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Vasculitis

Systemic Vasculitis

Vasculitis is defined as inflammation and damage of blood vessels. It may be a primary disease or a manifestation of another disease.


Presentation

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.

Fever
  • Clinical manifestations including fever, polyarthralgia, and lung hemorrhage were on remission with a decrease of dysregulated NETs.[ncbi.nlm.nih.gov]
  • Early inflammatory symptoms of the disease include fever, arthralgia, myalgia and pain over the involved area.[symptoma.com]
  • […] vasculitis itchy skin eruption fever loss of appetite fatigue Wegener's granulomatosis / Wegener's granulomatosis fever pulmonary congestion cough weight loss blood in the urine and nose anorexia inflammation of the nostrils and throat pain in muscles[healthcentral.com]
Fatigue
  • Aspects of disease relevant for AAV patients were: fatigue and lack of energy; visual abnormalities; neuropathy; renal impairment; arthritis, and sinusitis. Greater total score on MFI-20 (p[ncbi.nlm.nih.gov]
  • Incorporating a holistic approach utilizing natural treatments and lifestyle changes will build up the immune system while alleviating fatigue and other symptoms. Reader Interactions[earthclinic.com]
  • People may have general symptoms, such as fever or fatigue, followed by other symptoms depending on which organs are affected.[msdmanuals.com]
Rapidly Progressive Glomerulonephritis
  • Here, we describe a case of hydralazine-associated rapidly progressive glomerulonephritis and pulmonary haemorrhage.[ncbi.nlm.nih.gov]
  • They include alveolar hemorrhage, rapidly progressive glomerulonephritis, mesenteric ischemia, and vision loss in patients with giant cell arteritis.[web.archive.org]
  • Rapidly progressive glomerulonephritis This condition is a syndrome of the kidney that rapidly progresses into acute renal (kidney) failure if untreated. Complications Complications can vary depending on the type of vasculitis.[medicalnewstoday.com]
  • This is also called rapidly progressive glomerulonephritis (RPGN) because of the speed with which it can cause kidney failure.[edren.org]
  • Goodpasture syndrome clinically manifests with rapidly progressive glomerulonephritis and/or pulmonary hemorrhage (pulmonary renal syndrome). Thrombophlebitis refers to inflammation of a vein associated with the formation of a blood clot.[emedicine.medscape.com]
Chills
  • Abstract A previously healthy 77-year-old Japanese man presented with a 2-week history of daily fevers peaking at 38 C, chills, hearing loss, and almost 10 kg of unintentional weight loss over 2 months.[ncbi.nlm.nih.gov]
  • With any type of vasculitis, people generally don't feel well, and may experience: Fever Chills Fatigue Loss of appetite Weight loss Specific organ systems of the body affected by vasculitis may cause the following signs or symptoms: Brain Symptoms: Stroke[upmc.com]
  • These include: chills fatigue fever weight loss Other early symptoms are only detectable through a blood test. These include leukocytosis, which involves having a high number of white blood cells (WBCs) and anemia.[healthline.com]
Rectal Bleeding
  • 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.[symptoma.com]
Hypertension
  • Hydralazine is a direct-acting vasodilator, which has been used in treatment for hypertension (HTN) since the 1950s.[ncbi.nlm.nih.gov]
  • 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.[symptoma.com]
Vascular Disease
  • Página 249 - Omenn GS, Motulsky AG: A quantitative assessment of plasma homocysteine as a risk factor for vascular disease. ‎[books.google.es]
  • disease, PLEVA and erythema multiforme Fibrin thrombi: present in segmented, hyalinizing vasculitis Infarct of skin: associated with malignant atrophic papulosis (Dego's disease); intimal proliferation of deep-seated arteriole Purpura: non-blanching,[pathologyoutlines.com]
  • To learn more about how we use cookies, please see our cookies policy Close Intended for healthcare professionals Education Vasculitis Vasculitis Clinical Review ABC of arterial and vascular disease BMJ 2000 ; 320 doi: (Published 13 May 2000) Cite this[bmj.com]
  • disease (eg, rheumatoid arthritis, Sjögren's syndrome, SLE) Inflammatory bowel disease Neoplastic (eg, hairy cell leukaemia) Causes of larger-vessel vasculitis (uncommon) Wegener's vasculitis Churg-Strauss syndrome Polyarteritis nodosa Kawasaki disease[patient.info]
Left Ventricular Dysfunction
  • Echocardiography demonstrated severe left ventricular dysfunction with hypokinesis of the apical area. Moreover, coronary angiography revealed no noticeable stenotic or obstructive lesions. These findings indicate the onset of takotsubo syndrome.[ncbi.nlm.nih.gov]
Musculoskeletal Pain
  • Adverse effects of denosumab include musculoskeletal pain, hypercholesterolaemia, symptomatic hypocalcaemia, osteonecrosis of the jaw and cutaneous events such as angioedema, cellulitis and pustular dermatitis.[ncbi.nlm.nih.gov]
Blurred Vision
  • Eyes may be red, itchy and burning, increased sensitivity to light, blurred vision. Brain may experience headaches, changes in mental function, stroke-like symptoms such as muscle weakness and paralysis.[allergy.org.au]
  • Red, itchy, burning eyes; light sensitivity; blurred vision; rarely, blindness Brain. Headaches; problems thinking clearly; changes in mental function; stroke-like symptoms, such as muscle weakness and paralysis Nerves.[arthritis.org]
