Vasculities, Polymyalgia Rheumatica, Giant Cell Arteritis (Temporal Arteritis)

1. Vasculities, Polymyalgia Rheumatica, Giant Cell Arteritis (Temporal Arteritis) Victor Politi, M.D. FACP Medical Director, SVCMC Physician Assistant Program

2. Vasculitis • “Vasculitis” is a general term for a group of diseases that involve inflammation in blood vessels. • Blood vessels of all sizes may be affected, from the largest vessel in the body (the aorta) to the smallest blood vessels in the skin (capillaries). • The size of blood vessel affected varies according to the specific type of vasculitis.

3. Overview • There are many different types of blood vessels in the body. • Any particular vessel is part of a large vascular "tree" that includes large and medium sized arteries and smaller and smallest arterial branches (arterioles).

4. Overview • These branches eventually reach all the tissues of the body, delivering oxygen and nutrients to a network of tiny vessels, called capillaries, that also remove wastes.

5. Overview • The capillaries drain into the venous system. • The smallest veins are venules; these connect like the tributaries of a river to form larger and larger veins.

6. Overview • Vasculitis can affect any of these different types of blood vessels. Inflammation can affect the lining of the vessels (endothelium) or the wall of an artery or vein. The damaged vessel does not function normally, and tissues that the vessel normally serves may then be affected.

7. Overview • The effects of vasculitis that result from damage to the blood vessel include decreased function due to decreased blood flow (ischemia), death of some or all of an organ due to absent blood flow (infarction), or bleeding into the skin or other part of the body due to rupture of the blood vessel wall.

8. Overview • Because vasculitis is a process that involves inflammation, it is usually accompanied by other features such as fever, or symptoms of involuntary weight loss and fatigue.

9. Overview • Fortunately, available treatments for vasculitis are helpful, especially in the acute phase. • Long term therapy presents challenges because of side effects of the medications typically used to treat the disorder.

10. WHAT CAUSES VASCULITIS? • In most cases, the cause is unknown. • It is likely that a combination of factors causes the inflammatory process to be set in motion.

11. WHAT CAUSES VASCULITIS? • Vasculitis can occur in conjunction with another illness, such as lupus erythematosus or rheumatoid arthritis.

12. WHAT CAUSES VASCULITIS? • Sometimes, it is precipitated by a reaction to a drug or other substance. (This is known as hypersensitivity vasculitis.)

13. WHAT CAUSES VASCULITIS? • In still other cases, it occurs in conjunction with a viral illness, such as hepatitis B or C, HIV, cytomegalovirus, Epstein-Barr virus, and Parvo B19 virus.

14. WHAT ARE THE SYMPTOMS? • Symptoms vary from patient to patient, and are dependent in part on the type of vasculitis.

15. Some common symptoms include: • Fatigue • Weakness • Fever • Joint pains • Abdominal pain • Kidney problems (bloody urine, dark urine) • Nerve problems (numbness, weakness, pain)

16. The diagnosis of vasculitis is often difficult, as the patient's symptoms may suggest many other illnesses. • Begin with a careful history and physical exam • You may be able to detect signs of organ problems suggestive of a vasculitic process.

17. Laboratory tests can help pinpoint the areas of the body affected. • Tests may include those that examine muscle, liver, or kidney function.

18. Other common tests that may provide useful information are additional blood tests, urinalysis, chest x-ray, and electrocardiogram. • Tests of lung function may be needed in some cases.

19. Patients with evidence of nerve or muscle involvement may undergo nerve conduction studies and an electromyogram (a test of muscle function).

20. Tissue biopsy is a critical component of the diagnostic process. • The tissue sample is taken from an area thought to be involved in the vasculitis. • In vasculitis affecting the larger vessels, an arteriogram may be useful. This test involves injecting dye into the arteries, which makes them visible on x-ray.

21. The diagnostic tests that are used vary widely depending on the type of vasculitis that is suspected.

22. TYPES OF VASCULITIS • There are many different types of vasculitis. • They are classified according to the type and location of the blood vessels that are generally involved.

23. Large vessel vasculitis • Large vessel vasculitis — The types of vasculitis that affect large arteries include Takayasu arteritis and giant cell (temporal) arteritis.

24. Large vessel vasculitis • Involvement of the main artery of the body, the aorta, can sometimes occur in association with other illnesses such as ankylosing spondylitis, rheumatoid arthritis, and relapsing polychondritis.

25. Large vessel vasculitis Takayasu arteritis • Takayasu arteritis primarily affects the main artery that receives blood from the heart (aorta) and its branches. • The inflammation may be localized to a portion of the aorta in the chest or abdomen and branches.

26. Large vessel vasculitis Takayasu arteritis • Only 1-3 cases are diagnosed in a year among 1 million people in North America. • The disease most often affects women during the years from age 10 to about 40. There is a 9:1 female predominance in this disease. • Although the disease has a worldwide distribution, it appears to occur more often in Asian women.

27. Large vessel vasculitis Takayasu arteritis • The involvement of large arteries may lead to symptoms such as pain and weakness with use of the arms or legs (claudication). • Other organs such as the intestines (abdominal pain after eating), heart (chest pain with exertion), or brain.

