CT scans show aortic wall thickness and its treatment-induced changes. Early inflammatory phase can be well imaged by positron emission tomography (PET). Histologically, the granulomatous phase represents the period of active inflammation with changes in media and adventitia with a mixed cellular infiltrate of lymphocytes, plasma cells and histiocytes together with typical giant cells. In the late stage, the disease is represented by a mild fibrous intimal hyperplasia, medial degeneration and fibrosis of the adventitia.

TA diagnosis is based on typical distribution of changes in main branches of the aorta, primarily in the left subclavian artery, superior mesenteric artery and other branches of the abdominal aorta. About 85 % of patients exhibit only stenosis and the rest have combined changes.

In the first place it is necessary to exclude atherosclerosis – TA primarily affects young individuals without risk factors of this disease. Other diseases to be excluded are SLE, Behçet’s disease, aortitis in ankylosing spondylitis, rheumatic fever, Crohn’s disease and syphilitic aortitis. TA may also resemble GCA, congenital coarctation of the aorta, ergotism and thromboangiitis obliterans.

The prognosis depends on specific manifestations (Takayasu’s retinopathy, secondary hypertension, aortic insufficiency, aortic and arterial aneurysms).

Polyarteritis nodosa (PAN) is a disease of small- and medium-sized arteries involving all three arterial wall layers, leading to multiple aneurysms, thrombi and tissue infarction. PAN may be a manifestation or a complication of RA, SSc and other connective tissue disorders.

Aetiology is unknown. The pathogenesis is attributed to deposition of immune complexes in various tissues, probably also to hepatitis C (less frequently B) virus, HIV and cytomegalovirus and parvovirus. The role of ANCA antibodies and hypersensitive reaction has not been clarified, yet.

The severity of the disease may range from a limited condition up to a rapidly progressing form which is usually fatal [6]. Most patients present with general symptoms, such as fever, weakness, weight loss and sometimes rash, peripheral neuropathy and polyarthritis. Sometimes only a single organ is affected, e.g. the skin, peripheral nerves or kidneys. Cutaneous manifestations may include palpable purpura, ulceration, livedo reticularis and ischaemic changes in distal phalanges. Joint involvement includes arthralgia or arthritis. Asymmetrical episodic nondeforming polyarthritis affects the joints of lower extremities. Neurological symptoms may develop in the form of peripheral neuropathy with an abrupt onset including pain and paresthesia of the peripheral nerve, followed by loss of motor function. Gradual involvement of nerves may result in asymmetrical polyneuropathy. Renal insufficiency is manifested by proteinuria; in the early stages, there may occur haematuria. Involvement of renal arteries or glomeruli often results in hypertension. The disease-related GIT disorders are associated with abdominal pain, and their location correlates with the affected organ. In case of diffuse pain, mesenteric artery thrombosis should be considered.

Include elevated ESR, normochromic anaemia, thrombocytosis and lower serum albumin levels. Part of the patients is HCV or HBsAg positive. The sign of disease activity is decreased complement levels. In the active phase, circulating immune complexes (CICs) are usually elevated.

Diagnosis should be always verified by biopsy, if the affected organ is accessible or by arteriography which shows typical aneurysms.

It is important to distinguish between IgA vasculitis (IGAV) (formerly Henoch-Schönlein purpura) and anti-GBM nephritis. The renal biopsy finding may be identical to granulomatosis with polyangiitis (GPA) (formerly Wegener’s granulomatosis) and to eosinophilic granulomatosis with polyangiitis (EGPA) (formerly Churg-Strauss syndrome). A number of PAN clinical symptoms may occur also in other diseases. Unless aneurysms are clearly seen, a similar angiographic image can be found in GPA, vasculitis in SLE, thrombotic thrombocytopenic purpura, infective endocarditis and tumours metastasizing to peritoneum. A problem may be dissecting arterial aneurysms.

All patients receive corticosteroids, at the beginning preferably in the form of intravenous bolus of methylprednisolone. In case of rapidly progressing extensive visceral involvement, cyclophosphamide pulse therapy should be added. In case of contraindication of cyclophosphamide, azathioprine or methotrexate may be used instead. If these combinations are ineffective, plasmapheresis is used as an adjuvant therapy. In cases associated with hepatitis B, corticosteroids are administered for several days, in combination with antiviral medications. Local, primarily cutaneous, forms respond well to colchicine in combination with topical corticosteroids.

The prognosis depends on the scope of involvement of vital organs. Uncontrollable vasculitis (heart failure and stroke) or infectious complications during immunosuppressive treatment may have fatal consequences.

Kawasaki syndrome (KS) is a vasculitis affecting coronary arteries with formation of aneurysms and occlusions and a tendency to spontaneous remission. It is characterised by long-term fever, oral mucosal inflammation, skin disorders and cervical lymphadenopathy. It most often affects children younger than 5 years, with a slight predominance in boys.