Cardiovascular Diseases › Vasculitic Disorders

Vasculitis - Small Vessel

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Vasculitis - Small Vessel

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Overview

Vasculitides are characterized by blood vessel inflammation with possible necrosis, ischemia, and organ damage.
The vessels and organs affected vary by the specific vasculitic disorder – the ones we'll address in this tutorial affect the small arteries, veins, and capillaries.
General symptoms are due to systemic inflammation, and include fever, arthritis, arthralgia, fatigue, and weight loss.
Cutaneous manifestations can occur in isolation.
Treatments include corticosteroids and immunosuppressants.

ANCA – Associated vasculitides

Granulomatosis with polyangiitis

Note that this was formerly called Wegener's granulomatosis, but this nomenclature is being phased out due to Wegener's associations with the Nazi party.

Characterized by necrotic granulomatous inflammation; granulomas comprise giant cells, plasma cells, lymphocytes, neutrophils, and eosinophils.

The respiratory tract is typically involved, which can lead to sinusitis, otitis media, rhinorrhea, and epistaxis; the mucosa can become granular and crusted.
– Erosion and destruction of the nasal septum can case the nasal bridge to collapse.
– Destruction of the tracheobronchial tree can lead to stenosis and airway obstruction.
– When the lungs are involved, patients can develop a cough, difficulty breathing, hemoptysis, and hemorrhaging.

The kidneys are also commonly affected, leading to necrotizing crescent focal glomerulonephritis and thrombosis.

Cutaneous manifestations vary, and include palpable purpura, livedo reticularis, ulcers, and the formation of deep, tender nodules.

The eyes, nervous system, heart, and musculoskeletal system can also be involved.

Granulomatosis with polyangiitis most commonly occurs in Caucasians; the average age of onset is 40 years.

Eosinophilic granulomatosis with polyangiitis

Aka, Churg-Strauss
ANCA-associated disorder characterized by necrotizing granulomatous inflammation.

Manifests as both vascular and extravascular necrotizing granulomas with eosinophil blood and tissue infiltrations.

Three stages of eosinophilc granulomatosis with polyangiitis:

The allergic stage is often marked by adult-onset asthma, which can develop years before full presentation of the disorder.
Patients may also develop sinusitis, hemoptysis, and pulmonary infiltrates as part of the allergic stage.

The eosinophilic stage is characterized by blood and tissue infiltration of eosinophils.

The vasculitic stage can affect multiple organ systems:
– Nervous system involvement is associated with multiple mononeuropathy.
– Cutaneous involvement manifests as nodules or papules on the extensor surfaces, especially around the elbows.
– GI involvement can produce pain, diarrhea, and bleeding.
– Cardiac involvement often leads to inflammation and/or cardiomyopathies.

Be aware that the kidneys are less commonly affected than in the other ANCA-associated vasculitides.

Disease onset is usually during the late 40s.

Microscopic polyangiitis

Characterized by necrotizing pauci-immune inflammation without granulomas.

Renal involvement is associated with glomerulonephritis and rapid progression to renal failure.

Purpuric rash is common.

The lungs are less commonly affected, but, when they are, alveolar hemorrhage and fibrosis can be serious.

Microscopic polyangiitis usually develops in patients 50-60 years old.

Non-ANCA-associated small vessel vasculitides

Immunoglobulin A-associated vasculitis

Aka, Henoch-Schonlein purpura
Occurs when IgA immune complexes are deposited in the small vessels.
Patients develop palpable purpura, especially on the lower extremities, arthralgias, abdominal pain, dark stools, and focal glomerulonephritis.
It's common and self-limited in children; in adults, it often becomes chronic.

Cryoglobulinemia

Occurs when cryoglobulins in the blood clump at cold temperatures.
Patients experience notable fatigue, palpable purpura in the legs, arthralgias in the knees and hands, glomerulonephritis, and PNS disturbances.

Type I cryoglobulinemia is associated with B-cell lymphoproliferative disorder.

Types II and III (aka, mixed cryoglobulinemia) is assoc. with Hepatitis C virus.

Autoimmune Disorders

The autoimmune disorders systemic lupus erythematosus and rheumatoid arthritis are also associated with small-vessel vasculitis.

Board Review

Small Vessel Vasculitis

Getting ready for boards? Review these concise, bulleted high yield reviews for your exam.

