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

  […] and joints Giant cell arteritis/Temporal arteritis fever muscle pain jaw muscle dysfunction visual changes headache Other vasculitic syndromes Kawasaki disease fever swelling congested conjunctivas scaly skin nonsuppurative cervical adenitis Behcet's [healthcentral.com]

  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]

  Its early symptoms include fever, weight loss, and a general feeling of tiredness. The disease may not be diagnosed for months or even years, however. [encyclopedia.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]

  Vasculitis occurs at any age, may be acute or chronic, and manifests with general symptoms such as fever, weight loss and fatigue, as well as more specific clinical signs depending on the type of vessels and organs affected. [orpha.net]

  People may have general symptoms, such as fever or fatigue, followed by other symptoms depending on which organs are affected. [merckmanuals.com]

• Rapidly Progressive Glomerulonephritis

  When presented as the pulmonary-renal syndrome, it could have a rapidly progressive course which can be fatal. Here, we describe a case of hydralazine-associated rapidly progressive glomerulonephritis and pulmonary haemorrhage. [ncbi.nlm.nih.gov]

  Severe, rapidly progressive and life- or organ-threatening vasculitis (eg, causing alveolar hemorrhage, rapidly progressive glomerulonephritis, or mesenteric ischemia) is a medical emergency requiring hospital admission and immediate treatment. [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]

  Auto-antibodies to GBM and neutrophil cytoplasm in rapidly progressive glomerulonephritis. Kidney Int 1990 ; 37(3): 965 – 70. Google Scholar | Crossref 2. Rutgers, A., Slot, M. [journals.sagepub.com]

• Congestive Heart Failure

  Vasculitis affecting the heart, however, can cause congestive heart failure. Large Vessel Vasculitis Large vessel vasculitis affects the body’s large arteries, including the aorta and carotid arteries. [nyulangone.org]

  Vasculitis affecting the heart can cause congestive heart failure. Vasculitis affecting the brain can cause headaches, confusion, seizures, stroke, paralysis, numbness, and lightheadedness. Vasculitis affecting the kidneys can cause kidney failure. [medicinenet.com]

  Depending on the organ(s) involved, the child may develop aneurysms in the arteries supplying the kidneys, kidney failure, aneurysms in the coronary artery, congestive heart failure, massive bleeding in the digestive tract, aneurysms in the arteries supplying [encyclopedia.com]

  Nicola PJ, Maradit-Kremers H, Roger VL, Jacobsen SJ, Crowson CS, Ballman KV, Gabriel SE: The risk of congestive heart failure in rheumatoid arthritis: a population-based study over 46 years. Arthritis Rheum. 2005, 52: 412-420. 10.1002/art.20855. 19. [arthritis-research.biomedcentral.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]

• Easy Bruising

  Other prominent side effects include increased blood sugar (diabetes), increased blood pressure, loss of bone density (osteoporosis), easy bruising, and poor healing. [health.clevelandclinic.org]

• Painful Cough

  Common symptoms include: Skin rashes Fatigue Weakness Fever Joint pains Abdominal pain Kidney problems (including dark or bloody urine ) Nerve problems (including numbness, weakness and pain) Cough and/or shortness of breath Additional symptoms can occur [my.clevelandclinic.org]

  Nose or mouth sores Nose bleeding Change in voice Hoarseness Nasal congestion Lungs Symptoms: Shortness of breath Cough Chest pain Coughing up blood Abnormal chest x-ray or CT scan Heart Symptoms: Heart attack Angina Stomach and colon Symptoms: Bleeding [upmc.com]

  […] in trouble breathing including asthma-like symptoms, chest pain, cough, coughing up blood, and sinus congestion. [rheumaderm-society.org]

  The clinical manifestations due to lower tract affectation include cough, dyspnea and/or hemoptysis with pleuritic chest pain. [archbronconeumol.org]

• Loss of Appetite

  It manifests with fever, fatigue, loss of appetite, weakness, skin rash, joint pain, numbness, burning sensation and renal failure. [icd9data.com]

