Vasculitis

Vasculitis is a group of disorders that destroy blood vessels by inflammation.[2] Both arteries and veins are affected. Lymphangitis (inflammation of lymphatic vessels) is sometimes considered a type of vasculitis.[3] Vasculitis is primarily caused by leukocyte migration and resultant damage. Although both occur in vasculitis, inflammation of veins (phlebitis) or arteries (arteritis) on their own are separate entities.

Vasculitis
Other namesVasculitides[1]
Petechia and purpura on the lower limb due to medication-induced vasculitis.
Pronunciation
  • /væskjʊˈlaɪtɪs/
SpecialtyRheumatology, Immunology
SymptomsWeight loss, fever, myalgia, purpura
ComplicationsGangrene, Myocardial infarction

Signs and symptoms

Possible signs and symptoms include:[4]

Cause

Classification

Vasculitis can be classified by the cause, the location, the type of vessel or the size of vessel.

  • Underlying cause. For example, the cause of syphilitic aortitis is infectious (aortitis simply refers to inflammation of the aorta, which is an artery.) However, the causes of many forms of vasculitis are poorly understood. There is usually an immune component, but the trigger is often not identified. In these cases, the antibody found is sometimes used in classification, as in ANCA-associated vasculitides. Clinical studies with immunosuppressive drugs targeting specific cytokines and cells can also be used to understand the heterogeneous immunopathogenic mechanisms of vasculitis and support a mechanistic immunological classification.[5]
  • Location of the affected vessels. For example, ICD-10 classifies "vasculitis limited to skin" with skin conditions (under "L"), and "necrotizing vasculopathies" (corresponding to systemic vasculitis) with musculoskeletal system and connective tissue conditions (under "M"). Arteritis/phlebitis on their own are classified with circulatory conditions (under "I").
  • Type or size of the blood vessels that they predominantly affect.[6] Apart from the arteritis/phlebitis distinction mentioned above, vasculitis is often classified by the caliber of the vessel affected. However, there can be some variation in the size of the vessels affected.

A small number have been shown to have a genetic basis. These include adenosine deaminase 2 deficiency and haploinsufficiency of A20.

According to the size of the vessel affected, vasculitis can be classified into:[7][8]

Comparison of major types of vasculitis
VasculitisAffected organsHistopathology
Cutaneous small-vessel vasculitisSkin, kidneysNeutrophils, fibrinoid necrosis
Granulomatosis with polyangiitisNose, lungs, kidneysNeutrophils, giant cells
Eosinophilic granulomatosis with polyangiitisLungs, kidneys, heart, skinHistiocytes, eosinophils
Behçet's diseaseCommonly sinuses, brain, eyes and skin; can affect other organs such as lungs, kidneys, jointsLymphocytes, macrophages, neutrophils
Kawasaki diseaseSkin, heart, mouth, eyesLymphocytes, endothelial necrosis
Buerger's diseaseLeg arteries and veins (gangrene)Neutrophils, granulomas
"Limited" granulomatosis with polyangiitis vasculitisCommonly sinuses, brain, and skin; can affect other organs such as lungs, kidneys, joints;

Takayasu's arteritis, polyarteritis nodosa and giant cell arteritis mainly involve arteries and are thus sometimes classed specifically under arteritis.

There are also many conditions that have vasculitis as an accompanying or atypical feature, including:

In pediatric patients varicella inflammation may be followed by vasculitis of intracranial vessels. This condition is called post varicella angiopathy and this may be responsible for arterial ischaemic strokes in children.[12]

Several of these vasculitides are associated with antineutrophil cytoplasmic antibodies.[13] These are:

  • Granulomatosis with polyangiitis
  • Eosinophilic granulomatosis with polyangiitis
  • Microscopic polyangiitis

