Thirty Flashcards
Describe temporal vasculitis. Location? Etiology? Symptoms? Association? What is biopsy like? Treatment? What can happen if not treated?
1 . Granulomatous vasculitis that classically involves branches of the carotid artery
2. Most commo n 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.
- 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. - Treatment is corticosteroids; high risk of blindness without treatment
What is takayasu arteritis like pathologically? Presentation? Epidemiology? Treatment?
B. Takayasu Arteritis
1 . 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.
3 . Treatment is corticosteroids.
What is polyarteritis nodosa like pathologically? Location? Presentation? Association? Describe the pathophysiology including progression? What is the pathology? Treatment?
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
3 . Lesions of varying stages are present. Early lesion consists of transmural inflammatio n with fibrinoid necrosis (Fig. 7.3); eventually heals with fibrosis,
producing a ‘string-of-pearls’ appearanc e on imaging
4. Treatment is corticosteroids and cyclophosphamide; fatal if not treated
What is the epidemiology of kawasaki disease? How does it present? Location/results? Treatment?
1 . Classically affects Asian children < 4 years old
2 . Presents with nonspecific signs including fever, conjunctivitis, strawberry tongue, erythematous rash of palms and soles, and enlarged cervicai lymph nodes
3 . Coronary artery involvement is c o m m o n and leads to risk for (1) thrombosi s with myocardial infarctio n and (2) aneurys m with rupture.
4. Treatment is aspirin and 1VIG; disease is self-limited.
What is Buerger disease? How does it present? What is it associated with? Treatment?
C, Buerger Disease
1 . Necrotizing vasculitis involving digits
2. Presents with ulceration, gangrene, and autoamputatio n of fingers and toes; Raynaud phenomeno n is often present,
3 . Highly associated with heavy smoking; treatment is smoking cessation.
What is wegener’s granulomatosis? Location? Presentation? What marker is present? What does biopsy reveal? Treatment?
A. Wegener Granulomatosi s
1 . Necrotizing granulomatous vasculitis involving nasopharynx, lungs, and kid neys
2. Classic presentation is a middle-aged male with sinusitis or nasopharyngeal ulceration, hemoptysis with bilateral nodular lung infiltrates, and hematuri a due
to rapidly progressive glomerulonephritis .
3 . Serum c-ANC A levels correlate with disease activity,
4. Biopsy reveals large necrotizing granulomas with adjacent necrotizing vasculitis
(Fig. 7.4).
5 . Treatment is cyclophosphamide and steroids; relapses are common,
What is microscopic polyangiitis? Presentation? Serum marker? Biopsy? Treatment?
B. Microscopic Polyangiitis
1 . Necrotizing vasculitis involving multiple organs, especially lung and kidney
2. Presentation is similar to Wegener granulomatosis , but nasopharyngeal involvement and granulomas are absent.
3 . Serum p-ANC A levels correlate with disease activity.
4. Treatment is corticosteroids and cyclophosphamide; relapses are common .
What is the pathology of churgg strauss syndrome? Location? Associations? Serum marker?
1 . Necrotizing granulomatous inflammatio n with eosinophils involving multiple organs, especially lungs and heart
2 . Asthma and peripheral eosinophilia are often present.
3 . Serum p-ANC A levels correlate with disease activity.
What is the pathophysiology of Henoch-Schoenlein Purpura? How does it present? Treatment?
1 . Vasculitis due to IgA i m m u n e complex deposition; most commo n vasculitis in children
2 . Presents with palpable purpura on buttocks and legs, GI pain and bleeding, and hematuri a (IgA nephropathy) ; usually occurs following an upper respiratory tract infection
3 . Disease is self-limited, but may recur; treated with steroids, if severe
What is essential mixed cryoglobulinemia? What can it be associated with? Clinical findings? Lab findings?
Essential Mixed cryoglobulinemia: cryoglobulins are cold precipitable monoclonal or polyclonal immunoglobulins and may be associated with vasculitis. It is a small vessel
vasculitis. Many cases are idiopathic. It can be associated with:
Multiple myeloma
Lymphoproliferative disorders
Connective tissue disease
Infection: hepatitis C (since the discovery of hep C vast majority of essential mixed cryoglobulinemia is found to be secondary to hep C virus)
Liver disease
Clinical findings and lab evaluation:
Palpable purpura Arthralgias Weakness Neuropathy Glomerulonephritis: 10-30%
Circulating cyroprecipitates RF + Hypocomplementemia 90% Elevated ESR Hepatitis C in majority
What is behcet’s syndrome? Epidemiology? Clinical findings?
Behcet’s Syndrome: small vessel vasculitis (ANCA negative) characterized by leukocytoclastic venulitis with fibrinoid necrosis. All size vessels may be involved.
It is most commonly seen in males and females from the Mediterranean region, Middle
East, and the Far East. Though males and females are affected equally, males then to have a more aggressive disease course.
Clinical findings:
Recurrent aphthous ulcers: painful, shallow or deep with central necrosis. Ulcers may occur singly or in crops and are located anywhere in the oral cavity. The ulcers persist
for 1-2 weeks and do not leave a scar.
Genital ulcers are less common: do not involve the glans penis or urethra and leave a scrotal scar.
Skin involvement include: folliculitis, erythema nodosum, acne like exanthem, vasculitis.
Abnormal pathergy test: nonspecific skin reaction after intradermal saline injection.
Eye involvement: bilateral panuveitis with scarring rapidly progressing to blindness.
Retinal vessel occlusion and optic neuritis
Arthritis: knees and ankles
Superficial or deep vein thrombosis in 25%
Superior vena cava obstruction may be seen
Aortitis or peripheral arterial aneurysm
Pulmonary artery vasculitis in 5%: dyspnea, cough, chest pain, hemoptysis, and
pulmonary infiltrates
CNS 5-10%: brainstem involvement
GI: mucosal ulceration of the gut
Elevated ESR/ APR
Leukocytosis
ASCA anti-Saccharomyces cerevisiae antibodies (seen in crohn’s disease)
Anti- enolase antibodies
