SM_249a: Vasculitis Flashcards
Vasculitides may be classified by ____
Vasculitides may be classified by vessel size
Large vessel vasculitis includes ____ and ____
Large vessel vasculitis includes Takayasu arteritis and giant cell arteritis
Giant cell arteritis affects ____ ____ most commonly
Giant cell arteritis affects older women most commonly
(most common form of arteritis, average onset 73 years, Northern European ancestry)
Giant cell arteritis clinical features include ____, ____, ____, ____, ____, and ____
Giant cell arteritis clinical features include new headache, constitutional symptoms, jaw claudication, symptoms of polymyalgia, partial or complete loss of vision, and arm claudication
Describe giant cell arteritis diagnostics
Giant cell arteritis diagnostics
- Laboratory studies: marked elevation in ESR and CRP, thrombocytosis may be seen
- Imaging: temporal artery ultrasound, MRA, PET
- Biopsy: temporal artery, should be done urgently on all patients with suspected GCA, yield remains high even after several weeks of treatment
____ should be done urgently in all patients with suspected giant cell arteritis
Temporal artery biopsy on the affected side should be done urgently in all patients with suspected giant cell arteritis
Giant cell arteritis pathology involves ____, ____, ____, and ____
Giant cell arteritis pathology involves inflammation often centered on internal elastic lamina, multinucleated giant cells, granulomas, and focal fibrinoid necrosis
Giant cell arteritis treatment includes ____ and ____
Giant cell arteritis treatment includes high-dose corticosteroids and tocilizumab (anti IL-6)
Takayasu arterities is ____ but most commonly affects the ____, ____, or ____ arteries
Takayasu arteritis is a large vessel vasculitis that can affect any artery but most commony affects aorta, subclavia, or carotid arteries
(pulmonary artery involvement also common)
Takayasu arteritis is characterized by ____, ____, or ____
Takayasu arteritis is characterized by segmental stenosis, dilatation, or aneurysm formation
Takayasu arteritis is seen in patients aged ____, ____, and has greatest prevalence in ____
Takayasu arteritis is seen in patients aged ≤ 40 years old, females, and has greatest prevalence in Asia
Describe Takayasu arteritis clinical features
Takayasu arteritis clinical features
- Depend on vascular bed
- Symptoms to suspect Takayasu arteritis in patient ≤ 40: constitutional symptoms (absent in up to 50% at onset), unexplained lightheadedness, extemity pain / claudication, bruits, or absent/diminished pulses or BP difference > 10 mmHg
Takayasu arteritis laboratory findings show ____ and ESR and CRP ____
Takayasu arteritis laboratory findings show no specific lab abnormality and ESR and CRP are often elevated but can be negative despite active disease
Takayasu arteritis pathology includes ____, ____, ____, and ____
Takayasu arteritis pathology includes granulomatous panarteritis, intimal hyperplasia, fixed stenosis and occlusions, and occasional aneurysm
___ is crucial for Takayasu arteritis diagnosis
Imaging is crucial for Takayasu arteritis diagnosis
(noninvasive such as CTA / MRA / PET most common)
Describe Takayasu arteritis treatment
Takayasu arteritis treatment
- High-dose corticosteroids initially: high relapse rate if tapered
- Methotrexate or azathioprine are preferred steroid-sparing agents to help maintain remission
Surgical bypass with good results in certain specific situations - Angioplasty ± stenting has generally been associated with high failure rates
- Early recognition and initiation of therapy are critical because aortic regurgiation and acute MI are common and can cause death
Compare and contrast giant cell arteritis and Takayasu arteritis
Gziant cell arteritis and Takayasu arteritis
Medium sized vasculitides include ____ and ____
Medium sized vasculitides include polyarteritis nodosa and Kawasaki disease
Polyarteritis nodosa is ____, more common in ____, and typically presents between ages ____
Polyarteritis nodosa is a systemic necrotizing vasculitis of medium-sized muscular arteries (often at branch points), more common in males, and typically presents between ages 40-60
(but can affect any age group, primary or secondary)
Secondary polyarteritis nodosa is primarily associated with ____
Secondary polyarteritis nodosa is primarily associated with HBV
(also HCV, HIV, hairy cell leukemia)
Polyarteritis nodosa involves ____, ____, and ____
Polyarteritis nodosa involves transmural inflammation, no giant cells or granulomas, and often dfiferent phases of vasculitis occurring within different vessels or one vessel at the same time
Polyarteritis nodosa clinical features include ____, ____, ____, ____, ____, and ____
Polyarteritis nodosa clinical features include cutaneous, renal disease (not glomerular), neuropathy, mesenteric ischemia, orchitis, and myalgia/weakness
- Cutaneous: nodules, purpura, livedo reticularis, ulcers