Diagnosis
Micrograph showing a vasculitis (eosinophilic granulomatosis with polyangiitis). H&E stain.
Severe vasculitis of the major vessels, displayed on FDG-PET/CT
  • Laboratory tests of blood or body fluids are performed for patients with active vasculitis. Their results will generally show signs of inflammation in the body, such as increased erythrocyte sedimentation rate (ESR), elevated C-reactive protein (CRP), anemia, increased white blood cell count and eosinophilia. Other possible findings are elevated antineutrophil cytoplasmic antibody (ANCA) levels and hematuria.
  • Other organ functional tests may be abnormal. Specific abnormalities depend on the degree of various organs involvement. A brain SPECT can show decreased blood flow to the brain and brain damage.
  • The definite diagnosis of vasculitis is established after a biopsy of involved organ or tissue, such as skin, sinuses, lung, nerve, brain, and kidney. The biopsy elucidates the pattern of blood vessel inflammation.
  • Some types of vasculitis display leukocytoclasis, which is vascular damage caused by nuclear debris from infiltrating neutrophils.[14] It typically presents as palpable purpura.[14] Conditions with leucocytoclasis mainly include hypersensitivity vasculitis (also called leukocytoclastic vasculitis) and cutaneous small-vessel vasculitis (also called cutaneous leukocytoclastic angiitis).
  • An alternative to biopsy can be an angiogram (x-ray test of the blood vessels). It can demonstrate characteristic patterns of inflammation in affected blood vessels.
  • 18F-fluorodeoxyglucose positron emission tomography/computed tomography (FDG-PET/CT)has become a widely used imaging tool in patients with suspected Large Vessel Vasculitis, due to the enhanced glucose metabolism of inflamed vessel walls.[15] The combined evaluation of the intensity and the extension of FDG vessel uptake at diagnosis can predict the clinical course of the disease, separating patients with favourable or complicated progress.[16]
  • Acute onset of vasculitis-like symptoms in small children or babies may instead be the life-threatening purpura fulminans, usually associated with severe infection.
Laboratory Investigation of Vasculitic Syndromes[17]
DiseaseSerologic testAntigenAssociated laboratory features
Systemic lupus erythematosusANA including antibodies to dsDNA and ENA [including SM, Ro (SSA), La (SSB), and RNP]Nuclear antigensLeukopenia, thrombocytopenia, Coombs' test, complement activation: low serum concentrations of C3 and C4, positive immunofluorescence using Crithidia luciliae as substrate, antiphospholipid antibodies (i.e. anticardiolipin, lupus anticoagulant, false-positive VDRL)
Goodpasture's diseaseAnti-glomerular basement membrane antibodyEpitope on noncollagen domain of type IV collagen
Small vessel vasculitis
Microscopic polyangiitisPerinuclear antineutrophil cytoplasmic antibodyMyeloperoxidaseElevated CRP
Granulomatosis with polyangiiitisCytoplasmic antineutrophil cytoplasmic antibodyProteinase 3 (PR3)Elevated CRP
Eosinophilic granulomatosis with polyangiitisperinuclear antineutrophil cytoplasmic antibody in some casesMyeloperoxidaseElevated CRP and eosinophilia
IgA vasculitis (Henoch-Schönlein purpura)None
CryoglobulinemiaCryoglobulins, rheumatoid factor, complement components, hepatitis C
Medium vessel vasculitis
Classical polyarteritis nodosaNoneElevated CRP and eosinophilia
Kawasaki's DiseaseNoneElevated CRP and ESR

In this table: ANA = antinuclear antibodies, CRP = C-reactive protein, ESR = erythrocyte sedimentation rate, dsDNA = double-stranded DNA, ENA = extractable nuclear antigens, RNP = ribonucleoproteins; VDRL = Venereal Disease Research Laboratory

Treatment

Treatments are generally directed toward stopping the inflammation and suppressing the immune system. Typically, corticosteroids such as prednisone are used. Additionally, other immune suppression medications, such as cyclophosphamide and others, are considered. In case of an infection, antimicrobial agents including cephalexin may be prescribed. Affected organs (such as the heart or lungs) may require specific medical treatment intended to improve their function during the active phase of the disease.