  The early symptoms of vasculitis frequently include fever, weakness, loss of appetite, weight loss, tiring easily, pains in the muscles or joints, and swollen joints. [encyclopedia.com]

  […] of appetite fatigue Granulomatosis with Polyangiitis/Granulomatosis with Polyangiitis fever pulmonary congestion cough weight loss blood in the urine and nose anorexia inflammation of the nostrils and throat pain in muscles and joints Giant cell arteritis [healthcentral.com]

  However, general signs and symptoms that most people with vasculitis experience include: Fever Fatigue Weight loss and/or loss of appetite Muscle and joint pain or general achiness with headache Night sweats Rash Nerve problems, such as numbness or weakness [columbiadoctors.org]

  It can cause: asthma allergic rhinitis ( cold-like symptoms caused by allergies ) a high temperature (fever) muscle and joint pain tiredness loss of appetite and weight loss It can also affect the nerves, causing weakness, pins and needles or numbness [nhs.uk]

• 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

  Intended for healthcare professionals Education Vasculitis Vasculitis Clinical Review ABC of arterial and vascular disease BMJ 2000; 320 doi: https://doi.org/10.1136/bmj.320.7245.1325 (Published 13 May 2000) Cite this as: BMJ 2000;320:1325 C O S Savage [bmj.com]

  some viral infections, collagen vascular 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 [pathologyoutlines.com]

  Página 249 - Omenn GS, Motulsky AG: A quantitative assessment of plasma homocysteine as a risk factor for vascular disease. ‎ [books.google.es]

  The clinical and radiological presentation can mimic vasculitis, fibromuscular dysplasia or collagen vascular diseases. SAM is often self-limited and may be managed conservatively with adequate blood pressure control in the majority of cases. [archive-ouverte.unige.ch]

• Osteopenia

  […] a National,Randomised, Single-masked Controlled Trial (INCYTOB) Completed NCT00167583 Phase 3 Cyclosporin A;Interferon-alpha2a 42 Double-Blinded Controlled Trial of Alendronate for the Treatment of Childhood and Adolescent Glucocorticoid- Associated Osteopenia [malacards.org]

• Blurred Vision

  Symptoms based on blood supply of organ involvement can include: Brain : Alteration of consciousness, severe headache, stroke-like symptoms (paralysis) Eyes: Blurred vision, seeing floaters, sensitive to light Skin: Rash, chronic ulcer, bruise Bowel: [my.clevelandclinic.org]

  Von der Harnwege: pharma-base.ru  From the side of the central nervous system and peripheral nervous system : headache, dizziness, agitation, disturbances of perception, paresthesia, loss of memory, disorientation, blurred vision, hearing, taste, tinnitus [sl.pons.com]

  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]

  Eyes – 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. [arthritis.org]

• Redness of Eye

  Symptoms include high fever; swollen hands and feet; red lips, eyes, and mouth; rash and swollen lymph nodes. Microscopic polyangiitis (MPA) — Can affect any organ in the body, but is most commonly found in the skin, kidneys and nerves. [pennmedicine.org]

  Eye problems may result in blindness. [merckmanuals.com]

  […] are affected- Red, irritated eyes Eye pain Blurred vision Vision loss in one (or both) eyes If the ears are affected- Ringing in one or both ears (tinnitus) Infection/inflammation in the middle ear Hearing loss If nerves are affected- Numbness, tingling [unckidneycenter.org]

• Purpura

  Classification of cutaneous vasculitis Capillaritis Progressive pigmented purpura : the most common form of capillaritis Itching purpura Pigmented purpuric lichenoid dermatosis Purpura annularis telangiectodes Capillaritis in association with contact [dermnetnz.org]

  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]

  Henoch-Sch ö nlein purpura (HSP). [encyclopedia.com]

  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]

• Ulcer

  Skin rash: e.g., 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]

