Necrotizing arteriolitis

Necrotizing arteriolitis
Pronunciation
  • nek'rō-tīz-ing ahr-tēr'ē-ō-lī'tis [1]
SpecialtyNephrology
SymptomsNecrosis, numbness, peripheral neuropathy and severe inflammation of the arteries
CausesPolyarteritis nodosa; Granulomatosis with polyangiitis; Lupus erythematosus; Hepatitis C; Autoimmune response
PreventionThere is no known way of preventing the disease.
TreatmentAdministering corticosteroids and immunosuppresive drugs

Necrotizing arteriolitis, also called necrotizing arteritis is a life-threatening[2] inflammation of medium-sized blood vessels and arterial walls, also called vasculitis, that leads to tissue necrosis. It presents with symptoms such as fever, inflammation, muscle weakness, and abdominal pain.

It encompasses conditions like polyarteritis nodosa and is different from other forms of its type.[3] It is one of the three major medium vessel vasculitis.[3]

History

Kussmaul and Maier gave the first detailed description of systemic necrotizing arteritis in 1866.[4]

In 1919 Karl Theodor Fahr[5] described acute arterial lesions which were always present in malignant nephrosclerosis. These injuries were the most severe and widespread in the kidneys and occurred less frequently and severely in other organs, particularly in the pancreas, adrenals and intestines. Fahr believed that necrotizing arteriolitis was the primary cause of the lesions, malignant necrosis and hypertension in these cases by narrowing the renal vascular bed.[4]

Symptoms

The symptoms include:

Emergency symptoms

The organ most damaged by the disease is the kidney.

Causes

Necrotizing arteriolitis may be the primary condition in patients with polyarteritis nodosa or granulomatosis with polyangiitis. It could also be a result of another disorder, such as lupus erythematosus or hepatitis C. What causes the inflammation is not known, but is likely related to the autoimmune response.[9]

The intestinal version of the disease may be a reaction in collagen disease. [12]

Treatment

In most cases, corticosteroids are adminstered. For severe cases, Cytoxan used to be administered, but is being fased out in favor of Rituxan, a less toxic alternative. Some immunosupressive drugs such as azathioprine, methotrexate and mycophenolate have been used to treat the disease,[9] at the cost of exhacerbating the risk of infections and autoimmune diseases.[13]