Describe polyarteritis nodosa treatment
Polyarteritis nodosa treatment
- Mild: glucocorticoid monotherapy, methotrexate or azathioprine for resistant disease
- Moderate to severe: glucorticoid and cyclophosphamide
- Viral associated secondary polyarteritis nodosa: check for HBV, appropriate antiviral therapy (± glucocorticoids and/or immunosuppression)
Kawasaki disease is ____ and that is more common in ____ ____
Kawasaki disease is an acute febrile typically self-limited vasculitis seen in young children that is more common in Asian boys
(#1 cause of acquired heart disease in kids, presents betwen 6 months and 5 years of age)
Kawasaki disease clinical manifestations include ____ and ____
Kawasaki disease clinical manifestations include strawberry tongue and erythematous rash
Kawasaki disease pathology primarily affects ____ and includes ___, leading to ___
Kawasaki disease pathology primarily affects medium-sized muscular arteries (coronary arteries) and includes destruction of luminal endothelial cells, elastic lamina, and smooth muscle cells of media, leading to arterial wall dilatation ± aneurysm formation
Kawasaki disease laboratory finds are ____ and include ____, ____, and ____
Kawasaki disease laboratory finds are generally nonspecific and include leukocytosis with left shift, normocytic anemia and often marked thrombocytosis, and transaminitis or mild hyperbilirubinemia due to intrahepatic congestion
Kawasaki disease treatment includes ____ and ____
Kawasaki disease treatment includes aspirin and IVIG
- Aspirin: initially high-dose until fever resolves, anti-thrombotic dose continued for 6-8 weeks or until lab abnormalities resolve and there are no coronary abnormalities on echo
- IVIG: significant reduction in risk of mortality related to coronary artery aneurysms if begun within 1st 10 days
ANCA-associated small vessel vasculitis includes ____, ____, and ____
ANCA-associated small vessel vasculitis includes microscopic polyangiitis, granulomatosis with polyangiitis, and eosinophilic granulomatosis with polyangiitis
____ is associated with p-ANCA while ____ is associated with c-ANCA
MPO is associated with p-ANCA while PR3 is associated with c-ANCA
Describe pathogenesis of ANCA-associated small vessel vasculitis
ANCA-associated small vessel vasculitis pathogenesis
- Activated neutrophils -> express PR3 or MPO -> antibodies stick to them -> release cytokines -> migrate into vessel wall -> release more cytokines -> cause inflammation
Granulomatosis with polyangiitis is a ____ vasculitis generally associated with ____, has average age onset of ____, and is more common in ____ patients
Granulomatosis with polyangiitis is a small-medium vasculitis generally associated with c-ANCA, has average age onset of 40-50s with second peak in elderly 70s, and is more common in white patients
Granulomatosis with polyangiitis pathology includes ____ and ____
Granulomatosis with polyangiitis pathology includes necrotizing granulomas and few or no immune deposits
Describe granulomatosis with polyangiitis clinical features
Granulomatosis with polyangiitis clinical features
- Upper airway disease: nasal septum, sinuses, subglottic stenosis
- Pulmonary disease: nodules, alveolitis/capillaritis, ILD-fibrosis
- Glomerulonephritis
- Arthritis/arthralgia
- Vascular: gangrene, VTE
- Neuropathy: sensory, motor, CNS (rare), sensorineural hearing loss
- Ocular disease: scleritis/episcleritis, pseudotumor
- Skin/mucosa: oral ulcers, purpura, nodules
- Cardiac
- GI disease
Microscopic polyangiitis is a ____ vasculitis most common in ____
Microscopic polyangiitis is a small vessel vasculitis most common in middle age
Microscopic polyangiitis pathology includes ____, ____, and ____
Microscopic polyangiitis pathology includes necrotizing arteritis, few or no immune deposits, and no granulomatous inflammation
Microscopic polyangiitis clinical features include ____, ____, ____, ____, ____, and ____
Microscopic polyangiitis clinical features include glomerulonephritis, pulmonary capillaries, peripheral neuropathy, skin lesions, weight loss, and fevers
Granulomatosis with polyangiitis and microscopic polyangiitis are treated initially with ____, ____, and ____
Granulomatosis with polyangiitis and microscopic polyangiitis are treated initially with steroids, rituximab or cyclophosphamide for severe disease, and methotrexate for non-severe disease
Eosinophilic granulomatosis with polyangiitis has ___ predominance, average age of ___, is associated with ___, and has ___ and ____
Eosinophilic granulomatosis with polyangiitis has a mild male predominance, average of 35-45, is associated with p-ANCA in 40% of cases but is often ANCA negative, and has eosinophilia/elevated IgE and adult onset asthma
(renal involvement is less common)
Eosinophilic granulomatosis with polyangiitis pathology involves ____, ____, and ____
Eosinophilic granulomatosis with polyangiitis pathology involves necrotizing vasculitis, eosinophilic infiltration, and extravascular granuloma
