Vascular: Pathoma, BRS, First Aid Flashcards
What is vasculitis?
Inflammation of blood vessel wall
BV wall has what 3 components?
- endothelial intima
- smooth muscle media
- CT adventitia
Etiology of vasculitis is usually what?
Unknown or immune mediated.
Clinical features of vasculitis? 2
- Nonspecific symptoms of inflammation
2. Symptoms of organ ischemia
Nonspecific symptoms of inflammation include? (4)
- Fever
- Fatigue
- Weight loss
- Myalgias
Symptoms of organ ischemia are due to what?
Luminal narrowing or thrombosis of inflamed vessels
Large-vessel vasculitis involves what?
Aorta and its branches
Medium vessel vasculitis involves what?
Muscular arteries that supply organs
Small vessel vasculitis involves what? (3)
- Arterioles
- Capillaries
- venules
Temporal Giant Cell arteritis is what?
Granulomatous vasculitis that classically involves branches of the carotid artery
Temporal giant cell arteritis is the most common vasculitis in what patients?
Older adults (especially females)
How does temporal giant cell arteritis present? 6
- Unilateral Headache: Due to temporal artery
- Visual distrubance: Ophthalmic artery (can go blind)
- Jaw claudication
- Polymyalgia rheumatica
- Elevated ESR
- Nodules along course of artery involved
Biopsy of temporal giant cell arteritis reveals what? 4
Inflamed vessel wall (fibrosis)
Giant cells
Lesions are segmental
Many granulocytes
Treatment of temporal giant cell arteritis is?
Corticosteroids
Takayasu arteritis is what?
Granulomatous vasculitis that involves aortic arch at branch points
What demographic has takayasu arteritis the most?
Young asian females
How does takayasu arteritis present?
- Visual symptoms
- Neurologic smptoms
- Weak or absent pulse in upper extremity
- ESR elevated
Treatment of takayasu arteritis?
Corticosteroids
What syndrome does takayasu arteritis produce?
Aortic arch syndrome
What are the two medium vessel vasculitises?
Polyarteritis Nodosa
Kawasaki Disease
Buerger Disease
Polyarteritis nodosa is what?
Necrotizing vasculitis involving muscular arteries that supply organs.
What organ is spared in polyarteritis nodosa?
Lungs
How does polyarteritis nodosa classically present?
Young adult Hypertension: Renal artery involvement Abdominal pain with melena: Mesenteric artery IHD: Coronary arteries Neurologic dysfunction Skin lesions
What serum antigen is associated with Polyarteritis nodosa?
Hepatitis B surface antigen
Early lesion of polyarteritis nodosa consists of what?
Transmural inflammation with fibrinoid necrosis
How do early lesions in polyarteritis nodosa heal?
What does this produce?
Fibrosis
String of pearls appearance
Treatment for polyarteritis nodosa? 2
What happens if not treated?
- Corticosteroids
- Cyclophosphamide
Fatal
Fibrinoid necrosis is clasically seen in what two scenarios?
- Malignant HTN
2. Vasculitis
Early lesions have what effect in polyarteritis nodosa?
What can result?
Weaken vessel wall
Aneurysm
What mediates polyarteritis nodosa?
Immune complexes
What organ causes most deaths in polyarteritis nodosa?
Kidneys: Vasculitis in arterioles and glomeruli and renal artery lesions
Kawasaki disease typically affects what demographic?
Asian children under 4 years old.
How does kawasaki disease present? 5
- Fever
- Conjunctivitis
- Red rash of palms and soles
- Enlarged cervical lymph nodes
- Strawberry tongue
What artery is dangerous for kawasaki disease?
Why?
Coronary artery
Can lead to:
- Thrombosis with MI
- Aneurysm with rupture
Treatment for kawasaki disease is what?
- Aspirin
2. IV immunoglobulin
Buerger disease is what?
Necrotizing vasculitis involving digits
How does Buerger disease present?
Ulceration
Gangrene
Autoamputation of fingers and toes
Raynaud phenomenon
What is Buerger’s disease heavily associated with?
Which leads into what is the best treatment for Buerger disease?
Smoking
Cessation of smoking
What demographic typically presents with Buerger’s disease?
Young Jewish men
What are the four small vessel vasculitis?
Wegener Granulomatosis
Microscopic Polyangiitis
Churg-Strauss syndrome
Henoch-Schonlein purpura
Wegener Granulomatosis is what?
Fibrinoid-Necrotizing granulomatous vasculitis involving nasopharynx, lungs, and kidneys
Classic presentation of Wegener granulomatosis?
- Middle-aged male
- Sinusitis/nasopharyngeal ulceration
- Hemoptysis with bilateral nodular lung infiltrates
- Hematuria: Glomerulonephritis
Treatment of Wegener granulomatosis 2
What happens despite treatment
Cyclophosphamide
Steroids
Relapse
Wegener granulomatosis correlates with what serum level?
Circulating-Antineutrophil cytoplasmic antibodies (C-ANCA)
Biopsy of Wegener granulomatosis reveals what?
Large necrotizing granulomas (with giant cells) with adjacent necrotizing vasculitis
The TRIAD of Wegener granulomatosis?
- Focal necrotizing vasculitis
- Necrotizing granulomas in lung and URT
- Necrotizing glomerulonephritis
Microscopic polyangiitis is what?
Necrotizing vasculitis of multiple organs especially lung and kidney?
How do microscopic polyangiitis symptoms differ from wegener’s? 2
No nasopharyngeal symptoms
No granulomas
Treatment of microscopic polyangiitis? 2
Corticosteroids and cyclophosphamide
What serum level correlates with microscopic polyangiitis AND Churg-Strauss syndrome?
Serum p-ANCA
Churg-strauss syndrome is what?
Necrotizing granulomatous inflammation with eosinophils involving multiple organs especially lungs and heart
How does Churg-strauss syndrome present? 4
- Asthma
- Peripheral eosinophilia
- Palpable purpura
- Glomerulonephritis
What is elevated in Churg-Strauss syndrome? 2
- p-ANCA
2. IgE
Henoch-Schoenlein Purpura is what?
Vasculitis due to IgA immune complex deposition
Henoch-Schoenlein Purpura is the most common vasculitis in what demographic?
Children
How does Henoch-Schoenlein Purpura present? 5
- Palpable purpura on buttocks and legs
- GI pain and bleeding
- Hematuria (IgA nephropathy)
- Follows URT infection (Causes excess IgA)
- Anthralgia
How to treat Henoch-Schonlein Purpura?
Usually goes away on own but steroids if needed
What is Raynaud’s disease?
Recurrent vasospasm of small arteries and arterioles
Symptom of Raynaud’s disease?
What precedes it
Pallor/Cyanosis especially fingers and toes
Chilling
Raynaud disease is most common in who?
Young, healthy women
What is Raynaud phenomenon?
Similar to Raynaud disease but is secondary to an underlying disorder
What is the underlying disorder in Raynaud’s phenomenon? 2
SLE = Lupus
CREST syndrome = Systemic sclerosis
Hypertension is what?
Increased blood pressure
Does hypertension involve pulmonary or systemic circulation?
Either
Systemic hypertension is defined how?
greater than 140/90