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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
  • A 33-year-old man presented with hemoptysis and a low-grade fever and was diagnosed to have IgAV based on the results of a renal biopsy. Treatment with corticosteroids, cyclophosphamide, and plasmapheresis was effective.[ncbi.nlm.nih.gov]
  • Early inflammatory symptoms of the disease include fever, arthralgia, myalgia and pain over the involved area.[symptoma.com]
  • 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]
  • This is a case of a 60-year-old man with chronic idiopathic pulmonary fibrosis, with fever, arthralgias and rash characterised by extensive erythematous plaques on his limbs and trunk 5â ‰days after influenza vaccination.[ncbi.nlm.nih.gov]
  • We report an F-FDG linear uptake of the right popliteal artery in a 25-year-old man who underwent a PET/CT scan for unexplained chronic asthenia associated with fever symptoms, arthralgia, and subcutaneous nodes, in favor of vasculitis.[ncbi.nlm.nih.gov]
Chills
  • 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]
  • 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]
  • 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]
  • Now along with these local symptoms patients might also experience general symptoms such as night sweats or fever, so there's a little thermometer right here, as the patient might have a fever or the patient might have chills or generalized muscle aches[khanacademy.org]
Rapidly Progressive Glomerulonephritis
  • 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]
  • 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.[msdmanuals.com]
  • Patients with rapidly progressive glomerulonephritis should be treated with high dosages of glucocorticoids in combination with cyclophosphamide.[lupusinternational.com]
Hypertension
  • A 60-year-old man with a history of hypertension, type 2 diabetes, and reflux esophagitis was admitted to our hospital with hemoptysis, dyspnea, and leg edema.[ncbi.nlm.nih.gov]
  • The most common risk factor of mortality was hypertension (42.4%), followed by chronic kidney disease stage 3 (25.9%). Fourteen patients (8.2%) died during the mean follow-up of 56.7 months.[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]
  • Systemic polyarteritis nodosa involves all organ systems and the presentation widely varies. [ 4 ] Features include vasculitic skin lesions, hypertension, neuropathy, and myalgia.[emedicine.medscape.com]
  • Hypertension, cardiac failure, unequal pulses. Testicular pain. Oliguria, oedema. Pulmonary infiltrates or haemorrhage. Laboratory: Increased acute-phase reactants (ESR, CRP). Anaemia, Leukocytosis, Eosinophilia.[pmmonline.org]
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]
  • According to the journal Autoimmune Diseases , studies show that vascular disease is common in patients who suffer from lupus. Vasculitis may be recognized in as many as 56 percent of lupus patients throughout their lives.[belmarrahealth.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]
Blurred Vision
  • 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]
  • vision; most common type of vasculitis in adults over age 50; half of those with GCA also develop polymyalgia rheumatica Polymyalgia Rheumatica Large Characterized by pain and stiffness in the shoulders and hips; found mostly in those over age 50; 10[labtestsonline.org]
Purpura
  • IgA vasculitis (IgAV) commonly occurs in young children, who present with a tetrad of purpura, abdominal pain, arthralgia and nephritis. Diffuse alveolar hemorrhage (DAH) is a rare complication of IgAV.[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]
  • Twelve days after the first chemotherapy administration and after 8 sessions of radiotherapy (total of 16 Gy), the patient presented with acute onset of palpable purpura in the lower limbs.[ncbi.nlm.nih.gov]
  • 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]
  • After excluding more common causes of CSVV and abdominal pain in children, including immunoglobulin A vasculitis (previously Henoch-Schönlein purpura), infectious colitis, and drug-induced vasculitis, alternative diagnoses such as CSVV secondary to IBD[ncbi.nlm.nih.gov]
Palpable Purpura
  • 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]
  • Leukocytoclastic vasculitis (LCV) is a disease characterized by inflammation of small vessels presenting with petechiae and palpable purpura.[ncbi.nlm.nih.gov]
  • Twelve days after the first chemotherapy administration and after 8 sessions of radiotherapy (total of 16 Gy), the patient presented with acute onset of palpable purpura in the lower limbs.[ncbi.nlm.nih.gov]
  • This disorder usually presents as palpable purpura, although lesions may occasionally be urticarial or ulcerative ( Figure 2 ) . Skin biopsy usually shows leukocytoclastic angiitis.[aafp.org]
  • ., 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]
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]
  • Systemic vasculitis commonly causes general symptoms such as feeling unwell, weight loss, night sweats and joint pains. Sometimes it may come on very suddenly and dramatically, but it commonly comes on gradually over days and weeks.[edren.org]
Urticaria
  • 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]
  • […] 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]
  • Diagnostic methods Diagnosis requires the presence of two major criteria (recurrent urticaria for 6 months and hypocomplementemia) and at least two minor criteria (leukocytoclastic vasculitis on biopsy, arthralgia and arthritis, ocular inflammation, abdominal[orpha.net]
  • However, the differential diagnosis of the different types of rashes associated with LCV is broad, and includes the following: urticaria, urticarial vasculitis, arthropod bites, morbilliform drug eruption, erythema multiforme, urticaria, scurvy, amyloidosis[clinicaladvisor.com]
Petechiae
  • Leukocytoclastic vasculitis (LCV) is a disease characterized by inflammation of small vessels presenting with petechiae and palpable purpura.[ncbi.nlm.nih.gov]
  • Large vessel vasculitis can present symptoms ranging from acneiform lesions, spontaneous bruising, purpura, petechiae and peripheral or periorbital edema Small vessel inflammation can present symptoms of tenderness, bruising, petechiae, and extremity[ehcd.com]
  • Vasculitis affecting the small vessels of the skin (eg, arterioles, capillaries, postcapillary venules) tends to cause lesions such as purpura, petechiae, and possibly shallow ulcers.[merckmanuals.com]
  • Capillaritis presents as pigmented purpura , characterised by petechiae resolving with haemosiderin deposition . Small vessel vasculitis is characterised by palpable purpura .[dermnetnz.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.

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: 2017-08-09 17:56