USMLE & COMLEX-USA

Nurse Practitioner (NP)

Physician Assistant (PA)

Internal Medicine (ABIM)

References

  • "Aitc201611010_table_1_spectrum_of_clinical_features_of_giant_cell_arteritis.Jpeg (714×1200)." Accessed August 5, 2019.
  • Alba, Marco A., Luis Felipe Flores-Suárez, Ashley G. Henderson, Hong Xiao, Peiqi Hu, Patrick H. Nachman, Ronald J. Falk, and J. Charles Jennette. "Interstital Lung Disease in ANCA Vasculitis." Autoimmunity Reviews 16, no. 7 (July 2017): 722–29. https://doi.org/10.1016/j.autrev.2017.05.008.
  • "ANCA-Associated Vasculitis: Pathogenesis, Models, and Preclinical Testing- ClinicalKey." Accessed August 5, 2019. https://www-clinicalkey-com.proxy.medlib.uits.iu.edu/#!/content/playContent/1-s2.0-S0270929517300554.
  • "Autoinflammatory Associated Vasculitis- ClinicalKey." Accessed August 5, 2019. https://www-clinicalkey-com.proxy.medlib.uits.iu.edu/#!/content/playContent/1-s2.0-S0049017216301226.
  • Baigrie, Dana, and Jonathan S. Crane. "Leukocytoclastic Vasculitis (Hypersensitivity Vasculitis)." In StatPearls. Treasure Island (FL): StatPearls Publishing, 2019. http://www.ncbi.nlm.nih.gov/books/NBK482159/.
  • Barut, Kenan, Sezgin Sahin, and Ozgur Kasapcopur. "Pediatric Vasculitis:" Current Opinion in Rheumatology 28, no. 1 (January 2016): 29–38. https://doi.org/10.1097/BOR.0000000000000236.
  • Brogan, Paul, and Despina Eleftheriou. "Vasculitis Update: Pathogenesis and Biomarkers." Pediatric Nephrology 33, no. 2 (February 1, 2018): 187–98. https://doi.org/10.1007/s00467-017-3597-4.
  • Cacoub, Patrice, Cloe Comarmond, Fanny Domont, Léa Savey, and David Saadoun. "Cryoglobulinemia Vasculitis." The American Journal of Medicine 128, no. 9 (September 2015): 950–55. https://doi.org/10.1016/j.amjmed.2015.02.017.
  • "Cutaneous Vasculitis - Musculoskeletal and Connective Tissue Disorders." Merck Manuals Professional Edition. Accessed August 5, 2019. https://www.merckmanuals.com/professional/musculoskeletal-and-connective-tissue-disorders/vasculitis/cutaneous-vasculitis.
  • De Virgilio, Armando, Antonio Greco, Giuseppe Magliulo, Andrea Gallo, Giovanni Ruoppolo, Michela Conte, Salvatore Martellucci, and Marco de Vincentiis. "Polyarteritis Nodosa: A Contemporary Overview." Autoimmunity Reviews 15, no. 6 (June 1, 2016): 564–70. https://doi.org/10.1016/j.autrev.2016.02.015.
  • Einhorn, Joseph, and Joel T Levis. "Dermatologic Diagnosis: Leukocytoclastic Vasculitis." The Permanente Journal 19, no. 3 (2015): 77–78. https://doi.org/10.7812/TPP/15-001.
  • "Eosinophilic Granulomatosis with Polyangiitis: Clinical Pathology Conference and Review- ClinicalKey." Accessed August 6, 2019. https://www-clinicalkey-com.proxy.medlib.uits.iu.edu/#!/content/playContent/1-s2.0-S2213219818304239.
  • "File:Crescentic Glomerulonephritis (2).Jpg - Wikimedia Commons." Accessed August 8, 2019. https://commons.wikimedia.org/wiki/File:Crescentic_glomerulonephritis_(2).jpg.
  • "Giant Cell Arteritis (Temporal Arteritis)." Vasculitis Foundation (blog). Accessed August 5, 2019. https://www.vasculitisfoundation.org/education/forms/giant-cell-arteritis/.
  • Girard, Charlotte, Pierre Charles, Benjamin Terrier, Guillaume Bussonne, Pascal Cohen, Christian Pagnoux, Vincent Cottin, Jean-François Cordier, and Loïc Guillevin. "Tracheobronchial Stenoses in Granulomatosis With Polyangiitis (Wegener's)." Medicine 94, no. 32 (August 14, 2015). https://doi.org/10.1097/MD.0000000000001088.