28. On the right is an example of an abnormal aortic arch in a patient with Takayasu's, with obvious dilation of the ascending aorta on the left side of the picture

29. Large vessel vasculitis Takayasu arteritis • The narrowing and irregularities that occur at several sites, and the “corkscrew” configuration of one vessel segment near the junction of the two arteries. • These changes, caused by inflammation in the blood vessel wall, sometimes cause complete blockage of the artery

30. Large vessel vasculitis Giant cell arteritis • Giant cell arteritis may also affect the aorta and its branches. • Frequent involvement of the arteries of the face and scalp, particularly those near the temples, accounts for the other common name for this disorder, temporal arteritis.

31. Large vessel vasculitis Giant cell arteritis • Giant cell arteritis is a disease that nearly always affects people older than 50 years of age. • Among a million people 50 or older, approximately 2000 may be affected at any one time.

32. Large vessel vasculitis Giant cell arteritis • In addition to general symptoms associated with inflammation, headache, tiring of jaw muscles during chewing, and visual changes or loss of vision are suggestive of this disease.

33. Large vessel vasculitis Giant cell arteritis • The diagnosis is suspected based upon symptoms, the finding of an elevated level of a blood test (erythrocyte sedimentation rate or C-reactive protein), and a confirmatory biopsy of an artery (usually one or both temporal arteries).

34. Medium sized vessel vasculitis • Some types of vasculitis appear to spare the aorta and affect medium sized arteries instead. • Polyarteritis is the term used for this disorder when it occurs in the absence of any other disease.

35. Medium sized vessel vasculitis • But similar involvement of blood vessels can occur when vasculitis develops in some people with rheumatoid arthritis, systemic lupus erythematosus, scleroderma (systemic sclerosis), hairy cell leukemia, and infectious forms of hepatitis (hepatitis B and C).

36. Medium sized vessel vasculitis Polyarteritis nodosa (PAN) • Polyarteritis nodosa is a term that refers to inflammation of medium to small arteries. • In the skin the inflammation results in thickened nodular (nodose) vessels that can be felt or sometimes seen.

37. Medium sized vessel vasculitis Polyarteritis nodosa (PAN) • Polyarteritis nodosa is sometimes termed “systemic necrotizing vasculitis”, but this term is non-specific as other forms of vasculitis also have systemic and necrotizing features.

38. Medium sized vessel vasculitis Polyarteritis nodosa • General symptoms due to inflammation, and specific symptoms due to bleeding, decreased blood flow (ischemia), or irreversible damage to organs due to the absence of blood flow (infarction), suggest the presence of polyarteritis.

39. Medium sized vessel vasculitis Polyarteritis nodosa • Most cases of PAN occur in the 4th or 5th decade, although it can occur at any age. • Men are twice as likely to be affected than women. • A minority of patients with PAN have an active hepatitis B infection. • In the rest of the cases, the cause(s) is presently unknown, and the disease is said to be “idiopathic” in nature.

40. Medium sized vessel vasculitis Polyarteritis nodosa • Damage to the nerves of the arms or legs, to the kidneys, the intestines, and the heart may occur. • The diagnosis is suspected when several organs of the body are being damaged at the same time.

41. Medium sized vessel vasculitis Polyarteritis nodosa • Peripheral neuropathies are very common (50 to 70%). This includes tingling, numbness and/or pain in the hands, arms, feet, and legs. • Central nervous system (CNS) lesions may occur 2 to 3 years after the onset of PAN and may lead to cognitive dysfunction, decreased alertness, seizures and neurologic deficits.

42. Medium sized vessel vasculitis Polyarteritis nodosa • Skin abnormalities are very common in PAN and may include purpura, livedo reticularis, ulcers, nodules or gangrene. • Skin involvement occurs most often on the legs and is very painful.

43. Medium sized vessel vasculitis Polyarteritis nodosa • Testing for the presence of antineutrophil cytoplasmic antibodies (ANCA) in the blood is helpful because these are frequently present in patients with polyarteritis or polyangiitis.

44. Medium sized vessel vasculitis Polyarteritis nodosa • Arteriography or biopsy of an involved blood vessel is often necessary to confirm the diagnosis.

45. Medium sized vessel vasculitis Polyarteritis nodosa • Treatment of PAN has improved dramatically in the past couple of decades. • Before the availability of effective therapy, untreated PAN was usually fatal within weeks to months. • Most deaths occurred as a result of kidney failure, heart or gastrointestinal complications.

46. Medium sized vessel vasculitis Polyarteritis nodosa • However, effective treatment is now available for PAN. After diagnosis, patients are treated with high doses of corticosteroids. • Other immunosuppressive drugs are also added for patients who are especially ill. In most cases of PAN now, if diagnosed early enough the disease can be controlled, and often cured.

47. Medium sized vessel vasculitis • Other diseases that can affect the medium sized arteries include Kawasaki disease and isolated central nervous system vasculitis

48. Small vessel vasculitis • Several different types of vasculitis can affect small vessels such as arterioles, capillaries, and small veins (venules). • These disorders may appear very similar based upon biopsy results, but are distinguished from one another by other features.

49. Small vessel vasculitisChurg-Strauss Vasculitis • Churg-Strauss vasculitis occurs almost exclusively in people who have asthma. • It is likely to cause lung damage. • ANCA testing is valuable. • Biopsy is useful to confirm the diagnosis.

50. Small vessel vasculitis Wegener's granulomatosis • Wegener's granulomatosis characteristically affects the nose and sinuses, the lungs, and the kidneys. • Almost all those with Wegener's granulomatosis have a positive ANCA blood test. • Biopsy of the lining of the nose, a sinus, part of a lung, or kidney may confirm the diagnosis.