Chapter 7 Flashcards
What are the clinical features of a vasculitis?
- Nonspecific features of inflammation (e.g. fever, fatieu, weight loss, myalgias)
- Symptoms of organ ischemia due to luminla narrowing or thrombosis
Note that vasculites are divided into large (the aorta and its major branches), medium (Muscular arteries that supply organs), and small vessel vasculitis (arterioles, capillaries, and venules).
What are the large vessel vasculites?
- Temporal (Giant cell) arteritis
- Takayasu Arteritis
What is Giant cell arteritis?
Granulomatous vasculitis that classically involves branches of the carotid artery (most common form of vaculitis in 50+; affects females more)
Presents as a temporal HA, visual disturbances, and jaw claudication. ESR will be elevated and joint and muscle pain due to polymyalgia rheumatica may be present
What is Takayasu Arteritis?
granuomatous vasculitis that classically involves the aortic arch at branch pts. Presents in adult LESS than 50 yo (classically young Asian females) as visual and neurologic symptoms with a weak or absnet pulse in the upper extremity
Tx with steroids
What are the medium sized vasculites?
Polyarteritis Nodosa
Kawasaki Disease
Buerger Disease
Describe Polyarteritis Nodosa
Necrotizing vasculitis involving multiple organs (lungs are SPARED) classically presenting in young adults as HTN (renal a. involvment), abdominal pain with melena, neurologic disturbances, and skin lesions
Polyarteritis Nodosa is associated with ______
HBsAg
What is this?
Lesions of PN may vary in stages. Early lesions consist of transmural inflammation with fibrinoid necrosis that eventually heal with fibrosis, producing a ‘string of pearl’s appearance’ on imaging
How is PN tx?
Steroids and cyclophosphamide (fatal if not tx)
Describe Kawasaki disease
Disease of (classically) less than 4 yo Asian children presenting with nonspecific signs including fever, conjunctivitis, erythematous rash on the palms and soles, and enlarged lymph nodes
Coronary artery involvemnt is common and leads to risk for thrombosis with MI and aneurysm with rupture
How is Kawasaki disease tx?
ASPIRIN and IVIG
Describe Buerger Disease
Necrotizing vasculitis of the digits presenting with ulceration, gangrene, and autoamputation
Highly associated with smoking
What are the small vessel vasculities?
- Wegener Granulomatosis
- Microscopic Polyangiitis
- Churg-Strauss Syndrome
- Henoch-Schonlein Purpura
Describe Wegener Granulomatosis
Necrotizing granulomatous vasculitis involving the nasopharynx, lungs, and kidneys
Classically presents in MIDDLE AGED MEN with sinusitis or nasopharyngeal ulceration, hemoptysis with bilateral nodular lung infiltrates, and hematuria due to rapidly progressive glomerulonephritis
Associated with c-ANCA
How is Wegener Granulomatosis tx?
Steroids and cyclophosphamide; relapses are common
Describe Microscopic Polyangiitis
Necrotizing vaculitis involving multiple oragns, especially the LUNGS and KIDNEYS with a presentation similar to Wegener’s, but without nasopharyngeal involvment and no granulomaes
Associated with p-ANCA
Tx with cyclophosphamide and steroids; relapses common
Describe Churg-Strauss Syndrome
Necrotizing granulomatous inflammation with EOSINOPHILS involving multiple oragsn, especially the lungs and HEART. ASthma and peripheral eosinophilia are often present
Associated with p-ANCA
Describe Henoch-Schenlein Purpura
Vasculitis due to IgA complex deposition (most common vaculitis in CHILDREN) presenting with palpable purpura on buttocks and legs, GI pain and bleeding, and hematuria (IgA nephropathy) usually following an URTI
Self limited but may recur; tx wit steroids
Systemic HTN is defined as BP > 140/90 and its causes are divided into primary and 2ndary causes. Primary causes (95%) are of unknown etiology and risk factors include AA, age, obesity, stress, and a high-salt diet.
What are some common causes of 2ndary HTN (5%)?
Renal artery stenosis- stenosis decreases blood flow to the glomeruli. the Juxtagomerular apparatus responds by secreting renin, which converts angiotensinogen to angiotensin I. ATI to ATII by ACE. ATII then raises BP by contracting ateriolar smooth muscle increasing TPR, and promoting adrenal release of aldosterone.
All of this leads to HTN with increased plasma renin and unilaterla atrophy of the affected kidney
Important causes of renal artery stenosis include:
- Atherosclerosis in elderly
- Fibromuscular dysplasia (young females)
What is Fibromuscular dysplasia?
A developmental defect of the blood vessel wall, resuting in irregular thickening of large and medium sized arteries, especially the renal artery
Difference between benign and malignant HTN
Benign: Mild elevation that is clinically silent and slowly leads to organ damage
Malignant: Severe elevation that precipitates end organ failure
Atherosclerosis
What is arteriosclerosis?
Hard arteries due to the blood vessel wall thickening
Three pathologic patterns- atherosclerosis, arteriolosclerosis, and Monckeberg medial calcific sclerosis