References

  1. ^ "nec·ro·tiz·ing ar·ter·i·o·li·tis". medical-dictionary.thefreedictionary.com. The Free Dictionary by Farlex. Retrieved 12 August 2025.
  2. ^ Kelly, H. D. Brown (May 1956). "Granuloma of the Nose and Necrotizing Arteriolitis". The Journal of Laryngology & Otology. 70 (5): 313–316. doi:10.1017/S0022215100052956. PMID 13319935.
  3. ^ a b c Jennette, J. Charles; Falk, Ronald J. (2006). "Necrotizing Arteritis and Small Vessel Vasculitis". The Autoimmune Diseases. pp. 899–920. doi:10.1016/B978-012595961-2/50068-8. ISBN 978-0-12-595961-2.
  4. ^ a b c "Pathogenesis of Hypertensive Arterial Necrosis". JAMA. 141 (4): 4. September 24, 1949. doi:10.1001/jama.1949.02910040028008.
  5. ^ Mellon, Ralph R.; Baker, Margaret R.; McIlroy, Alice P. (October 1935). "Experimental Necrotizing Arteriolitis Induced by a Protein Cleavage Product". Proceedings of the Society for Experimental Biology and Medicine. 33 (1): 92–95. doi:10.3181/00379727-33-8263C.
  6. ^ Raval, Prafulla (2007). "Systemic Necrotizing Vasculitis". XPharm: The Comprehensive Pharmacology Reference. pp. 1–5. doi:10.1016/B978-008055232-3.62933-2. ISBN 978-0-08-055232-3.
  7. ^ Atkinson, James B.; Johnson, Mahlon D.; Bouldin, Thomas W.; Whetsell, William O. (2009). "Muscle and Nerve Biopsy". Modern Surgical Pathology. pp. 2069–2088. doi:10.1016/B978-1-4160-3966-2.00053-9. ISBN 978-1-4160-3966-2.
  8. ^ Charles Jennette, J.; Falk, Ronald J. (2014). "Necrotizing Arteritis and Small Vessel Vasculitis". The Autoimmune Diseases. pp. 1067–1086. doi:10.1016/B978-0-12-384929-8.00072-1. ISBN 978-0-12-384929-8.
  9. ^ a b c d MedlinePlus Encyclopedia: Necrotizing vasculitis
  10. ^ Luger, Alan M.; Bauer, John H.; Neviackas, James A.; Lee, Hun Tae; Nichols, Kin (22 October 2008). "Primary Necrotizing Arteritis of the Main Renal Artery Presenting as Accelerated Renovascular Hypertension". American Journal of Nephrology. 1 (3–4): 168–172. doi:10.1159/000166534. PMID 7349049.
  11. ^ "arteriolitis". Oxford Reference.
  12. ^ Trumer, Commander (23 May 1963). "More on Necrotizing Arteriolitis". New England Journal of Medicine. 268 (21): 1201. doi:10.1056/NEJM196305232682131.
  13. ^ Vergara Serpa, Oscar Vicente; Velásquez-Franco, Carlos Jaime; Pinto, Luis Fernando; Pertuz Charris, Neil Smith; Echeverri García, Andrés Felipe; Cardona Palacio, Alejandro; Márquez Hernández, Javier Darío (July 2025). "De la inmunosupresión a la autoinmunidad: vasculitis ANCA-PR3+ en una paciente inmunosuprimida por trasplante hepático. Reporte de un caso" [From immunosuppression to autoimmunity: PR3+ ANCA-associated vasculitis in a patient immunosuppressed for hepatic transplant. A case report]. Revista Colombiana de Reumatología (in Spanish). 32 (3): 286–293. doi:10.1016/j.rcreu.2024.05.005.

Further reading

  • Luthra, Harvinder S. (2009). "Rheumatoid Arthritis". Morrey's the Elbow and Its Disorders. pp. 1025–1038. doi:10.1016/B978-1-4160-2902-1.50079-6. ISBN 978-1-4160-2902-1.
  • Zachary, James F., ed. (2017). Pathologic basis of veterinary disease (6th ed.). St. Louis, Missouri: Elsevier. ISBN 978-0-323-35775-3.

1940s Papers

  • Goldblatt, Harry (January 1947). "The renal origin of hypertension". Physiological Reviews. 27 (1): 120–165. doi:10.1152/physrev.1947.27.1.120. PMID 20282156.
  • Wilson, C; Pickering, G. W. (15 August 1938). "Acute Arterial Lesions in Rabbits with Experimental Renal Hypertension". Clinical Science. 3: 343–355.
  • Byrom, F. B.; Dodson, L. F. (July 1948). "The causation of acute arterial necrosis in hypertensive disease". The Journal of Pathology and Bacteriology. 60 (3): 357–368. doi:10.1002/path.1700600302.

2020s Papers

  • Saha MK, Pendergraft WF, Jennette C, Falk RJ. Primaryu glomerular disease. Brenner and Rector's The Kidney. 11th ed. Philadelphia, PA: Elsevier; 2020 chap 31.
  • Free M, Jennette JC, Falk RJ, Jain K. Renal and systemic vasculitis. Comprehensive Clinical Nephrology. 7th ed. Philadelphia, PA: Elsevier; 2024 chap 26.
  • Dinulos JGH. Hypersensitivity syndromes and vasculitis. Habif's Clinical Dermatology. 7th ed. Philadelphia, PA: Elsevier; 2021 chap 18.
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