  • "Granulomatosis with Polyangiitis (Wegener's) - Johns Hopkins." Johns Hopkins Vasculitis Center (blog). Accessed August 6, 2019. https://www.hopkinsvasculitis.org/types-vasculitis/granulomatosis-with-polyangiitis/.
  • Hid Cadena, Rebeca, Wayel H. Abdulahad, G. A. P. Hospers, T. T. Wind, Annemieke M. H. Boots, Peter Heeringa, and Elisabeth Brouwer. "Checks and Balances in Autoimmune Vasculitis." Frontiers in Immunology 9 (February 22, 2018): 315. https://doi.org/10.3389/fimmu.2018.00315.
  • Hilhorst, Marc, Pieter van Paassen, and Jan Willem Cohen Tervaert. "Proteinase 3-ANCA Vasculitis versus Myeloperoxidase-ANCA Vasculitis." Journal of the American Society of Nephrology 26, no. 10 (October 2015): 2314–27. https://doi.org/10.1681/ASN.2014090903.
  • "Histopathology Images of Giant Cell (Temporal) Arteritis by PathPedia.Com: Pathology e-Atlas." Accessed August 5, 2019. https://www.pathpedia.com/education/eatlas/histopathology/blood_vessels/giant_cell_(temporal)_arteritis.aspx.
  • Jarrot, Pierre-André, and Gilles Kaplanski. "Pathogenesis of ANCA-Associated Vasculitis: An Update." Autoimmunity Reviews 15, no. 7 (July 1, 2016): 704–13. https://doi.org/10.1016/j.autrev.2016.03.007.
  • Jennette, J. Charles, and Patrick H. Nachman. "ANCA Glomerulonephritis and Vasculitis." Clinical Journal of the American Society of Nephrology 12, no. 10 (October 6, 2017): 1680–91. https://doi.org/10.2215/CJN.02500317.
  • Johnston, S L, R J Lock, and M M Gompels. "Takayasu Arteritis: A Review." Journal of Clinical Pathology 55, no. 7 (July 2002): 481–86.
  • Kamesh, Lavanya, Lorraine Harper, and Caroline O. S. Savage. "ANCA-Positive Vasculitis." Journal of the American Society of Nephrology 13, no. 7 (July 1, 2002): 1953–60. https://doi.org/10.1097/01.ASN.0000016442.33680.3E.
  • Kesten, F, M Aschwanden, P Gubser, K Glatz, T Daikeler, and C Hess. "Giant Cell Arteritis- a Changing Entity." Swiss Medical Weekly, September 28, 2011. https://doi.org/10.4414/smw.2011.13272.
  • Ly, Kim-Heang, Alexis Régent, Mathieu C. Tamby, and Luc Mouthon. "Pathogenesis of Giant Cell Arteritis: More than Just an Inflammatory Condition?" Autoimmunity Reviews 9, no. 10 (August 1, 2010): 635–45. https://doi.org/10.1016/j.autrev.2010.05.002.
  • Makol, Ashima, Eric L. Matteson, and Kenneth J. Warrington. "Rheumatoid Vasculitis: An Update." Current Opinion in Rheumatology 27, no. 1 (January 2015): 63–70. https://doi.org/10.1097/BOR.0000000000000126.
  • Marques, Camila Carneiro, Elizabeth Leocadia Fernandes, Gabriela Momente Miquelin, and Mariana Morais Tavares Colferai. "Cutaneous Manifestations of Churg-Strauss Syndrome: Key to Diagnosis." Anais Brasileiros de Dermatologia 92, no. 5 Suppl 1 (2017): 56–58. https://doi.org/10.1590/abd1806-4841.20175522.
  • Nantsupawat, Teerapat, Charoen Mankongpaisarnrung, Suthipong Soontrapa, Chok Limsuwat, and Kenneth Nugent. "Obscure Severe Infrarenal Aortoiliac Stenosis With Severe Transient Lactic Acidosis." Journal of Investigative Medicine High Impact Case Reports 1, no. 1 (January 2013): 232470961347994. https://doi.org/10.1177/2324709613479940.
  • Nephron. English: High Magnification Micrograph of Eosinophilic Vasculitis Consistent with Churg-Strauss Syndrome, Abbreviated CSS. H&E Stain. [object HTMLTableCellElement]. Own work. https://commons.wikimedia.org/wiki/File:Churg-Strauss_syndrome_-_high_mag.jpg.