References
  1. "Vasculitis - Definition from the Merriam-Webster Online Dictionary". Archived from the original on 1 July 2016. Retrieved 8 January 2009.
  2. "Glossary of dermatopathological terms. DermNet NZ". Archived from the original on 20 December 2008. Retrieved 8 January 2009.
  3. "Vasculitis" at Dorland's Medical Dictionary
  4. "The Johns Hopkins Vasculitis Center - Symptoms of Vasculitis". Archived from the original on 27 February 2009. Retrieved 7 May 2009.
  5. Torp, Christopher Kirkegaard; Brüner, Mads; Keller, Kresten Krarup; Brouwer, Elisabeth; Hauge, Ellen-Margrethe; McGonagle, Dennis; Kragstrup, Tue Wenzel (2021). "Vasculitis therapy refines vasculitis mechanistic classification". Autoimmunity Reviews. 20 (6): 102829. doi:10.1016/j.autrev.2021.102829. PMID 33872767.
  6. Jennette JC, Falk RJ, Andrassy K, et al. (1994). "Nomenclature of systemic vasculitides. Proposal of an international consensus conference". Arthritis Rheum. 37 (2): 187–92. doi:10.1002/art.1780370206. PMID 8129773.
  7. "Overview of Vasculitis". Merck Manuals Professional Edition. Archived from the original on 3 April 2015. Retrieved 5 October 2016.
  8. Gündüz, Özgür (18 October 2011). "Histopathological Evaluation of Behçet's Disease and Identification of New Skin Lesions". Pathology Research International. 2012: 209316. doi:10.1155/2012/209316. ISSN 2090-8091. PMC 3199096. PMID 22028988.
  9. Meyts, Isabelle; Aksentijevich, Ivona (July 2018). "Deficiency of Adenosine Deaminase 2 (DADA2): Updates on the Phenotype, Genetics, Pathogenesis, and Treatment". Journal of Clinical Immunology. 38 (5): 569–578. doi:10.1007/s10875-018-0525-8. ISSN 0271-9142. PMC 6061100. PMID 29951947.
  10. Donadieu, Jean; Lamant, Marie; Fieschi, Claire; de Fontbrune, Flore Sicre; Caye, Aurélie; Ouachee, Marie; Beaupain, Blandine; Bustamante, Jacinta; Poirel, Hélène A.; Isidor, Bertrand; Van Den Neste, Eric (August 2018). "Natural history of GATA2 deficiency in a survey of 79 French and Belgian patients". Haematologica. 103 (8): 1278–1287. doi:10.3324/haematol.2017.181909. ISSN 0390-6078. PMC 6068047. PMID 29724903.
  11. Geier, Christoph B.; Farmer, Jocelyn R.; Foldvari, Zsofia; Ujhazi, Boglarka; Steininger, Jolanda; Sleasman, John W.; Parikh, Suhag; Dilley, Meredith A.; Pai, Sung-Yun; Henderson, Lauren; Hazen, Melissa (21 October 2020). "Vasculitis as a Major Morbidity Factor in Patients With Partial RAG Deficiency". Frontiers in Immunology. 11: 574738. doi:10.3389/fimmu.2020.574738. ISSN 1664-3224. PMC 7609967. PMID 33193364.
  12. Nita R Sutay, Md Ashfaque Tinmaswala, Shilpa Hegde . "International Journal of Medical Research and Health Sciences | 404 Page". Archived from the original on 17 November 2015. Retrieved 19 August 2015.
  13. Millet A, Pederzoli-Ribeil M, Guillevin L, Witko-Sarsat V, Mouthon L (2013) Antineutrophil cytoplasmic antibody-associated vasculitides: is it time to split up the group? Ann Rheum Dis
  14. A Brooke W Eastham, Ruth Ann Vleugels and Jeffrey P Callen (12 July 2021). "Leukocytoclastic Vasculitis". Medscape. Updated: Oct 25, 2018
  15. Maffioli L, Mazzone A (2014). "Giant-Cell Arteritis and Polymyalgia Rheumatica". NEJM. 371 (17): 1652–1653. doi:10.1056/NEJMc1409206. PMC 4277693. PMID 25337761.
  16. Dellavedova L, Carletto M, Faggioli P, Sciascera A, Del Sole A, Mazzone A, Maffioli LS (2015). "The prognostic value of baseline 18F-FDG PET/CT in steroid-naïve large-vessel vasculitis: introduction of volume-based parameters". European Journal of Nuclear Medicine and Molecular Imaging. 55 (2): 340–8. doi:10.1007/s00259-015-3148-9. PMID 26250689. S2CID 21446786.
  17. Burtis CA, Ashwood ER, Bruns DE (2012). Tietz Textbook of Clinical Chemistry and Molecular Diagnostics, 5th edition. Elsevier Saunders. p. 1568. ISBN 978-1-4160-6164-9.
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