  • Symptoms of this condition can include headaches, scalp tenderness, jaw pain, blurred vision, double vision, and acute (sudden) vision loss. Giant cell arteritis is the most common form of vasculitis in adults older than 50.[nhlbi.nih.gov]
  • The most common symptoms are: headache shoulder pain pain in the hips pain in the jaw after chewing fever blurred vision Less common symptoms include: scalp tenderness cough throat pain tongue pain weight loss depression pain in the arms during exercise[medicalnewstoday.com]
Purpura
  • Pictured below is an example of Henoch-Schönlein purpura: cutaneous vasculitis manifested by palpable purpura and arthritis (note the right ankle swelling).[web.archive.org]
  • KEYWORDS: Henöch-Schonlein purpura; IgA vasculitis; adult; diffuse alveolar hemorrhage; plasmapheresis; pulmonary renal syndrome[ncbi.nlm.nih.gov]
  • 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.[symptoma.com]
  • Infection (15-20%) - eg, Henoch-Schönlein purpura, septic vasculitis, upper respiratory tract flares of granulomatosis with polyangiitis (Wegener's granulomatosis), polyarteritis nodosa (PAN).[patient.info]
Ulcer
  • ., palpable purpura, vasculitic urticaria, nodules, ulcers, mouth ulcers. Neurologic signs: e.g., headache, mononeuritis multiplex, focal neurological lesions (stroke). Arthritis or arthralgia, myalgia or myositis.[pmmonline.org]
  • Livedo reticularis, nodules, and deep ulcers are usually caused by vasculitis of deeper, medium or large vessels.[merckmanuals.com]
  • Inflammatory disease (15-20%) - eg, systemic lupus erythematosus (SLE), rheumatoid arthritis, Crohn's disease and ulcerative colitis.[patient.info]
  • We report two cases of CSVV associated with ulcerative colitis, review the literature, and discuss the diagnostic evaluation of children who present with CSVV and abdominal pain.[ncbi.nlm.nih.gov]
Palpable Purpura
  • Pictured below is an example of Henoch-Schönlein purpura: cutaneous vasculitis manifested by palpable purpura and arthritis (note the right ankle swelling).[web.archive.org]
  • Abstract Cutaneous leukocytoclastic vasculitis (LCV) is type of small vessel vasculitis that commonly presents as palpable purpura involving the lower extremities and buttocks.[ncbi.nlm.nih.gov]
  • This is an example of Henoch-Schönlein purpura: cutaneous vasculitis manifested by palpable purpura and arthritis (note the right ankle swelling).[hopkinsvasculitis.org]
Urticaria
  • Urticaria lesions may be huge and are usually larger than those of UV [ 2 ]. Chronic urticarial lesions are clinically more indurated than that of acute urticaria [ 4 ].[intechopen.com]
  • The most frequent manifestations at diagnosis were urticaria (100%), arthritis (88%), followed by biopsy-proven glomerulonephritis (19%), episcleritis/scleritis (19%) and recurrent abdominal pain (13%).[ncbi.nlm.nih.gov]
  • ., palpable purpura, vasculitic urticaria, nodules, ulcers, mouth ulcers. Neurologic signs: e.g., headache, mononeuritis multiplex, focal neurological lesions (stroke). Arthritis or arthralgia, myalgia or myositis.[pmmonline.org]
  • Patients may present with skin symptoms such as lesions, including palpable purpura, petechiae, urticaria, ulcers, livedo reticularis, and nodules.[merckmanuals.com]
  • […] necrosis and fragmented neutrophilic nuclei (leukocytoclasis); it presents as purpuric palpable lesions of lower legs; systemic cases are associated with Henoch-Schonlein pupura (fever, arthralgias, abdominal pain, hematura); also chronic idiopathic urticaria[pathologyoutlines.com]
Night Sweats
  • After completion of 4 cycles of ipilimumab for the treatment of metastatic melanoma, our patient complained of increasing fatigue, drenching night sweats, and chills. Imaging revealed diffuse adenopathy involving several lymph nodes.[ncbi.nlm.nih.gov]
  • Signs and symptoms include joint pain, loss of pulse, high blood pressure, night sweats, fever, general malaise, appetite loss, headaches and visual changes.[mayoclinic.org]
  • Anemia Fatigue Fever Muscle pain, numbness or tingling Night sweats Pain when chewing Severe headaches Stiffness Tenderness in the temple area Weight loss Vision loss Also might include: Red or purple rash on skin, usually on the legs Aching in joints[peripheralneuropathycenter.uchicago.edu]
  • Fever, night sweats, malaise, myalgia, and arthralgia are common in all types of vasculitis.[bmj.com]
  • Regardless of the size of the vessels involved, patients can present with symptoms and signs of systemic inflammation (eg, fever, night sweats, fatigue, anorexia, weight loss, arthralgias, arthritis).[web.archive.org]
Recurrent Rhinitis
  • 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.[symptoma.com]
Kidney Failure
  • Vasculitis affecting the kidneys can cause kidney failure. What tests diagnose vasculitis? Laboratory testing of blood or body fluids in a patient with active vasculitis generally indicates inflammation in the body.[medicinenet.com]
  • Finally, renal involvement can cause glomerulonephritis, which can cause both hematuria and proteinuria, with or without a rise in serum creatinine, and can progress to kidney failure.[osmosis.org]
  • This is also called rapidly progressive glomerulonephritis (RPGN) because of the speed with which it can cause kidney failure.[edren.org]
  • A patient with ANCA disease who gets a kidney transplant can expect a similar life span and kidney transplant survival as a transplant patient who has kidney failure from another disease.[unckidneycenter.org]