  • ———. English: Intermediate Magnification Micrograph of Granulomatosis with Polyangiitis and ANCA-Associated Granulomatous Vasculitis. Lung Biopsy. H&E Stain. [object HTMLTableCellElement]. Own work. https://commons.wikimedia.org/wiki/File:Wegener%27s_granulomatosis_-b-intermed_mag.jpg#/media/File:Wegener's_granulomatosis-b-_very_high_mag.jpg.
  • ———. English: Micrograph of Giant Cell Arteritis (Also Temporal Arteritis). H&E Stain. December 14, 2014. Own work. https://commons.wikimedia.org/wiki/File:Giant_cell_arteritis_--_low_mag.jpg.
  • Ozen, Seza. "The Changing Face of Polyarteritis Nodosa and Necrotizing Vasculitis." Nature Reviews. Rheumatology; London 13, no. 6 (June 2017): 381–86. http://dx.doi.org.proxy.ulib.uits.iu.edu/10.1038/nrrheum.2017.68.
  • Pagnoux, Christian. "Updates in ANCA-Associated Vasculitis." European Journal of Rheumatology 3, no. 3 (September 2016): 122–33. https://doi.org/10.5152/eurjrheum.2015.0043.
  • "Polyarteritis Nodosa." Johns Hopkins Vasculitis Center (blog). Accessed August 5, 2019. https://www.hopkinsvasculitis.org/types-vasculitis/polyarteritis-nodosa/.
  • Reference, Genetics Home. "Behçet Disease." Genetics Home Reference. Accessed August 6, 2019. https://ghr.nlm.nih.gov/condition/behcet-disease.
  • Rosenbaum, James T., Cailin H. Sibley, and Phoebe Lin. "Retinal Vasculitis." Current Opinion in Rheumatology 28, no. 3 (May 2016): 228–35. https://doi.org/10.1097/BOR.0000000000000271.
  • Rowley, Anne H., and Stanford T. Shulman. "The Epidemiology and Pathogenesis of Kawasaki Disease." Frontiers in Pediatrics 6 (2018). https://doi.org/10.3389/fped.2018.00374.
  • Samson, Maxime, Marc Corbera-Bellalta, Sylvain Audia, Ester Planas-Rigol, Laurent Martin, Maria Cinta Cid, and Bernard Bonnotte. "Recent Advances in Our Understanding of Giant Cell Arteritis Pathogenesis." Autoimmunity Reviews 16, no. 8 (August 1, 2017): 833–44. https://doi.org/10.1016/j.autrev.2017.05.014.
  • Shirai, Tsuyoshi, Marc Hilhorst, David G. Harrison, Jörg J. Goronzy, and Cornelia M. Weyand. "Macrophages in Vascular Inflammation – From Atherosclerosis to Vasculitis." Autoimmunity 48, no. 3 (May 2015): 139–51. https://doi.org/10.3109/08916934.2015.1027815.
  • Söderberg, Daniel, and Mårten Segelmark. "Neutrophil Extracellular Traps in ANCA-Associated Vasculitis." Frontiers in Immunology 7 (2016). https://doi.org/10.3389/fimmu.2016.00256.
  • Takahashi, Kei, Toshiaki Oharaseki, and Yuki Yokouchi. "Histopathological Aspects of Cardiovascular Lesions in Kawasaki Disease." International Journal of Rheumatic Diseases 21, no. 1 (January 2018): 31–35. https://doi.org/10.1111/1756-185X.13207.
  • Takahashi, Kei, Toshiaki Oharaseki, Yuki Yokouchi, Nobuyuki Hiruta, and Shiro Naoe. "Kawasaki Disease as a Systemic Vasculitis in Childhood." Annals of Vascular Diseases 3, no. 3 (2010): 173–81. https://doi.org/10.3400/avd.sasvp01003.
  • "Takayasu's Arteritis." In Wikipedia, May 29, 2019. (Image) https://en.wikipedia.org/w/index.php?title=Takayasu%27s_arteritis&oldid=899366891.
  • "Takayasu's Arteritis." Accessed August 5, 2019. https://www.rheumatology.org/I-Am-A/Patient-Caregiver/Diseases-Conditions/Takayasus-Arteritis.
  • "Takayasu's Arteritis: Pathogenesis and Clinical Findings | Calgary Guide." Accessed August 6, 2019. https://calgaryguide.ucalgary.ca/takayasus-arteritis-pathogenesis-and-clinical-findings/.
  • Yates, M., and R. Watts. "ANCA-Associated Vasculitis." Clinical Medicine (London, England) 17, no. 1 (February 2017): 60–64. https://doi.org/10.7861/clinmedicine.17-1-60.