Vasculitides
This article deals with the vasculitides (singular vasculitis). Vascular disease that is not vasculitides is covered in the article vascular disease.
Overview
Most common[1]
- Polyarteritis nodosa (PAN).
- Microscopic polyangiitis.
- Wegener's granulomatosis.
- Predominantly cutaneous vasculitis.
- Giant cell arteritis (GCA).
Grouping by size
Small vessel vasculitides
- Predominantly cutaneous vasculitis.
- Henoch-Schoenlein purpura.
- Essential cryoglobulinemic vasculitis.
- ANCA-associated:
- Wegener's granulomatosis (c-ANCA > p-ANCA).
- Churg-Strauss syndrome (50% ANCA +ve).
- Microscopic polyangiitis (usually p-ANCA).
Notes:
- ANCA = anti-neutrophil cytoplasmic antibodies.
Medium vessel vasculitides[2]
- Polyarteritis nodosa (PAN).
- Kawasaki disease.
Large vessel vasculitides[2]
- Giant cell arteritis (AKA temporal arteritis).
- Takayasu's arteritis.
Pathologist's role in the diagnosis of vasculitis
General
- Pathologists often cannot, based on morphology alone, arrive at the definitive diagnosis.
- Presentation & distribution are more characteristic than pathology.[3][4]
Microscopic
Features:[5]
- Inflammatory cells within the blood vessel wall.
- Vessel injury:
- Frank fibrinoid necrosis or nuclear dust:
- Fibrinoid necrosis = anucleate amorphous intensely eosinophilic material.
- Amorphous = no definite form.[6]
- "Nuclear dust" = punctate hyperchromatic material ~ 1 micrometre.
- Fibrinoid necrosis = anucleate amorphous intensely eosinophilic material.
- Frank fibrinoid necrosis or nuclear dust:
Giant cell arteritis
General
- AKA temporal arteritis.
Clinical
Features:
- Classic finding: jaw claudication, in a patient older than 50 years.
- Other findings: headache, vision loss or diplopia, scalp tenderness, polymyalgia, weight loss, chills, fever.
Work-up:
- CRP, ESR, temporal artery biopsy.
Treatment:
- Treat right away with high dose steroids.
- Biopsy is confirmatory.
Micrograph
Features:
- Classical: granulomas.
Image(s):
Takayasu's arteritis
General
Features:[7]
- Disease of medium/large arteries.
- Classically involves the aortic arch (leading to decreased pulses in the upper limbs).
- Typically in patients <40 yrs old.
- Usually asian.
Microscopic
Features:[7]
- Adventitial mononuclear infiltrate with perivascular cuffing of the vasa vasorum.
- Mononuclear inflammation in media.
- Granulomas, giant cells.
- +/-Patchy necrosis of media.
Polyarteritis nodosa
General
- Abbreviated PAN.
- Involves small and medium sized vessels.
- Often - renal vessels, mesenteric vessels.[8]
- Strong association with hepatitis B (see medical liver diseases); ~1/3 of patients with PAN have HBV.
Serology:
- ANCA is usually negative.
Microscopic
Features:
- Inflammatory cells (neutrophils, lymphocytes) within the tunica media.
- Fibroid necrosis: dead vessel wall - pink anucleate stuff, nuclear debris (black specks of nuclear material).
Image: PAN (immunologyclinic.com).
Wegener's granulomatosis
Clinical
- Epistasis.
- Renal failure - present as nephritic syndrome.
- Renal biopsy: crescentic glomerulonephritis (AKA rapidly progressive glomerulonephritis).
- Pulmonary hemorrhage.
Serology:
- c-ANCA +ve.[9]
Notes:
- Pulmonary hemorrhage syndromes:[10]
- Goodpasture syndrome.
- Idiopathic pulmonary hemosiderosis.
- Vasculitis-assoc. hemorrhage (hypersensitivity angiitis, Wegener's granulomatosis).
- Systemic lupus erythematosus.
Microscopic
Features:
- Granulomas typically poorly formed.[11]
Image: Wegener's granulomatosis (flickr.com).
See also
- Cardiovascular pathology.
- Vascular disease - covers atherosclerosis, medial cystic degeneration.
References
- ↑ TN05 RH3.
- ↑ 2.0 2.1 Kumar, Vinay; Abbas, Abul K.; Fausto, Nelson; Aster, Jon (2009). Robbins and Cotran pathologic basis of disease (8th ed.). Elsevier Saunders. pp. 512. ISBN 978-1416031215.
- ↑ URL: http://www.pathology.ubc.ca/path425/PrincipleofPathophysiology/CirculatoryDisorders/SystemicVasculitisDrBWalker.doc. Accessed on: 26 November 2010.
- ↑ URL: http://www.icapture.ubc.ca/who/who_bios_david_walker.shtml. Accessed on: 26 November 2010.
- ↑ URL: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2908435/. Accessed on: 26 November 2010.
- ↑ URL: http://dictionary.weather.net/dictionary/amorphous. Accessed on: 26 November 2010.
- ↑ 7.0 7.1 Cotran, Ramzi S.; Kumar, Vinay; Fausto, Nelson; Nelso Fausto; Robbins, Stanley L.; Abbas, Abul K. (2005). Robbins and Cotran pathologic basis of disease (7th ed.). St. Louis, Mo: Elsevier Saunders. pp. 538. ISBN 0-7216-0187-1.
- ↑ Klatt, Edward C. (2006). Robbins and Cotran Atlas of Pathology (1st ed.). Saunders. pp. 14. ISBN 978-1416002741.
- ↑ TN05 RH6.
- ↑ Cotran, Ramzi S.; Kumar, Vinay; Fausto, Nelson; Nelso Fausto; Robbins, Stanley L.; Abbas, Abul K. (2005). Robbins and Cotran pathologic basis of disease (7th ed.). St. Louis, Mo: Elsevier Saunders. pp. 745. ISBN 0-7216-0187-1.
- ↑ Cotran, Ramzi S.; Kumar, Vinay; Fausto, Nelson; Nelso Fausto; Robbins, Stanley L.; Abbas, Abul K. (2005). Robbins and Cotran pathologic basis of disease (7th ed.). St. Louis, Mo: Elsevier Saunders. pp. 747. ISBN 0-7216-0187-1.