29 vascular pathology Flashcards
main immunologic mechanism that initiate non infectious vasculitis
immune complex deposition
anti nuetrophil cytoplasmic antibody
anti endothelail cell aab
autoreactive t cells
types of vasculitis plus included
large
temporal or giant cell
takaysu
medium
polyartritis nodosa
kawasaki mucocutaneous LN syndrome
bergers disease thromboangitis obliterans
large
wegner granulomatosis with polyangitis
microscopic polyangitis
Churg-Strauss Syndrome
Henoch-Schönlein Purpura
Temporal (Giant Cell) Arteritis
age
inloves
affects or presents as
Granulomatous vasculitis that classically involves branches of the carotid artery
2. Most common form of vasculitis in older adults (> 50 years); usually affects
females
3. Presents as headache (temporal artery involvement), visual disturbances
(ophthalmic artery involvement), and jaw claudication. Flu-like symptoms
with joint and muscle pain (polymyalgia rheumatica) are often present. ESR is
elevated.
4. Biopsy reveals inflamed vessel wall with giant cells and intima! fibrosis (Fig. 7.2).
i. Lesions are segmental; diagnosis requires biopsy of a long segment of vessel,
and a negative biopsy does not exclude disease.
Takayasu Arteritis
age
involes
other name or causes
- Granulomatous vasculitis that classically involves the aortic arch at branch
points - Presents in adults < 50 years old (classically, young Asian females) as visual
and neurologic symptoms with a weak or absent pulse in the upper extremity
(‘pulseless disease’). ESR is elevated.
Polyarteritis Nodosa
involves
age
associated with
lesion type
appearance in imaging
Polyarteritis Nodosa
1. Necrotizing vasculitis involving multiple organs; lungs are spared.
2. Classically presents in young adults as hypertension (renal artery involvement),
abdominal pain with melena (mesenteric artery involvement), neurologic
disturbances, and skin lesions. Associated with serum HBsAg
Lesions of varying stages are present. Early lesion consists of transmural
inflammation with fibrinoid necrosis (Fig. 7.3); eventually heals with fibrosis,
producing a ‘string-of-pearls’ appearance on imaging
Kawasaki Disease
age
presentaion
most involved
AKA
Classically affects Asian children < 4 years old
2. Presents with nonspecific signs including fever, conjunctivitis, erythematous
rash of palms and soles, and enlarged cervicai lymph nodes
3. Coronary artery involvement is commo n and leads to risk for (1) thrombosis
with myocardial infarction and (2) aneurysm with rupture.
Buerger Disease
age and who
presentation
where
AKA
Necrotizing vasculitis involving digits
2. Presents with ulceration, gangrene, and autoamputation of fingers and toes;
Raynaud phenomenon is often present,
3. Highly associated with heavy smoking; treatment is smoking cessation.
Wegener Granulomatosis
triad
age
correlates with
AKA
- Necrotizing granulomatous vasculitis involving nasopharynx, lungs, and
kid neys - Classic presentation is a middle-aged male with sinusitis or nasopharyngeal
ulceration, hemoptysis with bilateral nodular lung infiltrates, and hematuria due
to rapidly progressive glomerulonephritis. - Serum c-ANCA levels correlate with disease activity,
Microscopic Polyangiitis
- Necrotizing vasculitis involving multiple organs, especially lung and kidney
- Presentation is similar to Wegener granulomatosis, but nasopharyngeal
involvement and granulomas are absent. - Serum p-ANCA levels correlate with disease activity.
Churg-Strauss Syndrome
AKA
type
presents also with
Necrotizing granulomatous inflammation with eosinophils involving multiple
organs, especially lungs and heart
2. Asthma and peripheral eosinophilia are often present.
3. Serum p-ANCA levels correlate with disease activity.