Eosinophilic granulomatosis with polyangiitis clinical presentation includes the ____, ____, and ____ phases
Eosinophilic granulomatosis with polyangiitis clinical presentation includes the prodrome, eosinophilic, and vasculitis phases
- Prodrome: asthma, allergic rhinitis, nasal polyp
- Eosinophilic phase: tissue and periphery
- Vasculitis phase: cutaneous, cardiac, neurologic, renal, and gastroenteritis
Eosinophilic granulomatosis with polyangiitis is treated initially with ____, ____, and ____
Eosinophilic granulomatosis with polyangiitis is treated initially with glucocorticoids, cyclophosphamide, and meopolizumab (anti IL-5)
Compare the contrast the ANCA vasculitides
ANCA vasculitides
Immune complex small vessel vasculitis includes ____, ____, ____, and ____
Immune complex small vessel vasculitis includes cryoglobulinemic vasculitis, IgA vasculitis (Henoch-Schonlein), hypocomplementemic urticarial vasculitis, and anti-GBM disease
Describe Anti-GBM disease (Goodpasture’s)
Anti-GBM disease (Goodpasture’s)
- Immune complex small vessel vasculitis
- Older children and adults
- Usually idiopathic but can occur after URI
- Diagnosis made by checking anti-GBM antibodies
Anti-GBM disease (Goodpasture’s) clinical presentation includes ____, ____, and ____
Anti-GBM disease (Goodpasture’s) clinical presentation includes acute renal failure, alveolar hemorrhage, and NO constitutional symptoms
(can occur in addition to ANCA vasculitis)
Anti-GBM disease (Goodpasture’s) is treated with ____, ____, and ____
Anti-GBM disease (Goodpasture’s) is treated with plasmapheresis, glucocorticoids, and cyclophosphamide
Describe cryoglobulinemia
Cryoglobulinemia
- Cryoglobulins: immunoglobulins or mixtures of immunoglobulins and complement that precipitate at temperatures less than 37C
- 3 types: Type 1 - monoclonal Ig, Type II - mixed monoclonal Igs with and polyclonal Ig, Type III - polyclonal IgG and IgM
- Hyperviscositiy (digital ischemia, livedo reticularis, skin necrosis) vs vasculitis (arthralgias, fatigue, myalgia, palpable purpura, peripheral neurpathy, glomerulonephritis)
- Associated conditions: Type I is MGUS or B cell malignancy, Type II is HCV / HBV / HIV / autoimmune disease
- Treatment depends on underlying condition and severity of disease
Cryoglobulinemia type II is associated with ____
Cryoglobulinemia type II is associated with Hepatitis C
IgA vasculitis (Henoch-Schonlein purpura) most commonly affects ____ and 2/3 of cases present after ____
IgA vasculitis (Henoch-Schonlein purpura) most commonly affects children and 2/3 of cases present after URI
(children have better prognosis)
IgA vasculitis (Henoch-Schonlein purpura) pathology includes ____, ____, and ____
IgA vasculitis (Henoch-Schonlein purpura) pathology includes IgA and C3 complexes in blood vessel, granulocytes in walls of arterioles or venules, and necrotizing lesions
IgA vasculitis (Henoch-Schonlein purpura) clinical features include ____, ____, ____, and ____
IgA vasculitis (Henoch-Schonlein purpura) clinical features include rash / purpura, intestines (abdominal pain prior to rash, vomiting diarrhea), joint pains/myalgias, and kidney involvement (hematuria)
IgA vasculitis (Henoch-Schonlein purpura) treatment includes ___ and ___
IgA vasculitis (Henoch-Schonlein purpura) treatment includes supportive care and glucorticoids
(glucocorticoids: pulse steroids for severe disease, used to prevent nephritic / nephrotic CKD)
Variable vessel vasculitis includes ____
Variable vessel vasculitis includes Behcets syndrome
Behcets syndrome is a ____ vasculitis with highest prevalence in ____ and onset at ____
Behcets syndrome is a mixed vessel vasculitis with highest prevalence in Mediterranean / Middle East / Far East and onset at 3rd-4th decade
Describe Behcets disease clinical features
Behcets disease clinical features
- Oral ulcers
- Genital ulcers
- Cutaneous lesions (including pathergy)
- Ocular involvement
- Cardiovascular
- Arthralgias
- Neurologic
- Gastrointestinal
- Inner ear involvement
Behcets disease treatment is ____
Behcets disease treatment is organ based
- Topical glucorticoids, colchicine, apremilast (phosphodiesterase 4 inhibitor), systemic glucocorticoids, azathioprine, TNF inhibitors, and cyclophosphamide
Secondary vasculitis etiology includes ____, ____, ____, and ____
Secondary vasculitis etiology includes infectious, drug-induced, connective tissue disease, and malignancy associated
Vasculitis mimics include ___, ___, and ___
Vasculitis mimics include congenital vascular disorders, atheroembolic disease, and thrombotic disorders
Describe the approach for vasculitis
Approach for vasculitis
Most likely diagnosis is ____
Most likely diagnosis is IgA vasculitis (Henoch-Schonlein purpura)
Most likely diagnosis is ____
Most likely diagnosis is granulomatosis with polyangiitis
Most likely diagnosis is ____
Most likely diagnosis is granulomatosis with polyangiitis
