Difference between revisions of "Vasculitides"
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==Wegener's granulomatosis== | ==Wegener's granulomatosis== | ||
* | ===Clinical==== | ||
*Renal. | *Epistasis. | ||
*Renal failure - present as ''nephritic syndrome''. | |||
**Renal biopsy: crescentic glomerulonephritis ([[AKA]] rapidly progressive glomerulonephritis). | |||
*Pulmonary hemorrhage. | |||
* | Serology: | ||
*c-ANCA +ve.<ref>TN05 RH6.</ref> | |||
Notes: | Notes: | ||
*Pulmonary hemorrhage syndromes:<ref>PBoD p.745.</ref> | *Pulmonary hemorrhage syndromes:<ref>PBoD p.745.</ref> | ||
**Goodpasture. | **Goodpasture syndrome. | ||
**Idiopathic pulmonary hemosiderosis. | **Idiopathic pulmonary hemosiderosis. | ||
**Vasculitis-assoc. hemorrhage (hypersensitivity angiitis, Wegener's granulomatosis). | **Vasculitis-assoc. hemorrhage (hypersensitivity angiitis, Wegener's granulomatosis). | ||
Revision as of 02:30, 22 May 2010
This article deals with the vasculitides (singular vasculitis).
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.
Large vessel vasculitides[2]
- Giant cell arteritis (AKA temporal arteritis).
- Takayasu's arteritis.
Giant cell arteritis
- 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.
Micrograph
Features:
- Classical: granulomas.
Takayasu's arteritis
General:[3]
- 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:[4]
- Adventitial mononuclear infiltrate with perivascular cuffing of the vasa vasorum.
- Mononuclear inflammation in media.
- Granulomas, giant cells.
- +/-Patchy necrosis of media.
Polyarteritis nodosa
- Abbreviated PAN
- Involves small and medium sized vessels.
- Often - renal vessels, mesenteric vessels.[5]
- 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.
- Necrosis: dead vessel wall - pink anucleate stuff, nuclear debris (black specks of nuclear material).
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.[6]
Notes:
- Pulmonary hemorrhage syndromes:[7]
- Goodpasture syndrome.
- Idiopathic pulmonary hemosiderosis.
- Vasculitis-assoc. hemorrhage (hypersensitivity angiitis, Wegener's granulomatosis).
- Systemic lupus erythematosus.
Microscopic
Features:
- Granulomas typically poorly formed.[8]