  Behçet’s Skin ulcer Behcets rash Skin lesion Lip ulcer Lip ulcer Mouth ulcer Central Nervous System Vasculitis Primary cerebral vasculitis Churg Strauss Syndrome (EPGA) Skin involvement Skin involvement Giant Cell Arteritis Swollen temporal artery Henoch-Schönlein [vasculitis.org.uk]

  The ulcers start as raised erythematous swellings which then break down into an ulcer with a grey pseudo membrane over. Usually on the mucosa, they may spread to the hard palate, pharynx and tonsils. [nature.com]

  Interestingly, the patient had been initially diagnosed as having diabetic foot ulcer at our outpatient clinic. Case A 64-year-old male patient was hospitalized in our clinic for diabetic foot ulcer (initial diagnosis).... [go.gale.com]

• Palpable Purpura

  These spots can usually be felt by the examiner's fingertips, hence the descriptor "palpable". The image below is a classic example of palpable purpura. [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]

  Skin rash: e.g., 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]

• Sweating

  Fever, night sweats, malaise, myalgia, and arthralgia are common in all types of vasculitis. [bmj.com]

  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]

  Inflammatory infiltrate in wall of dermal or subcutaneous vessels; may be neutrophilic, lymphocytic or granulomatous Often red blood cell extravasation Variable fibrinoid necrosis of vessel walls Variable secondary changes in overlying epidermis and sweat [pathologyoutlines.com]

• Skin Rash

  However, vancomycin treatment is associated adverse reactions, including skin rashes and nephrotoxicity. [ncbi.nlm.nih.gov]

  […] purpura (HSP) Henoch-Schönlein purpura (HSP) Unspecified or Other Vasculitis Rash in LCV (Leukocytoclastic vasculitis Skin rash in unspecified pANCA vasculitis Vasculitis rash Skin rash in unspecified pANCA vasculitis [vasculitis.org.uk]

  You have a skin rash with palpable purpura. You have a skin rash that is maculopapular (contains both flat and raised spots). You used a drug before developing a skin rash. [healthline.com]

  Skin rash: e.g., 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]

  It manifests with fever, fatigue, loss of appetite, weakness, skin rash, joint pain, numbness, burning sensation and renal failure. [icd9data.com]

• Kidney Failure

  Microscopic polyarteritis—affects the smaller blood vessels of the body, causing kidney failure and lung hemorrhage. A type of antibody called ANCA (antineutrophil cytoplasmic antibody) is commonly found. [jamanetwork.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]

  Vasculitis affecting the heart can cause congestive heart failure. Vasculitis affecting the brain can cause headaches, confusion, seizures, stroke, paralysis, numbness, and lightheadedness. Vasculitis affecting the kidneys can cause kidney failure. [medicinenet.com]

  Kidney failure, decrease amount of urine, blood and protein leakage from kidney Get useful, helpful and relevant health + wellness information enews More health news + info [my.clevelandclinic.org]

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 [merckmanuals.com]

  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

  Mild leukocytosis—with or without eosinophilia—and increased acute-phase indicators, such as erythrocyte sedimentation rate (ESR) or C-reactive protein, are typical but non-specific. [worldallergy.org]

• Leukocytosis

  Anaemia, Leukocytosis, Eosinophilia. Antineutrophil cytoplasmic antibodies (ANCA). Elevated factor VIII - related antigen (von Willebrand factor). Haematuria and active urine sediment. [pmmonline.org]

  These include leukocytosis, which involves having a high number of white blood cells (WBCs) and anemia. As the disease progresses, symptoms can worsen and become more varied. Your specific symptoms depend on what parts of your body are affected. [healthline.com]

  The univariate analysis demonstrated a significant association between systemic vasculitic involvement and elevated erythrocyte sedimentation rate (cRR, 4.15), leukocytosis (cRR, 4.33), and thrombocytosis (cRR, 6.9). [jamanetwork.com]

  The routine lab studies were notable for high ESR (45 mm), C-reactive protein (40 g/l), leukocytosis with neutrophillia on background corticosteroid therapy, high BUN (15 g/l), low grade proteinuria up to 0.6 g/l. [actarheuma.com]

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.
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