Workup

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.
Complete Blood Count Abnormal
  • The following blood and urine tests are usually done: Complete blood count : Abnormalities, such as lower counts of red blood cells (anemia), too many platelets, too many white blood cells, or a high proportion of certain kinds of white blood cells, can[msdmanuals.com]
C-Reactive Protein Increased
  • Laboratory findings include increased erythrocyte sedimentation rate, normochromic or slightly hypochromic anemia, increased gamma globulin and complement, elevated C-reactive protein, increased blood IL-6 concentration, and mildly abnormal liver function[worldallergy.org]
Creatinine Decreased
  • OUTCOMES: The serum creatinine decreased to 2.17 mg/dL a week later. MPO-ANCA turned negative when re-examined 3 weeks later. No relapse has been observed during follow-up for 6 months.[ncbi.nlm.nih.gov]
T Wave Inversion
  • Electrocardiography indicated newly developed T wave inversions. Echocardiography demonstrated severe left ventricular dysfunction with hypokinesis of the apical area.[ncbi.nlm.nih.gov]

Treatment

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.

Prognosis

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.

Etiology

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.

Epidemiology

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].

Sex distribution
Age distribution

Pathophysiology

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.

Prevention

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.

Summary

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].

Patient Information

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.

References

Article

  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.

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Last updated: 2019-07-11 22:32